Neurology

A 60-year-old male complains of recurrent and worsening bouts of foot drop and difficulty picking up objects. He denies any trauma or pain but admits to muscle cramps and twitching. Family members have noticed that he has

been forgetful recently with inappropriate bouts of laughing and crying. A diagnosis of amyotrophic lateral sclerosis (ALS) is suspected. Which of the following is

TRUE concerning this disease? • A:• The process involves the degeneration of both upper and

lower motor neurons.• B:• The majority of ALS cases have a familial basis.• C:• No treatment has been found to improve survival.• D:• The initial symptoms are usually muscle cramps, twitching and

drooling.• E:• Extraocular muscles and bladder muscles are usually affected.•

Answer

• A:• The process involves the

degeneration of both upper and lower motor neurons.

Amyotrophic Lateral Sclerosis•  Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressiveneurologic disease of uncertain etiology. It is

comprised of two types, familial and sporadic, with the latter accounting for approximately 90 percent of the cases. In the familial form, a genetic mutation is usually transmitted in an autosomal dominant fashion. In the sporadic form, advanced age is the only established risk factor, although smoking, high fat or glutamate diets, exposures (herbicides, pesticides, heavy metals) and service in the Gulf War (1990-1991) are being cited as possible risk factors. Annual incidence is 1-3/100,000 with a slight male predominance.

•  • The pathophysiology consists of the degeneration of both upper motor neurons and lower motor neurons. In addition, there is sclerosis of

the lateral motor column of the spinal cord. This leads to decreased nerve transmission to the muscles and subsequent muscle weakness and atrophy. The initial manifestation of the disease is commonly painless muscle weakness in the distal extremities. Weakness is asymmetric in 80 percent of patients. Patients may complain of a foot drop (slapping gait) or a weak grip (difficulty writing or manipulating fingers). As the disease progresses, there may be muscle cramps, spasticity, twitching, loss of voluntary limb movement, drooling, speech and swallowing difficulties and the inability to regulate laughter or crying (pseudobulbar affect). Anxiety, dementia and Parkinsonism may also accompany ALS. Death usually occurs when respiratory muscles become too weak to support adequate ventilation. Survival is usually 3-5 years from the time of diagnosis. Face and neck weakness are rarely the initial symptoms of ALS. Extraocular muscles and bladder muscles are typically not affected by the disease. Frontotemporal dementia can be seen.

•  • The diagnosis of ALS is made clinically by the findings of both lower (weakness, atrophy,fasciculations) and upper motor deficits (increased

tone and reflexes, presence of any reflexes in muscles that are very weak or wasted) with a wide distribution. No particular test is confirmatory, but electromyography can be used to confirm lower motor neuron involvement. The management of ALS consists of a multidisciplinary team (speech therapist, nutritionist, occupational therapist, physician therapist, physician, etc.) and medications to provide supportive and symptomatic care. Examples of supportive medicines include atropine for drooling, fluvoxamine for pseudobulbar affect, lorazepam for anxiety, oxygen for hypoxia,baclofen for muscle cramps and spasticity and laxatives for constipation. Riluzole 100 mg daily is reasonably safe and probably prolongs median survival by about 2 to 3 months in patients with amyotrophic lateral sclerosis (SOR A; Ref. 1). Riluzole (Rilutek®) is a sodium channel blocker and glutamate antagonist. Its use is limited by its high cost and routine liver function testing requirement.

•  • Selected references:• 1. Miller RG, Mitchell JD, Moore DH. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane

Database Syst Rev 2007 Jan 24; (1):CD001447.http://www.cochrane.org/reviews/en/ab001447.html  Accessed March 2008• 2. Strong M, Rosenfeld J. Amyotrophic lateral sclerosis: A review of current concepts. Amyotroph Lateral SclerOther Motor

Neuron Disord 2003; 4(3):136-143.• 3. Vanderhoff BT, Delphia M, Pemmering TL. Amyotrophic lateral sclerosis. In: Rake RE, ed, Textbook of Family Practice.

6th ed. Philadelphia: EB Saunders Co., 2002: electronic version.• 4. Wicklund MP. Amyotrophic lateral sclerosis: possible role of environmental influences. Neurol Clin 2005; 23(2):462-484.

A 27-year-old woman with epilepsy comes to the office to discuss her current contraception needs

and also to ask about the implications of her epilepsy on future pregnancies. She is currently on carbamazepine (generic, Tegretol®) 400 mg orally twice daily. All of the following statements to her

are true EXCEPT • A:• She is not a good candidate for low-dose oral contraceptive pills.• B:• Fetuses exposed to any antiepileptic drugs have higher rates of minor

and major malformations than the general population.• C:• Once she decides to become pregnant, she should be switched to lower

doses of 2 antiepileptic drugs because it is safer to keep a woman on low doses of more than one antiepileptic drug rather than a higher dose of just one.

• D:• Folic acid supplementation prior to conception is especially important.• E:• Women with epilepsy in their reproductive years may be considered for

withdrawal of their antiepileptic drugs if they have been seizure-free for 2-5 years, have a normal exam, single type of seizure and normalized electroencephalogram (EEG).

Answer

• C:• Once she decides to become

pregnant, she should be switched to lower doses of 2 antiepileptic drugs because it is safer to keep a woman on low doses of more than one antiepileptic drug rather than a higher dose of just one.

Assuming that the patient in the previous question becomes pregnant and remains on carbamazepine,

which of the following is NOT appropriate management during her pregnancy?

• A:• She should take oral vitamin K from 36 weeks until

delivery.• B:• She should be offered screening for neural tube defects.• C:• Drug levels of her carbamazepine should be monitored

periodically during the pregnancy.• D:• She should be delivered by cesarean section as the stress

of the second stage of labor may precipitate a seizure.• E:• Epidural analgesia can be administered without increased

risk.

Answer

• D:• She should be delivered by

cesarean section as the stress of the second stage of labor may precipitate a seizure.

Antiepileptic Drugs And Pregnancy•  Up to 1 percent of the population has epilepsy. Therefore, most family physicians will care for women on antiepileptic drugs (AEDs). There are special considerations when considering the use of oral

contraceptive pills (OCPs) in patients taking AEDs. Unfortunately, physicians are often unaware of decreased efficacy of OCPs when given to patients taking some of the most commonly used AEDs. In one study only 4 percent of neurologists and no obstetrician/gynecologists were aware of the interaction. It is thought that the effect is the result of hepatic enzyme induction caused by progesterone and estradiol. Contraceptive failure is particularly noted with the lower estrogen-containing pills commonly prescribed now. In one study the failure rate was 25 times greater than expected. The Table shows which AEDs are associated with increased OCP failure and those that are not.

•  Antiepileptic Drugs and Oral Contraceptive Pill Interactions•  Significant InteractionNo Carbamazepine (generic, Tegretol®)

• Ethosuximide (generic, Zarontin®)• Oxcarbazepine (Trileptal®)• Phenobarbital• Phenytoin (generic, Dilantin®)• Primidone (generic, Mysoline®)• No Significant Interaction• Gabapentin (generic, Neurontin®)• Lamotrigine (Lamictal®)• Levetiracetam (Keppra®)• Tiagabine (Gabitril®)• Valproic acid (Depakene®)• Vigabatrin (Sabril®)•  Physicians must remember that the use of these medications is not limited to the treatment of epilepsy. Some of them are also used for treatment of migraine headache, pain disorders, bipolar

disorder and other disorders. Therefore, women using these medications for these purposes also must be informed properly about potential contraceptive failure.•  Pregnant women with epilepsy may experience a higher seizure frequency and may need higher doses of AEDs. Physiologic increases in blood volume with resultant lower serum drug levels during

pregnancy necessitate frequent measurement of drug levels and higher medication doses in many women. Women should have drug levels tested at least once a trimester and then within the first two to four weeks after delivery.

•  Women with epilepsy have an increased chance of giving birth to a baby with a major malformation compared to women in the general population (4-11.5 percent vs. 2-4 percent). A substantial part of this risk is due to taking AEDs. Fetal anticonvulsant syndrome is a term used to describe all of the abnormalities found in babies born to women on anticonvulsant medications. Comparison studies of women with epilepsy not on any medications compared to women taking AEDs show lower rates of malformation, suggesting that the medication is responsible for much of the risk. The risk of minor malformations is also higher. Therefore, pregnant women on AEDs for other indications should also be counseled about the risks.

•  Therapy with more than one AED has been shown to be related to even higher rates of malformations. The Cochrane Database states that based on currently available evidence women who are pregnant should continue medication during pregnancy and that monotherapy at the lowest effective dose. Polypharmacy should be avoided (SORA; Ref: 1).  It is not recommended to change medications once a woman is pregnant because the exposure to multiple medications may further increase the risk of malformations and changing medications may predispose the patient to seizures.

•  Various types of anomalies have been described in women on virtually all AEDs. The older anticonvulsants generate free radicals and may predispose to folic acid deficiency. TheAmerican Academy of Neurology (AAN) recommends that all pregnant women on AEDs should receive folic acid supplementation. The dose of folic acid is between 0.4 mg to 4 mg per day. Women on valproic acid (generic, Depakene®, Depakote®) or carbamazepine (generic,Tegretol®) should receive the highest dose of folic acid. As in other pregnant women, folic acid is most effective in preventing neural tube defects if taken before conception and during the first few weeks of pregnancy. Women on AEDs should be offered aggressive prenatal diagnostic testing including maternal serum testing and targeted ultrasound at 15-20 weeks.

•  Many AEDs can inhibit the transport of vitamin K across the placenta. Therefore, it is recommended that all women on AEDs should receive oral vitamin K (10-20 mg) daily from 36 weeks gestation until delivery. Infants should then also receive intramuscular vitamin K after delivery. Neonatal hemorrhage and shock from vitamin K deficiency carries a mortality rate of 30 percent.

•  Selected references:• 1. Adab N, Tudur Smith C, Vinten J, et al. Common antiepileptic drugs in pregnancy in women with epilepsy.Cochrane Database of Systematic Reviews 2004; 3:CD004848. http://www.cochrane

.org/reviews/en/ab004848.html   Accessed March 2008• 2. Kaplan PW. Reproductive health effects and teratogenicity of antiepileptic drugs. Neurology 2004; 63(10Suppl 4):S13-S23.• 3. Morrell MJ. Epilepsy in women. Am Fam Physician 2002; 66:1489-1494. http://www.aafp.org/afp/20021015/1489.html   Accessed March 2008• 4. Pennell PB. The importance of monotherapy in pregnancy. Neurology 2003; 60(11 Suppl 4):S31-S38.• 5. Pennell PB. Pregnancy in women who have epilepsy. Neurol Clin North Am 2004; 22(4):799-820.• 6. Pschirrer ER. Seizure disorders in pregnancy. Obstet Gynecol Clin North Am 2004; 31(2):373-384.

Rhabdomyolysis is a potentially life-threatening condition with a variety of etiologies. Adverse drug

reactions can present with rhabdomyolysis. Antipsychotic medications can induce

rhabdomyolysis and should be considered in the differential diagnosis of this condition. Which of the following statements regarding rhabdomyolysis is

TRUE? • A:• Rhabdomyolysis occurs only with older antipsychotics such as

chlorpromazine (generic, Thorazine®) but has not been associated with the newer atypical antipsychotics such as olanzapine.

• B:• Rhabdomyolysis is unlikely to occur with a second exposure to an

antipsychotic that caused rhabdomyolysis in a first exposure.• C:• Alcoholism is not considered a risk factor for neuroleptic malignant

syndrome.• D:• A patient with antipsychotic-induced rhabdomyolysis would meet

criteria for neuroleptic malignant syndrome if fever, muscle rigidity and delirium are also present.

• E:• The primary cause of death associated with rhabdomyolysis is stroke.

Answer

• D:• A patient with antipsychotic-

induced rhabdomyolysis would meet criteria for neuroleptic malignant syndrome if fever, muscle rigidity and delirium are also present.

Antipsychotic-Induced Rhabdomyolysis•  Antipsychotic drugs represent one of the pharmacologic classes most commonly associated with toxic rhabdomyolysis. Rhabdomyolysis is a clinical condition of acute muscle injury with

evidence of muscle catabolism. This catabolism produces increased levels of creatinephosphokinase (CPK), myoglobin and other muscle components in the bloodstream and urine. The nephrotoxic effects of these compounds on the kidney can produce acute renal failure.

•  A study of more than 400 patients presenting with rhabdomyolysis found that 46 percent has a significant toxic insult from illicit drugs, alcohol or a variety of pharmacologic agents. Along with antipsychotic agents, statins, zidovudine, colchicine, selective serotonin reuptake inhibitors (SSRIs) and lithium were contributing agents. In the majority of cases, multiple factors were identified as contributing to the rhabdomyolysis. This suggests that the risk ofrhabdomyolysis may be increased by the presence of chronic or acute contributing factors (risk factors). Contributing factors may include systemic infection, dehydration, electrolyte disturbance, alcoholism, rapid antipsychotic dose escalation, antipsychotic-inducedextrapyramidal symptoms and previous brain injury.

•  Conventional antipsychotics such as chlorpromazine (generic, Thorazine®) and haloperidol (generic, Haldol®) have been felt to be more likely to induce rhabdomyolysis than newer agents such as olanzapine (Zyprexa®), risperidone (Risperdal®) and ziprasidone (Geodon®). However, rhabdomyolysis has been described with many of the newer agents and so these compounds do not represent a completely safe alternative to the older agents. A previous episode of antipsychotic-induced rhabdomyolysis increases the risk for recurrentrhabdomyolysis with a second exposure to an offending drug.

•  Antipsychotic-induced rhabdomyolysis may be part of a broader clinical presentation that has been described as neuroleptic (antipsychotic) malignant syndrome (NMS). Antipsychotic-induced rhabdomyolysis can meet criteria for NMS when additional signs and symptoms are clinically present. The diagnostic classification criteria for NMS are listed below.

•  DSM-IV Diagnostic Criteria for Neuroleptic Malignant Syndrome•  - Severe muscle rigidity and fever associated with use of neuroleptic medication

• - Two or more of the following:•           Diaphoresis•           Dysphagia•           Tremor•           Incontinence•           Fluctuating consciousness (delirium)•           Mutism•           Tachycardia•           Elevated or labile blood pressure•           Leucocytosis•           Laboratory evidence (e.g., elevated CPK) of muscle injury – rhabdomyolysis• - Symptoms that are not better explained by another medical or psychiatric condition•  Acute renal failure from medication-induced rhabdomyolysis (not just antipsychotics) is quite common (approximately 50 percent in one study). Death can occur and is usually related to

renal failure or multisystem organ failure. Other serious complications of rhabdomyolysis are disseminated intravascular coagulation and compartment syndrome. Effective treatment forrhabdomyolysis includes removing exposure to the antipsychotic drug, ruling out other treatable causes for rhabdomyolysis, hydration, electrolyte normalization, dialysis and cardiopulmonary support.

•  • Selected references:• 1. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders. 4 th ed. Washington,DC: American Psychiatric Association, 2000.• 2. Ananth J, Parameswaran S, Gunatilake S, et al. Neuroleptic malignant syndrome and atypical antipsychotic drugs. J Clin Psychiatry 2004; 65:1722-1723.• 3. Gupta S, Nihalani N. Neuroleptic malignant syndrome: a primary care perspective. Prim Care Companion JClin Psychiatry 2004; 6:191-184.• 4. Melli G, Chaudhry V, Cornblath DR. Rhabdomyolysis: an evaluation of 475 patients. Medicine 2005; 84:377-385.• 5. Rosebraugh CJ, Flockhart DA, Yasuda SU, et al. Olanzapine-induced rhabdomyolysis. Ann Pharmacother2001; 35:1020-1023.• 6. Sauret JM, Marindes G, Wang GK. Rhabdomyolysis. Am Fam Physician 2002; 65(5):907-912.http://www.aafp.org/afp/20020301/907.html  Accessed March 2008• 7. Webber MA, Mahmud W, Lightfoot JD, et al. Rhabdomyolysis and compartment syndrome withcoadministration of risperidone and simvastin. J Psychopharmacol 2004; 18:432-434.•  

Which one of the following statements concerning insomnia is correct?

A. There is little correlation between insomnia and depression 

B. Daytime drowsiness, excessive snoring, and confusion if awakened from sleep are

associated with restless legs syndrome C. Getting up at the same time every day can

increase the restfulness of sleep D. Patients must take hypnotic drugs for a

prolonged period to cure insomnia E. Insomnia rarely occurs in the elderly

Answer• C. Getting up at the same time every day can increase the

restfulness of sleep  • Explanation: Getting up at the same time every day

stabilizes the sleep-wake schedule and improves the restfulness of actual sleep in bed. Insomnia is more prevalent among the elderly and is associated with depression. Daytime drowsiness, excessive snoring, and confusion when awakened are associated with sleep apnea. Hypnotics should be prescribed only for short-term use. Ref: Kupfer DJ, Reynolds CF III: Management of insomnia. N Engl J Med 1997;336(5):341-346.

A 27-year-old female complains of palpitations. Your clinical evaluation finds no

abnormalities. You recommend that she discontinue her daily regimen of 3–4 cups of regular coffee. Which one of the following symptoms is she most likely to develop?

A. Anxiety B. Depression C. Headache D. Fatigue 

E. Flu-like illness

Answer

• C. Headache• Explanation: In a study of patients with low to

moderate caffeine intake, discontinuing caffeine resulted in moderate to severe headache in 52%. About 10% had depression or anxiety, and less than 10% had fatigue or flu-like symptoms. Ref: Silverman K, Evans SM, Strain EC, et al: Withdrawal syndrome after the double-blind cessation of caffeine consumption. N Engl J Med 1992;327(16):1109-1114. 2) Tierney LM Jr, McPhee SJ, Papadakis MA (eds): Current Medical Diagnosis & Treatment, ed 38. Appleton & La

 A 27-year-old white male has been in rehabilitation for C6 complete quadriplegia. His health had been good prior to a

diving accident 2 months ago which caused his paralysis. The patient has been catheterized since admission and his

recovery has been steady. His vital signs have been normal and stable. The nurse calls and tells you that for the past hour

the patient has experienced sweating, rhinorrhea, and a pounding headache. His heart rate is 55/min and his blood

pressure is 220/115 mm Hg. His temperature and respirations are reported as normal. There has been no vomiting and his

neurologic examination is unchanged. The most likely diagnosis is:

A. Cluster headache B. Autonomic hyperreflexia 

C. Sepsis D. Intracranial hemorrhage 

E. Progression of the spinal cord lesion

Answer• B. Autonomic hyperreflexia  • Explanation: Autonomic hyperreflexia is characterized by the sudden onset of headache

and hypertension in a patient with a lesion above the T6 level. There may be associated bradycardia, sweating, dilated pupils, blurred vision, nasal stuffiness, flushing, or piloerection. It usually occurs several months after the injury and has an incidence as high as 85% in quadriplegic patients. Frequently, it subsides within 3 years of injury, but it can recur at any time. Bowel and bladder distention are common causes. Hypertension is the major concern because of associated seizures and cerebral hemorrhage. Cluster headaches have a constant unilateral orbital localization. The pain is steady (non-throbbing) and lacrimation and rhinorrhea may be part of the syndrome. Sepsis is usually manifested by chills, fever, nausea, and vomiting. Common signs include tachycardia and hypotension rather than bradycardia and hypertension. Signs and symptoms of intracranial hemorrhage vary depending upon the site of the hemorrhage, but the unchanged neurologic status and the lack of a history of hypertension decrease the likelihood of this diagnosis. There are no neurologic findings or history which suggest progression of the patient’s lesion at C6. Ref: Braunwald E, Fauci AS, Kasper DL, et al (eds): Harrison’s Principles of Internal Medicine, ed 15. McGraw-Hill, 2001, pp 70, 78, 799, 2385, 2419.

 Contraindications to thrombolytic therapy in acute

stroke include which one of the following?

A. Age >80 B. Resolving transient ischemic

attack C. Blood glucose >200 mg/dL D. Deficit present for >1 hour

Answer• B. Resolving transient ischemic attack  • Explanation: Thrombolysis is now an approved treatment

for acute stroke. The critical time frame is 3 hours after the onset of the deficit. Beyond that time span, the use of thrombolytic agents is contraindicated. Advanced age per se is not a contraindication to thrombolytic therapy. Contraindications include blood glucose levels <50 mg/dL or >400 mg/dL, resolving transient ischemic attack, and hemorrhage visible on a CT scan. Ref: Rakel RE, Bope ET (eds): Conn’s Current Therapy 2002. WB Saunders Co, 2002, pp 874-876.

Which one of the following therapeutic agents is most appropriate for daily use in the prevention of migraine headache?

A. Dihydroergotamine (D.H.E. 45) B. Amitriptyline (Elavil) C. Sumatriptan (Imitrex) 

D. Aspirin/caffeine/butalbital (Fiorinal) E. Acetaminophen/hydrocodone

bitartrate (Vicodin)

Answer• B. Amitriptyline (Elavil)  • Explanation: Beta-adrenergic blockers, antidepressants,

anticonvulsants, calcium channel blockers, NSAIDs, and serotonin antagonists are the major classes of drugs used for preventive migraine therapy. All of these medications result in about a 50% reduction in the frequency of headaches. The other drugs listed are useful for the treatment of acute migraine, but not for prevention. Ref: Noble SL, Moore KL: Drug treatment of migraine: Part II. Preventive therapy. Am Fam Physician 1997;56(9):2279-2286. 2) Goadsby PJ, Lipton RB, Ferrari MD: Migraine—Current understanding and treatment. N Engl J Med 2002;346(4):257-270.

A 72-year-old woman cuts herself with a clean knife. The wound is 4 cm long on the volar surface of the right forearm. A reliable history of which one of the following would

make tetanus toxoid UNNECESSARY at this time?

A. Tetanus toxoid 1 year ago B. Tetanus toxoid 5 years ago 

C. Tetanus toxoid 11 years ago D. Tetanus toxoid 5 years ago and 3 tetanus

toxoid shots over her lifetime E. Tetanus toxoid 11 years ago and 3 tetanus

toxoid shots over her lifetime

Answer

• D. Tetanus toxoid 5 years ago and 3 tetanus toxoid shots over her lifetime

• Explanation: The Advisory Committee on Immunization Practices of the Centers for Disease Control recommends that for a clean, minor wound, tetanus toxoid should be given if the patient has not had a tetanus toxoid shot within 10 years, with a total of at least 3 prior tetanus toxoid shots. Ref: Tintinalli JE, Kelen GD, Stapczynski JS (eds): Emergency Medicine: A Comprehensive Study Guide, ed 5. McGraw-Hill, 2

The physician counseling a 4-year-old child about the death of a loved one should keep in

mind that children in this age group:A. Often feel no sense of loss 

B. Often believe they are somehow responsible for the death 

C. Should not attend a funeral D. Should usually be told the loved one is

having a long sleep E. Usually accept the finality of death with

little question

Answer• B. Often believe they are somehow responsible for the death • Explanation: Children from the ages of 2 to 6 often believe

they are somehow responsible for the death of a loved one. The emotional pain may be so intense that the child may react by denying the death, or may somehow feel that the death is reversible. If children wish to attend a funeral, or if their parents want them to, they should be accompanied by an adult who can provide comfort and support. Telling a child the loved one is asleep or that he or she “went away” usually creates false hopes for return, or it may foster a sleep phobia. Ref: Behrman RE, Kliegman RM, Jenson HB (eds): Nelson Textbook of Pediatrics, ed 16. WB Saunders Co, 2000, p 109.

 A 40-year-old sedentary white male with a family history of stroke sees you for a health maintenance visit. His blood pressure averages 150/100 mm Hg

over several visits. His LDL cholesterol level is 170 mg/dL. Which one of the following would have the

greatest impact on decreasing his future risk of stroke?

A. Reducing his blood pressure to normal levels B. Reducing his LDL cholesterol level to <130

mg/dL C. Aspirin, 81 mg daily 

D. A program of regular physical exercise

Answer• A. Reducing his blood pressure to normal levels  • Explanation: Stroke is the third leading cause of death in the United

States, and hypertension is the most consistently powerful predictor of stroke. There is strong and consistent evidence supporting the efficacy of antihypertensive therapy in reducing stroke risk. Several other interventions have been shown to lower stroke risk to a lesser extent, including statin treatment of high cholesterol, daily aspirin, and regular exercise. Ref: Bronner LL, Kanter DS, Manson JE: Primary prevention of stroke. N Engl J Med 1995;333(21):1392, 1395, 1397. 2) Goldman L, Bennett JC (eds): Cecil Textbook of Medicine, ed 21. WB Saunders Co, 2000, pp 2103-2104. 3) Braunwald E, Fauci AS, Kasper DL, et al (eds): Harrison’s Principles of Internal Medicine, ed 15. McGraw-Hill, 2001, p 2383.

 A mother gives her 3-year-old child a cookie whenever he starts

to whine. In behavioral terms, she is using:

A. Positive reinforcement B. Negative reinforcement 

C. Extinction D. Bonding

Answer• A. Positive reinforcement • Explanation: This situation illustrates a common problem in the

behavioral management of children, namely positive reinforcement or reward for a negative or undesirable behavior. By giving the child a cookie, the mother is actually increasing the likelihood that the child will whine. Putting a child in time-out for whining would be an example of negative reinforcement. Letting the child continue to whine by ignoring the behavior is termed extinction. Bonding is not a term used in behavioral management, but it describes the affectional relationship between parents and infants. Bonding occurs rapidly and shortly after birth and reflects the feelings of the parents toward a newborn (unidirectional). Ref: Behrman RE, Kliegman RM, Jenson HB (eds): Nelson Textbook of Pediatrics, ed 16. WB Saunders Co, 2000, pp 15-16.

Which one of the following is associated with an increased risk of developing

Alzheimer’s disease?A. A positive homozygous genotype for

apolipoprotein E4 B. Elevated serum aluminum 

C. Elevated serum alpha-tocopherol D. Decreased serum beta-carotene 

E. A low-fiber diet

Answer

• A. A positive homozygous genotype for apolipoprotein E4  • Explanation: Recent genetic work has demonstrated a high risk

for the development of Alzheimer’s disease in patients with the apolipoprotein E4 genotype. Other predictors of the disease have been elusive. Serum aluminum is not predictive, although higher than normal brain aluminum levels have been studied for many years. Eating a low-fiber diet has no known correlation, and serum levels of beta-carotene and vitamin E are not useful for predicting the development of Alzheimer’s disease, although supplementation is considered helpful by some sources. Ref: Martinez M, Campion D, Brice A, et al: Apolipoprotein E epsilon4 allele and familial aggregation of Alzheimer disease. Arch Neurol 1998;55(6):810-816.

A previously alert, otherwise healthy 74-year-old African-American male has a history of slowly developing progressive memory loss

and dementia associated with urinary incontinence and gait disturbance resembling ataxia. This presentation is most consistent

with:A. Normal pressure hydrocephalus 

B. Alzheimer’s disease C. Subacute sclerosing panencephalitis 

D. Multiple sclerosi

Answer• A. Normal pressure hydrocephalus  • Explanation: In normal pressure hydrocephalus a mild impairment of memory typically

develops gradually over weeks or months, accompanied by mental and physical slowness. The condition progresses insidiously to severe dementia. Patients also develop an unsteady gait and urinary incontinence, but there are no signs of increased intracranial pressure. In Alzheimer’s disease the brain very gradually atrophies. A disturbance in memory for recent events is usually the first symptom, along with some disorientation to time and place; otherwise, there are no symptoms for some period of time. Subacute sclerosing panencephalitis usually occurs in children and young adults between the ages of 4 and 20 years and is characterized by deterioration in behavior and work. The most characteristic neurologic sign is mild clonus. Multiple sclerosis is characteristically marked by recurrent attacks of demyelinization. The clinical picture is pleomorphic, but there are usually sufficient typical features of incoordination, paresthesias, and visual complaints. Mental changes may occur in the advanced stages of the disease. About two-thirds of those affected are between the ages of 20 and 40. Ref: Humes HD (ed): Kelley’s Textbook of Internal Medicine, ed 4. Lippincott Williams & Wilkins, 2000, pp 2919-2921, 2954-2955. 2) Victor M, Ropper AH: Adams and Victor’s Principles of Neurology, ed 7. McGraw-Hill, 2001, pp 663-665

When evaluating a patient on chronic phenytoin (Dilantin)

therapy for a seizure disorder, which one of the following is a

sign of toxicity?A. Peripheral neuropathy 

B. Ataxia C. Clonus 

D. Ballistic movements E. Photophobia

Answer

• B. Ataxia • Explanation: Family physicians often see patients

with seizure disorders well controlled on phenytoin. However, due to its many side effects and associated illnesses, careful monitoring is required. Screening for ataxia, which is often subtle, must be performed at each visit, even when following blood levels at regular intervals. Ref: Victor M, Ropper AH: Adams and Victor’s Principles of Neurology, ed 7. McGraw-Hill, 2001, pp 356-361.

A 70-year-old white female who has been your patient for 10 years had an emergency cholecystectomy 2 days ago. When you see her today while

making rounds, she appears to be confused. When you ask her how she is, she just stares at your stethoscope, and then says, “That snake may bite you.” When you ask further questions she seems distracted and does not

answer the question asked. At times, she closes her eyes and seems to fall asleep unless questioned. She does not know her daughter, who is in the room when you are. Which one of the following additional observations

would help you differentiate delirium from dementia?A. Her pulse, blood pressure, temperature, and respiratory rate are all

normal B. She cannot remember today’s date or the day of the month, interpret

proverbs, name the president, or even remember your name C. Her neurologic examination is normal, except for the noted mental

status changes D. Her mental status was normal before surgery, and on successive visits

it fluctuates

Answer

• D. Her mental status was normal before surgery, and on successive visits it fluctuates

• Explanation: An acute onset and fluctuating course, along with an altered level of consciousness, illusions, and distractibility are consistent with delirium according to current diagnostic criteria. A normal neurologic and general physical examination, as well as memory and orientation problems, are common to both states. Ref: Bross MH, Tatum NO: Delirium in the elderly patient. Am Fam Physician 1994;50(6):1325-1332. 2) Hazzard WR, Blass JP, Ettinger WH Jr, et al (eds): Principles of Geriatric Medicine and Gerontology, ed 4. McGraw-Hill, 1999, pp 1229-1237. 3) Rakel RE: Textbook of Family Practice, ed 6. WB Saunders Co, 2002, pp 1365-1367.

You have diagnosed tardive dyskinesia in a 72-year-old white female with schizophrenia. She resides in a nursing home and has been treated with haloperidol (Haldol), 1 mg twice a day, for 5 years. She also has

a hiatal hernia. Which one of the following statements is true regarding this patient?

A. The chances of symptom remission after withdrawal of the haloperidol are better than for a

younger patient B. Quickly reducing the dosage of haloperidol will lead to prompt worsening of her tardive dyskinesia 

C. Long-term metoclopramide (Reglan) would be the best treatment for her hiatal hernia 

D. Risperidone (Risperdal) would be more likely than haloperidol to cause tardive dyskinesia

Answer• B. Quickly reducing the dosage of haloperidol will lead to prompt

worsening of her tardive dyskinesia  • Explanation: Symptom remission is more likely to occur after

neuroleptic withdrawal in young patients than in the elderly. Tardive dyskinesia is initially exacerbated by a reduction in neuroleptic dosage, and dyskinesias decrease following an increase in the dosage. Metoclopramide has been shown to cause tardive dyskinesia with long-term treatment, and therefore would not be the best drug for the patient’s hiatal hernia. There is no convincing evidence that any of the traditional antipsychotic drugs is less likely to produce tardive dyskinesia than any other, but the newer atypical agents such as clozapine, risperidone, and olanzapine offer some hope for a reduced incidence. Ref: Sadock BJ, Sadock VA (eds): Kaplan & Sadock’s Comprehensive Textbook of Psychiatry, ed 7. Lippincott Williams & Wilkins, 2000, pp 295-296, 2295-2296, 3075-3076.

A 38-year-old alcoholic male has successfully completed outpatient alcohol detoxification and has plans to participate in Alcoholics Anonymous. Which one of the following pharmacologic agents can aid in relapse

prevention?A. Naltrexone (ReVia) B. Naloxone (Narcan) 

C. Bupropion (Wellbutrin) D. Mirtazapine (Remeron) E. Flumazenil (Romazicon)

Answer• A. Naltrexone (ReVia) • Explanation: Pharmacologic agents can be a useful

adjunct to counseling in preventing relapse in patients with alcohol dependence. Naltrexone and disulfiram are currently approved by the FDA for the treatment of alcohol-dependent patients. Bupropion is of value for smoking cessation and mirtazapine is an antidepressant. Naloxone is used to treat opioid overdose and flumazenil to treat benzodiazepine overdose. Ref: Fiellin DA, Reid MC, O’Connor PG: Outpatient management of patients with alcohol problems. Ann Intern Med 2000;133(10):815-827.

It would be most appropriate to WITHHOLD rabies prophylaxis for

which one of the following?A. A rat bite occurring in the patient’s

basement B. A bat bite sustained on a hiking trip 

C. A dog bite from an unprovoked cocker spaniel not found for observation D. A raccoon bite occurring on a hunting

trip

Answer• A. A rat bite occurring in the patient’s basement  • Explanation: Rabies postexposure prophylaxis (RPEP) should

be given for all bat bites and most raccoon bites unless brain test results will be available within 48 hours. Bites from small rodents (e.g., rats, mice, and squirrels) never require RPEP. RPEP should be given after a domestic animal bite if it was unprovoked and/or the animal demonstrated abnormal behavior and is not available for observation. Ref: Moran GJ, Talan DA, Mower W, et al: Appropriateness of rabies postexposure prophylaxis treatment for animal exposures. JAMA 2000;284(8):1001-1007. 2) Human rabies prevention—United States, 1999: Recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR 1999;48(

Children typically manifest anxiety toward strangers at what

age?A. 3 months B. 9 months 

C. 18 months D. 24 months E. 30 months

Answer

• B. 9 months  • Explanation: At 3–4 months of age, a normal term

baby will smile at almost anyone. By 9 months of age, there is a developmentally normal anxiety over separation from the mother (or primary caregiver), as well as anxiety at the sight of an unfamiliar face. Coincident with the increased mobility gained by walking (usually at 12–15 months of age), these anxieties normally abate. Ref: Behrman RE, Kliegman RM, Jenson HB (eds): Nelson Textbook of Pediatrics, ed 16. WB Saunders Co, 2000, p 36.

A 30-year-old gravida 3 para 2 at 28 weeks' gestation is a restrained passenger in a high-speed motor vehicle accident.

After initial stabilization in the field with supplemental oxygen and intravenous fluids, she is brought into the

emergency department on a backboard and wearing a cervical collar. Until you are able to rule out a spinal injury, in what

position should the patient be kept?A. Supine 

B. Supine, with the uterus manually deflected laterally C. Prone 

D. Trendelenburg’s position E. Left lateral decubitu

Answer• B. Supine, with the uterus manually deflected laterally• Explanation: In general, it is best to place a woman who is greater than

20 weeks pregnant in the left lateral decubitus position because the uterus can compress the great vessels, resulting in decreased systolic blood pressure and uterine blood flow. However, in the case of trauma where a spinal cord injury cannot be ruled out, the woman needs to be kept supine on a backboard. The weight of the uterus can be shifted off the great vessels by either manual deflection laterally or by elevating the right hip 4–6 inches by placing towels under the backboard. The Trendelenburg position does not relieve the weight of the uterus on the great vessels. The prone position does not provide adequate spinal cord protection, and would be extremely awkward in a large pregnant woman. Ref: Advanced Trauma Life Support for Doctors, ed 3. American College of Surgeons, 2001, p 319. 2) Grossman NB: Blunt trauma in pregnancy. Am Fam Physician 2004;70(7):1303-1310.

Promoting good sleep hygiene is basic in the treatment of insomnia. Which one of the following

measures will aid in promoting healthy sleep habits?A. Vigorous evening exercise 

B. Taking an enjoyable book or magazine to bed to read 

C. Drinking a glass of wine as a sedative before retiring 

D. Eating the heaviest meal of the day close to bedtime 

E. Maintaining a regular sleep/wake schedule

Answer• E. Maintaining a regular sleep/wake schedule • Explanation: Maintaining a regular sleep/wake schedule

helps prevent insomnia. While a light snack before bed may be sleep inducing, heavy meals close to bedtime may be counterproductive. Alcohol should be avoided as a sedative, to prevent midsleep awakenings. Hours spent reading or watching television in bed can lead to long awakenings in the middle of the night. Ref: Kasper DL, Braunwald E, Fauci AS, et al (eds): Harrison’s Principles of Internal Medicine, ed 16. McGraw-Hill, 2005, pp 156-158. 2) Doghramji K, Kuritzky L: Strategies For Managing Insomnia. AAFP Video CME Program Monograph, 1999, p

You are asked to perform a preoperative evaluation on a 75-year-old male

scheduled for a cholecystectomy. Which one of the following would be most

predictive of postoperative delirium?A. Anxiety 

B. Dementia C. Depression D. Psychosis

Answer

• B. Dementia  • Explanation: Older patients have a high incidence of

post-anesthesia delirium and thus should have a mental status examination before and after surgery. Although patients with anxiety, depression, and psychosis may have particular perioperative problems, patients with dementia are more likely to develop postoperative delirium. Ref: Evans JG, Williams TF, Beattie BL, et al: Oxford Textbook of Geriatric Medicine, ed 2. Oxford University Press, 2000, p 1037.

. A 75-year-old white male complains of a tremor which has been progressive over the past 2 years.

The tremor interferes with writing, pouring liquids, and eating soup. He has no other medical problems.

He abstains from alcohol and tobacco products. Physical examination is remarkable for an action

tremor of the upper extremities and a head tremor. No rigidity or gait disorder is noted. Of the following agents, which one is most appropriate as initial drug

therapy for this problem?A. Alprazolam (Xanax) 

B. Clonazepam (Klonopin) C. Carbamazepine (Tegretol) 

D. Propranolol (Inderal) E. Theophylline

Answer• D. Propranolol (Inderal)  • Explanation: Essential tremor is the most likely cause of a disabling

action tremor in this age group. A resting tremor, rigidity, and other associated problems are seen with Parkinson’s disease. Propranolol and primidone are the agents of choice. Alprazolam may have beneficial effects, but it is not a first-line agent because of the risk of sedation and habituation. Clonazepam has not been found to be effective in this disorder. Low doses of theophylline have been found to be somewhat beneficial, but it is considered a second-line agent for essential tremor. Selective beta-blockers such as metoprolol are not as effective as propranolol. Ref: Duthie EH Jr: Practice of Geriatrics, ed 3. WB Saunders Co, 1998, pp 339-340. 2) Goroll AH: Primary Care Medicine, ed 4. Lippincott Williams & Wilkins, 2000, pp 954-955. 3) Louis ED: Essential tremor. N Engl J Med 2001;345(12):887-891.

The parents of a 20-month-old female bring her to your office because she has lost consciousness twice recently. They

describe two episodes where the child was crying vigorously then “turned purple and passed out.” The child is an

otherwise healthy product of a term delivery. There is no history of head trauma and no family history of seizures or

cardiac problems. The episodes are not associated with fever or other symptoms. Physical examination of the child is

normal. Which one of the following would be most appropriate at this point?

A. Reassurance B. A CT scan of the brain 

C. An EKG and chest radiograp D. Measurement of serum glucose, electrolytes, and

hematocrit E. Echocardiography

Answer• A. Reassurance • Explanation: The parents are describing classic breath-holding spells. These

are a form of autonomic syncope frequently misdiagnosed as seizures. They occur in early childhood and infancy. They can be of two forms: cyanotic, as described here, and pallid. The cyanotic form usually occurs after vigorous crying, while the pallid form commonly occurs after a sudden fright or minor injury. The history of a prodrome of injury, vigorous crying, or sudden fright is key to distinguishing a breath-holding spell from a seizure. Parents can be reassured that no brain damage occurs and, in the presence of a classic history, no further workup is necessary. An EKG and chest radiograph would be indicated if the history or examination suggested cardiac syncope. Blood testing would be indicated if the history suggested orthostatic hypotension or diabetes. A head CT scan would be indicated in the evaluation of seizures. Ref: Tintinalli JE, Kelen GD, Stapczynski JS (eds): Emergency Medicine: A Comprehensive Study Guide, ed 5. McGraw-Hill, 2000, p 872.

A 40-year-old male professional consults you about his recent onset of depression. He generally feels well, but sometimes

feels “high and out of control.” This is followed by significant depression which usually remits after a long

weekend of sleep at his cabin on the lake. He also complains of persistent nasal congestion and a 10-lb weight loss. His

psychiatric history is negative, but he is suspicious and feels that people are against him. His mental status otherwise

reveals normal thought content and processes. His physical examination is normal except for inflamed nares and enlarged

nasal turbinates. The most likely diagnosis is:A. Schizophrenia 

B. Generalized anxiety disorder C. Panic disorder D. Alcohol abuse E. Cocaine abuse

Answer• E. Cocaine abuse • Explanation: A chronic user of cocaine, like the chronic user of

alcohol, does not always fit the classic description of dependence, and the physician must therefore consider the diagnosis in all patients with episodic depression and peculiar mood swings. Organic symptoms are like those of amphetamine use, mainly hyperpyrexia, tachycardia, and even cardiac arrhythmias. Routine, continued cocaine “snorting” often leads to nasal mucosal congestion and occasional septal perforation. Paranoid ideation is sometimes seen with the use of cocaine and other stimulants. The patient’s age and normal mental status make schizophrenia unlikely. Panic disorder is not complicated by paranoid behavior. Ref: Sadock BJ, Sadock VA (eds): Kaplan & Sadock’s Comprehensive Textbook of Psychiatry, ed 7. Lippincott Williams & Wilkins, 2000, pp 1003-1004.

An 85-year-old white male is brought to you for the first time by his son. The father has recently seen a neurologist who performed a workup for dementia

and diagnosed moderate Alzheimer’s disease. Which one of the following is true regarding the use of a

cholinesterase inhibitor in this patient?A. It is too late to initiate cholinesterase therapy B. Agitation is often intensified by these agents 

C. Memory is likely to improve significantly D. If the patient has a vascular dementia rather than Alzheimer’s dementia the drug will not be useful 

E. Nursing-home placement may be delayed a year or longer

Answer• E. Nursing-home placement may be delayed a year or longer • Explanation: Medications for dementia should be prescribed with caution, and

the patient watched closely for side effects. Currently available cholinesterase inhibitors are at least as effective for vascular dementia as they are for Alzheimer’s dementia, although they are not approved for this use by the FDA. These agents include donepezil, rivastigmine, and galantamine, and they are often helpful in Alzheimer’s disease patients with agitation. Anticholinesterase therapy is considered the standard of care for Alzheimer’s disease, and therapy can begin at any stage in the disease process, although early therapy is the most beneficial. While the medication will not restore memory, it does prevent the rapid loss of more memory. Long-term studies on effectiveness are still in progress, but most evidence at present indicates that nursing-home placement can be delayed a year and possibly longer. Ref: Cefalu C, Grossberg GT: Diagnosis and Management of Dementia. American Family Physician monograph series, 2001, no 2, pp 13-15. 2) Bonner LT, Peskind ER: Pharmacologic treatments of dementia. Med Clin North Am 2002;86(3):657-674.

You are treating an 89-year-old white male who has lived alone since his wife died 5 years ago. His niece found him helpless in his apartment. The patient is filthy, listless, and

weak, and complains of thirst. He is oriented to self, but he is sure that you are his pastor and that Nixon is president. His

general physical examination reveals cardiomegaly and peripheral edema. Findings on neurologic examination include horizontal and vertical nystagmus, weakness of

lateral recti, ataxia, and peripheral areflexia. Plantar responses are downpointing. A CBC is pending; electrolyte,

BUN, and glucose levels obtained in the emergency department reveal hypertonic dehydration for which 5% dextrose in ½-normal saline is running at 200 cc/hr. The

patient’s drowsiness increases during your examination. You order which one of the following?

A. Cyanocobalamin (vitamin B12) B. Thiamine 

C. Methylprednisolone sodium succinate (Solu-Medrol) D. A stat carboxyhemoglobin determination

Answer• B. Thiamine • Explanation: Alcoholism, while less frequent in the elderly, is often

masked by isolation. Elderly widowers are in the highest risk group. Several features of this case, including the long-term inattention to self, gaze disturbance, cerebellar signs, confabulation (confidence in the face of confusion), and better past than present memory, all suggest Wernicke’s encephalopathy. The presence of signs of wet beriberi related to the same nutritional deficiency support the diagnosis. The patient is at immediate risk and thiamine should be administered right away. Other diagnoses are less likely. Pernicious anemia causes no prominent eye motor signs; temporal arteritis, lupus, and Takayasu’s vasculitis cause lateralizing signs; lead poisoning doesn’t generally cause cardiomegaly; and carbon monoxide intoxication is more acute, causing headache and nonselective confusion. Ref: Braunwald E, Fauci AS, Kasper DL, et al (eds): Harrison’s Principles of Internal Medicine, ed 15. McGraw-Hill, 2001, p 2496.

An 18-year-old patient presents with his father for a pre-college physical examination. The student plans to attend college in a nearby state and will live in a university-owned residence hall. A review of his record shows that he received all of the currently recommended immunizations on time throughout

childhood. The benefits of vaccination against which one of the following organisms should be discussed

during this visit?A. Measles B. Tetanus C. Pertussis 

D. Meningococcus E. Polio

Answer• D. Meningococcus  • Explanation: College freshmen, especially those who live in dormitories,

are at a modestly increased risk for meningococcal disease compared with other persons of the same age, and vaccination with the currently available quadrivalent meningococcal polysaccharide vaccine will decrease their risk. The Advisory Committee on Immunization Practices of the Centers for Disease Control and Prevention recommends that incoming and current college freshmen, and their parents, be informed about meningococcal disease and the benefits of vaccination. This is particularly true for those who live in dormitories and residence halls. In this case, the patient has received vaccinations against measles, polio, pertussis, and tetanus at the recommended times, and booster vaccinations are not indicated now. Ref: Meningococcal disease and college students: Recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR 2000;49(RR-7):13-20.

Compared with younger adults, healthy older adults:

A. Fall asleep more quickly at bedtime 

B. Awaken less frequently during the night 

C. Spend less time awake in bed D. Spend fewer hours in stages of

deep sleep

Answer• D. Spend fewer hours in stages of deep sleep • Explanation: Normal older adults require less total sleep

time. This change begins by the age of 50, and gradually increases with time. By age 75, total sleep time is reduced to 6 or 7 hours per night, and by age 85, 5 to 6 hours of total sleep time is biologically and physiologically normal. Older adults take longer to fall asleep, awaken more frequently at night, spend more time awake in bed, and spend far less time in stages of deep sleep. Ref: Lantz MS: Insomnia and sleep complaints: What is normal in the older adult? Clin Geriatr 2002;10

A 62-year-old male with a previous history of hypertension, smoking, and hypercholesterolemia

comes to your office after being seen in the emergency department with an episode of dysarthria and weakness on the left side of his body. Physical examination reveals a right carotid bruit. Carotid ultrasonography shows 70%–80% stenosis of the

right carotid artery, which is confirmed by magnetic resonance angiography. In addition to management

of risk factors, which one of the following is appropriate?

A. Long-term aspirin therapy B. Aspirin plus clopidogrel (Plavix) 

C. Warfarin (Coumadin) D. Carotid endarterectomy

Answer• D. Carotid endarterectomy • Explanation: Carotid stenosis is an important cause of transient

ischemic attacks and stroke. For patients with symptomatic carotid stenosis of more than 70%, the value of carotid endarterectomy has been firmly established on the basis of three major randomized trials. Both the North American Symptomatic Carotid Endarterectomy Trial (NASET) and the European Carotid Surgery Trial (ECST) showed that only seven or eight patients would need to undergo endarterectomy to prevent one stroke in a 5-year period. The trial by the Veterans Affairs Cooperative Studies Program also showed that endarterectomy was the best treatment in this situation. Ref: Sacco RL: Extracranial carotid stenosis. N Engl J Med 2001;345(15):1113-1118.

 An 82-year-old white male has a transient ischemic attack. A carotid duplex study

reveals an 85% stenosis in the affected carotid artery. Which one of the following would be

the most appropriate management at this time?A. Aspirin 

B. Aspirin plus extended-release dipyridamole (Aggrenox) 

C. Warfarin (Coumadin) D. Clopidogrel (Plavix) 

E. Surgical referral for carotid endarterectomy

Answer

• E. Surgical referral for carotid endarterectomy • Explanation: Carotid endarterectomy is beneficial in patients

with internal carotid artery stenosis of 70%–99% who have had a stroke or a transient ischemic attack attributable to the stenosis. The short-term risk of stroke is high among patients with severe stenosis of the internal carotid artery. Although data are lacking on the optimal timing of endarterectomy, when infarction is absent or limited, urgent endarterectomy is probably indicated for patients with internal carotid artery stenosis of 70%–99% and in selected patients with stenosis of 50%–69% who can be treated surgically with a low risk of complications. Ref: Johnston SC: Transient ischemic attack. N Engl J Med 2002;347(21):1687-1692.

For the past 3 days, an 11-year-old white male has had several small, circular lesions on the left side of his lower

forehead and nose, extending to the tip of the nose. On examination you note grouped vesicles, several of which are scabbed. The patient also complains of burning pain in the area of the lesions. He had a sore throat with a fever 5 days ago, but is now improved. His stepfather reports he is up-to-date on immunizations, but a specific immunization record is

not available. His history and examination are otherwise unremarkable. The most likely diagnosis is:

A. Herpes zoster B. Undetected immune deficiency C. Primary herpes simplex type 1 

D. Impetigo E. Erythema multiforme

Answer

• A. Herpes zoster • Explanation: Clustered circular lesions with

accompanying dysesthesia in a dermatome are characteristic of herpes zoster, which may occur after a stressful event or infection in both children and adults. For herpes zoster to occur there must be a previous primary varicella infection or immunization. Herpes zoster is less likely to be associated with significant postherpetic neuralgia in children than in adults. Ref: Behrman RE, Kliegman RM, Jenson HB (eds): Nelson Textbook of Pediatrics, ed 16. WB Saunders Co, 2000, pp 9

In differentiating early Parkinson’s disease from variants such as drug-induced parkinsonism, progressive

supranuclear palsy, and multiple system atrophy, which one of the following is

the most important clue?A. A resting tremor 

B. Bradykinesia C. Rigidity 

D. Gait difficulty E. Loss of postural reflexes

Answer

• A. A resting tremor• Explanation: Resting tremor is the most common

presentation of Parkinson’s disease. It is rare in progressive supranuclear palsy and multiple system atrophy, and less common in drug-induced parkinsonism. Ref: Hazzard WR, Blass JP, Ettinger WH Jr, et al (eds): Principles of Geriatric Medicine and Gerontology, ed 4. McGraw-Hill, 1999, pp 1272-1274  

 A 28-year-old female complains of generalized headache, dizziness (characterized as lightheadedness), and generally not feeling well for 3 days. This started at the same time as her menses and coincided with a

major examination in a college class she is taking. Her review of symptoms is otherwise negative. Her past medical history includes a

recent acute onset of low back pain related to lifting, and a recent depressive episode which responded well to medication. Her current

medications include an oral contraceptive which she has taken for 2 years, a corticosteroid nasal spray, and ibuprofen for the past 2 weeks. She was on paroxetine (Paxil), 30 mg/day, for 7 months, but this was stopped 5 days ago because of sexual dysfunction. Because of her symptoms she

has not taken any medications for the past 2 days. Since then the headache has eased substantially, but the feeling of lightheadedness has remained. A physical examination is unremarkable. Which one of the following is

the most likely cause of her symptoms?A. Allergic rhinitis 

B. Paroxetine withdrawal C. Serotonin syndrome 

D. Viral infection E. Stress

Answer

• B. Paroxetine withdrawal  • Explanation: The timing of the symptoms (starting

about 2 days after paroxetine was stopped) and the symptoms (headache, lightheadedness) are consistent with SSRI discontinuation syndrome. This syndrome is more likely with abrupt withdrawal, after prolonged treatment, and at higher doses. Ref: Rosenbaum JF, Fava M, Hoog SL, et al: Selective serotonin reuptake inhibitor discontinuation syndrome: A randomized clinical trial. Biol Psychiatry 1998;44(2):77-87.

A 66-year-old white female consults you because she has developed a tremor of her right hand that interferes with her

ability to do needlework. She has noticed that the tremor improves when she rests her hands in her lap and gets worse when she holds them up against gravity. She has developed a

slight quiver to her voice as well. Her symptoms started gradually over 6 months ago and have progressed slowly. She

remembers her mother having similar problems in her later years. She takes no medications, and her physical

examination corroborates her history. No other abnormalities are noted. A multiple chemistry screen and TSH level are

normal. Which one of the following is most likely to alleviate her tremor?

A. Propranolol (Inderal) B. Paroxetine (Paxil) 

C. Carbidopa/levodopa (Sinemet) D. Bromocriptine (Parlodel) E. Carbamazepine (Tegretol)

Answer• A. Propranolol (Inderal)• Explanation: This patient has essential tremor, which is

frequently a familial condition. Primidone and propranolol are the drugs most likely to provide relief of essential tremor. Other beta-blockers such as atenolol and metroprolol may not be as effective, although results of trials are mixed. Antiparkinsonian medications such as carbidopa and bromocriptine have no effect on essential tremor. Carbamazepine is occasionally useful, but is much less likely to be effective than primidone. Ref: Louis ED: Essential tremor. N Engl J Med 2001;345(12):887-891  

Which one of the following is a common early side effect of

fluoxetine (Prozac)?A. Constipation 

B. Loss of appetite C. Orthostatic hypotension D. Atrioventricular block 

E. Skin rash

Answer• B. Loss of appetite • Explanation: Fluoxetine, a selective serotonin reuptake

inhibitor, has no effect on the norepinephrine system; therefore, it does not produce the side effects common to the tricyclic antidepressants. These include anticholinergic side effects (dry mouth, constipation), orthostatic hypotension, cardiac conduction disturbances, and drowsiness. Loss of appetite is often seen in patients who take fluoxetine, and can be especially troublesome in the elderly. Skin rash is uncommon. Ref: Tasman A, Kay J, Lieberman JA (eds): Psychiatry. WB Saunders Co, 1997, pp 1616-1619. 2) Physicians’ Desk Reference, ed 54. Medical Economics Co, 2000, pp 962-967.

Which one of the following is useful in migraine prophylaxis?

A. Cyanocobalamin (vitamin B12) 

B. Riboflavin (vitamin B2) C. Ascorbic acid (vitamin C) 

D. Cholecalciferol (vitamin D) E. Tocopherol (vitamin E)

Answer

• B. Riboflavin (vitamin B2) • Explanation: A daily oral dose of 400 mg of riboflavin

has been shown to be superior to placebo for migraine prophylaxis. The effect of riboflavin begins after 1 month of treatment and is maximal after 3 months of treatment. Its effect is most pronounced on attack frequency and the number of days patients have a headache. Ref: Schoenen J, Jacquy J, Lenaerts M: Effectiveness of high-dose riboflavin in migraine prophylaxis: A randomized controlled trial. Neurology 1998;50(2):466-470.

Which one of the following sleep disorders is in the general class of circadian sleep disorders and may respond to bright-light therapy?

A. Shift-work insomnia B. Alcohol-dependent sleep

disorder C. Inadequate sleep hygiene D. Sleep-related myoclonus

Answer• A. Shift-work insomnia • Explanation: Shift-work insomnia is the only circadian sleep

disorder listed. It may respond to bright-light therapy. Alcoholism is a behavioral disorder that may respond to gradual discontinuance. Inadequate sleep hygiene (use of stimulants at night, sleeping other than at bedtime, etc.) may respond to habit changes. Sleep-related myoclonus is an intrinsic sleep disorder and can be treated with levodopa or clonazepam. Ref: Sadock BJ, Sadock VA (eds): Kaplan & Sadock’s Comprehensive Textbook of Psychiatry, ed 8. Lippincott Williams & Wilkins, 2005, pp 2023-2030. 2) Kasper DL, Braunwald E, Fauci AS, et al (eds): Harrison’s Principles of Internal Medicine, ed 16. McGraw-Hill, 2005, pp 153-162.

Pallidotomy is a surgical therapy for:

A. Alzheimer’s disease B. Parkinson’s disease C. Huntington’s chorea D. Vascular dementia 

E. Temporal lobe epilepsy

Answer

• B. Parkinson’s disease • Explanation: Thalamotomy and pallidotomy,

contralateral to the side of the body that is most affected, are most effective for the treatment of disabling unilateral tremor and dyskinesia from Parkinson’s disease. Ref: Brownlee HJ, Hubble JP: Current Approaches to the Management of Parkinson’s Disease. AAFP Video CME Program Monographs, 2000, p 13. 2) Victor M, Ropper AH: Adams and Victor’s Principles of Neurology, ed 7. McGraw-Hill, 2001, pp 1136-1137.

 In healthy adults, performance on the Folstein Mini-Mental State Examination is affected by which

one of the following?A. Educational attainment B. Socioeconomic status 

C. Gender D. Race

Answer• A. Educational attainment  • Explanation: The Mini-Mental State Examination,

developed by Folstein in 1975, has become a standard tool for rapid clinical assessment of cognitive impairment. The score is known to be affected by the patient’s educational attainment. Given the same level of cognitive impairment, those with higher education levels score somewhat better than those with less education. Race, sex, and socioeconomic status per se do not affect patients’ scores. Ref: Ross GW, Bowen JD: The diagnosis and differential diagnosis of dementia. Med Clin North Am 2002;86(3):455-476.

Which one of the following would support a diagnosis of carpal tunnel

syndrome?A. Absence of symptoms at night 

B. Numbness in the fourth and fifth digits 

C. Wasting of the thenar compartment 

D. Provocation of symptoms by sustained wrist extension

Answer• C. Wasting of the thenar compartment  • Explanation: Carpal tunnel syndrome (CTS) is an entrapment neuropathy of the

median nerve at the wrist, producing paresthesias and weakness of the hands. The syndrome is caused by pressure on the median nerve where it and the flexor tendons of the fingers pass through the tunnel formed by the carpal bones and the transverse carpal ligament. It usually begins with a gradual onset of numbness, tingling, and pain in the hand and wrist. Symptoms are often present at night, during sleep, and when the wrists are flexed. The symptoms occur in the thumb and the index and middle fingers, and occasionally in part of the fourth finger. The fifth finger is never involved. The thenar compartment is innervated by the median nerve and may atrophy as the syndrome progresses. The hypothenar musculature is not involved. Physical signs of CTS include a positive Phalen’s maneuver, which is a provocation of symptoms by sustained wrist flexion. Symptoms can be precipitated by activities which require repeated flexion, pronation, and supination of the wrist, e.g., sewing, driving, operating computers and cash registers, and playing golf. Ref: Goldman L, Bennett JC (eds): Cecil Textbook of Medicine, ed 21. WB Saunders Co, 2000, p 1559. 2) Victor M, Ropper AH: Adams and Victor’s Principles of Neurology, ed 7. McGraw-Hill, 2001, pp 1433-1434.

A 15-month-old male is brought to the emergency department following a generalized tonic-clonic seizure at home. The

parents report that the seizure lasted 5 minutes, with confusion for the next 15 minutes. This is the child’s first

seizure. There is no family history of seizures. His medical history is normal except for a 1-day history of a URI. While initially lethargic in the emergency department, the child is

now awake and playful, with a temperature of 39.5 degrees C (103.2 degrees F) and a normal examination. Appropriate diagnostic tests are performed, including a blood glucose

level, which is 96 mg/dL. Which one of the following would be most appropriate to administer at this point?

A. Phenytoin (Dilantin) intravenously B. Ceftriaxone (Rocephin) intravenously 

C. Acetaminophen orally D. Carbamazepine (Tegretol) orally 

E. Phenobarbital orally

Answer• C. Acetaminophen orally • Explanation: This child has had a simple febrile seizure, the most

common seizure disorder of childhood. Treatment includes finding a source for the fever; this should include a lumbar puncture if meningitis is suspected. The most common infections associated with febrile seizures include viral upper respiratory infections, otitis media, and roseola. Antipyretics are the first-line treatment. Antibiotics are indicated only for appropriate treatment of underlying infections. Phenytoin and carbamazepine are ineffective for febrile seizures. Phenobarbital is sometimes used for prevention of recurrent febrile seizures, but is not indicated as an initial therapy. Only 30%–50% of children with an initial febrile seizure will have recurrent seizures. Ref: Behrman RE, Kliegman RM, Jenson HB (eds): Nelson Textbook of Pediatrics, ed 17. Saunders, 2004, p 1994.

You evaluate an 80-year-old white male who is a heavily medicated chronic schizophrenic.

You note constant, involuntary chewing motions and repetitive movements of his legs. Which one of the following is the most likely

diagnosis?A. Neuroleptic malignant syndrome 

B. Acute dystonia C. Huntington’s disease D. Tardive dyskinesia 

E. Oculogyric crisis

Answer• D. Tardive dyskinesia • Explanation: The patient has classic signs of tardive dyskinesia.

Repetitive movement of the mouth and legs is caused by antipsychotic agents such as phenothiazines and haloperidol. Neuroleptic malignant syndrome consists of fever, autonomic dysfunction, and movement disorder. Acute dystonia involves twisting of the neck, trunk, and limbs into uncomfortable positions. Huntington’s disease causes choreic movements, which are flowing, not repetitive. Oculogyric crisis involves the eyes. Ref: Sadock BJ, Sadock VA (eds): Kaplan & Sadock’s Comprehensive Textbook of Psychiatry, ed 7. Lippincott Williams & Wilkins, 2000, pp 295-296. 2) Ross GW, Bowen JD: The diagnosis and differential diagnosis of dementia. Med Clin North Am 2002;86(3):455-476.

The most effective way to diagnose chronic alcoholism is to:

A. Ask the patient directly if he/she is an alcoholic 

B. Obtain a careful history of alcohol intake from the patient 

C. Inquire about problems resulting from drinking 

D. Confront the patient when he/she is intoxicated

Answer

• C. Inquire about problems resulting from drinking  • Explanation: Because denial is a key aspect of

alcoholism, eliciting examples of loss of control as a consequence of drinking is an effective interview strategy. Little can be accomplished when the patient is intoxicated. Ref: Sadock BJ, Sadock VA (eds): Kaplan & Sadock’s Comprehensive Textbook of Psychiatry, ed 7. Lippincott Williams & Wilkins, 2000, p 959.

A 30-year-old white female returns to your office for a 6-week follow-up for depression. Six weeks ago

she started fluoxetine (Prozac) and she now complains that her libido, which was decreased when she started the drug, has become significantly worse.

Which one of the following is appropriate?A. Continue fluoxetine and tell her that her libido

will improve B. Continue fluoxetine but increase the dosage 

C. Stop fluoxetine and start imipramine (Tofranil) D. Stop fluoxetine and start bupropion (Wellbutrin) 

E. Stop fluoxetine and start sertraline (Zoloft)

Answer

• D. Stop fluoxetine and start bupropion (Wellbutrin) 

• Explanation: Fluoxetine and other SSRIs (e.g., sertraline) can cause or worsen loss of libido. This is also true with tricyclics such as imipramine. Bupropion does not inhibit libido. Ref: Phillips RL Jr, Slaughter JR: Depression and sexual desire. Am Fam Physician 2000;62(4):782-786.

As a member of the local emergency response management team you are asked about the

treatment of nerve gas (e.g., sarin) poisoning. Which one of the following is most effective

in reversing the symptoms of nerve gas toxicity?

A. Albuterol (Proventil, Ventolin) via inhalation 

B. Ciprofloxacin (Cipro) C. Atropine 

D. Parenteral verapamil (Calan, Isoptin) E. Parenteral corticosteroids

Answer• C. Atropine  • Explanation: Nerve gas agents such as sarin resemble organophosphate

insecticides and inactivate anticholinesterase, leading to the accumulation of acetylcholine at nerve endings. Respiratory symptoms include rhinorrhea, bronchorrhea, bronchospasm, and respiratory muscle paralysis. Gastrointestinal symptoms include nausea, vomiting, and diarrhea. Central nervous system symptoms include headache, vertigo, agitation, seizures, and coma. Exposed patients benefit from treatment with atropine, which competitively inhibits acetylcholine. Pralidoxine chloride and diazepam are also beneficial. Although beta-agonists and corticosteroids are beneficial in the general treatment of bronchospasm, atropine is preferred in this situation. Verapamil and ciprofloxacin have no role in the treatment of nerve gas exposure. Ref: Prevention and treatment of injury from chemical warfare agents. Med Lett Drugs Ther 2002;44:1-4.

 Which one of the following is the most effective drug for the

treatment of alcohol dependence?A. Disulfiram (Antabuse) 

B. Diazepam (Valium) C. Amitriptyline (Elavil) D. Fluoxetine (Prozac) E. Naltrexone (ReVia)

Answer• E. Naltrexone (ReVia) • Explanation: Drug therapy should be considered for all patients

with alcohol dependence who do not have medical contraindications to the use of the drug and who are willing to take it. Of the several drugs studied for the treatment of dependence, the evidence of efficacy is strongest for naltrexone and acamprosate. Naltrexone is currently available in the U.S.; acamprosate and tiapride are currently available in Europe but not in the U.S. Ref: Swift RM: Drug therapy for alcohol dependence. N Engl J Med 1999;340(19):1482-1490. 2) Kasper DL, Braunwald E, Fauci AS, et al (eds): Harrison’s Principles of Internal Medicine, ed 16. McGraw-Hill, 2005, pp 1810, 2562-2564.

The Mini-Mental State Examination (MMSE) tests for:

A. Mood B. Behavior 

C. Intelligence quotient D. Cognitive function 

E. Functional impairment

Answer• D. Cognitive function • Explanation: The MMSE is most commonly used in clinical

settings. It is considered valuable because it assesses a broad range of cognitive abilities (i.e., memory, language, spatial ability, set shifting) in a simple and straightforward manner. In addition, the wide use of the MMSE in epidemiologic studies has yielded cutoff scores that facilitate the identification of patients with cognitive dysfunction. Ref: Geldmacher DS, Gordon B: Dementia in the elderly: Is it Alzheimer’s disease? The AD Letter 2000;1(1):1-4. 2) Cassel CK, Leipzig RM, Cohen HJ, et al (eds): Geriatric Medicine: An Evidence-Based Approach, ed 4. Springer, 2003, p 206.

The most common cause of fainting is:

A. Cardiac dysrhythmia B. Medications 

C. Orthostatic hypotension D. Psychiatric disorders E. Vasovagal syncope

Answer

• E. Vasovagal syncope • Explanation: Neurally mediated syncope (also

termed neurocardiogenic or vasovagal syncope) comprises the largest group of disorders causing syncope. These disorders result from reflex-mediated changes in vascular tone or heart rate. Ref: Kapoor WN: Syncope. N Engl J Med 2000;343(25):1856-1862. 2) Kasper DL, Braunwald E, Fauci AS, et al (eds): Harrison’s Principles of Internal Medicine, ed 16. McGraw-Hill, 2005, pp 126-128.

A 15-year-old white male is being evaluated after a fall down one flight of stairs. He was transported by the local rescue

squad with his cervical spine immobilized. He walked briefly at the scene and did not lose consciousness. His only

complaint is a mild, generalized headache. One episode of vomiting occurred shortly after the accident. No weakness or

numbness has been noted. Vital signs, mental status, and neurologic findings are normal. Radiologic evaluation of the cervical spine is remarkable only for an air-fluid level in the sphenoid sinus. Which one of the following abnormalities is

most likely to be associated with this radiologic finding?A. A basilar skull fracture B. An orbital floor fracture C. An epidural hematoma 

D. A zygomatic arch fracture E. A mandible fracture

Answer• A. A basilar skull fracture  • Explanation: A post-traumatic air-fluid level in the sphenoid sinus

is associated with basilar skull fractures. This finding is frequently noted on cervical spine films. Orbital floor fractures may be associated with double vision, fluid in the maxillary sinus, an air-fluid level in the maxillary sinus, and diplopia. Epidural hematomas are more frequently associated with skull fractures in the area of the meningeal artery. Zygomatic arch fractures are more visible on Towne’s view. Characteristic swelling and lateral orbital bruising are typically present. Mandible fractures may be associated with dental misalignment or bleeding. Panoramic views are often diagnostic. Ref: Barkin RM, Rosen P (eds): Emergency Pediatrics: A Guide to Ambulatory Care, ed 6. Mosby, 2003, pp 428, 433.

 An otherwise healthy 72-year-old white male presents with pain on the right side of his head,

increasing in severity over the past 2 days. Today he broke out in a rash consisting of grouped vesicles on an erythematous base in the distribution of the first

division of the fifth cranial nerve. The right eyelid is involved, but the patient complains of no pain in the eye itself, or of any visual disturbance. There are no

lesions on any part of the nose. Appropriate management at this time would include the

administration of which one of the following?A. Intravenous acyclovir (Zovirax) 

B. Oral famciclovir (Famvir) C. Topical capsaicin (Zostrix) 

D. Varicella-zoster immune globulin (VZIG) E. Idoxuridine (Herplex) eye drops

Answer• B. Oral famciclovir (Famvir) • Explanation: Herpes zoster, or shingles, is a common condition which results from the

reactivation of varicella virus acquired during an earlier episode of chickenpox. The distribution of the characteristic rash typically follows a single dermatome and does not cross the midline. The lesions are typically painful, and postherpetic neuralgia can become a disabling chronic problem. When any branch of the opthalmic nerve is involved, the condition is called herpes zoster ophthalmicus. Vesicles on the side or tip of the nose (Hutchinson’s sign) that occur during an episode of zoster suggest involvement of the nasociliary branch, and are associated with the most serious ocular complications. Involvement of the other sensory branches of the trigeminal nerve may affect the eyelid but rarely involve the eye itself. Treatment of uncomplicated herpes zoster ophthalmicus in an immunocompetent patient includes oral acyclovir, famciclovir, or valacyclovir. Corticosteroids can be used for acute pain but have no effect on the development of postherpetic neuralgia. Intravenous antiviral therapy is indicated for immunosuppressed patients with extensive cutaneous disease, and those at high risk for ocular complications. Treatment is most effective when started within 48 hours of the onset of the disease. Capsaicin cream is used in the treatment of postherpetic neuralgia, and varicella zoster immunoglobulin (VZIG) is used in high-risk immunocompromised patients to prevent varicella infection. Idoxuridine is indicated for herpes simplex keratitis. Ref: Habif TP: Clinical Dermatology, ed 3. Mosby-Year Book Inc, 1996, pp 355-359.

A 12-year-old white male is brought to your office after accidentally cutting his left hand with a pocketknife. On examination you find

a deep 2-cm laceration at the base of the thenar eminence. To test for motor injury to

the median nerve you would have the patient:A. Extend the thumb and fingers 

B. Oppose the thumb and little finger C. Flex the wrist 

D. Abduct the thumb and index finge

Answer• B. Oppose the thumb and little finger  • Explanation: The ability to touch the tip of the thumb

to the tip of the little finger indicates normal motor function of the median nerve. The radial nerve controls extension of the thumb and fingers. The median nerve partially controls flexion of the wrist, but the site of innervation is proximal to the wound site at the base of the thumb. Abduction of the thumb is a function of the radial nerve. Finger abduction is a function of the ulnar nerve. Ref: Marx JA (ed): Rosen’s Emergency Medicine, ed 5. Mosby Inc, 2002, p 505.

 A 16-year-old white female is brought to your office because she has been “passing out.” She tells you that on several occasions while playing in the high-school band at the end of the half-time show she has “blacked

out.” She describes feeling lightheaded with spots before her eyes and tunnel vision just prior to falling. Friends in the band have told her that

she appears to be pale and sweaty when these episodes occur. No seizure activity has ever been observed. In each instance she regains

consciousness almost immediately; there is no postictal state. She has been seen in the emergency department for this on two occasions with

normal vital signs, physical findings, and neurologic findings. A CBC, a metabolic profile, and an EKG are also normal. Which one of the

following tests is most likely to yield the correct diagnosis?A. A sleep-deprived EEG 

B. 24-hour Holter monitoring C. A pulmonary/cardiac stress test 

D. An echocardiogram E. Tilt table testing

Answer• E. Tilt table testing • Explanation: Reflex syncope is a strong diagnostic consideration for episodes of

syncope associated with a characteristic precipitating factor. The major categories of syncope include carotid sinus hypersensitivity, and neurally mediated and situational syncopes. The most common and benign forms of syncope are neurally mediated or vasovagal types with sudden hypotension, frequently accompanied by bradycardia. Other terms for this include neurocardiogenic, vasomotor, neurovascular, or vasodepressive syncope. Most patients are young and otherwise healthy. The mechanism of the syncope seems to be a period of high sympathetic tone (often induced by pain or fear), followed by sudden sympathetic withdrawal, which then triggers a paradoxical vasodilatation and hypotension. Attacks occur with upright posture, often accompanied by a feeling of warmth or cold sweating, lightheadedness, yawning, or dimming of vision. If the patient does not lie down quickly he or she will fall, with the horizontal position allowing a rapid restoration of central profusion. Recovery is rapid, with no focal neurologic sense of confusion or headache. The event can be duplicated with tilt testing, demonstrating hypotension and bradycardia. Ref: Weimer LH, Williams O: Syncope and orthostatic intolerance. Med Clin North Am 2003;87(4):835-

A 65-year-old male has recently undergone coronary artery bypass graft (CABG) surgery. Generally, he has recovered well from his surgery. However, his

cardiac surgeon referred him back to you because of symptoms suggestive of depression. Which one of

the following is true in this situation?A. Patients with chronic cardiac symptoms prior to surgery are more likely to develop postoperative

depression B. Postoperative depression increases the risk for

subsequent cardiovascular events C. Treatment of postoperative depression with

antidepressants decreases the rate of subsequent cardiovascular events 

D. Enrollment in a cardiac rehabilitation program often worsens depression

Answer• B. Postoperative depression increases the risk for subsequent cardiovascular

events  • Explanation: In patients who are depressed after coronary artery bypass graft

(CABG) surgery, impaired memory and cognition are seen more frequently than other depressive symptoms. Patients with rapid progression of cardiac symptoms before surgery are at particular risk of depressive symptoms after surgery. Newly depressed patients are at higher risk than non-depressed patients for long-term cardiovascular events and death from cardiovascular causes. The Sertraline AntiDepressant Heart Attack Randomized Trial (SADHART) showed that antidepressant use was associated with a slight, but not significant, reduction in the rates of cardiovascular events. The Enhancing Recovery in Coronary Heart Disease (ENRICHD) trial showed that although it did not reduce the risk of cardiac events, participation in a cardiac rehabilitation program reduced depressive symptoms and increased social ties. Ref: Charlson ME, Isom OW: Care after coronary-artery bypass surgery. N Engl J Med 2003;348(15):1456-1463.

 A 33-year-old white female has a 12-year history of headache occurring 3–4 times per

month, accompanied by nausea and vomiting. She takes over-the-counter analgesics, but

relief is usually obtained only when she falls asleep. This is her first visit to you for this problem. You diagnose migraine without

aura. Although the patient is willing to consider prescription drugs, she says that she would prefer “something that is natural and

without side effects.” Which one of the following would be the best recommendation?

Answer• B. Ma huang  • Explanation: Of the listed options covering the realm of complementary and

alternative medicine, only biofeedback has been shown to have a therapeutic effect on migraine. Specifically, the modality that seeks to control physiologic response to skin temperature and skin conductance appears to be the most successful. It is best performed in a medical office by caring, supportive staff members under physician supervision. Oxygen is used to treat cluster headaches. The Epley maneuver is used for managing benign positional vertigo, and phototherapy is useful in seasonal affective disorder. Ma huang, a Chinese herb, has ephedrine properties but is not useful in treating migraine headaches. Ref: Novey DW (ed): Clinician’s Complete Reference to Complementary/Alternative Medicine. Mosby, 2000, pp 32-35. 2) Dambro MR (ed): Griffith’s 5 Minute Clinical Consult. Lippincott Williams & Wilkins, 2002, pp 690-691. 3) Kasper DL, Braunwald E, Fauci AS, et al (eds): Harrison’s Principles of Internal Medicine, ed 16. McGraw-Hill, 2005, pp 88-93.

A 27-year-old white male presents to the emergency department 2 hours after being bitten by a rattlesnake. He complains of weakness, abdominal cramping, left leg pain, and left leg swelling. His speech is slurred, and

his breath smells of alcohol. Physical Findings Temperature 37.0° C (98.6° F) Blood pressure 100/60 mm Hg Pulse 122 beats/min Respirations 24/min Skin diaphoretic; ecchymoses on both forearms; bite puncture site

just above left lateral malleolus Lungs clear to auscultation Cardiac normal heart tones, 1+ posterior tibial pulses Abdomen flat; hypoactive bowel sounds; no masses or guarding Extremities visible swelling of left leg and thigh; skin tightness of left leg Neurologic decreased sensation to

light touch and sharp sensation in left foot Which one of the following therapeutic interventions is indicated?

A. Antivenin administration B. Venom extractor use 

C. Tourniquet application at the upper thigh D. Surgical consultation for decompression fasciotomy E. Administration of platelets and fresh frozen plasma

Answer• A. Antivenin administration • Explanation: This patient presents with a history of snakebite, swelling of an entire

extremity, weakness, and ecchymosis. This is consistent with a grade III envenomation and merits antivenin therapy. Production of equine-derived antivenin has stopped, but may still be indicated where available. The ovine product, CroFab, is less allergenic but still scarce due to limited production. Venom extractors are thought to be useful only in the first few minutes after a bite. Two hours is too late to be of any use. Tourniquets are thought to be contraindicated when used to compress an artery. Low-pressure constriction of lymphatic and venous vessels is controversial. Fasciotomy has not proved useful. Antivenin is indicated before any consideration of compartment syndrome. Pressure measurements would be required because of the clinical similarities between envenomation injury and compartment syndrome. Coagulation factors and blood products are rapidly inactivated. They are indicated only in the presence of exsanguination. Ref: Walter FG, Bilden EF, Gibly RL: Envenomations. Crit Care Clin 1999;15(2):353-386. 2) Juckett G, Hancox JG: Venomous snakebites in the United States: Management review and update. Am Fam Physician 2002;65(7):1367-1374, 1377. 3) Marx JA (ed): Rosen’s Emergency Medicine, ed 5. Mosby Inc, 2002, pp 786-793. 4) Rakel RE, Bope ET (eds): Conn’s Current Therapy 2004. Saunders, 2004, pp 1192-1193.

 A 66-year-old white male is brought to your office for evaluation of progressive memory loss over the last several months. The problem seems to wax and wane significantly

over the course of days and weeks. At times when he is more confused, he tends to have visual and auditory hallucinations

that he is back fighting in Vietnam, thinking a ringing telephone is calling in fighter jets. He has also been falling

occasionally. On physical examination, he has a resting tremor in his left leg, and rigidity of his upper body and face. A full medical workup, including standard blood work and a

CT scan, shows no abnormalities that suggest delirium, stroke, or other primary etiologies. Which one of the

following is the most likely diagnosis?A. Alzheimer’s disease 

B. Dementia with Lewy bodies C. Fronto-temporal dementia 

D. Multi-infarct dementia E. Pseudodementia

Answer

• B. Dementia with Lewy bodies  • Explanation: Dementia with Lewy bodies is currently considered one

of the most common etiologies of dementia in elderly patients, representing up to 20%-30% of those with significant memory loss. The clinical presentation consists of parkinsonian symptoms (rigidity, tremor), fluctuating levels of alertness and cognitive abilities, and behavior sometimes mimicking acute delirium. Significant visual hallucinations are common, and delusions and auditory hallucinations are seen to a lesser degree. On pathologic examination, Lewy bodies (seen in the substantia nigra in patients with Parkinson’s disease) are present diffusely in the cortex. There is currently no specific treatment. Ref: Galvin JE: Dementia with Lewy bodies. Arch Neurol 2003;60(9):1332-1335. 2) Kasper DL, Braunwald E, Fauci AS, et al (eds): Harrison’s Principles of Internal Medicine, ed 16. McGraw-Hill, 2005, pp 2402-2403.

An 87-year-old African-American female is admitted to your hospital with a hip fracture. She lives alone and has been self-sufficient. She has been able to drive, go to the grocery, and balance her own checkbook. She does well in the hospital

until the second postoperative day, when she develops agitated behavior, tremor, and disorientation. She attempts to remove her Foley catheter repeatedly. She exhibits alternating

periods of somnolence and agitation, and describes seeing things in the room that are not there. Which one of the

following is the most likely diagnosis?A. Delirium 

B. Alzheimer’s disease C. Senile dementia D. Schizophrenia 

E. Psychosis

Answer• A. Delirium  • Explanation: This individual is exhibiting symptoms of delirium. Diagnostic criteria

for delirium, according to the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR), include the following: A. Disturbance of consciousness (i.e., reduced clarity of awareness about the environment) with reduced ability to focus, sustain, or shift awareness. B. A change in cognition (e.g., memory deficit, disorientation, language disturbance) or development of a perceptual disturbance that is not better accounted for by a preexisting, established, or evolving dementia. C. Development over a short period of time (usually hours to days) with a tendency to fluctuate during the course of a day. D. Evidence from the history, physical examination, or laboratory findings that indicates the disturbance is caused by direct physiologic consequences of a general medical condition. In the case described, the patient’s history does not indicate preexisting problems and she had a relatively abrupt onset of disturbance of consciousness and change in cognition, related to the hospitalization for hip fracture. Ref: American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, ed 4. American Psychiatric Association, 1994, pp 132-133. 2) Gleason OC: Delirium. Am Fam Physician 2003;67(5):1025-1034.

A 75-year-old male has not seen a physician in 25 years and presents with advanced Parkinson’s disease. The best initial treatment would be:A. Referral to a neurosurgeon for

thalamotomy B. Amantadine (Symmetrel) C. Benztropine (Cogentin) D. Pramipexole (Mirapex) 

E. Carbidopa/levodopa (Sinemet)

Answer• E. Carbidopa/levodopa (Sinemet) • Explanation: While anticholinergics such as benztropine and amantadine

may provide some improvement of symptoms, these effects wane within a few months. Such medications are not a good option in this patient with advanced disease. Dopamine agonists provide some improvement in motor complications, but are mainly used to delay the introduction of levodopa in younger patients, to avoid levodopa-related adverse reactions. Carbidopa/levodopa is better for initial therapy in older patients, and those who present with more severe symptoms. Slow-release versions of this combination may decrease motor fluctuations. Stereotactic thalamotomy is used to ameliorate tremors that have become disabling. This procedure has been replaced by other surgical options such as pallidotomy and high-frequency, deep-brain stimulation of specific nuclei. Ref: Siderowf A, Stern M: Update on Parkinson disease. Ann Intern Med 2003;138(8):651-658. 2) Goldman L, Ausiello D (eds): Cecil Textbook of Medicine, ed 22. Saunders, 2004, pp 2306-2310.

Which one of the following should be avoided in the

treatment and prophylaxis of migraine during early pregnancy?

A. Calcium channel blockers B. Beta-blockers 

C. Triptans D. NSAIDS

Answer• C. Triptans  • Explanation: Headaches, and migraines in particular, are very common in

women of childbearing age. Migraine sufferers usually have improvement of symptoms in pregnancy and many have complete remission. Most medications used for prophylaxis and abortive treatment of migraines in the nonpregnant patient can also be used in pregnant patients. Most beta-blockers and calcium channel blockers are safe. Acetaminophen and narcotics can be used for acute pain. Ibuprofen can also be used but should be avoided late in pregnancy because it is associated with premature closure of the ductus arteriosus and oligohydramnios. Ergotamines should be avoided as they are uterotonic and have abortifacient properties. They have also been associated with case reports of fetal birth defects. Triptans have the potential to cause vasoconstriction of the placental and uterine vessels and should be used only if the benefit clearly outweighs the harm. Ref: Gabbe SG, Niebyl JR, Simpson JL (eds): Obstetrics: Normal and Problem Pregnancies, ed 4. Churchill Livingstone, 2002, pp 1244-1246.

 Which one of the following side effects induced by traditional neuroleptic agents responds to treatment with beta-blockers?

A. Akathisia B. Rigidity C. Dystonia 

D. Sialorrhea E. Stooped posture

Answer• A. Akathisia • Explanation: Rigidity, sialorrhea, and stooped posture are

parkinsonian side effects of neuroleptic drugs. These are treated with anticholinergic drugs such as benztropine or amantadine. Dystonia, often manifested as an acute spasm of the muscles of the head and neck, also responds to anticholinergics. Akathisia (motor restlessness and an inability to sit still) can be treated with either anticholinergic drugs or beta-blockers. Ref: Sadock JB, Sadock VA (eds): Kaplan & Sadock’s Synopsis of Psychiatry: Behavioral Sciences/Clinical Psychiatry, ed 9. Lippincott Williams & Wilkins, 2003, pp 1009, 1012-1015.

•

. A case of meningococcal meningitis has just been confirmed at a day-care center. The

susceptibility of the microorganism is not yet known. At this point, you should do which one of the following for the day-care center

contacts?A. Culture their nasopharyngeal secretions 

B. Administer meningococcal vaccine C. Prescribe sulfadiazine 

D. Prescribe chloramphenicol (Chloromycetin) 

E. Prescribe rifampin (Rifadin)

Answer

• E. Prescribe rifampin (Rifadin) • Explanation: Rifampin, in the absence of major contraindications, is the drug

of choice for preventing the spread of meningococcal disease when the susceptibility of the organism is not known. In this situation, meningococcal vaccines are of no value because their protective effects take a few days to develop, and because they do not protect against group B meningococci, the most prevalent strain for meningococcal disease. Sulfadiazine is the drug of choice if the meningococcus is known to be susceptible to it. Chloramphenicol and penicillin, which are effective in treating the disease, are ineffective in eliminating nasopharyngeal carriers of meningococci, possibly because they do not appear in high concentrations in saliva. Culturing contacts for meningococcal carriage in the nasopharynx has no value for identifying those at risk for meningococcal disease. Ref: Pickering LK (ed): 2003 Red Book: Report of the Committee on Infectious Diseases, ed 26. American Academy of Pediatrics, 2003, pp 123-137, 430-436

DIRECTIONS: The following series of questions concern two diseases ? babesiosis and Lyme

disease. For the following questions, select the answer most closely associated with the statement. 

There are documented cases of transmission via blood transfusion.

• A:• if babesiosis is associated with the statement• B:• if Lyme disease is associated with the

statement• C:• if BOTH babesiosis AND Lyme disease are

associated with the statement• D:• if NEITHER babesiosis NOR Lyme disease is

associated with the statement