RITE Review

Sergei Kashirny, MD02-11-2011

1. Which of the following statements about the saphenous nerve is most accurate?

A) The saphenous nerve is a motor nerve B) The saphenous nerve supplies sensation to the medial aspect of the leg

C) The saphenous nerve arises from the deep peroneal nerve D) The saphenous nerve supplies sensation to the posterior aspect of the thigh

E) The saphenous nerve is a mixed motor and sensory nerve

B) The saphenous nerve supplies sensation to the medial aspect of the leg

The saphenous nerve is a sensory nerve which arises from the femoral nerve. It supplies sensation to the medial aspect of the leg.

• 2. A 35 year-old female presents to your office accompanied by a family member, who helps provide the history. The patient has had a 3 year history of worsening psychiatric and neurologic issues, including odd movements and memory problems and uncharacteristic depression. The patient is on atorvastatin for dyslipidemia, and has no other past medical history. The patient confirms that other family members have had similar symptoms, and some have died at an early age. On exam, you note cognitive slowing, motor impersistence, generalized choreiform movements, slightly impaired memory, and oculomotor dysfunction. MRI of the brain is remarkable only for slight atrophy of the caudate bilaterally. The patient's diagnosis was confirmed by genetic testing, which showed a CAG trinucleotide repeat on chromosome 4. Of the following statements, which is most accurate about this patient's disease?

• A) The earliest cognitive change in this disorder is usually memory loss B) Tetrabenazine can delay the progression of this disease C) This disorder has a higher prevalence among blacks than among caucasians or asians

D) Psychosis is common in this disease E) The degree of chorea is a reliable marker of the severity of this disease

• D) Psychosis is common in this disease

This patient has Huntington disease (HD). Approximately 25% of patients with HD develop psychosis.

3.

These anterior horn cells from this 63 year old who died with ALS contain which of the following proteins? A) Paired helical filament Tau protein B) TAR-DNA binding protein 43 C) Huntingtin D) Alpha-synuclein E) Lou Gherin

• B) TAR-DNA binding protein 43

The major component of intraneuronal inclusions in familial and sporadic ALS

• 4. A 6-year-old male has been referred to you for further evaluation of a possible Wilson's disease because of dystonic gait and limbs. History taking and thorough physical examination are carried out by you and you think that Wilson's disease is not the correct diagnosis in this child. Why?

• A) Flexor planters B) Favorable response to low dose L-dopa C) Wing-beating tremor D) Dysphagia E) Cataract

• B) Favorable response to low dose L-dopa

One of the most important differential diagnosis of Wilson's is dopa responsive dystonia (DRD). All cases of childhood dystonia should receive a trial of a low dose L-dopa; if they respond, the diagnosis of DRD can be done.

• 5. Which of the following is a hallmark side effect of valproate?

• A) Gum hyperplasia B) Leukopenia C) Hair loss D) Depression E) Blindness

• C) hair loss

Side effects of valproate include thrombocytopenia, weight gain, hepatotoxicity, pancreatitis, hyperammonemia, tremor, and hair loss.

• Depression is a hallmark side effect of vigabatrin• Blindness is a hallmark side effect of vigabatrin• Leukopenia is a hallmark side effect of carbamazepine• Gum hyperplasia is a hallmark side effect of phenytoin

6.

The concentric structures seen within this nerve biopsy indicate: A) Peripheral nerve involvement in a patient with adrenomyeloneuropathy B) Plexiform neurofibroma C) Repeated trauma with perineurial damage D) Repeated demyelination and remyelination E) Hereditary neuropathy with liability to pressure palsies

• D) Repeated demyelination and remyelination

Repeated demyelination and remyelination, such as is seen in patients with CIDP and HSMN1, result in these concentric structures often referred to as "onion bulbs"

• 7. A 17-year-old high school girl has been referred from the psychiatry department as a difficult-to-manage case of affective psychosis. She is on chlorpromazine for the past 3 months. Examination reveals resting tremor, rigidity, and dysarthria. Her work-up shows ALT 61 iu/L, total serum bilirubin 2.3 mg/dl, serum bicarbonate 14 mEq/L, blood urea nitrogen 15 mg/dl, and positive urine glucose testing. What does the girl have?

• A) Chlorpromazine-induced Parkinsonism B) Wilson's disease C) Juvenile Huntington's disease (HD) D) Occult drug abuse E) Chlorpromazine-induced cholestatic hepatitis

• B) Wilson's disease

Up to 20% of Wilson's disease patients may have predominant psychiatric manifestations and psychiatrists might be the first who see those patients. Note the profound acidosis with normal renal function and urine sugar (normal anion gap metabolic acidosis of proximal renal tubular acidosis). You may assume her liver function abnormalities to be due to chlorpromazine therapy, but the latter would not explain other tests.

• 8. The damage is confined to the reticular nucleus, which is essentially destroyed. Of the following options, what function is most likely to be affected?

• A) Cortical arousal B) Vision C) Audition D) Emotional expression and normal memory function E) Somatic sensation from the face

• A) cortical arousal

The reticular nucleus of the thalamus is associated with cortical arousal

• 9. A patient with a congenital myopathy has developed malignant hyperthermia after anesthesia. Of the following choices, which type of congenital myopathy is most likely to be the one that this patient has?

• A) Central core disease B) Fingerprint myopathy C) Cytoplasmic body myopathy D) Centronuclear myopathy E) Nemaline myopathy

• A) central core disease

Malignant hyperthermia after general anesthesia is characteristic of central core disease. It is not characteristic of any of the other options.

• 10. A person with a major depressive episode suffers from depressed mood or loss of interest in pleasure for a period of at least:

• A) 2 months B) 7 days C) 3 weeks D) 2 weeks E) 20 days

• D) 2 weeks

The DSM-IV-TR definition of a major depressive episode includes the specific criterion that symptoms must be present for at least 2 weeks.

• 11. A patient presents with a history of several months of numbness in his fingers and toes. Prior workup, including folate, B12, homocysteine, MHATP, RPR, and hemoglobin A1C have been unremarkable. The patient states that he used to work for a manufacturing company, and his job involved exposure to many various potentially toxic compounds. Of the following choices, which chemical is most likely to produce peripheral neuropathy in this patient?

• A) Aluminum B) Bismuth C) Cyanide D) Manganese E) Mercury

• E) Mercury

Mercury can produce peripheral neuropathy.

• 12. Which of the following muscles is innervated by the median nerve? A) Flexor digitorum profundus II B) Infraspinatus C) Trapezius D) Extensor pollicis brevis E) The palmar interossei

• A) flexor digitorum profundus II Flexor digitorum profundus II is innervated by the median nerve

• Infraspinatus is innervated by the suprascapular nerve, which arises from the upper trunk of the brachial plexus

• Trapezius is innervated by the spinal accessory nerve • Extensor pollicis brevis is innervated by the radial

nerve• The palmar interossei is innervated by the ulnar

nerve

13. After performing a lumbar puncture for an inpatient, the lab calls you to tell you that the protein content of the CSF sample is 13 mg/dl. All, but one, of the following can result in this CSF protein value?

• A) Removal of large volume of CSF B) Hyperparathyroidism C) Pseudotumor cerebri D) CSF fistulae E) Leukemias

• B) Hyperparathyroidism

Hyperthyroidism can result in low CSF protein, that usually normalizes when the patient is rendered euthyroid. Note that the definition of low CSF protein is a protein value between 3-20 mg/dl.

14. The most common location for this tumor is: A) Basal ganglia

B) Pineal region C) Suprasellar D) Cerebellum E) Temporal lobe

• B) Pineal region

Germinoma is the most common pineal region tumor, generally presenting in adolescent boys.

15. Which of the following is a hallmark side effect of oxcarbazepine?

A) Irritability B) Hepatoxicity C) Pancreatitis D) Coarsening of the facies E) Hyponatremia

E) hyponatremia

• Coarsening of the facies - phenytoin• Pancreatitis - valproate• Hepatoxicity - felbamate• Irritability - vigabatrin

16. This pattern of spinal cord damage is most consistent with which of the following diseases? A) Subacute combined degeneration B) ALS C) Ataxia-telangiectasia D) Tabes dorsalis E) Fredreich ataxia

• E) Fredreich ataxia

Combined spinocerebellar and posterior column degeneration is characteristic of Fredreich ataxia

• 17. A 34 male in otherwise good health is standing, telling jokes with his roommate. In response to a humorous statement, he begins laughing hysterically. Suddenly, he collapses to the ground. He remains awake and alert, but is very weak for several seconds. The most accurate term for this phenomenon is:

A) Complex partial seizure B) Hypnagogic paralysis C) Cataplexy D) Gelastic seizure E) Non-epileptic seizure

• C) Cataplexy

This sudden loss of muscle tone, with preserved consciousness is called cataplexy. Cataplexy usually occurs in response to an emotional stimulus like laughter, although less frequently it can occur without stimulus.

• 18. You examine a child with pes cavus, diminished ankle reflexes, scoliosis, and weakness with wasting of the calf muscles. On nerve conduction study, you find severe slowing of the conduction velocity. Of the following, which of the following syndromes is the most appropriate diagnosis? A) Charcot Marie Tooth type 2B B) Charcot Marie Tooth type 2E C) Charcot Marie Tooth type 1B D) Charcot Marie Tooth type 2G E) Charcot Marie Tooth type 2C

• C) Charcot Marie Tooth type 1B Charcot Marie Tooth type 1 (including subtypes A-D) is a demyelinating inherited sensorimotor polyneuropathy. Nerve conduction velocities are slow on NCS. It has autosomal dominant inheritance.

Charcot Marie Tooth type 2 is a primarily axonal sensorimotor polyneuropathy. NCS would show normal or near-normal conduction velocities.

19. This MRI most likely comes from a patient with which of the following findings? A) Ataxia, nystagmus, jaundice, and insomniaB) Confabulation, nystagmus, vertigo, and nauseaC) Ataxia, telangiactasias, dementia, and hallucinationsD) Confusion, nuchal rigidity, headache, and nauseaE) Ophthalmoparesis, nystagmus, confusion, and ataxia

E) ophthalmoparesis, nystagmus, confusion, and ataxia This FLAIR MRI shows hyperintensity in the medial thalamic nuclei. This finding is characteristic of both Wernicke encephalopathy and Korsakoff dementia. The typical symptoms of Wernicke encephalopathy include ophthalmoparesis, nystagmus, ataxia, and a change in mental status characterized by confusion and apathy. Wernicke encephalopathy and Korsakoff dementia are both due to a deficiency of thiamine, and are both more common in alcoholics. Treatment of Wernicke encephalopathy requires administration of Thiamine. Adams and Victor recommend 50mg IV and 50mg IM immediately, followed by 50mg IM daily.

• 20. A patient you see in clinic is suffering from major depression. You start him on an antidepressant. A week later, you receive a panic-stricken phone call from him. He complains that he has a painful erection that has persisted for the last 24 hours. Of the following medications, which is most likely to have caused this reaction?

A) Bupropion B) Tranylcypromine C) Amitriptyline D) Trazodone E) Duloxetine

• D) Trazodone

Priapism is a rare but well-known side effect of trazodone.

21. "I ate his liver with fava beans and a fine chianti." - H. LecterHannibal Lecter is cast in a Silence of the Lambs sitcom spinoff complaining of

a severe headache, nausea, dizziness, and blurred vision. His skin is a dark yellow, although his sclera are anicteric. Funduscopic evaluation demonstrates swollen, edematous optic nerves bilaterally. LP opening pressure is elevated, but CSF composition is normal and there is no evidence of infection. CT and MRI are both normal. Oddly, he has no eyebrows. From what neurological or metabolic disorder does Dr. Lecter suffer, and what unusual dietary habits may have led to this presentation?

A) Wernicke's encephalopathy / chronic abuse of a nice chianti B) Creutzfeldt-Jakob disease / remote ingestion of infected neural tissueC) Anemia of glucose-6-phosphate dehydrogenase deficiency / ingestion of fava beansD) Hypervitaminosis A / overconsumption of liverE) Malingering in order to entrap and eat the examiner / chronic cannibalism

• D) Hypervitaminosis A / overconsumption of liver At very high levels of vitamin A consumption (>25000 IU/day), vitamin A toxicity results in decreased CSF absorption, leading to elevated intracranial pressure and papilledema (pseudotumor cerebri). Early symptoms of hypervitaminosis A include sparsely distributed, coarse hair and a specific alopecia of the eyebrows. In this case, apparent jaundice of the skin is due to high levels of carotenoid pigment.

• 22. A 16-year-old male has been referred to the neurology outpatient clinic because of "recurrent loss of consciousness." He has reeling gait, lost ankles, and up-planters with pes cavus. His speech is dysarthric. Which one of the following is consistent with the diagnosis of Friedreich's ataxia?

A) Bilateral ptoses B) Wasting of right thenar eminence and left-sided wrist drop C) Recurrent hypoglycemia D) Minimental status examination score of 29 E) Retinal AV nipping and silver-wiring of retinal arterioles

D) Minimental status examination score of 29

Friedreich's ataxia is not a dementing illness; therefore MMSE score is expected to be normal. Note that his lapses of consciousness are due to cardiac involvement with dysrrhythmias (and are not due to seizures).

• 23. A 85 year-old female presents to your office accompanied by her young boyfriend, who provides most of the history.The boyfriend states that for quite some time, the patient has had trouble keeping his balance, has been increasingly slow getting around, has complained of seeing visions of odorless brown doves, has had periods when she is lethargic and drowsy, and others when she is more alert, has complained of a tremor in his hands, and has been increasingly forgetful.Which of the following pathology findings is MOST characteristic of this patient's disease? A) Inclusions comprised primarily of Huntingtin B) Inclusions comprised primarily of alpha-synuclein C) Lesions in the mammillary bodies D) Punctate hemorrhages in periaqueductal grey and in the grey matter surrounding the 3rd and 4th ventricles E) Neurofibrillary tangles,

B) inclusions comprised primarily of alpha-synuclein This patient's history is consistent with dementia with Lewy bodies (DLB). Patients with DLB develop Parkinsonian symptoms and dementia symptoms at approximately the same time. Other key clinical features include recurrent visual hallucinations and fluctuations in mental status. Lewy bodies are eosinophilic cytoplasmic inclusions which contain alpha-synuclein.

• Huntingtin is a protein associated with Huntington chorea. • Lesions in the mammillary bodies are seen in Korsakoff amnestic state.• Punctate hemorrhages in periaqueductal grey and in the grey matter

surrounding the 3rd and 4th ventricles are seen in Wenicke encephalopathy.

• Neurofibrillary tangels are more commonly associated with Alzheimer disease.

24. Which of the following muscles is innervated by the radial nerve?

A) Lumbricals III B) Serratus anterior C) Biceps brachii D) Flexor digitorum profundus I E) Extensor carpi ulnaris

E) extensor carpi ulnaris

Flexor digitorum profundus I - median nerveBiceps brachii - musculocutaneous nerve Serratus anterior - long thoracic nerve, which

arises from the brachial plexus Lumbricals III - ulnar nerve

25. A 45 year-old immunocompetent female presents with bacterial meningitis. Of the following options, which is the most likely pathogen?

A) E. coli B) S. pneumoniae C) L. monocytogenes D) S. agalactiae E) H. influenzae

B) S. pneumoniae S. pneumoniae and N. meningitidis are the most common pathogens isolated from CSF in immunocompetent patients aged 18 to 50 years who have bacterial meningitis.

S. agalactiae, E. coli, and L. monocytogenes are the most common pathogens in infants less than 2 months of age. L. monocytogenes is also commonly seen in patients more than 50 years old, or patients with impaired cellular immunity. H. influenzae is more commonly seen in patients 4 months to 17 years of age.

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