KNOW all the possible complications of STROKES from the following branches in the objectives, along with the superior & inferior division of the MCA, and the circle of willis in it’s entirety. Know the lobes & all the information they could lose, especially all the lobes involved with vision, voluntary eye movements, etc. Also, aphasia & all the different types and which ones are in which lobes and infarction present how, expression, conductive, word salad, you get the idea. Also, know what to do & what not to do w/ a patient with an acute head injury, in a stepwise fashion, who may be unresponsive. Order does matter and was specific. Know the thyroid and its implications to neurology in detail. This stuff was tested and not really covered well in the objectives, but remember this is only a few peoples opinion. Julie Scott’s notes are pretty well used by most but with a disk you can cut, delete, add & edit, not to mention print for free in the LRC! Good luck ! Kim Northup, 2002

Unit One Cerebrovascular Disease

3 Categories of stroke- ischemic (usually thrombotic > embolic) - hemorrhage (usually embolotic at bifurcations of large arteries like MCA)- lacunar or deep infarcts in the Internal capsule, or Grey matter i.e. potential seizure foci

Risk Factors:Natural: Preventable:-age, gender, &race HTN, hyperlipidemia, diabetes, valve abnormalities

smoking, ETOH

Artery distribution:

ACA CL weakness or numbness Legs> arm, MIXED motor& sensoryLoss of decision making capacity (abulia), Urinary Incontinence

MCA CL weakness or numbness Arms & face, CL hemianopsia, CL gazeSuperior branch Palsy (Eyes Look to the Lesion), Aphasia for dominant and neglect if

Nondominant, MIXED motor and sensory Inferior branch of MCA interrupted visual radiation, Wernicke’s aphasia

PCA CL hemianopsia, pure sensory or pure motor syndromePCA bilaterally Cortical blindness, prosoagnosiaPCA branches CL hemibalism if the subthalamic nucleus is involvedVerterbrobasilar Ataxia, vertigo, diplopia, dsyarthria, dysphagia, ipilateral CN, with

Weakness and numbness in extremeties (crossed finding)AICA Pons, gaze palsy, ipsilateral facial weakness, deafness possiblyPICA branch of vertebral Wallenberg’s syndrome of CL loss of P & T ipsilateral CN findings,

Ipsilateral Horners, ataxia, nystagmus, hoarseness, dysphagiaLacunar Infarcts of deep Basal ganglia, Pons, cerbellum, and anterior limb of ICGrey cortical matter CL pure motor or pure sensory hemiparesis Ipsilateral ataxia

Anterior Spinal Artery CL UMN hemiplegia near C5 w/ infarct of spinal cord, CN XII nucleus1st branch of the vertebral

Carotid dissection usually due to trauma & can present with a Horner’s from SNS fibersBasal brainstem infarct CN III, VI, VIII , CL hemiparesis

Thrombotic occulsions are almost always atherosclerotic and occur at IC & carotid bifurcation or in the vertebrobasilar system depending upon the normal functioning of the Circle of Willis.

Asymptomatic Carotid Bruit- between 30-70% occulded usually

TIA- stroke w/neurological signs resolving in < 24 hrs ; usually in 30 min, and recurrent TIAs could be due to cardiac or thromobosis or embolism in the cerebral circulationLacunar Infarct- occlusion of small penetrating branches from hyalinosis due to HTN, DM, and atherosclerosisHemorrhagic infarct- vessel imcompetence leading to destructive or compressive bleeding that can lead to ischemic infarct or edema, presents w/ a severe HA and LOCCarotid Endarterectomy- surgical removal of thrombus from stenotic carotid arteries w/ stenosis > 70% used for unstable TIA’s w/ major risk of stroke, not done in vertebrobasilar system

Spontaneous Hemmorhage-- ruptured cerebral artery aneurysm- AVM’s men > women 2:1, ages 20-40 yo- Berry aneurysms 50-60 yo ass’d w/ APKD- Cocaine or Amphetamine use - Trauma

Clincial findings of hemmorhage- increased ICP, finding is papilledema- “worst Headache of my life!!”- LOC, vomiting, neck stiffness, confusion, stupor, coma- Increased BP, TWork up - CT/MRI- LP (increased opening pressure, pleocytosis w/ xanthrochromia post hemorrhage)- ECG short PR interval w/tall inverted T waves- Arteriography- Nimodipine (decreases cerebral vasospasm but can cause bradycardia)- Surgical clipping or wrapping/ En Bloc resection of AVM if accessible- Bedrest w/ head up, manage pain w/ analgesics NOT Aspirin- Watch IV fluids to make sure they aren’t increasing ICP

Cerebral Infarcts- 1-4 hrs no changes possibly detected by MRI- detectable at 12 hrs w/ softening of the gray matter seen on MRI swelling, dead red neurons + PMN

more apparent swelling 48-72 hrs ** may cause herniation WATCH DAY 3 MRI/CT visible most likely

- 2-6 days macrophages are the predominate response w/ maximal edema achieved- signs of edema are altered conciousness, projectile vomiting, pupillary changes

- 1-3 wks reactive astrocytes, small BV perfusion, beginning of liquifaction w/ macrophages- months to years resolution via liquifaction, cystic degeneration or cavitation w/ astrocytes around the

edge, or glial formation

Work up for Ischemic Stroke- ECG to look for cardiac source MI or A- fib- CT or MRI to look for infarct or hemorrhage and R/O lesions or tumors- LP to r/o SAH- Carotid Doppler Ultrasound study- Echocardiogram for embolic source- EEG to distinguish seizure vs. TIA or Lacunar vs. cortical- MR Angiography

Classic Carotid Emboli= ipsilateral blindness (amarousis fugax approx 10 mins), CL hemiparesisClassic Vertebrobasilar= drop attacks, bilateral blindness, confusion, vertigo

Watershed infarcts secondary to hypoperfusion near MCA-ACA border; with reperfusion can be bilateral and/or hemorrhagic

Hemorrhage can be intraparenchymal, subarachnoid, or mixed usually due to vascular damage VS. Hemorrhagic infarct- a white infarct that has been reperfused, looks like petechial hemorrhages

Pathogenesis of Atherosclerosis- development of focal area of endothelial damage with increased permeability to lipid insudation,

monocyte and platelet adherence- intimal aggregate of monocytes and macrophages- chemotactic and growth factors causing smooth muscle (intimal) proliferation- macrophages and smooth muscle cells engulf lipids forming “foam cells”- surface plaque can ulcerate, break off and lead to embolus and thrombotic infarction

DDx of Intercranial HemorhagesHypertensive hemorhages- intraparenchymal bleeds in regions served by perforator arteries caused by rupture of charcot-bouchard microaneurysm most frequently in basal ganglia, pons, cerebellum, 40% mortality, with resolution there may be some restoration of functionGross Pathology- expansion of affected area - flattening of the gyri- herniation, midbrain displacement- ventricular distortion

Berry Aneurysms- bleeding into subarachnoid space occurs at the bifurcation of cerebral arteries or at sites of media weakness, rupture can be ass’d w/ acute increased ICP, common in APKD, fibromuscular dysplasia, AVM, but MOST are sporadic.SX: sudden severe occipital HA, rapid LOC w/ 50% of patients dyingOther complications: infarction, hydrocephalus, herniation, brainstem hemorrhage, arterial vasospasm

AVM- 1% of intercranial tumors can rupture and bleed into grey matter of brain (setting up a seizure foci) or subarachnoid space, tortuous tangled vessels of various sizes, 90% are w/in the cerebral hemispheres, most common bleeds between the ages of 10-30 Males 2: 1 femalesSX: Intercerebral hemorrhage W/ seizures

Supratentorial Hemorrhages- lead to progressive hemiplegiaPosterior Fossa/ Cerebellar Hematomas- lead to intractable vomiting, increased ICP, herniation, & coma

Epidural Hematoma- trauma, middle menigeal artery, lense shaped on CT that does not cross midlineSubdural Hematoma- in young and elderly, tearing of the bridging veins, cresent shape and can cross cranial sutures, shifting midline structures

Unit Two

CT scan- Indicated in a stroke > 48 hrs or early to R/O acute bleed, tumor, trauma, dementia, cerebral hemorrhage- detection of structural abnormalities- speedy- good for evaluating progressive neurological disease or focal deficits due to strxl lesion- contrast improves detection of tumors & abscesses, AVM, chronic subdural hematomas MRI- Indicated in early stroke (w/1st day), hematoma, tumor, trauma (parenchymal pathology), dementia, MS or demyelinating diseases, and infections- better contrast between gray and white matter- better visualization of the posterior fossa and spinal cord

- better detection of MS plaques or seizure foci- gadolinium enhancement (contrast) can separate small tumors vs. edema, identify leptomeningio

disease, and integrity of the BBB- Superior to CT for MS, early stroke, tumors at vertex, posterior fossa, and acoustic neuromas

Arteriography- for visualizing intercranial circulation- catherization into femoral or brachial a. to a major cervical vessel w/ contrast to visualize cerebral

vessels- risks 1% morbidity/mortality and considerable radiation exposure

MR Angiography (MRA)- for visualizing carotids, and proximal areas of intercranial circulation where flow is fast- screening tests for stenosis, plaques, & venous sinus occlusion- if more than 70% consider carotid endarterectomyEEG- non invasive recording of electrical activity of brain w/leads placed on scalp- for evaluation of epilepsy, abnl rhythmic electrocerebral activity of abrupt onset and termination a

negative EEG can not R/O epilepsy not matter how many days it is followed- for classification of seizures disorder to determine type for proper anticonvulsant therapy- for assessment and prognosis of seizures & management of status epilepticus- detection of structural brain lesions (foci)- diagnosis of neurological diseases, i.e. HSV encephalitis repetitive slow wave complexes & CJK/SSPE

w/ presence of periodic complexes- evaluation of alterations of conscisouness, details of the level of comaEvoked Potentials- potentials evoked by non invasive stimulation of specific afferent pathways to monitor functional

activity of the pathway- common used in detection of MS lesions and monitoring functional status, spinocerebellar

degeneration, familial spastic paraplegia, Vit E or Vit B12 def’y, assessment following CNS trauma or hypoxia, intraoperative monitoring, evaluation of auditory and visual

- Types:- Visual w/ checkboard pattern stimulation recording potential from mid occipital region the clinical

importance is the P100 response.- Auditory – monoaural stimulation w/ repetitive clicks elicites BS evoked potential recorded at the

vertex of the scalp, senses potential in the first 10 ms, check for latency or increased intervals- Somatosensory- stimulation of peripheral nerves recorded at spine or scalp

EMG- assesses regional muscle activity via an electrode placed in muscle in passive and active statesmuscle activity at rest (passive state)- relaxed muscle, normally no spontaneous activity except at NMJ- fibrillation potentials = + sharp waves, deinnervated muscle seen in myopathic disease such as

inflammatory myositis and poliomyositis, not seen on PEX- fasiculation potential= characteristic of neuropathic disease, especially ALS- myotonic discharge=myotonic dystrophy, congenital myotonia, and occasionally poliomuscle in active state- myopathic disease= short, small duration, polyphasic motor units in affected (proximal) muscles- neuropathic disease= loss of motor units w/ decreased # of activated fiber with and increased rate of

firing (in distal muscles)secondary myopathies common w/ statins & steroidal therapyPoliomyositis is always AsymmetricalDermatomyosistis in kids is usually rheumatological & in adults it is probably a paraneoplastic syndrome

Nerve Conduction StudiesMotor Nerve conduction studies & Sensory nerve conduction studies- to comfirm the presence & extent of peripheral nerve damage- Carpal tunnel syndrome Dx’d with EMG/NC studies- sensory and motor velocities slowed at rest w/

possible signs of chronic, partial denervation of the median suppplied muscles of the hand

Lumbar Puncture- indicated for diagnosis of meningitis or other infective or inflammatory disorder such as SAH, hepatic

encephalopathy, Guillan Barre, psuedotumor cerebri, & MS- assessment of treatment response- administeration of intrathecal meds or contrast - to reduce ICP pressure (rarely done)- contraindications: suspected intracranial mass lesion, local infection, coagulopathy, suspected spinal

cord mass lesion, & a infarct w/ hydrocephalus- procedure= position pt in lateral decubitus position w/ maximal back flexion, site is between L3-L4 or

L4-L5 approximated by pt’s iliac crest, sterilized procedure w/ local anesthesia, bevel up, pop through tegmental flavum, enter subarachnoid space, measure opening pressure w/ stop cock. Pt may get post LP HA.

Clinical uses of BiopsiesBrain-- primary tumors, metastatic tumors, HSV encephalitis, abscesses, CJDMuscle- to distinguish neurogenic from myogenic causes: myositis, polio, DMD, BMD, myopathies, &

mitochondrial d/oNerve - for metabolic storage diseases (Fabry’s, Tangier’s), infection (leprosy), inflammatory changes

(vasculitis) or neoplastic involvementArtery biopsy- Temporal artery for Giant cell arteritis

Unit 3 Disorders of Consciousness

Seizures- excessive, oversynchronized discharges of cerebral neuronsEpilepsy- group of disorders characterized by recurrent seizures, episodic loss of consciousness, affecting 0.3% of population at one time in life. Possible etiologies include hereditary, perinatal injury, infection, post infarction stroke, and neoplasms (mass effect).

Benign Febrile Convulsions- 2-4% of children between 3 mo – 5yrs, on the 1st day of a non CNS febrile illness, lasts < 15 mins, non focal 2/3 have one seizure w/ < 10% having more than 3. Seizures occuring early in illness or w/ a family hx of seizures have a 90% risk of a recurrence w/in 2 years of the first seizure.

Generalized Seizures- (i.e. involving both hemispheres) & including absence seizure- primary generalized are usually in people < 20 and do not have an associated aura- tonic clonic- loss of consciouness, usually w/ a symptomatic aura or warning- tonic phase is unconsciousness w/ 10 Hz or faster waves, tonic contraction of limbs 10-30 sec,

extension of limbs, arching of body, crying, cyanosis, contraction of muscles of massication (toungue biting)

- clonic phase is alternating contraction /relaxation of muscle for 30-60 sec w/ slow waves & w/ return of ventilatory effort and clearing of cyanosis, frothy saliva, possible muscle flaccidity and incontinence.

- Recovery or post ictal phase, can have confusion and HA, > 30 mins at times, positive Babinski at times, Todd’s paralysis (transient unilateral weakness suggesting focal brain lesion)

Absence Seizures-- genetic, always begin in childhood and usually end by age 20- brief LOC w/ loss of postural tone- subtle motor manifestations= eye blinking or head turning “automatisms”- no post ictal phase, full orientation- inducible by hyperventilation- 3Hz/sec spike wave during seizure usually < 30 sec

Partial Epilepsy (i.e. focal)Simple Partial- begins w/ motor, sensory, or autonomic pheonomenon or aura (i.e. pallor, flushing, sweating, pupil

dilation, piloerection, vomiting, and incontinence)- psychiatric symptoms, dysphasia, distorted memory, affect disturbance, cognitive defecits, labored

thought processes, & hallucinations w/o LOCComplex Partial- temporal lobe or psychomotor episodes of impaired conscionnesss BUT NOT LOC- arises mostly from medial temporal or medial frontal ; possible HC formation- symptoms epigastric sensations, fear, déjà vu, olfactory hallucinations, before impaired

consciousnesss- usually < 30 min w/ characteristic coordinated motor activities “automatisim” orobuccolingual

movements, pupillary dilation or salivation.- Secondary generalization may occur if motor area is involved it will be a tonic-clonic

Systemic Diseases that predispose to seizures**know these- metabolic= hypoglycemia, hyponatremia, hypocalcemia- HTN, hepatic encephalopathy, uremia, porphyia- Drug overdose/withdrawal- Eclampsia/hyperthermia

Syncope- LOC due to decreased blood flow to brain, light headedness, dimming of vision, usually <15 sec w/ no

post ictal confusion- Causes cardiac abnormalities; orthostatic hypotension, vasovagal reflex, cerebrovascular syncope

EEG- useful confirmatory test to distinguish seizure from other causes of LOC, can NOT exclude seizuresEpilepsy EEG findings- abnormal spikes, polyspike discharge, and spike wave complexesAbsence seizures- 3Hz spike and wave activity, bilaterally symmetric and synchronousOther Brain Disease detectable by EEG- structural brain lesions- evaluation of altered conscousness- herpes encephalitis- repetitive slow wave complexes- CJD/SSPE- periodic complexes

MRI/CT is indicated in all partial or focal seizure patients

Status Epilepticus- Seizures that fail to cease and recur w/o full consciousness being restored in between- THIS IS A MEDICAL EMERGENCY.. and can cause permanent brain damage- Most common cause is seizure drug N/C or withdrawal & alcohol WD, metabolic, infarcts, tumor- RX: ABC’s, IV Diazepam 10 mg/ 2 minutes, Phenytoin, Phenobarbital- Labs: CBC, metabolic panel, tox screen, ESR, UA- IV 50ml of 50% dextrose + thiamine

- ABG & LP **know order

Primary Seizures vs Secondary Seizures

Phenytoin (Na Blocker) used in partial and GTC ataxia, nystagmus, somulenceCarbamazepine (Na B) used in partial and GTC ataxia, naseau, diplopiaPhenobarbital (GABA) used in GTC ataxia, somolence, memory problemsValproic acid (Na B) used in GTC & absence & M ataxia, drowsiness, tremorEthosuximide (Ca+ B) used in absence nausea, anorexia @ onset

Coma- no purposeful response to the environment, unable to arouse, eyes typically closed, no speaking, may

or may not withdraw to painful stimuli- caused by lesion above the midpons of the brainstem or w/ bilateral hemispheric involvementSupratentorial causes- mass lesion - subdural hematoma (more common in elderly w/ atrophic brains and increased sheer force on cortical

bridging veins with falling)- SX’s HA, alteration of consciousness, CL hemiparesis, ipsilateral pupillary dilation- RX: surgical evacuation- DX: CT shows crescent shaped hemorrhage that can cranial suture lines, & show midline shift of

ventricles- epidural hematoma (more common in head traumas ass’d w/ lateral skull fracture tearing of the middle

meningeal a. tear)- SX: Fine one minute, obtunded the next- RX: Medical emergency- DX: CT w/ lentiform (lens) shaped hemorrhage w/o crossing the suture lines

Subtentorial causes- basilar artery thrombosis or occlusion

- RX: anti coagulation- pontine hemorrhage

- Hypertensive patients w/ symptoms such as ocular bobbing, pinpoint pupils, w/loss of horizontal gaze

- cerebellar hemorrhage or infarct- posterior fossa subdural or epidural hematomas

Global causes of ComaHypoglycemia- in insulin overdose, alcoholism, insulinoma, or large retroperitoneal neoplasms, liver disease- condition must be treated w/ 50% dextrose w/in 60-90 minutes to prevent further damageGlobal Cerebral Ischemia-post cardiac arrestDrug IntoxicationHepatic Encephalopathy

Examination /Treatment of a Comatose Patient

Coma Cocktail- 25 g Dextrose (corrects hypoglycemia), 100 mg thiamine (Rx Wernicke’s), 0.4-1.2 mg Naloxone (Rx for opiod OD) HX:- sudden onset= vascular (stroke or hemorrhage)- rapid progression from hemispheric Sx to coma = intracerebral hemorrhage- protracted course to coma= tumor, abscess, chronic subdural bleed

- proceeded by confusional state= metabolicPEX:- signs of basilar skull fracture: raccoon eyes, Battles sign, hemotympaneum, CSF rhinorrhea/otorrhea- decreased in basal body temperature w/ alcoholic Wernicke’s, sedative OD, or hepatic encephalopathy- increased in basal body temperature w/ heat stroke or status epilepticus

Pupil- nl is 3-4 mm- thalamic= smaller but reactive- CN III compression, anticholinergic OD, transtentorial herniation of medial temporal lobe=fixed

dilated > 7mm non reactive- Brainstem at the midbrain level= Fixed mid sized pupils =5 mm- Opoid OD, focal pontine, Organophosphates, Neurosyphillis= Pinpoint 1- 1.5 mm- Midbrain structural lesion, CN III =Anisoconia, asymmetric pupils

Oculocephalic reflex “doll head” & Oculovestibular reflex (caloric) normal movement’s with intact brainstem fxnNormal movements COWS ( cold opposite warm same)Abnormal movements downward deviation w/ cold water= sedative ODNO OVR = structural lesion at PonsABNL OVR = CNIII or midbrain lesion

Motor responses to PainDecorticate response- subcortical lesion involves the thalamus, elbow flexed, legs extendedDecerebrate response- midbrain lesion elbow & forearm extension, leg extension

Irreversible Loss of Brainstem Function:- apneic coma- PCO2 > 60- Negative corneal, pupillary, gag, negative OVR/OCR, with + Doll’s- W/o underlying metabolic or drug induced etiology

Unit 4 Headache, Neck & Back Pain

Tension HA- usually after age 20 female > male, non throbbing bilateral occipital head pain “like a tight band around head”, not ass’d with N/V, prodromes, or auras. Chronic, frequent, sometimes involving tension in scalp or neck muscles, and may overlap with migraine type HA

RX: ASA, NSAIDs, ergot derivatives, Prophylaxis: relaxation, PT, psychotherapy or TCA, SSRI’s, propranol

Migraine HA- early onset, females> males or + Family Hx, pulsatile unilateral pain often ass’d with N/V, photophobia, and aura (intracranial vasoconstriction) vs HA (extracranial vasodilation), auras can be visual alterations such as hemanopic field defects, scotomas, scinnilations. Diagnostic Test: compression of ipsilateral carotid or superficial temporal artery may decrease severity of HA. Precipitating factors tyramine, nitrites, phenyethylamine, MSG, fasting, caffeine, emotion, menses, OCPs, nitroglycerin, bright lights.

RX: NSAIDS, ergot derivativesProphylaxis: propanol, TCA, methylsergide, CCB

Cluster HA- Men> women, > 25 year age @ onset, 10 mins-2hrs duration, Awakens patient at night, burning sensation lateral aspect of nose, pressure behind eye, lacrimation, nasal d/c, & Horner’s are common w/these. Precipitated by Etoh and vasodilating drugs, smoking.

Abortive: Sumatriptan SQ, 100% O2, dihydroergotamine SQRX for Chronicity: Prednisone, IndomethacinProphylaxis: ergotomine, methysergide, CCB

Trigeminal Neuralgia (Tic douloureux)- facial pain syndrome of CN V roots possibly due to arterial compression of nerve roots- V2 V3 unilaterally lighting like jabs of pain, spontaneously cease w/ sensory triggers on face by touch,

cold, wind, talking or chewing- Simillar pain w/ MS or brainstem tumors possible- RX: Tegretol or Posterior Fossa decompression surgery

Temporal Arteritis- subacute granulomatous inflammation of temporal or vertebral artery- HA, severe boring temporal face pain, > women over 50, uni or bilateral- 50% of untreated pt will develop blindness due to opthalmic artery involvement- DX w/ biopsy, ESR > 100- RX: prednisone for 1-2 years

Cervical Spondylosis- can include neck stiffness, pain in arms, segmental motor/sensory deficit in arms, UMN in legs,

cervical disc degeneration and herniation, secondary ossification, and osteophytic outgrowths- can involve > 1 nerve root & be uni or bilateral- presents w/ neck pain, limited head movement, occipital HA, radicular pain, sensory change in

arms/legs- C5, C6 commonly involved- DX: Xray, myelography, CT/MRI- DDX: myelopathy of MS, motor neuron disease, SCD, cord tumor, syringomyelia, hereditary spastic

paraplegia- RX: cervical collar, surgery

Radicular Syndrome- localized to one or more nerve roots (segmental paresthesias & numbness & weakness & reflex

changes)- exacerbated by coughing, sneezing, or other increases in ICP

C5 C6 Radiculopathy- weakness in deltoid, supra/infraspinatus, biceps, brachioradialis- pain & sensory loss in shoulder, outer arm and forearm- reflexes : decreased biceps and brachioradialis- ass’d myelopathy UMN weakness in 1 or both legs, change in tone or reflex, PC or STT sensory

defectsL5 S1 Radiculopathy- weakness dorsiflexion of foot, toes, eversion, plantar flexion, - Reflexes : decreased ankle jerk- Spine movement restricted w/ local back tenderness, decreased straight leg raising

Low Back Pain

Trauma-Musculoskeletal pain w/ lumbar muscle spasms, decreased spinal movement, DX: Xray to r/o vertebral fractures RX: bed rest, local heat, NSAIDs, and muscle relaxants Prolapsed Lumbar intervertebral disk- most common L5-S1 and L4-L5 - usually follows normal activity or strain, can be related to trauma- can cause radicular pain, segmental motor/senosory deficits, & sphincter disturbances- pain reproducible upon percussion over spine & passive straight leg raising

- Pelvic exam, rectal exam, & Xray to r/o other diagnosis- RX: analgesics, diazepam, bedrest w/ PT, if not fixed consider CT/surgery, etc

Lumbar Osteoarthropathy- later in life an increase in activity leads to pain- mild Sx Tx w/ surgical corsett & severe treat w/ surgery- can present as root/cord pain, spinal stenosis (intermittant claudication of the cord or cauda equina)

requiring surgical compression

Ankylosing Spondylitis- backache and stiffness, progressive limitation of movement, young men HLA B27- RX: Pain – ASA, indomethacin, PT, postural excercises

Neoplasms- suspect if symptoms are persistent and worsen despite bedrest- MRI to r/o mets, cord compression and cauda equina

Infectious- TB, pyogenic, cocci, of the vertebra or disc- LABS: increased WBC’s, increased ESR- RX: surgical debridment, long term (IV) antimicrobials, drainage

Osteoporosis- vertebral compression fractures- RX: NSAIDS, adequate activity, diet, HRT for post menopausal women

Paget’s disease of the spine - increased alk phosphatase w/ a NL Ca++ and NL PO4- urine hydroxyproline and urine Ca++ increase w/ active disease- Xray show increased density and expansion of bone, possible fissure fractures - RX: high Protein diet, Ca++, Vit C, Vit D, Anabolic hormones, calcitonin, bisphosphates, mithromycin

to decrease osteoclast activity.

Congenital Anomalies of Lower Back/SpineSpinal Dysraphism- defect in spinal fusion presenting w/ pain, neurologic deficit in 1 or both legs & sphincter disturbancesSpinal Stenosis- can lead to syndrome of neurogenic claudication later in lifeArachnoiditis- severe pain in back and legs due to inflammation and fibrosis of the arachnoid areaReferred Pain- Disease of hip joints, back & thigh pain w/activity w/ limitation of movement, + Patrick’s sign (pain

on external rotation), & Xrays show degeneration of hip joint- Other causes: Triple A, Cardiac ischemia, visceral/retroperitoneal masses, GU disease

Unit 5 Movement disorders

Postural tremor- tremor w/ sustained posture continuing w/ movement w/o increasing in severity- physiological 8-12 Hz commonly w/outstretched hand- enhanced physiological – as above but worsened w/ stress, anxiety, thyroidtoxicosis- Familial Benign essential tremor- AD inheritance involving 1 or both hands, head or voice

w/sparing of legs, medically treated w/ beta blocker Propanolol or anticonvul. Primidone- Drug induced, metabolic d/o (Wilson’s) or cerebellar d/o also produce tremor

Intentional tremor- tremor w/ activity, due to lesion in SCP or as a drug toxicity and can result in fxnl disability, treament is surgical procedure= sterotactic surgery of VLN of the thalamus;CEREBELLAR

Resting tremor- tremor w/o movement, characteristic of Parkinson’s but can be idiopathic or 2ndary to heavy metal poisoning (Pb+/Hg+) or Cu+ (Wilson’s disease); BASAL GANGLIA

Chorea- rapid, irregular, involuntary muscle jerks in various parts of bodyHuntington’s disease- due to loss of neurons in caudate nucleus & putamenDrug induced / SE of Dopa agonists- levadopa, antipsychotics, lithium, phenytoin, OCP’sVascular- vasculitis d/o, ischemic/hemorrhagic stroke, subdural hematomaStatic encephalopathy (Cerebral Palsy) secondary to anoxia, hemorrhage, trauma, or kernicterus

Dyskinesia- movement disorder that w/excessive abnl, involuntary movements i.e. tremors, chorea, hemiballismus, myoclonus, dystonia, tic, or athetosis w/ various etiologics including hereditary, vascular, or drug induced.

Isolated Focal dystonias- often treated with Low dose Levadopa, trihexphenidyl (anti cholinergic Artane), diazepam, baclofen or tegretol or surgically w/ stereotactic thalamotomy esp. w/ unilateral limb dystonia

1. Blepharospasm- involuntary closure of eyelids2. Oromandibular dystonia3. Torticollis- neck twisting, tx as above w/ botox injections possible CNXI sectioning4. Writers cramp- drugs not very therapeutic, alternating hand therapy, possibly botox

Important points in evaluation of Movement disorders

Age & Mode of Onset: Infancy think birth trauma, kernicterus, anoxic damage or CP, inherited d/oChildhood common for tic development, Tourette’sAbrupt onset think drug induced or ischemic eventSlow onset think degenerative disease

Drug & Medical Hx: anti psychotics(phenothiazine/haldol) commonly cause tardive dyskinesiaLevadopa (dopaminergic agonists) can cause choreaLithium, TCA, valproic acid (depakote), bronchodilators can cause tremorSLE, Thyrotoxicosis can induce choreaAIDS can cause tremor, chorea, dystonia, hemiballismus

Family Hx: History of demyelinating d/oHuntington’s disease

Idiopathic Parkinson’s Disease- loss of pigementation (cells) or presence of intracytoplasmic Lewy bodies in the substantia

nigra or other brain stem centers such as the globus pallidus, putamen, spinal cord or sympathetic ganglia

- imbalance between dopamine and Ach allows excess Ach to excite the inhibitory (GABA) release in the globus pallidus inhibiting the thalamus and cortex causing bradykinesia

5 Features of PD- bradykinesia, rigidity, resting tremor, masklike facies, stooped posture w/shuffling gait- dx is clinical

Mainstay Treatment of Parkinson’s disease1. Early PD- no drug Rx2. Progressive/symptomatic Rx

Levadopa/Carbidopa (Sinemet)- helpful for hypokinesia SE: N/V, hypotension, chorea, restlessness, confusionBromocriptine/& Pergolide (dopamine agonists)- ergot derivative, SE: psychiatric , Contraindication in pts w/ psychiatric d/o’s, MI, severe peripheral vascular disease, and active PUDAmantadine- antiviral that increases the release of dopamine SE: restlessness, confusion, skin rash, edema, arhythmiasAnticholinergic (muscarinic agonists) – to alleviate tremor & rigidity

Ex’s hexyphenidyl, benztropine, procyclidine, phenadrine

SE’s dry mouth, urinary retention, constipation, defective pupillary accomodation & confusion

Parkinson Plus Syndromes1. Shy-Drager Syndrome- Parkinson’s with ANS instability & other neurological involvement2. Cortical Basal Ganglionic Degeneration- Parkinson’s w/ sensory loss, apraxia, focal reflex myoclonus

w/asymmetric symptoms & no ANS instability or cognitive loss. Some Pt’s respond to sinemet (levidopa/carbidopa)

Tardive Dykinesia: Pt w/ abnormal choreoathetoid movements usually of face or mouth in adults or limbs in kids that can develop w/ long term Rx w/ antipsychotics (anti-dopaminergics)

Neuroleptic Malignant Syndrome: Patient develops rigidity, fever, altered mental status, and autonomic dysfunction over 1-3 days of antipsychotic treatments ESP’LLY WITH HALOPERIDOL, RX w/ dantrolene or manage symptoms with antipyretics, anticholinergics, antipsychotics lithium. DDx is infectious cause.

Unit 6 Motor and Sensory Deficits

Jaw - mandibular branch V3 3and ponsBiceps - Musculocutaneous and C5, C6Brachioradialis – radial and C5, C6Triceps- Radial and C7, C8Finger- Median and C8, T1Knee- Femoral and L3, L4Ankle- Tibial and S1, S2

Pyramidal System from Motor cortex to IC- medullary pyramid decussation to descend in CL CST : lesions here will cause difficulty in fine motor movements. (UMN)

Extrapyramidal System- UMN input from Basal Ganglia or cerebellum : lesions here will have uncoordinated rate, rhythmic, and irregular amplitude movementsLMN motor fibers are cranial nerves or peripheral nerves

AppearanceFasiculations are to LMN injuries (or anterior horn disease) as flexor and extensor spasms are to UMN.Psuedohypertrophy is usually due to myopathies w/ replacement of muscle by adipose or other tissue.

ToneTwo types of hypertonia- Spasticity (clasp knife-velocity dependent w/ arm F>E and legs E>F most likely due to UMN damage) & rigidity (lead pipe- seen in passive movement affecting all muscles equally most likely due to BG/cerebellar lesion with cogwheeling superimposed ) Paratonia- seen in pts with frontal lobe or diffuse cerebellar disease where patient is unable to relax and will move limbs as examiner does.

Power (or weakness 0 –no contraction 1 flicker 2 active w/ gravity eliminated 3 active but no resistance 4 active to gravity and resistance & 5 nl)UMN weakness pattern affects Extensors and Abductors in the ARMS and flexors in the legs.LMN weakness pattern of supplied muscles at SC, root or peripheral level with distal > proximalProximal more than distal suggests a myopathy or NMJ Variability in pattern of severity and distribution over time suggest Myasthenia gravis/Lambert Eaton syndrome.

ReflexesAreflexia- root lesion, or peripheral neuropathy or early UMN disease, deep comaHypereflexia- (clonus)- rhythmic contractions in response to a stretch Lateralized asymetrical of reflex responses (UMN disease) Focal reflex deficits relating to root, plexus, or peripheral nerve lesionsLoss of distal DTR common in polyneuropathies & can be normal finding in elderly.

GaitsApraxic (dominant frontal lobe d/o- hydrocephalus, dementing processes, w/o weakness or coordination problems) usually can’t stand unsupported, feet glued to ground, unsteady short stepped gait

Hemiparetic w/ circumduction- unilateral lesion of CST pt tilts at waist and swings affected leg around

Scissoring- w/severe bilateral spasticity, legs brought stiffly forward & adducted w/ compensatory trunk movements (UMN sign)

Parkinsonian gait- small steps taken at a increased rate (festination), problems with starting and stopping, forward stooping, reduced arm swing

Steppage gait in persons w/ impaired sensation.

UMN SYNDROMES (damage to cortical areas where UMN orginate pyramidal & extrapyramidal CST)- hypertonia “spasticity”- arms flexed (w/ weakness of extensors and abductor) legs extended (w/ weaker flexors)- hyperreflexia “sustained or nl clonus ”- changes in gait- + Babinski

Radiculopathy- lesions affecting specific nerve roots yielding characteristics changes in muscle innervated, location of sensory and reflex changes, dermatone distributionNeuropathy- pathology of a nerve that causes weakness in innervated muscles (distal >proximal) w/ a decreased reflex or sensory change, fasiculations and muscle wasting can occur later in diseaseMyelopathy- lesion affecting spinal cordMyopathy- d/o affecting the muscles, usually presenting with weakness in proximal area vs. distal, w/ NO sensory loss, no sphincter disturbances, nl abdominal and plantar reflexes, with muscle wasting & decreased/absent reflexes only late in disease

ALS- Pure motor syndrome mixed UMN & LMN signs w/ NO sensory deficit usually beginning in the

limbs- (40%) c/o weakness, cramping, twitching, stiffness, etc in upper extremites and (40%) c/o of the same

type of stuff in lower extremities- (20%) present with bulbar signs; which has a worse prognosis- Progressive, fatal w/in 3-5 yrs, mainly due to respiratory problems, infections- Clinical Dx., CSF is normal, no sensory change or mental dysfunction- Rx of Symptoms

- Drooling- treat w/ anticholinergics- Mobility – walkers, braces- Contractures- prophylaxis PT

Myasthenia Gravis- occurs at ANY age; Females > males & often ass’d w/ Thymomas, thyroidtoxicosis, or SLE

- characterized by fluctuating weakness of voluntary muscles w/ a predilection for extraocular muscles (bilateral opthalmoplegia) and other CN muscles (massetor, facial, laryngeal, pharyngeal) but can get limbs & diaphragm as well

- Pt present w/ ptosis, diplopia, difficulty swallowing or chewing, nasal voice, weak limbs and respiratory difficulties w/o sensory changes & w/o reflex changes

- autoimmune etiology- Ab’s against the Ach receptor at the postsynaptic NMJ causing a relapsing, remitting course

- can have insidious onset or become symptomatic in times of stress (infection, menses, pregnancy)- drug induced onset w/quinine, quinidine, procainamide, propranolol, Lithium, phenytoin, PCN &

aminoglycosides- Dx is clinical and the edrophonium (anticholinesterase drug- Tensilon) test is useful to check for

improvement right away. If improved, prescribe a longer acting pyridostigmine (Mestinon)- Other Rx’s & tests

- Other anticholinesterase drugs(Mestinon), corticosteroids, azathioprine, plasmapharesis, IVIG- CXR for thymomas, or thymic hyperplasia w/ thymectomy if indicated- EMG

Symmetric Polyneuropathies:Guillen – Barre Syndrome-- weakness usually begins in legs, may have sensory and autonomic complaints- often follows infective illnesses, innoculations, or surgical procedures. May be associated with

camplobacter jejuni- speed and progression variable but can be fatal- Dx: acute onset w/progressive weakness of > one limb up to 4wks, symmetrical, distal areflexia w/

proximal hyporeflexia, mild sensory changes, CN VII involvement, autonomic dysfunction, with recovery beginning 4wks after onset.

- Tests: CSF w/ increased protein BUT NO Cellls; EMG shows slowing- Rx’s: plasmaphoresis, IVIG (400mg/kg/d X 5d)- Prognosis: Sx’s decrease after 4 weeks, 75% recover, 25% have neurodeficits w/ 5% dying acutely

Critical Illness Polyneuropathy-- pt w/ sepsis or multi-system failure- wasting of extremities w/ absent DTR’s and sensory changes

Diptheria Polyneuritis-- neurotoxin produced by C. diptheria 2-3 wks following a palate or muscle infection- within 4-5 weeks pupillary response impaired- 1-3 months generalized sensory polyneuropathy; weakness proximally> distal- Dx. CSF w/ increased protein, pleocytosis & EMG showing decreased conduction- Rx Antitoxin + 2 weeks of PCN

Paralytic Shellfish Poisoning-- poison blocks Na+ channels causing a rapidly progressing sensory & motor ascending paralysis

Porphyria-- weakness usually proximal > distal; beginning in upper extremities and later involving leg and trunk- decreased or absent DTR’s, sensory changes- also causes abdominal pain, psychosis, fever/chills, HTN, hyponatremia, peripheral leukocytosis

Arsenic/ Thallium poisoning- - sensorimotor polyneuropathy w/ GI disturbance- distal > proximal & legs > arms

Organophosphate poisoning-- cramping pain to distal numbness progressing to leg weakness & decreased DTR- sensory changes 1st in legs then arms

Sensory Disorders

Complete loss of touch= anethesia of pain= analgesiaPartial loss of touch= hyesthesis of pain= hypalgesiaIncreased sensitivity= hyperesthesia of pain= hyperalgesia/hyperpathia

Light touch= ipsilateral posterior columns (large fibers) to medial lemiscus of brain and on the contralateral anterior spinothalamic tract

Pinprick & Temperature= ipsilateral lateral spinothalamic tracts for 2-3 segments after entry and then cross over infront of central canal

Deep Pressure= evaluated on tendons (achilles)

Vibration= 128 Hz tuning fork on bony prominence, often decreased in elderly below the knees

Joint Position= passive movement of DIP joints thought to be carried in fibers also in the posterior columns

Complex sensory tests

Romberg’s Test= + w/ unsteadiness to evaluate impairment of joint sense in legs ( tabes dorsalis )

Two point discrimination= depends on intergration between the CNS & PNS and sensory compenent of the area stimulated. Threshold is 4mm on fingers to several cm on back. If PNS is intact w/decreased two point discrimination d/o in sensory cortex (CL parietal is suggested).

Graphesthesis, Stereognosis, & barognosis= able to identify a number traced in palm, shapes presented or pressures applied in area w/ normal sensation implies problem in CL parietal lobe.

Bilateral sensory discrimination (EDSS) = neglect/or decreased reaction to 1 side of stimulus suggest CL cerebral lesion

Sensory changes often develop well before onset of sensory signs and should not be considered psychogenic.

Peripheral nerve lesions: Mononeuropathy= not usually noticed b/c of overlap but can be caused by compression lesions that preferentially involve the large touch fibers.

Polyneuropathy= symmetric and distally> proximally (glove and stocking sensory loss) feet before hands usually. EX’s Tangier disease (metabolic AR d/o w/ little or no HDL) tends to involve small pain & temperature fibers. Can also involve motor and reflex changes.

Distal axonpathies > myelinopathies ; including acute idiopathic GBS, CIDP, infective (AIDS, leprosy, diptheria, sarcoid), vasculitic ( PAN, RA, SLE), paraneoplastic syndromes, drug induced ( alcohol, INH, Dilantin, dapsone, vincristine, etc.), toxins, heavy metals (Pb), metabolic and nutritional neuropathies (DM, hypothyroid, uremia. B 12), entrapment, & hereditary neuropathies often w/ motor involvment (metachromatic leukodystrophy, Krabbe’s disease, Freidreich’s ataxia, Fabry’s, Refsum’s, etc)

Radicular lesion/Root involvement= impairment of a segemental cutaneous patternRoot + pain suggests compression lesionLoss of reflexes at specific root levels C56 bi/brachio C78 tri L34 knee and S1 ankleW/ weaknes and muscle atrophy= suggest anterior root involvement

Central Cord lesion= syringomyelia, cord tumors, loss of P & T 1st, bilateral, segmental, and possibly w/ LMN weakness & pyramidal or PC deficit below level of lesion.

Anterolateral cord lesions= CL impairment of P & T below level of lesion, with sacral segments involved first with extramedullary lesions and can be spared in intrinsic or intramedullary lesions.

Anterior Cord lesions: CL impairment of P & T below level of lesion w/ LMN weakness of paralysis of muscles supplied by the segment due to damage of the anterior horns

Posterior Column Lesion= tight or bandlike sensation at level of the lesion w/ paresthesias (electrical shocks) shooting down extremities on neck flexion (Lhermitte’s sign). Loss of vibration and position sense w/ preservation of sensory.

Cord Hemisection or Brown-Sequard Syndrome= ipsilateral pyramidal deficit (UMN signs) w/ disturbed vibration and positional sense w/ CL loss of P & T 2-3 segments below the lesion

Crossed Sensory Deficit in Medullary lesions= P & T lost in CL limbs w/ Ipsilateral facial pain & T lost Wallenberg type

Brain Stem lesions= usually sensory combined w/ motor, cerebellar signs, & CN palsiesPons= P&T lost in CL limbs and trunkMedulla= P & T lost in CL limbs and trunk w/ ipsilateral P & T loss of facial sensation (spinal

Trigeminal nucleus) (Wallenberg syndrome)Medial Lemiscus= CL loss of touch and propioception or w/ upper brainstem involvement

Complete CL loss of sensation

Thalamic lesions= CL impairment of all forms of sensation except in some cases where there is a painful sensation with any stimulation (Dejerine-Rousy syndrome) This can result from lesions of white matter of the parietal lobe or SC. (also know as thalamic pain syndrome).

Sensory Cortex Lesions: CL impairment of discrimatory sensory function usually worse distally more than proximally

Psychogenic disturbances could be conversion disorder & are usually cutaneous.Non organic tends to surround an area defined by surface landmarks, sharp margins & commonly stops at midlineOrganic does not usually extend to midline b/c of overlapping innervation and has surrounding areas of altered sensation.

Examples of types of Peripheral NeuropathiesMetabolic: DM, hypothyroidism , acromegaly, uremia, liver disease, vit B 12 def’y, hyponatremia, hypoglycemiaInfectious: AIDS, TB Leprosy #1, diptheria, sarcoidosis, sepsis, multiorgan failureHereditary: HMSPN, Friedreich’s ataxia, Familial Amyloidosis, Porphryia, Krabbe’s, Tangier’sPN’s associated with PAIN: DM, Etoh, porphyria, Fabry’s, amyloidosis, HSN, paraneoplastic senosry neuropathy.

Entrapment Neuropathies:

Carpal Tunnel Syndrome: compression of median nerve @ writst- often during pregnancy, or seen w/ trauma, degenerative arthritis, myexedema, acromegaly,

amyloidosis- pain & paraesthesia of median n. distribution of hand (thumb, index, middle, & lateral ½ of ring) can

radiate up to neck and can keep pts awake at night, weakness and thenar atrophy may occur later- Dx. Tinnel’s sign (pressing over median nerve) & Phalan’s sign (wrist flexion) both positive- EMG

- Rx: corticosteroids, splint, surgery

Ulnar nerve palsy: compression @ elbow- may result from ext.pressure, entrapment @ cubital tunnel or from cubitus valgus deformity- nocturnal pain in little finger & ulnar border of hand +/- pain at elbow, possible weakness/sensory Sx’s- Rx: surgical decompression

Peroneal Palsy: secondary to trauma or pressure @ head of fibula- weakness or paralysis of foot and toe extension/foot eversion, impaired sensation over dorsum of foot

& lower ant. Aspect of leg, ankle DTR’s are present- Rx: supportive

Femoral Nerve Palsy: L3,L4 ass’n w/ DM, vascular ds bleeding diathesis, or retroperitoneal neoplasms- weak quadriceps, decreased patellar reflex, sensory changes of ant/med aspects of leg- Rx: treat underlying disease

Herpes Zoster-- viral d/o that causes an inflammatory rxn in dorsal root ganglion or CN nuclei, in the affected root, or

in nerve itself, can also be in CSF- intial c/o burning or shooting pain w/in dermatome with rash 2-5 days later, rash resolves with scars

and possibly post herpectic neuralgia- common sites: thoracic dermatome, V1 of CNV which may lead to corneal scarring and anesthesia- Ramsey Hunt syndrome CNVII palsy w/ herpectic eruptions in ear, palate, pharynx and nose- Rx: Acyclovir or steroids for Sx’s and tegretol 1200 mg/d, phenytoin 300mg/day, and amitryptiline 10-

100 mg q hs.

Syringomyelia-Communicating ( hydrodynamic d/o of CSF pathways) opening between central cord and cavityNon Communication- cystic dilation of cord not communicating with CSF pathways- severity is site dependent, mainly w/ impaired P & T at that level with intact light touch- sensory loss commonly seen by presence of painless skin ulcers, neuropathic joints, edema, scars, etc- muscle wasting @ level of lesion with Extrapyramidal signs and sphincter disturbance possible- pyramidal lesion w/ gliosis or compression of CST- scoliosis is common- decreased DTR below lesion and Increase above lesion

Friedrich’s Ataxia- idiopathic degenerating disease of cerebellar ataxia- AR chrom 19 begin’s in childhood- Demyelination of Spinal cerebellar tract, posterior column, dorsal roots and neurons in clark’s nucleus- Large axons of periferal nerves as well as cell bodies of sensory nerves, and dorsal root ganglion- Sx’s: Gait ataxia, progression to involve all limbs, dsyarthias, w/2ndary features impaired flexor

/extensory response, pes cavus, scoliosis, and cardiomyopathy.

Unit 7 Multiple Sclerosis

CNS demyelination Signs in MS:- Demyelination slows AP’s or blocks them and effects heavily myelinated tracts i.e. optic nerves,

posterior columns, corpus callosum, brainstem pathways, and spinal cord.- Can present w/focal weakness, numbness, tingling or unsteadiness in a limb, sudden loss or blurring of

vision in 1eye (optic neuritis), diplopia, disequilibrium, or bladder fxn disturbance. Some patients present with mainly UMN sx’s as acute to gradual spastic paraparesis w/ sensory deficit.

- Mechanism considered to be autoimmune, with hypersensitive T cell (or IgG’s) attacking myelin & CNS myelin producing cells oligodendrocytes, HLA DR2 genetic relation, women > men, ages 20-40, and increased exacerbations with heat which sensitizes the Na+ channels that are responsible for AP conduction

- Impaired/blocked conduction can result in gliosis

6 common clinical presentations of MS1. optic neuritis- tested w/ swinging light test shows Marcus Gunn pupil or pupil w/ afferent defect which

will constrict less markedly w/ direct illumination and also have decrease in color vision a sign of retinal involvement

2. INO- demyelination of the MLF clinically seen as MR palsy or diplopia sign of Brainstem disease and if not MS DDx includes brainstem encephalitis, tumor, syringomyelia, drug intoxication, Wernicke’s

3. Disequilibruim- posterior column (+ Romberg’s test)4. Urinary incontinence or frequency, ANS dysfunction that causes spasms or retention5. Focal weakness (bilateral paraparesis of both legs)6. Paraesthesias- numbness or tingling or dyesthesias- painful sensation from a non painful stimulus

allodynia7. Spasticity- increased tone, clasp knife effect w/ velocity dep’t passive movement, UMN lesion

Clinical criteria for Dx of MS1. 2 signs or symptoms separated in time and space2. clinical picture w/ history and elimination of other possible cause for symptoms3. MRI (T2 w/contrast) -w/ evidence of plaques (areas of demyelination in the white matter;commonly

periventricular)

Paraclinical supporting tests1. LP w/ CSF showing increased IgG; oligoclonal bands (+ in 90% of pts)2. VEP, BAER, SSEP evoked potential showing slowing

Other conditions or DDx for MS1. isolated Optic neuritis (70% will go on to develop MS later)2. transverse myelitis (Brown Sequard Syndrome)3. vasculitis (SLE, Sjorgens, etc)4. AIDS5. Lyme disease (bilateral Bells or CN VII palsy)6. Inherited spinocerebellar degenerations (Freidrich’s Ataxia)7. Adrenoleukodystrophy/Metachromatic leukodystrophy8. CNS mass lesion (more likely if focal defecits clinically)

Prophalaxis drugs (ABC’s of MS)1. Avonex (beta interferon 1a)2. Betaseron (beta interferon 1b)3. Copaxone (glatiramir acetate) alters or interferes w/ T cell response to myelin w/ SE of flu like

syndrome, increased spasticity & side reactions w/ betaseronRx for acute exacerbations IV methylprednisolone (Solu-Medrol) 500-1000 mg/d, IVIG, plasmaphoresisManagement of MS ACTH- 80 U IM x 1wk, tapered for 2-3 wksSteroids: Methylprednisolone 15-20 mg/kg/d IV x 3days, taper 2 wk Prednisone 60-80 mg/day PO x 1 wk, taper 2 wks Dexamethasone 16 mg/d x 1wkImmunosuppressants: Cyclophosphamide, azathioprineIVIG, plasmapharesisPT/OT & speech therapySpasticity- Baclofen (GABA agonist), Tizanidene alpha agonist(zanaflex), Dantrolene, DiazepamNeuropathic Pain- TCA amitriptyline (elavil), or anti-convulsants gabapentin (neurontin), dilantin, tegretolFatigue- pemoline (cylert), amantadine (Dopa agonist), fluoxetine

Unit VIII DEMENTIA

Dementia- progressive deterioration in mental function that interferes w/ ADL’s

Need 2 or more of the following:-Defecits of memory, language, visualspatial skills, calculation, apraxia, agnosia, abstract reasoning, behavioral componentPersistance of deficits is the differentiating feature from delirium.Evaluation duration, progression, risk factors, ass’d neurological findings, Hx of psychiatric disease, head injury, or FHx of neuro/psychiatric diseaseDX work up: CT/MRI, SPECT/PET, EEG, LP, metabolic panel w/ CBC

Alzheimer’s- most commonly diagnosed form of dementia- risk factors= age, FHx, head trauma w/ LOC- present w/ loss of ST memory, language, orientation, & judgement- progresses over 2-20 year period ultimately involving all functioning- pathological dx senile (amyloid) plaques, NF (tau microtubule protein) tangles, neuronal degeneration- association cortex in parietal, temporal and frontal lobes particularly affected- loss of cholinergic activity in forebrain: nucleus basalis of Meynert- dx is one of exclusion, with diffuse cerebral atrophy possibly seen on CT/MRI- PET scan may show regional hypoperfusion in parietaltemporal region, EEG w/ slowing- Apo E4 Genotype testing & CSF levels of high Tau and lowAmyloid have poorer prognosis- RX: acetylcholinesterase inhibitors (Cognex & Aricept) - Symptomatic treatments: LD benzo’s, neuroleptics, AD’s- Genes involved in SDAT chrom 21 amyloid, chrom 14 is presenilin, chrom 1 is presenilin 2, 19 is Apo

E w/ E4 homozygotes at highest risk , Apo E2 may have protective benefit in caucasians

Multi Infaract Dementia- elements of both cortical and subcortical dementia- stepwise progression, psuedobulbar effect w/ impossible localization of a single focal lesion- DX: CT can miss small infarcts & MRI is more sensitive- Non invasive or invasive vascular studies are useful- RX: antiplatelets, anticoag, decrease risk factors

Infectious Dementia- resulting from meningitis or encephalitis- general paresis of the insane “treponemen pallidum” neurosphyllis delayed onset up to 30 yr, RX w/

PCN - chronic meningitis agents: syphillis, crytococcus, TB, borellia, other fungal/ parasites possibly

presenting w/ hydrocephalus- AIDS dementia complex – HIV 1 presents w/ psychomotor retardation progressing to global dementia

consider concurrent infections; cryto, PML- Herpes Zoster Encephalitis- diffuse slowing on EEG- CJD disease w/ characteristic EEG pattern of periodic complexes- DX : RPR, VDRL-ABS, lyme serology, MRI/CT, CSF culture- RX: acyclovir for herpes, Ab’s

Normal pressure Hydrocephalus (NPH)- Communicating & non communication Hydrocephalus are both able to produce dementia- Nl CSF pressure 100-200 mm H20- NPH can be caused by SAH, CSF infections or idiopathic- Present w/ ataxia, urinary incontinence, and dementia- DX: CT/MRI showing dilation of ventricles, or radionucleoside cisternography- RX: ventricular shunting improves 50% of cases

Dementia in Extra Pyramidal Syndromes (UMN Globus pallidus & cerebellum)Parkinson’s -- can present w/ tremor, cog-wheel rigidity, bradykinesia, shuffling gait, masked facies

RX: sinement, amantadine, bromocriptine, selegelline (MAO B inhibitor)Diffuse Lewy Body diseaseHuntingtons- choreoathetosis w/ positive FHx

RX: anti dopaminergic haloperidol & risperidone (antipsychotics)Wilsons- deposition of Cu++ seen on CT/MRI, genetic testing, serum copper levelsProgressive Supranuclear palsy- paralysis of vertical gaze and axial rigidityCorticalbasilar degeneration- apraxia & alien limb phenomenon

Random questions

Which extrapyramidal syndrome presents with dementia, paralysis of vertical gaze and axial rigidity?Progressive supranuclear palsy (PSP)

Which extrapyramidal syndrome that has dementia, choreoathetosis, can be sometimes diagnosed with MRI, and whose motor symptoms often respond to anti-dopaminergic agents such as Haloperidol and risperidone?Huntingtons disease, AD genetic disease, chromosome 4, atrophy of caudate nucleus

Which disease of dementia has the histopathological change of neuronal granulovacuolar degeneration, especially in the nucleus basalis of Meynert & favors the association cortex in the parietal, temporal, and frontal lobes and can be diagnosed sometimes on a volumetric analysis of the hippocampal/temporal regions?Alzheimer’s Disease, also with senile plaques and NF tangles but too obvious to include in the question.Associated with chromosome 1 (presenilin 2), 14 (presenilen 1), chromosome 19 (Apo E) and chromosome 21 (APP-amyloid precursor protein).

Which disease can be symptomatically treated with Cognex and Aricept, along with low dose benzodiazepines, AD’S trazodone, or neuroleptics for behavioral changes or paranoia?

AlzheimersWhich allele is associated with higher LDL and familial and sporadic AD?Apo E4, and combined with head injury it may increase the risk ten fold

Which protein that is associated with microtubules is elevated in AD w/ a 90% specificity?Tau

Is beta amyloid, the protein ass’d with neuritic plaques increased/decreased or nl in the CSF of pt with AD?They are decreased in pt’s with AD and if they are elevated in a patients CSF it can rule out AD.High Tau > 420 pg/ml and low Ab <1240 is diagnostic for AD

Which lobes are involved in Pick’s Disease?Frontal and Temporal Lobe (FTD) inappropiate bx W/O the three A’s No amnesia, NO aphasia, NO agnosia, ex. Is Kluver-Bucy syndrome

Multi-infarct dementia’s have what type of characteristic presentation?Progressive, psuedobulbar, or w/possible focal deficits in high risk people, most commonly due to subcortical lacunar infarcts

What is the preferred evalation for dx of MID?MRI or vascular studies

Infectious dementia can be caused by which key organisms?Treponema pallidum, TB, cryptococcus, borrelia, etc.

Which studies are done to rule out additional causes of dementia in patients with HIV 1 ass’d dementia?An LP to check for crypto or imaging to r/o PML, & if non AIDS pts Creutzfeldt-Jakob disease or HSV

A patient presents with a triad of dementia, gait ataxia and incontinence w/ a history a recent MVA what is a likely diagnosis?NPH (between 100 & 200) secondary to a subarachnoid bleed, caused by infection or AVM

Contusions of the grey matter in the orbitofrontal and temporal lobes along with diffuse axonal injury to white matter structures can cause which syndrome in young people?Post-traumatic dementia, often occuring after an MVA.

An elderly woman presents to the ER after falling with fluctuating levels of consicousness, impaired attention, and is irritable. What could be the cause?Subdural hematoma.

Which lobe is least likely to give focal deficits if involved w/ a tumor producing dementia?Frontal

What kind of paraneoplastic syndrome is caused mostly by lung, breast, ovarian, and testicular CA that presents with neurological signs such as amnestic syndrome, seizures, or an affective disorder up to two years prior to primary tumor is discovered?Paraneoplastic Limbic Encephalitis

Depressive dementia is typical cortical or subcortical in nature?Subcortical w/ Complaints of deficits or I don’t know or I can’t type answers

Ganser syndrome refers to what type of dementia?Hysterical w/ some somatomotor symptoms like conversion reactions & may be frank malingering.

Which cortices are primarily involved in Alzheimer’s disease?Temporoparietal which can cause fluent aphasias w/ comprehensive problems & frontal

Misc. stuff for the wardsIsolated Eye Findings

CNVI (Lateral Rectus)- affected eye deviates medially, head turns towards the lesion side- commonly presents as increased ICP- most common etiology in kids is pontine glioma- most common etiology in adults is Nasopharyngeal carcinoma (presents w/ sensory findings)

CN IV (Superior Oblique) - Most common cause of vertical diplopia, eye is up & in, head tilting to opposite, w/ straight line test arrow points to the lesion side.- most common cause is trauma or idiopathic

CN III lesion- ptosis, eye is “down & out”, dilated non-reactive pupils, cyclopegia (loss of PNS of CN III)- a diabetic ischemic infarct spares the pupil (the PNS fiber are on outer portion of nerve)- a compression lesion dilates the eye, painlessly and progressive (uncal herniation)- common etiologies are aneurysms, temporal lobe herniation, meningiomas in cavernous sinus

Painful OpthalmoplegiasTolosa Hunt Syndrome, psuedotumor cerebri, inflammation in the cavernous sinus

Acute Bilateral Opthalmoplegias-Wernicke’s, GBS, MG, TB meningitis, botulism

Psuedoparalysis of upward and downward gaze-Thyroid disease, myxedema infiltration of ocular muscles IR & SR

Duane’s Syndrome- congenital absence of CN VI “ retraction syndrome” eyes are sunken due to co-contraction of MR & SO causes globe retraction

Intranuclear Opthalmoplegia- If on the left there is no Left eye Adduction when patient looks to the Right, and the nystagmus is in the unaffected abducting eye (Right beating nystagmus)- lesion is in the MLF- commonly associated with MS

Down beating nystagmus is always central in origin- Wernicke’s, Arnold Chiari malformation, lesion in the medullary/cervical region

Vertical Nystagmus on upward gaze- commonly due to a demyelinating, or vascular disease, tumor or w/ Wernicke’s- lesion in the pontine medullary region of brainstem

Red Glass Test for Diplopia- false image is always the furthest image and the visual field where the two images are furthest apart is

the field of vision of the affected muscle i.e. if horizontal nystagmus is worse w/looking to the right it is most likely the R abductor (R CNVI) or the left adductor (L CNIII)

- for polymonocular disease of diplopia that is non specific DDx should include MG or thyroid opthalmopathy (w/ this eye may be protuding)

IF diplopia does not go away with covering of the eye you can call Psych b/c it is not likely neurological!

A CN III, IV & VI palsy is probably due to a brainstem lesion vs. a peripheral cause such as tumor at base of brain, ischemic infarction, or aneurysms within the Circle of Willis.

Common Brain TumorsAstrocytomas- most frequent & include Glioblastoma Multiforme, gliomas in BS (pontine gliomas in kids), and

pilocyticOligodendroglioma- most likely cerebral, focal hemorrhages & calcifications, variable prognosisEpendymoma- younger people 10-20’s; often presenting with hydrocephalus- most common in 4th ventricle> lateral ventricles > third ventricle> aqueduct of SylviusMedulloblastoma- occur in the cerbellum w/in the 1st two decades of life- dissseminate through CSF, neuroectodermal in origin- highly malignant but radiosensitiveMeningioma- most commonly appear in frontal hemispheres, near falx, the lesser wing of sphenoid, and near

olfactory groove- usually solitary but can be multiple if ass’d with NF Type 2- whorled pattern on histology, psammoma bodies

Common Mets to BrainLung, Breast, Colon, Melanoma, & Renal

Psuedotumor Cerebri- obese females w/ increased ICP, taking OC’s, that present with throbbing headache, visual changes (peripheral blurring/decreased acuity), and have papilledema on exam.- benign intracranial HTN w/ diffusely increased ICP- often present w/diplopia with CNVI palsy- females > males- CT/MRI shows small ventricles

RX: decrease ICP w/ furosemide, CA inhibitors or surgical optic nerve sheath fenestrationFor refractory cases prednisone can be used.

Major danger of not treating an elderly patient with steroids that presents w/ a throbbing, acute HA, jaw claudication, with a HX of Polymyalgia rheumatica, and a current ESR of 100 is loss of vision due to thrombosis of the opthalamic artery due to Temporal Arteritis.