NEUROLOGYInternal Medicine Board Review

Brad Ingram, MD

Neurology

• Dementia• Headache• Epilepsy• Neuro-oncology

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• Demyelinating diseases

• Stroke• Neuromuscular• Movement Disorders

Demyelinating Diseases

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26 y/o RH WF with no significant PMH presents to the ED with blurry vision and pain with eye movements x

2d. She has no other complaints but does report having had at transient R leg weakness and a

lightning-like sensation on neck flexion in the past. On exam, you find a well-appearing woman with

decreased visual acuity and impaired color vision OD. She has disc pallor OS, R ankle clonus, and a L INO.

Labs are all normal. Her MRI is shown below. What is the most appropriate next step?

A. LP with MS panel, OCBsB. Start her on copaxoneC. MRI c-spineD. C/s ophthalmologyE. Start Solumedrol 1

gram qday

Multiple Sclerosis• Diagnosis:

• Clinical—dissemination in time/space• Paraclinical—MRI brain/c-spine, LP, evoked potentials

• Treatment:o Exacerbation

• New si/sx lasting >48 hrs with infection/metabolic cause ruled out (pseudo-relapse)

• Solumedrol 1 gram/day x 3-5 dayso Prevent Relapse

• 1st line: IFN b-1a, IFN b-1b, glatiramer acetate• 2nd line: mitoxantrone (cardiotoxicity, leukemia);

natalizumab (PML); fingolimod (bradycardia, macular edema)

Symptomatic Treatment

• Fatigueo R/o medical causes: TSH, B12, CBCo Address sleep hygieneo Exercise and cooling measureso Amantadine, modafinil

• Spasticity: PT, baclofen, tizanidine• Bladder dysfunction: anticholinergics, timed

voiding, urology referral• Paroxysmal symptoms: cooling,

carbamazepine, gabapentin• Depression: TCAs, SSRIs

NMO (Devic’s disease)• Neuromyelitis optica• Recurrent optic neuritis

and transverse myelitis• Long segment of

demyelination• Peripheral Ab to

aquaporin4• Acute tx: steroids, PLEX• DMTx: rituximab,

azathioprine

STROKE

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70 y/o woman with HTN, DLD, Afib, prev lacunar strokes is brought to the ED for altered mental status. She afeb with HR 80, irreg BP 190/110 RR 16, irreg

and FSG 143. On general exam, her breathing is gurgly and she has an IR/IR HR with a 2/6 SEM at the RUSB. No meningismus. The pt is obtunded and only

briefly opens her eyes. No vocalization. Follows commands intermittently. L pupil smaller than R.

Absent L corneal. R gaze palsy. L facial weakness. R hemiparesis. Both toes are upgoing. CT head shows

“no acute intracranial abnormality.” What is the most likely diagnosis?A. Seizure with post-ictal paralysis

B. MeningoencephalitisC. Toxic ingestionD. Basilar artery occlusion

Basilar Artery Stroke• Symptoms

o 75% have prodrome w/stuttering course, presentation very variable

o Mild generalized weakness to comatoseo The 5 Ds: Dizziness, Diplopia, Dysarthria, Dysphagia,

Dystaxiao Hallmark of post. circ. = crossed findingso Locked-in = Quadriplegia, bilateral facial and

oropharyngeal palsy but preservation of cortical function and vertical gaze

• Treatment:o Respiratory supporto IV TPA w/i 4.5 H, IA interventions even beyond 8 hours, heparin

infusion in some patients after treatmento Angioplasty either acute or elective

• Very high mortality (>90% if locked-in)

Stroke etiology• Cardio-embolism (30-40%)• Athero-embolism (LVD) (30-50%)• Small Vessel Disease (SVD) (20-30%)• Non-atherosclerotic vasculopaties (Rare)• Thrombophila (up to 15%)• Hemodynamic stroke (Watershed infx.) (unkown)• Cryptogenic (unknown cause) (up to 30%)

Frequency does not add up to 100% b/c etiologic frequency varies by series/population)

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Basic Stroke Localization

Acute treatment of stroke

• IV tPAo <4.5 hrs after onseto CT head w/o contrast to r/o ICH absolute

mandatory for your hospital to consider stroke treatment!

o Dose 0.9mg/kg with max 90mg; 10% bolus then rest over 1 hour

o No ASA, NGT, Foley, other procedures x 24 h post-tPA

tPA criteriaInclusion Criteria Exclusion Criteria

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AIS by history and examTime of onset w/i 3/4.5 hoursNormal non-contrasted CT-head (early ischemic signs allowed)Age > 18 years

• Stroke or serious head trauma w/i 3 months

• Lifetime history of ICH• Major surgery w/i 14 days• SBP > 185 or DPB >110 (sustained!) OR

needing aggressive tx to maintain BP in above range

• Rapidly improving or minor Sx• Sx suggestive of SAH• GI or UT-hemorrhage w/i 21 days• Arterial puncture @ non-compressible site

w/i 7days• Seizure at onset of stroke symptoms• Coagulopathy: abnormal PT/PTT,

Plt.<100000• Hypoglycemia (BG<50) or Hyperglycemia

(BG>400)

Blurred gray-white junction

Dense MCA

Diffusion weighted MRI

ADC MAP

Apparent diffusion coefficient

Acute treatment of stroke

• IA tPA: less than 6 hours• Endovascular intervention up to 8 hours• If none of the above:

o Telemetry x 24 hourso Permissive HTN (to 220/120)o Maintain NPO until dysphagia screen passedo ASA 325mg in first 48 hourso PT/OT/Speecho TTE with bubble, vascular imaging

• Change in neuro examthink seizure, cerebral edema, infection, arrhythmia

BP Management in AIS

Post-tPA No tPA

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• Maintain SBP < 180, DPB < 105

• May continue use of Labetolol 10-20 mg, 1-2 times

• If BP control unsatisfactory, use Nicardipine infusion (start @ 5mg/hr)

• A-line not necessary, but preferred

• Do not treat BP unless SBP > 220 or DBP > 120

• Treat BP if patient shows signs of cardiac ischemia AND has clinical features of decompensation (pain, tachycardia, de novo arrhythmia etc.)

• If BP requires tx, monitor carefully for neurological deterioration

Secondary prevention• Risk factors are universal for vascular disease• Manage HTN, DLD, DM2• Antiplatelet agent

o Aspirin 81mg or 325mgo Aspirin + dipyridamoleo Clopidogrel 75mg

• Indications for warfarino Intracardiac thrombuso Artificial heart valveo Non-valvular Afib (lone Afib and secondary Afib may require other

considerations)o Large areas of cardiac hypokinesis

27 y/o WM with h/o migraine developed a R sided HA and R anterolateral cervical pain 4 days after chiropractic manipulation of the

neck. One day after becoming symptomatic, he c/o transient R eye blindness that

resolved within a few hours. Neuro exam demonstrates R miosis, ptosis, and mild R

tongue deviation. The most likely diagnosis is:A. Vertebral artery dissection

B. Basilar artery occlusionC. Complicated migraineD. Carotid artery dissectionE. Cluster headache

Carotid Artery Dissection

Intimal flap

Carotid artery dissection

• Unilateral HA in 2/3• Miosis/ptosis (oculosympathetic dysfunction) in

<50%• Transient monocular blindness in 50-95%• Causes:

o Trauma (50%)o Connective tissue diseaseo Fibromuscular dysplasia

• Dx: CTA, MRA, angiography• Tx: heparin infusioncoumadin, endovascular

stenting

A 71 y/o man presents to your clinic for a routine exam. You notice a L carotid bruit. You order a carotid ultrasound that shows 70% stenosis of the L ICA. All but which

of the following statements are true?

A. If this pt has symptoms of TIA, he will receive significant benefit from a CEA.

B. Without symptoms, his risk of stroke is ~2%/yr.C. Aggressive lipid control and antiplatelets are

equivalent to CEA.D. There is evidence that this pt will benefit from CEA

even in the absence of symptoms.E. Surgery is recommended only in centers with

perioperative complication rates <6%

Carotid artery stenosis• Symptomatic CAS:

o Either CEA or angioplasty + stento Stent if radiation anticipatedo Surgery is best done within 4-6 weeks after stroke

• Asymptomatic CAS. Consider CEA or stent if:o Stenosis >70%o age <80 yearso life expectancy > 5 yearso Center with low (<3%) perioperative complication rate

TIA

• Age ≥ 60 y: 1 pt• BP≥140/90: 1 pt• Clinical features: U/l

weakness (2 pts), speech change (1 pt)

• Duration: 10-59 min (1 pt), 60+ min (2 pts)

• DM: 1 pt

• < 4 low risk• 4-5 moderate risk• > 5 high risk

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ABCD2 score predicts risk at 2, 7, and 90 days after stroke; helps determine urgency of w/u

Neuromuscular

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65 y/o myasthenic woman is admitted with a productive cough. She is given a dose of

levofloxacin for CAP. The next morning, she feels weaker and complains of

shortness of breath worse with standing than with laying flat. She can count to 10

on a single breath and is unable to converse in full sentences. What is the

best next step?A. Increase Mestinon to 60mg q4h.B. Increase prednisone to 60mg qdayC. Increase supplemental O2.D. STAT pulmonary mechanicsE. Start plasma exchange.

Neuromuscular Respiratory

Failure¨ Respiratory failure does not occur until the respiratory muscle strength has fallen to 20-30% of normal.

¨ Signs of impending respiratory failure:

¨ Dysphagia¨ Progressive quadraparesis with inability

to lift head off bed¨ Weak voice¨ Bifacial weakness¨ Wet voice¨ Inability to complete sentences

Neuromuscular Respiratory Failure

• Pulmonary mechanics:oFVC <15mL/kgoNIF <-30 cmH20

• Rapid shallow breathing index• Do not wait for ABG changes or

sat drop!!!!• BiPAP doesn’t compensate for bulbar

dysfunction or evacuation of secretions.

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Myasthenia Gravis• Ab-mediated attack on post-synaptic NMJ • Dx: AchR Ab, MUSK Ab*, EMG, Tensilon test• Tx:

o Crisis—PLEX, IVIg, respiratory supporto Suppression—prednisone (low, slow—may cause

exacerbation), azathioprine, mycophenolateo Symptomatic—pyridostigmine (Mestinon)o Consider thymectomy if young, AchR Ab+

• Avoid FQ, Li, aminoglycosides, Mg, CCBs, and macrolides

25 y/o WM with no PMHx c/o R shoulder pain and weakness x 4 months. It began with sudden, severe, sharp pain in his shoulder and between his shoulder blades. There was no preceding trauma. On physical examination, he was unable to abduct his arm. On the right side, he had 4/5 muscle strength in the scapula-girdle musculature, 3/5 muscle strength in the infraspinatus and supraspinatus muscles, and normal strength in the elbow and hand. What is the most likely diagnosis?A. Parsonage-Turner Syndrome

(Neuralgic Amyotrophy)B. Erb palsyC. Fascioscapulo humeral

dystrophyD. ALSE. Thoracic outlet syndrome

Neuralgic amyotrophy• Brachial neuritis/plexitis (Parsonage-Turner)

o Postinfectious, post-vaccination, idiopathico Severe pain followed by subacute, progressive

weakness/atrophyo Tx: analgesics, PT/OT.

• Diabetic lumbosacral radiculopathy (Bruns-Garland)o Subacute, progressiveo Unilateral, pain in thigh, buttocks, low back with LE

weakness, sensory loss, and ↓reflexes without bladder/bowel involvement

o Assoc w/rapid wt loss or change in glycemic controlo MRI L-spine normal. EMG peripheral dysfunction. CSF

↑protein.o Tx: PT. Usually good recovery in months.

56-y/o WM c/o 1-month h/o intermittent weakness of the left foot, a 6-month h/o progressive R UE weakness, and 1-year h/o muscle cramps. He says he feels lately as if he is “catching” his foot on things when ambulating. Denies dyspnea, dysphagia, pain, bowel or bladder impairment. Neuro exam reveals normal speech, language, and mental status. His tongue appears atrophic with fasciculations. He has diffuse weakness and atrophy of the proximal muscles in the R UE; fascics are noted. L UE strength and bulk are normal. Mod weakness of the distal muscles in the L LE is noted, with fascics present in both LE. DTRs are brisk in the UE and LE, and toes are upgoing. Sensory exam is normal. Labs and imaging are normal. What diagnosis is most likely?

A. Amyotrophic lateral sclerosisB. Cervical myelopathyC. Chronic inflammatory demyelinating

polyradiculoneuropathyD. Primary lateral sclerosis

Motor neuron disease

• ALSo Both UMN and LMNo Multisegmental (bulbar + at least 2 spinal regions)o Cognition, sphincter function, sensory sparedo Likely to need trach/PEGo 50% die in 3 yearso Riluzole is the only disease modifying therapy.

Signs DDx

UMN HyperreflexiaSpasticityWeakness

ALSPLSMyelopathySpastic paraparesisB12 deficiency

LMN WeaknessAtrophyFasciculationsDecreased tone

Spinal muscular atrophyHeavy metal exposureKennedy disease

Cervical myelopathy• Symptoms:

o Intermittent neck/shoulder pain or stiffnesso May have pain, paresthesia, weakness in radicular

patterno “Numb, clumsy hands”o Intrinsic hand muscle atrophyo Spasticity, stiffness in LE. Gait change.o Loss of vib/proprioception +/- sensory levelo Hyperreflexic. +Babinski –jaw jerko Emergency if bowel/bladder change

• DDx: B12 myelopathy, Cu-deficiency, NO-toxicity, dural AV-fistula, spinal compression, HIV myelopathy, ALS

• MRI c-spine is best study• Tx: surgery, treat pain

Myopathies

¨ Critical illness Difficult to wean from vent Diffuse, flaccid weakness involving the limb, neck, diaphragm Avoid paralytics

¨ Steroid induced Normal CK, EMG Dx with muscle biopsy—type 2b atrophy Tx: PT, stop steroids

¨ Endocrine Proximal weakness, normal CK hyperthyroidism, hypothyroidism, hyperparathyroidism, or vitamin D

deficiency.

¨ Toxic—statins, AZT, colchicine, hydroxychloroquine

Inflammatory Myopathies

¨ Inclusion body myositis >50yo. Quads/hand & feet flexors. Dysphagia Mild ↑ CK mild Ubiquitin+ inclusion bodies, denervation

¨ Polymyositis May overlap with connective tissue disease ↑CK, antiJo-1 Ab Endomysial inflammation. +Necrosis

¨ Dermatomyositis• Older age = higher risk for underlying cancer• Painful proximal muscle weakness• Perivascular inflammation. No necrosis.• ↑CK; antiMi-2 Ab

Myotonic dystrophy¨ AD chr 19; CTG repeats¨ Difficulty releasing grip¨ Hand cramps¨ Hatchet facies

Frontal balding Bilat ptosis Drooping mouth

¨ Prog muscle weakness¨ Gynecomastia¨ Cataracts¨ Cardiac conduction

defects¨ Diabetes

Peripheral neuropathies

Classification Signs and Symptoms Major Diagnostic Considerations

Autonomic neuropathy

Orthostatic hypotensionConstipationEarly satietyErectile dysfunction

AmyloidosisDiabetes mellitusParaneoplastic syndrome

Small-fiber neuropathy

Burning extremity pain without weaknessNormal reflexes

Diabetes mellitusImpaired glucose tolerance

Acquired sensorimotor neuropathy

Distal sensory loss and ↓ reflexesDistal extremity weakness

Diabetes mellitusMonoclonal gammopathyToxic (chemotherapy)

Polyradiculoneuropathy

Severe weaknessSensory lossAreflexia

GBSCIDPAmyloidosis

Motor neuropathy Asymmetric weaknessMuscle atrophy

Multifocal motor neuropathyMotoneuron disease

Guillain-Barre Syndrome

• Ascending paralysis is classic• Evolves over days• May progress to resp failure, autonomic instability

or involve CN• CSF: ↑protein, normal cell count• EMG/NCS: Slowed conduction/block• Tx: Supportive, plasmapheresis, IVIg (NO

STEROIDS)• Good px: Young, mild disease, not hyperacute,

improvement within 1 week

MOVEMENT DISORDERS

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A 54 y/o man with Parkinson disease is brought by his wife, who is concerned about her husband’s recent excessive gambling. She says that in the past 6 mos, he has been spending increasing amounts of time at a casino, where he rarely enjoyed going before the diagnosis of Parkinson disease. His behavior is otherwise unchanged. The patient has been taking ropinirole since the diagnosis and has had a marked diminution in tremor as a result; he has had no difficulties with or change in mood, cognition, or sleep. Neurologic examination shows mild left UE rigidity and a minimal resting tremor. Which of the following is the most likely cause of this patient’s gambling problem?

A. Bipolar disorderB. Frontotemporal dementiaC. Medication-related compulsive behaviorD. Parkinson-related dementia

PD Medications• Dopamine agonists

o ropinirole, pramipexoleo Initial tx for early diseaseo AE: compulsive behavior, orthostatic hypotension, nausea, vomiting,

hallucinations, sleep attacks, pedal edema

• Levodopa-carbidopao Gold standardo Long-term use assoc with tolerance and dyskinesias, difficult to

distinguish from disease progressiono AE: nausea, orthostatic hypotension, hallucinationso **Watch out for NMS-like symptoms in pts who are NPO**

PD Medications• COMT inhibitors

o Entacapone, tolclaponeo Delay metabolism of Sinemet; give with Sinemeto AE: nausea, diarrhea, hepatotoxicity

• MAO-B inhibitorso Selegiline, rasagilineo AE: N/V, hallucinations, psychosis

• Amantadine, anticholinergicso Mostly for tremor, very early diseaseo AE: psychosis, cognitive declineo Stop 1st if pt has altered mental status

Parkinson disease• Tremor

o Restingo “pill-rolling”o asymmetric

• Rigidityo Cog-wheel, lead pipeo freezing

• Akinsesia/Bradykinesiao Festinationo Masked facieso Decreased blinking

• Postural Instabilityo Stooped postureo En bloc turnso Falls (often backwards)

• Non-motor symptoms: constipation, depression, poor sleep, autonomic instability, drooling, dementia (late)

CC: worsening gait unsteadiness and falls65 yo M with unsteadiness x 1 yr. Also has constipation, erectile dysfunction x 3 yrs.On exam: supine BP 190/105 , standing 76/50; no change in pulse rate. Normal mental status. CN afocal. Power 5/5. Mild rigidity of the extremities and mild appendicular ataxia. Slow gait with ↓arm swing. Marked postural instability.

Which of the following is the most likely diagnosis?

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A) Dementia with Lewy bodiesB) Multiple system atrophyC) Parkinson diseaseD) Progressive supranuclear palsy

Other diseases producing

Parkinsonism• Drug induced Parkinsonism

o Usually bilateral at presentationo Exposure to antipsycotics, dopamine-blockade

• Progressive Supranuclear Palsyo Early falls (backward), symmetric bradykinesia, axial

rigidity, vertical gaze impairment (can’t look up)o Lack of tremor or dopa-response

• Lewy Body Dementiao Parkinsonism, dementia, hallucinations, autonomic

instabilityo Worse with dopamine; sensitive to meds

• Multiple Systems Atrophyo Ataxia, dysautonomia

DEMENTIA

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Dementia• Must involve impairment in multiple

domains: memory, language, spatial skills, judgment, and problem solving

• Decline in cognitive function with preserved ADLs = mild cognitive impairmento 10-15%/yr progression to dementia

• Work-up of dementia includes:o CBC, TSH, B12, CMPo CT head or MRI braino Screening for depressiono Optional: RPR, HIV, LP

Alzheimer Dementia• By far most common type, Tau-opathy• 50% prevalence by age 90• Short term> long-term memory impairment• 1st language problem = anomia• Behavior impaired in late disease

(agitation/aggression)• Usually no focal signs or gait impairment• Tx:

o Acetycholinesterase inhibitors (donepezil, galantamine)

o NMDA receptor antagonists (memantine)—late disease only

Less common dementias

• Vascularo Step-wise deteriorationo Should have focal neuro deficits, but sometimes

difficult to determine due to multi-focalityo Will see multiple infarcts (typically) on MRI

• Frontotemporal lobar (Pick’s)o Insidious, onset <65 yrso Behavior change: hypersexuality, disinhibition,

irritability, poor attention, perseverationo Nonfluent aphasiao Can Dx by PET

Less common dementias

• Diffuse Lewy bodyo Fluctuating cognitiono Parkisonism, hallucinations, autonomic dysfunction

• Creutzfeldt-Jakob Diseaseo Rapidly progressive dementia with myoclonuso ↑ CSF 14-3-3

• Normal pressure hydrocephaluso Wet, wacky, wobblyo MRI brain then CSF drainageo Tx: low pressure VPS

• May improve gait and incontinence• Symptoms <1 yr in duration

Wernicke Encephalopathy

• Encephalopathy, eye movement abnormalities, ataxia

• Inadequate intake or absorption of thiamine

• At risk: alcoholics, malnourished patients (any reason), chronic gastritis, repetitive vomiting

• Always give thiamine before glucose!!• Prognosis depends on severity.

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67 y/o BM with HTN, DM2, h/o migraines was watching TV with his wife when he suddenly seemed very anxious

and kept repeating, “Who won the Super Bowl?” No other neuro symptoms and no headache. Upon arrival at the ED, his vitals and FSG were normal. He is anxiously

repeating the same question but is not confused. He cannot remember events for the past several weeks but he recognizes his family members and gives a detailed medical and surgical history. His neuro exam is afocal. CT head is normal. What is the most likely diagnosis?

A. TIAB. Nonconvulsive statusC. Transient global amnesiaD. Malingering

Transient global amnesia

• Age >50• Paroxysmal, transient loss of memory• Immediate recall and long-term memory spared• Pts usually anxious, repeat same question• Mental status and neuro exam normal, although

temporal “UBO’s” have been described• MRI brain and EEG usually normal• Spontaneously resolves in <24 hours

HEADACHE

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A 36-year-old woman is evaluated in the office for a 6-year history of headache. The patient says her headaches occur approximately three times each month, are associated with occasional nausea and pain that can be moderately severe and disabling, have a squeezing quality, and begin in the neck, radiating forward to involve the frontal head region bilaterally. Her headaches are preceded by yawning and irritability, last up to 24 hours, and can be triggered by stress or changes in barometric pressure. She has a history of mild depression treated with fluoxetine. Her mother and sister have a history of sinus headaches.

Results of general physical and neurologic examinations are normal. The patient’s mood appears euthymic. Which of the following is the most likely diagnosis?

A. Chronic migraineB. Medication overuse headacheC. Migraine without auraD. Sinus headacheE. Tension-type headache

Migraine Diagnosis• ~90% of pts w/CC of mod-sev recurrent HA

have migraine• At least 2 of the following:

o Unilateral, not side locked, pulsating, worsens with routine physical activity

• At least 1 of the following:o N/V, photophobia, phonophobia

• Classic (~20%): with aura• Common: no aura• Variants: hemiplegic, basilar, acephalgic

Migraine treatment• Abortive

o NSAIDS, caffeine containing analgesicso Triptanso Antiemetics + ergots

• Prophylactic—determined by freq of HA and degree of interference with daily activitieso Propranolol, verapamilo Topiramate, valproic acido SSRIs, amitriptyline

• Sleep hygiene and avoidance of triggers• In pregnancy: Tylenol, Reglan, Fioricet• Can use triptans in pts who are breast-feeding

42 yo F c/o occasional episodes of severe vertigo with nausea, vomiting, tinnitus, and a feeling of ear fullness. Her first episode occurred 3 years ago, and since then, she has had approximately six episodes, each of which may last from a few hours to 1 or 2 days. Meclizine and diazepam taken at the onset of symptoms provide partial relief, but she often must resort to bed rest during these episodes, missing 1 to 2 days of work. She has a family history of migraine headache, although the patient does not experience headache or visual symptoms with her episodes of dizziness.

Physical examination, including vital signs, is normal. An audiogram discloses a bilateral low-frequency sensorineural hearing loss. MRI of the head is normal.Which of the following is the most likely diagnosis in this patient?

A. Acephalgic migraineB. Ménière’s diseaseC. Acoustic neuromaD. Benign positional vertigoE. Vestibular neuritis

Ménière’s disease

• Recurrent, disabling vertigo• Usually 4th to 6th decades• Attacks last hours• N/V + cochlear symptoms (tinnitus, ear

fullness, and/or hearing loss)• Tx:

o Acute—meclizine, benzodiazepines, antiemeticso Prophylactic—diuretics, low-Na diet

Giant Cell Arteritis• Suspect if new onset HA after age 50• Sx: HA, scalp tenderness, painless sudden

blindness, jaw claudication, arthalgias (PMR), weight loss, fatigue

• ESR >100, low Hct, high plt, high CRP• Start prednisone 60mg qd when suspected• Dx with temporal artery bx• Complications: blindness, stroke, MI, aortic

dissection

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Benign positional vertigo

• Most common cause of dizziness• Sudden, brief episodes of mod-intense vertigo• Nausea +/- vomiting, gait instability• Triggered by change in head position• Dix-Hallpike+

o upbeating torsional nystagmus with fast component that rotates toward affected ear

o fatigable

• Tx: particle repositioning maneuvers, antihistamines, anticholinergics

Causes of VertigoBPPV Labyrinthitis Meniere’s CP angle

tumourBrain stem

Character of vertigo

Paroxysmal Gradual onset over hours

Paroxysmal, spontaneous

Episodic or persistent, insidious

Often constant

Course Frequent attacks then recovery

Constant then recovery

Severe attacks with remission

Progressive Usually improves with time

Duration 10-20 sec.(< 1 min)

Days, dynamic symptoms may persist weeks

Hours to days Persistent Can be persistent

Positional Yes, turning head to one side

No, may be exacerbated by movement

No, may be exacerbated by movement

No, may be exacerbated by movement

No, may be exacerbated by movement

Hearing loss No May or may not

Yes Yes Usually no

58 y/o WM with HTN, DM2 complains of double vision and pain in the right eye. He has a mild constant HA on the R side of his head. On exam, he has marked ptosis on the R. His eye is deviated down and out and the double vision is worse when he attempts to look upward and to the left. His pupils are equal in size and reacted equally to light. All other eye movements are normal. Corneal reflex is brisk; facial sensation is symmetric. VF full; VA 20/20 OU. What do you recommend?

A. MRA head/neckB. Cerebral angiogramC. STAT ophtho c/sD. Temporal artery biopsyE. Observation only

CN 3 palsy• Look at the pupil!

o Pupil sparing is usually ischemic and likely to be benign.o If pupil involved, aneurysm until proven otherwise.

• Examine CN IV, VI, V to r/o cavernous sinus involvement.

• Risk factors: age>50, DM, HTN, giant cell arteritis

• Diabetic 3rd nerve palsy:o Painful, ptosis, EOM weakness, pupil sparedo Full recovery by 3 months

CN 3 palsy• Just remember that any isolated 3rd

nerve palsy, partial or not, deserves a w/u

• Always consider the worst case scenario: aneuryms

• Less likely with pupil sparing partial 3rd, but if uncertain, perform w/u to r/u aneurysm

• Don’t let anybody tell you otherwise!

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EPILEPSY

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Seizures• Partial—originates from a focus

o Simpleretained consciousnesso Complexaltered consciousness

• Generalized: GTC, absence, atonic, etc• Symptomatic

o Hyper- or hypo- glycemiao Drug withdrawal—EtOH or benzoso Uremiao Meningitiso Tumor

• Risks: head injury w/LOC, h/o meningitis/encephalitis, febrile sz, family hx

Single Seizure• Was it a seizure?• Look for provoking factor

o UDS, CBC, Chem8, ?LP• Don’t usually start AED• Risk for recurrence:

o partial seizure, Todd paralysis, a family history of epilepsy, age greater than 65 years, and abnormal findings on neurologic examination

• MRI brain with and without• EEG

o Interictal EEG abnormal in 25-50% with epilepsyo Sensitivity ~90% with 4 EEGs

65 yo F comes to the office for her annual examination. She reports that she had a tonic-clonic seizure at age 24 years after the birth of her daughter but has been seizure-free on phenytoin since that time. The patient also has osteoporosis, diagnosed after a screening bone density scan. Current medications include phenytoin, alendronate, calcium, and vitamin D.

Physical examination findings are normal. Which of the following is the most appropriate next step in management?

A. Check the serum phenytoin levelB. Continue the current dosage of phenytoinC. Discontinue the phenytoin in a tapered fashionD. Substitute lamotrigine for the phenytoin

Epilepsy• 2 or more unprovoked seizures• Start with monotherapy. Push to toxicity before

adding 2nd AED • Trial of AED withdrawal if sz-free x 2 yrs• Driving laws vary by state.• Special considerations:

o Avoid valproate in child-bearing age ♀o Monotherapy in pregnancy (not necessary to change

to Lamictal)o Valproate for JME or myoclonuso Levetiracetam not metabolized by livero Ethosuximide for absence ONLYo Elderly, partial seizure: gabapentin, lamotrigine

Status Epilepticus• Incidence 60/100 000 (varies)• About 1% of patients with epilepsy will go

into status per year• Mortality across the board is 2-35%*• Factors associated with poor prognosis:

o Advanced ageo Prolonged (>60 min)o Hyperthermia o Associated hypoxia or stroke at onseto Other concurrent severe illness

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Status Epilepticus

Convulsive SE Nonconvulsive SE

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• Generalized seizure activity

• Subtle SE: dissociation of EEG activity and motor activity

• Mortality of > 30% (in some series)

• “Mental status change”

• Behavioral changes• Much lower morbidity

and mortality• Difficult to recognize

Status Epilepticus Tx• ABC’s

• Treat the seizures aggressively without worrying about causes initially

• While specific treatment for SE started, begin w/u for possible causes if no history available or no obvious cause apparent to you

• CT, LP and other labs must not delay treatment!

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Status Epilepticus Tx• First line = benzodiazepines

o 0.1 to 0.2 mg/kg (for LZP), but mostly administered as bolus of 2 to 6 mg IVP, up to 10 mg total

o DZP mostly used “on truck” IMo Midazolam not useful IVP, but as an infusion

• Second lineo Fosphenytoin 20 PE/kg @ up to 150 mg/min.

• Brady-arrhythmia, more profound in older patients• Goal level 15 to 25, check after 1 hour

o Phenytoin 20 mg/kg @ up to 100mg/hour (recommend 50)o Phenobarbital 20 mg/kg over 15 minutes**

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Status Epilepticus Tx• Any other AED (Depakote, Keppra, Vimpat)

administered in SE is “off label” use, which may be fine, but should be seen as adjunctive medication

• Your main goal is to stop SE w/i the hour of it’s onset, bad stuff starts to happen if permitted to persist longer

• Co-morbidities are the main determinant of Mortality/Morbidity related to SE

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NEURO-ONCOLOGY

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CNS Tumors• 50% tumors are primary:

o Astrocytoma—50%of primary• Low grade (WHO 1 and 2)

o Slow growing, present with seizureo Resection may be curative with certain subtype

• High grade (WHO 3 and 4)o Irregular enhancement, sig surrounding edemao Surgery, radiation, chemo (temozolamide)

o Meningioma• Benign, slow-growing, dural based• Surgery or sterotactic radiation

o Pituitary adenomao Schwannoma

• MRI brain with and without contrast is best study

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Primary CNS Lymphoma

• Rare; <4% of CNS tumors• B-cell NHL• Present with neuro deficits, seizures• Single homogeneously enhancing deep lesion• “Vanish” with steroids• Check toxo serology, CSF EBV and flow cytometry• Tx: IT MTX and WBRT

Brain Mets• More common than primary CNS tumors

• Lots of Bad Stuff Kills Glia• Lung• Breast• Skin (melanoma)• Kidney (renal cell)• GI

• 70-80% are multiple• Occur anywhere, but favor gray-white junction

early• Tx: steroids, surgery if single/few, whole brain

radiation if multiple

Mets from Renal Cell Cancer

CT MRI T1 with Gd

Paraneoplastic syndromesSyndrome Antibodies Associated tumor

Myasthenia gravis Nicotinic Ach ReceptorStriated muscle

Thymoma

Lambert-Eaton P/Q voltage-gated Ca channel

SCLC

Cerebellar degeneration HuYoother

SCLC, neuroblastomaSCLC, ovary, breastHodgkin lymphoma

Brainstem encephalitis Ma-1Ma-3

SCLCtesticle

Limbic encephalitis Voltage-gated K channelNMDA receptor

SCLCOvary

Neuropathy Mag

Hu

Waldenstrom macroglobulinemiaSCLC, prostate

Stiff-man syndrome Amphiphysin SCLC, breast

Opsoclonus/myoclonus Ri SCLC, breast, neuroblastoma