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NEUROLOGY Internal Medicine Board Review Brad Ingram, MD.

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1 NEUROLOGY Internal Medicine Board Review Brad Ingram, MD

2 Neurology Dementia Headache Epilepsy Neuro-oncology 2 Demyelinating diseases Stroke Neuromuscular Movement Disorders

3 Demyelinating Diseases 3

4 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, OCBs B. Start her on copaxone C. MRI c-spine D. C/s ophthalmology E. Start Solumedrol 1 gram qday

5 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 days o Prevent Relapse 1 st line: IFN b-1a, IFN b-1b, glatiramer acetate 2 nd line: mitoxantrone (cardiotoxicity, leukemia); natalizumab (PML); fingolimod (bradycardia, macular edema)

6 Symptomatic Treatment Fatigue o R/o medical causes: TSH, B12, CBC o Address sleep hygiene o Exercise and cooling measures o Amantadine, modafinil Spasticity : PT, baclofen, tizanidine Bladder dysfunction : anticholinergics, timed voiding, urology referral Paroxysmal symptoms : cooling, carbamazepine, gabapentin Depression : TCAs, SSRIs

7 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

8 STROKE 8

9 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. Meningoencephalitis C. Toxic ingestion D. Basilar artery occlusion

10 Basilar Artery Stroke Symptoms o 75% have prodrome w/stuttering course, presentation very variable o Mild generalized weakness to comatose o The 5 Ds: Dizziness, Diplopia, Dysarthria, Dysphagia, Dystaxia o Hallmark of post. circ. = crossed findings o Locked-in = Quadriplegia, bilateral facial and oropharyngeal palsy but preservation of cortical function and vertical gaze Treatment: o Respiratory support o IV TPA w/i 4.5 H, IA interventions even beyond 8 hours, heparin infusion in some patients after treatment o Angioplasty either acute or elective Very high mortality (>90% if locked-in)

11 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) 11

12 Basic Stroke Localization

13 Acute treatment of stroke IV tPA o <4.5 hrs after onset o 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

14 tPA criteria Inclusion CriteriaExclusion Criteria 14 AIS by history and exam Time of onset w/i 3/4.5 hours Normal 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 non-compressible site w/i 7days Seizure at onset of stroke symptoms Coagulopathy: abnormal PT/PTT, Plt.< Hypoglycemia (BG 400)

15 Blurred gray-white junction Dense MCA

16 Diffusion weighted MRI ADC MAP Apparent diffusion coefficient

17 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 hours o Permissive HTN (to 220/120) o Maintain NPO until dysphagia screen passed o ASA 325mg in first 48 hours o PT/OT/Speech o TTE with bubble, vascular imaging Change in neuro exam  think seizure, cerebral edema, infection, arrhythmia

18 BP Management in AIS Post-tPANo tPA 18 Maintain SBP < 180, DPB < 105 May continue use of Labetolol mg, 1-2 times If BP control unsatisfactory, use Nicardipine infusion 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

19 Secondary prevention Risk factors are universal for vascular disease Manage HTN, DLD, DM2 Antiplatelet agent o Aspirin 81mg or 325mg o Aspirin + dipyridamole o Clopidogrel 75mg Indications for warfarin o Intracardiac thrombus o Artificial heart valve o Non-valvular Afib (lone Afib and secondary Afib may require other considerations) o Large areas of cardiac hypokinesis

20 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 occlusion C. Complicated migraine D. Carotid artery dissection E. Cluster headache

21 Carotid Artery Dissection Intimal flap

22 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 disease o Fibromuscular dysplasia Dx: CTA, MRA, angiography Tx: heparin infusion  coumadin, endovascular stenting

23 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%

24 Carotid artery stenosis Symptomatic CAS: o Either CEA or angioplasty + stent o Stent if radiation anticipated o Surgery is best done within 4-6 weeks after stroke Asymptomatic CAS. Consider CEA or stent if: o Stenosis >70% o age <80 years o life expectancy > 5 years o Center with low (<3%) perioperative complication rate

25 TIA Age ≥ 60 y: 1 pt BP≥140/90: 1 pt Clinical features: U/l weakness (2 pts), speech change (1 pt) Duration: min (1 pt), 60+ min (2 pts) DM: 1 pt < 4 low risk 4-5 moderate risk > 5 high risk 25 ABCD 2 score predicts risk at 2, 7, and 90 days after stroke; helps determine urgency of w/u

26 Neuromuscular 26

27 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 qday C. Increase supplemental O2. D. STAT pulmonary mechanics E. Start plasma exchange.

28 Neuromuscular Respiratory Failure ¨ Respiratory failure does not occur until the respiratory muscle strength has fallen to % 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

29

30 Neuromuscular Respiratory Failure Pulmonary mechanics: o FVC <15mL/kg o NIF <-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. 30

31 Myasthenia Gravis Ab-mediated attack on post-synaptic NMJ Dx: AchR Ab, MUSK Ab*, EMG, Tensilon test Tx: o Crisis—PLEX, IVIg, respiratory support o Suppression—prednisone (low, slow—may cause exacerbation), azathioprine, mycophenolate o Symptomatic—pyridostigmine (Mestinon) o Consider thymectomy if young, AchR Ab+ Avoid FQ, Li, aminoglycosides, Mg, CCBs, and macrolides

32 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 palsy C. Fascioscapulo humeral dystrophy D. ALS E. Thoracic outlet syndrome

33 Neuralgic amyotrophy Brachial neuritis/plexitis (Parsonage-Turner) o Postinfectious, post-vaccination, idiopathic o Severe pain followed by subacute, progressive weakness/atrophy o Tx: analgesics, PT/OT. Diabetic lumbosacral radiculopathy (Bruns-Garland) o Subacute, progressive o 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 control o MRI L-spine normal. EMG peripheral dysfunction. CSF ↑ protein. o Tx: PT. Usually good recovery in months.

34 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 sclerosis B. Cervical myelopathy C. Chronic inflammatory demyelinating polyradiculoneuropathy D. Primary lateral sclerosis

35 Motor neuron disease ALS o Both UMN and LMN o Multisegmental (bulbar + at least 2 spinal regions) o Cognition, sphincter function, sensory spared o Likely to need trach/PEG o 50% die in 3 years o Riluzole is the only disease modifying therapy. SignsDDx UMNHyperreflexia Spasticity Weakness ALS PLS Myelopathy Spastic paraparesis B12 deficiency LMNWeakness Atrophy Fasciculations Decreased tone Spinal muscular atrophy Heavy metal exposure Kennedy disease

36 Cervical myelopathy Symptoms: o Intermittent neck/shoulder pain or stiffness o May have pain, paresthesia, weakness in radicular pattern o “Numb, clumsy hands” o Intrinsic hand muscle atrophy o Spasticity, stiffness in LE. Gait change. o Loss of vib/proprioception +/- sensory level o Hyperreflexic. +Babinski –jaw jerk o 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

37 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

38 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

39 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

40 Peripheral neuropathies ClassificationSigns and SymptomsMajor Diagnostic Considerations Autonomic neuropathyOrthostatic hypotension Constipation Early satiety Erectile dysfunction Amyloidosis Diabetes mellitus Paraneoplastic syndrome Small-fiber neuropathyBurning extremity pain without weakness Normal reflexes Diabetes mellitus Impaired glucose tolerance Acquired sensorimotor neuropathy Distal sensory loss and ↓ reflexes Distal extremity weakness Diabetes mellitus Monoclonal gammopathy Toxic (chemotherapy) PolyradiculoneuropathySevere weakness Sensory loss Areflexia GBS CIDP Amyloidosis Motor neuropathyAsymmetric weakness Muscle atrophy Multifocal motor neuropathy Motoneuron disease

41 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

42 MOVEMENT DISORDERS 42

43 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 disorder B. Frontotemporal dementia C. Medication-related compulsive behavior D. Parkinson-related dementia

44 PD Medications Dopamine agonists o ropinirole, pramipexole o Initial tx for early disease o AE: compulsive behavior, orthostatic hypotension, nausea, vomiting, hallucinations, sleep attacks, pedal edema Levodopa-carbidopa o Gold standard o Long-term use assoc with tolerance and dyskinesias, difficult to distinguish from disease progression o AE: nausea, orthostatic hypotension, hallucinations o **Watch out for NMS-like symptoms in pts who are NPO**

45 PD Medications COMT inhibitors o Entacapone, tolclapone o Delay metabolism of Sinemet; give with Sinemet o AE: nausea, diarrhea, hepatotoxicity MAO-B inhibitors o Selegiline, rasagiline o AE: N/V, hallucinations, psychosis Amantadine, anticholinergics o Mostly for tremor, very early disease o AE: psychosis, cognitive decline o Stop 1 st if pt has altered mental status

46 Parkinson disease T remor o Resting o “pill-rolling” o asymmetric R igidity o Cog-wheel, lead pipe o freezing A kinsesia/Bradykinesia o Festination o Masked facies o Decreased blinking P ostural Instability o Stooped posture o En bloc turns o Falls (often backwards) Non-motor symptoms: constipation, depression, poor sleep, autonomic instability, drooling, dementia (late)

47 CC: worsening gait unsteadiness and falls 65 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? 47 A) Dementia with Lewy bodies B) Multiple system atrophy C) Parkinson disease D) Progressive supranuclear palsy

48 Other diseases producing Parkinsonism Drug induced Parkinsonism o Usually bilateral at presentation o Exposure to antipsycotics, dopamine-blockade Progressive Supranuclear Palsy o Early falls (backward), symmetric bradykinesia, axial rigidity, vertical gaze impairment (can’t look up) o Lack of tremor or dopa-response Lewy Body Dementia o Parkinsonism, dementia, hallucinations, autonomic instability o Worse with dopamine; sensitive to meds Multiple Systems Atrophy o Ataxia, dysautonomia

49 DEMENTIA 49

50 Dementia Must involve impairment in multiple domains: memory, language, spatial skills, judgment, and problem solving Decline in cognitive function with preserved ADLs = mild cognitive impairment o 10-15%/yr progression to dementia Work-up of dementia includes: o CBC, TSH, B12, CMP o CT head or MRI brain o Screening for depression o Optional: RPR, HIV, LP

51 Alzheimer Dementia By far most common type, Tau-opathy 50% prevalence by age 90 Short term> long-term memory impairment 1 st 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

52 Less common dementias Vascular o Step-wise deterioration o Should have focal neuro deficits, but sometimes difficult to determine due to multi-focality o Will see multiple infarcts (typically) on MRI Frontotemporal lobar (Pick’s) o Insidious, onset <65 yrs o Behavior change: hypersexuality, disinhibition, irritability, poor attention, perseveration o Nonfluent aphasia o Can Dx by PET

53 Less common dementias Diffuse Lewy body o Fluctuating cognition o Parkisonism, hallucinations, autonomic dysfunction Creutzfeldt-Jakob Disease o Rapidly progressive dementia with myoclonus o ↑ CSF Normal pressure hydrocephalus o Wet, wacky, wobbly o MRI brain then CSF drainage o Tx: low pressure VPS May improve gait and incontinence Symptoms <1 yr in duration

54 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. 54

55 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. TIA B. Nonconvulsive status C. Transient global amnesia D. Malingering

56 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

57 HEADACHE 57

58 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 migraine B. Medication overuse headache C. Migraine without aura D. Sinus headache E. Tension-type headache

59 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

60 Migraine treatment Abortive o NSAIDS, caffeine containing analgesics o Triptans o Antiemetics + ergots Prophylactic—determined by freq of HA and degree of interference with daily activities o Propranolol, verapamil o Topiramate, valproic acid o SSRIs, amitriptyline Sleep hygiene and avoidance of triggers In pregnancy: Tylenol, Reglan, Fioricet Can use triptans in pts who are breast-feeding

61 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 migraine B. Ménière’s disease C. Acoustic neuroma D. Benign positional vertigo E. Vestibular neuritis

62 Ménière’s disease Recurrent, disabling vertigo Usually 4 th to 6 th decades Attacks last hours N/V + cochlear symptoms (tinnitus, ear fullness, and/or hearing loss) Tx: o Acute—meclizine, benzodiazepines, antiemetics o Prophylactic—diuretics, low-Na diet

63 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 63

64 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

65 Causes of Vertigo BPPVLabyrinthitisMeniere’sCP angle tumour Brain stem Character of vertigo ParoxysmalGradual onset over hours Paroxysmal, spontaneous Episodic or persistent, insidious Often constant CourseFrequent attacks then recovery Constant then recovery Severe attacks with remission ProgressiveUsually improves with time Duration10-20 sec. (< 1 min) Days, dynamic symptoms may persist weeks Hours to daysPersistentCan be persistent PositionalYes, turning head to one side No, may be exacerbated by movement Hearing lossNoMay or may not Yes Usually no

66 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/neck B. Cerebral angiogram C. STAT ophtho c/s D. Temporal artery biopsy E. Observation only

67 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 3 rd nerve palsy: o Painful, ptosis, EOM weakness, pupil spared o Full recovery by 3 months

68 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! 68

69 EPILEPSY 69

70 Seizures Partial—originates from a focus o Simple  retained consciousness o Complex  altered consciousness Generalized: GTC, absence, atonic, etc Symptomatic o Hyper- or hypo- glycemia o Drug withdrawal—EtOH or benzos o Uremia o Meningitis o Tumor Risks: head injury w/LOC, h/o meningitis/encephalitis, febrile sz, family hx

71 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 epilepsy o Sensitivity ~90% with 4 EEGs

72 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 level B. Continue the current dosage of phenytoin C. Discontinue the phenytoin in a tapered fashion D. Substitute lamotrigine for the phenytoin

73 Epilepsy 2 or more unprovoked seizures Start with monotherapy. Push to toxicity before adding 2 nd 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 myoclonus o Levetiracetam not metabolized by liver o Ethosuximide for absence ONLY o Elderly, partial seizure: gabapentin, lamotrigine

74 Status Epilepticus Incidence 60/ (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 age o Prolonged (>60 min) o Hyperthermia o Associated hypoxia or stroke at onset o Other concurrent severe illness 74

75 Status Epilepticus Convulsive SENonconvulsive SE 75 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

76 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! 76

77 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” IM o Midazolam not useful IVP, but as an infusion Second line o Fosphenytoin 20 up to 150 mg/min. Brady-arrhythmia, more profound in older patients Goal level 15 to 25, check after 1 hour o Phenytoin 20 up to 100mg/hour (recommend 50) o Phenobarbital 20 mg/kg over 15 minutes** 77

78 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 78

79 NEURO-ONCOLOGY 79

80 CNS Tumors 50% tumors are primary: o Astrocytoma—50%of primary Low grade (WHO 1 and 2) o Slow growing, present with seizure o Resection may be curative with certain subtype High grade (WHO 3 and 4) o Irregular enhancement, sig surrounding edema o Surgery, radiation, chemo (temozolamide) o Meningioma Benign, slow-growing, dural based Surgery or sterotactic radiation o Pituitary adenoma o Schwannoma MRI brain with and without contrast is best study

81 81

82 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

83 Brain Mets More common than primary CNS tumors L ots of B ad S tuff K ills G lia 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

84 Mets from Renal Cell Cancer CTMRI T1 with Gd

85 Paraneoplastic syndromes SyndromeAntibodiesAssociated tumor Myasthenia gravisNicotinic Ach Receptor Striated muscle Thymoma Lambert-EatonP/Q voltage-gated Ca channelSCLC Cerebellar degenerationHu Yo other SCLC, neuroblastoma SCLC, ovary, breast Hodgkin lymphoma Brainstem encephalitisMa-1 Ma-3 SCLC testicle Limbic encephalitisVoltage-gated K channel NMDA receptor SCLC Ovary NeuropathyMag Hu Waldenstrom macroglobulinemia SCLC, prostate Stiff-man syndromeAmphiphysinSCLC, breast Opsoclonus/myoclonusRiSCLC, breast, neuroblastoma


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