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NEUROLOGY

NEUROLOGY. Internal Medicine Board Review Brad Ingram, MD. Neurology. Demyelinating diseases Stroke Neuromuscular Movement Disorders. Dementia Headache Epilepsy Neuro-oncology. Demyelinating Diseases.

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NEUROLOGY

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  1. NEUROLOGY Internal Medicine Board Review Brad Ingram, MD

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

  3. Demyelinating Diseases

  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? • LP with MS panel, OCBs • Start her on copaxone • MRI c-spine • C/s ophthalmology • Start Solumedrol 1 gram qday

  5. Multiple Sclerosis • Diagnosis: • Clinical—dissemination in time/space • Paraclinical—MRI brain/c-spine, LP, evoked potentials • Treatment: • Exacerbation • New si/sx lasting >48 hrs with infection/metabolic cause ruled out (pseudo-relapse) • Solumedrol 1 gram/day x 3-5 days • Prevent Relapse • 1st line: IFN b-1a, IFN b-1b, glatiramer acetate • 2nd line: mitoxantrone (cardiotoxicity, leukemia); natalizumab (PML); fingolimod (bradycardia, macular edema)

  6. Symptomatic Treatment • Fatigue • R/o medical causes: TSH, B12, CBC • Address sleep hygiene • Exercise and cooling measures • Amantadine, modafinil • Spasticity: PT, baclofen, tizanidine • Bladderdysfunction: 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

  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? • Seizure with post-ictal paralysis • Meningoencephalitis • Toxic ingestion • Basilar artery occlusion

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

  12. Basic Stroke Localization

  13. Acute treatment of stroke • IV tPA • <4.5 hrs after onset • CT head w/o contrast to r/o ICH absolute mandatory for your hospital to consider stroke treatment! • Dose 0.9mg/kg with max 90mg; 10% bolus then rest over 1 hour • No ASA, NGT, Foley, other procedures x 24 h post-tPA

  14. tPA criteria Inclusion Criteria Exclusion Criteria 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 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)

  15. Dense MCA Blurred gray-white junction

  16. ADC MAP Apparent diffusion coefficient Diffusion weighted MRI

  17. Acute treatment of stroke • IA tPA: less than 6 hours • Endovascular intervention up to 8 hours • If none of the above: • Telemetry x 24 hours • Permissive HTN (to 220/120) • Maintain NPO until dysphagia screen passed • ASA 325mg in first 48 hours • PT/OT/Speech • TTE with bubble, vascular imaging • Change in neuro examthink seizure, cerebral edema, infection, arrhythmia

  18. BP Management in AIS Post-tPA No tPA 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

  19. Secondary prevention • Risk factors are universal for vascular disease • Manage HTN, DLD, DM2 • Antiplatelet agent • Aspirin 81mg or 325mg • Aspirin + dipyridamole • Clopidogrel 75mg • Indications for warfarin • Intracardiac thrombus • Artificial heart valve • Non-valvular Afib (lone Afib and secondary Afib may require other considerations) • 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: • Vertebral artery dissection • Basilar artery occlusion • Complicated migraine • Carotid artery dissection • 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: • Trauma (50%) • Connective tissue disease • 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? • If this pt has symptoms of TIA, he will receive significant benefit from a CEA. • Without symptoms, his risk of stroke is ~2%/yr. • Aggressive lipid control and antiplatelets are equivalent to CEA. • There is evidence that this pt will benefit from CEA even in the absence of symptoms. • Surgery is recommended only in centers with perioperative complication rates <6%

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

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

  26. Neuromuscular

  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? • Increase Mestinon to 60mg q4h. • Increase prednisone to 60mg qday • Increase supplemental O2. • STAT pulmonary mechanics • Start plasma exchange.

  28. 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

  29. Neuromuscular Respiratory Failure • Pulmonary mechanics: • FVC <15mL/kg • 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. Myasthenia Gravis • Ab-mediated attack on post-synaptic NMJ • Dx: AchR Ab, MUSK Ab*, EMG, Tensilon test • Tx: • Crisis—PLEX, IVIg, respiratory support • Suppression—prednisone (low, slow—may cause exacerbation), azathioprine, mycophenolate • Symptomatic—pyridostigmine (Mestinon) • Consider thymectomy if young, AchR Ab+ • Avoid FQ, Li, aminoglycosides, Mg, CCBs, and macrolides

  31. 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? • Parsonage-Turner Syndrome (Neuralgic Amyotrophy) • Erb palsy • Fascioscapulo humeral dystrophy • ALS • Thoracic outlet syndrome

  32. Neuralgic amyotrophy • Brachial neuritis/plexitis (Parsonage-Turner) • Postinfectious, post-vaccination, idiopathic • Severe pain followed by subacute, progressive weakness/atrophy • Tx: analgesics, PT/OT. • Diabetic lumbosacral radiculopathy (Bruns-Garland) • Subacute, progressive • Unilateral, pain in thigh, buttocks, low back with LE weakness, sensory loss, and ↓reflexes without bladder/bowel involvement • Assoc w/rapid wt loss or change in glycemic control • MRI L-spine normal. EMG peripheral dysfunction. CSF ↑protein. • Tx: PT. Usually good recovery in months.

  33. 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? • Amyotrophic lateral sclerosis • Cervical myelopathy • Chronic inflammatory demyelinating polyradiculoneuropathy • Primary lateral sclerosis

  34. Motor neuron disease • ALS • Both UMN and LMN • Multisegmental (bulbar + at least 2 spinal regions) • Cognition, sphincter function, sensory spared • Likely to need trach/PEG • 50% die in 3 years • Riluzole is the only disease modifying therapy.

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

  36. 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

  37. 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

  38. 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

  39. Peripheral neuropathies

  40. 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

  41. MOVEMENT DISORDERS

  42. 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? • Bipolar disorder • Frontotemporal dementia • Medication-related compulsive behavior • Parkinson-related dementia

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

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

  45. Parkinson disease • Tremor • Resting • “pill-rolling” • asymmetric • Rigidity • Cog-wheel, lead pipe • freezing • Akinsesia/Bradykinesia • Festination • Masked facies • Decreased blinking • Postural Instability • Stooped posture • En bloc turns • Falls (often backwards) • Non-motor symptoms: constipation, depression, poor sleep, autonomic instability, drooling, dementia (late)

  46. 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? A) Dementia with Lewy bodies B) Multiple system atrophy C) Parkinson disease D) Progressive supranuclear palsy

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

  48. DEMENTIA

  49. 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 • 10-15%/yr progression to dementia • Work-up of dementia includes: • CBC, TSH, B12, CMP • CT head or MRI brain • Screening for depression • Optional: RPR, HIV, LP

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