Neurology Core Curriculum - Contents from MKSAP, Medstudy and other sources to fulfill ABIM core knowledge subjects

1. Describe the Clinical Presentation of a patient that has an ischemic event to the following cerebrovascular territories: a. Anterior Cerebral Artery Contralateral Leg weakness, Amnesia, Personality Changes b. Middle Cerebral Artery Contralateral Face and Arm (sometimes Leg) Weakness with Contralateral visual defect, Aphagia, and Neglect c. Posterior Cerebral Artery Contralateral field vision loss d. Lacunar Contralateral motor/sensory deficits e. Thalamic Region (4 Syndromes) Pure Motor/Sensory dysfucntion, Clumsy Hand Dysarthria, Ataxic-Hemiparesis syndrome f. Basilar Oculomotor Deficits and/or ataxia with Crossed Sensory/Motor Signs g. Vertebral Dysphagia, Dysarthria, Palate/Tongue Deviation and/or Ataxia with Crossed sensory signs 2. Differentiate the three major categories of headaches (tension/cluster/migraine) based on location and quality of pain. How would you manage each type of headache? What medications can be used for migraine prophylaxis? List the major contraindications/side effects of medications used to treat migraine. Migraine Unilateral and Throbbing in Nature. Usually associated with nausea, anorexia, photophobia/phonophobia, occipital pain, and sinus pressure. Patients can present with aura in classic migraine, as opposed to "common" migraine which occurs without aura. Complicated migraine can presents with neurologic deficits (from weakness to CVA/TIA). Patients can have a prodrome (separate from aura) from up to one day prior to the Migraine. Migraine headaches are more common in females and most patients have a family history. Some patients can identify "triggers" such as stress, EtOH, weather changes, sleep deprivation, hunger and travel. Remember that patients with migraine, especially with aura, have a high risk of ischemic stroke in the future. Patients with mild and intermittent migraines can be treated with NSAIDS/Tylenol. Moderate to Severe migraines can be treated with triptans IV/oral/nasal. Side effects include chest pain, somnolence, flushing and dizziness. They are contraindicated with history of CAD or with multiple risk factors for CAD. Some triptans are contraindicated with severe renal or hepatic impairment. IV and nasal ergot derivates can be used in place of triptans. The major side effects are nausea and akathesia. It is contraindicated in CAD. Metaclopramide can be used in acute attacks. There is Level I evidence to support prophylaxis with: topiramate, valproic acid [divalproex sodium and sodium valproate], amitriptyline, metoprolol, propranolol, timolol, and extract of the plant Butterbur root Petasites hybridus. Evidence supports that patients can benefit from behavioural treatments relaxation training, biofeedback therapy, and cognitive-behavioral training. Tension Squeezing/Grip-like band around the head. Males = Females. Patients can have photophobia/phonophobia. Can use NSAIDS and Muscle relaxants. If severe enough to interfere with work, triptans and ergot derivates can be used Cluster Severe unilateral and temporal/orbital pain with one of the following: conjuctival symptoms, congestion/rhinorrhea, forehead sweating , ptosis or facial edema. Appears in clusters of 2-8 weeks of symptoms, and then pain-free intervals between episodes. Moving around and sometimes painful external stimuli to the face/head alleviate symptoms. Males affected more than females. Acute treatment includes Oxygen + triptan or ergotamine derivative. A brief course of oral corticosteroids (1 to 3 weeks) or an occipital nerve block in conjunction with verapamil as the preventive drug of choice.

3. A patient is found to have an acute occlusion in the Right MCA. You plan to administer t-PA. What is the time-frame during which treatment with t-PA is indicated? List some Absolute Contraindications to t-PA use. T-PA is effective within 4.5 Hours after exact onset of symptoms (per new guidelines) Contraindications: CT Evidence of Hemorrhage Seizure at Onset of Symptoms History of ICH GI/GU Bleeding within 3 weeks Major surgery or trauma within 2 weeks Pregnancy or Lactating Women BP> 185/110 Concurrent MI or Myocarditis Glucose <50 or >400 Platelet <100,000 On Coumadin with PT >15s PTT Elevated with history of Heparin Use 4. What clinical features differentiate Thoracic/Lumbar Spinal Stenosis vs. Vascular Claudication? Remember that Claudication is pain in the extremities upon ambultation. It has both neurogenic and vascular causes. Symptoms of lumbar stenosis (aka "neurogenic Claudication") appear or get worse with the onset of ambulation or on standing, and are promptly relieved by sitting or lying down or bending forward. (classic scenario is it is helped with use of of a shopping cart) Vascular Claudication is worse with ambulation and better with rest. Position usually does not affect the symptoms. Examination of the femoral, popliteal and pedal pulses, as well as inspection of the legs and feet for trophic changes, is essential in order to differentiate vascular from neurogenic claudication. 5. List the modifiable risk factors for stroke prevention. Also describe the BEST drugs of choice for each of these factors. 1) HTN ACEI/ARB +/- Thiazide Diuretic (esp Indapamide) 2) Dyslipidemia Statins (even with normal lipid panel) 3) Diabetes and Glycemic Control Try to reach A1c<7. There is no optimal medication to reach this, and patients should be managed like other diabetics. 4) Other modifiable risk factors include tobacco, obesity, and excessive EtOH use 6. List the general treatment approach for a patient with brain metastasis. How does this differ, in general, for patients with multiple brain mets? How would you treat Leptomeningeal involvement from Breast Cancer? Leptomeningeal involvement from Leukemia? Solitary: Steroids + Surgery then Whole Brain Radiation (WBRT) Multiple: Steroids then WBRT Leptomeningeal involvement from solid organs: Palliative Radiation - chemotherapy is not used for a lot of cases because it penetrates poorly into Blood-Brain Barrier Leptomeningeal involvement with leukemia/lymphoma: Chemotx then Rads

7.

Name some of the red flags of a patient presenting to the emergency department with a headache. Worst Headache of Life or New Onset Headache/ Atypical headache features Visual Changes with headache Headache that wakes patient up from sleep Immune Deficiency Neurologic Symptoms (Paralysis/Visual Loss) LOC/Syncope Age >50 Fever/Meningeal Signs Papilledema

8. What is the description of pain in a patient with Trigeminal Neuralgia. What are the causes. What is the workup? What is the treatment? Brief, lancinating, extreme pain around the distribution of the V nerve. Usually lasts a few seconds, in paroxysms, and sometimes triggered around the mouth/nose by stimuli such as wind, talking, shaving, chewing or brushing the teeth. Most of the time it is idiopathic, but one must think of secondary causes (such as carcinoma, multiple sclerosis, stroke or connective tissue disease) if it is persistent, bilateral, radiaties to the chest/neck, or has sensory symptoms. MRI should be part of the initial diagnostic workup. About half of patients have spontanoues remission. First line drug therapy is carbamazepine or oxcarbazepine, but other medications such as gabapentin, and other anti-seizure medications have been used. Surgery is used for the 1/3rd of patients refractory to drug therapy. 9. When is a Carotid Endarterectomy indicated in patients with carotid artherosclerosis? When would you choose stenting of the carotids? For patients with a recent TIA or ischemic stroke (within the past 6 months) who have ipsilateral severe (70%-99%) internal carotid artery stenosis and a life expectancy of greater than 5 years, carotid endarterectomy is recommended. For patients with a recent TIA or ischemic stroke and ipsilateral moderate (50%-69%) carotid artery stenosis, carotid endarterectomy is also usually recommended, depending on patient-specific factors (such as age, sex, comorbidities, and severity of initial symptoms). When the degree of stenosis is less than 50%, there is no indication for surgery Among patients with symptomatic severe (>70%) internal carotid artery stenosis, carotid endarterectomy is still considered the gold standard. RCTs are still evaluating if angioplasty or stenting in routine cases is comparable or superior. If the stenosis is difficult to access surgically or who have medical conditions that greatly increase the risk of surgery carotid angioplasty and stenting are preferable alternatives to endarterectomy. 10. Describe the initial imaging workup you would order of a patient that presents with acute stroke. Besides conventional imaging to diagnose the event, which further work-up would be indicated? What advantages does MRI have over CT? CT without contrast to evaluate for a bleed. Afterwards, one would perform either a CTA or MRA with Carotid Ultrasound and Cardiac Echo. MRI can show small cortical infarcts, and brainstem/cerebellar involvement better. It is also better for distinguishing acute from chronic strokes and pattern of the stroke can help identify the mechanism (vasculitis, embolic, etc)

11. List the various known causes of intracerebral hemorrhage. Which is the most common in all age/ethnic groups? Hypertension (the most common) Amyloid Angiopathy Cocaine Use Cerebral Vasculitis AVM 11. Describe clinical features of Acute inflammatorydemyelinating polyneuropathy (AIDP - also known as Guillain-Barr syndrome). What precedes the disease? How do you diagnose it and what do these tests show? What must you monitor in these patients? What is the treatment of choice? AIDP (aka GBS) Patients present with an ascending progressive distal -> proximal muscle weakness and absent/decreased DTRs. Usually there is a prodrome of pain. Patients can have concominant respiratory failure, autonomic disease and some with cranial nerve disease. This occurs "rapidly" from 2-4 weeks. Preceded by Infection (common ones include CMV/hepatitis/HIV/EBV/Campylobacter), Surgery or Immunization. Initial evaluation includes EMG, LP and PFTs. EMG shows demyelination, LP with normal glucose level and cell count and an elevated protein concentration Must check negative inspiratory force and vital capacity to monitor for impending respiratory failure. Treat the disease with plasmapheresis and/or IVIG. Steroids are not effective. 12. Describe CIDP. How does it differ from AIDP in presentation? How do you treat it?

CIDP is more gradual than AIDP - over a few months with Proximal and Distal Muscle Weakness, Areflexia and distal sensory loss. They usually do not have autonomic dysfunction, respiratory problems or Cranial Nerve disease. Treat with prednisone +/- cyclophosphamide. If refractory can use IVIG/Plasma Exchange 13. Describe and list the various causes of "cryptogenic stroke". Include how you would work-up a young patient (less than 55 years of age or one without risk factors) that presents with a stroke - Collagen Vascular Diseases (CNS Vasculitis/Takayasus) - Infections (Syphillis, Endocarditis) - Primary Vascular Disorders (Fibromuscular Dysplasia, Moya-Moya) - Mitochondrial Myopathies - Radiation Vasculopathy - Illicit Drug Use - Hypercoaguable Status - Cardiac Disease (Embolic Sources including PFO/ASD/MYXOMA) Workup would include: Cardiac Echo, Hypercoaguable Testing (Prot C/S/AT3, Factor V Leiden, Lupus AC, Anti CL Antibodies, Prothrombin Gene), Serum RPR, Urine Drug Screen, Lactate Levels, Cerebral Angiography, ANA and ANCAs. 14. What clinical scenario would you to believe a patient had narcotic overuse headache/rebound headache ? How do you treat it? Patients that have a headache >15 days out of the month, with analgesic use >10 days and without red flags. Headaches usually occur soon after a patient awakens. Analgesics only temporarily relieve the headache. Patients must not take medications to treat headaches for at least one month and be given migraine prophylaxis instead. Some use a brief course of steroids.

15. Describe the differences in the use of anti-platelet agents for the secondary prevention of stroke? Which are shown to be the least and most effective? when would you combine them? What is the major side effect of Dipyridamole? When would you use Coumadin for stroke? Aspirin reduces stroke by 20%. Plavix reduces stroke about 30% but is not significantly more significant than aspirin. Combination of ASA and Dypyridomole is the best with 43% risk reduction. Never use ASA and Plavix together solely for secondary stroke prevention (increases risk of bleeding without benefit!) Dipyridamole commonly causes Headache. Coumadin is usually only used if there is a cardioembolic source, or if a patient has recurrent strokes on ASA and Dypyridomole. Note that urgent anticoagulation should not be used even with presumed cardioembolism in patients with acute CVA may cause intracranial hemorrhagic complications. 16. A 73 year old African American Female Presents with the acute onset of Right sided weakness and an expressive aphagia. What would be the first diagnostic test to order? What would be the next option if this test is negative and your clinical suspicious for ischemic stroke is high? First step is a noncontrast CT. If negative or it is early in the course, the next step is a diffusion-weighted MRI. 17. A 55 year old man with a history of hypertension presented to the emergency department with the sudden onset of 10/10 headache that he states is the worst of his life. He also reports increasing frequency of tension-type headaches over the past 3 days, as well as severe neck stiffness. Vital Signs are normal except for a Blood Presure of 156/86. Laboratory values are normal. Physical examination reveals neck tenderness. CT Scan of the head is normal. -What is your next step to diagnose the patient? What are you looking for on this? Suspect subarachnoid headache - thurderclap headache, diffuse headache, and neck stiffness all lead you to suspect this. CT is positive in about 9/10 patients, but the effectiveness of this test decreases with time. Therefore, you must perform an LP to r/o SAH - you are looking for abnormally elevated erythrocyte count or xanthochromia -If this step confirms the suspected diagnosis then what would you do next to not only help confirm the diagnosis but offer therapeutics? MRI can be used to localize the lesion, and is an acceptable first step. However, patients will need aCerebral angiogram: Will also allow for clipping or coiling -In the meantime, while waiting for the test in step 2 what two medications must be administered to the patient? Need an antiseizure medicine (usually dilantin) and a CCB (nimodipine). 18. A 32 year old obese female presents to you with a complaint of constant headache for the past 4 days. It is unilateral and throbbing. Ibuprofen and acetaminophen only temporarily relieve the pain. She reports intermittent blurred vision and a ringing in her left hear made worse when laying down at night. Physical exam reveals an obese female with normal vital signs. She has flame hemorrhages on fundoscopic exam. CT head is normal. Opening Pressure was 36. 30 Minutes after the LP her headache was improves. What is the diagnosis? What is the long-term feared consequence? What medications are associated with the condition. What is the treatment? Pseudotumor cerebri (Intracranial Idiopathic HTN). Patients can get progressive optic atrophy and vision loss. May be associated with OCPs, Tetracycline therapy and Vitamin A (This is controversial !). Treatment is initially with lumbar pucture to decrease the pressure. Most patients are placed on acetazolamide. If there is vision compromise, steroids are given and if not helpful, then surgery is performed.

19. A 72 year old female is brought into your office by her husband because she is not herself. Over the past 4 months she has become more forgetful and does not answer him appropriately. MMSE is 22 and Geriatric depression Scale does not reveal that she has pseudodementia. What tests would you order to rule out secondary causes of dementia? TSH, B12, RPR & CT Head at a minimum. Other workup to consider includes EEG (to rule out nonconvulsive seizures, prion diseases, etc). Some may do LP, but it is not routine to perform. 20. What triad makes you suspicious for normal pressure hyodrocephalous? How do you diagnose NPH? Treat NPH? Shuffling gait apraxia, demential and urinary incontinence. CT/MRI usually show ventriculomegaly that is out of proportion to atrophy, but cannot be used to diagnose it. Treatment of suspected NPH, with resolution of symptoms, is actually the best way to "diagnose" the disorder. Rapid improvement of gait disorder after CSF drainage is suggestive. If this is the case, a VP shunt may be placed for further treatment. 21. How would you manage hypertension in a patient with ischemic stroke? How would blood pressure affect your use of tPA in ischemic stroke? How would your management of blood pressure differ if the patient had an intracranial hemorrhage? Ischemic: Consensus opinions state that patients with persistent elevations in systolic blood pressure higher than 220 mm Hg or diastolic blood pressure higher than 120 mm Hg should receive antihypertensive therapy. Beyond these levels, a reasonable goal would be to lower systolic blood pressure by approximately 15% during the first 24 hours after stroke onset. If the patient is eligible for thrombolysis, blood pressure must be lowered and stabilized below 185 mm Hg systolic and 110 mm Hg diastolic before thrombolytic therapy is started. Preferred antihypertensive agents include intravenous labetalol and intravenous nicardipine infusion. Patients with sustained hypertension above recommended levels, despite early antihypertensive therapy, should not be treated with intravenous thrombolysis. For patients with more elevated DBP (esp over 140): Sodium nitroprusside is preferred Hemorrhage: (1) If systolic BP is >200 mm Hg or MAP is >150 mm Hg, then consider aggressive reduction of BP with continuous intravenous infusion with frequent BP (q5min) checks. (2) If systolic BP is >180 mm Hg or MAP is >130 mm Hg and there is evidence or suspicion of elevated ICP, then consider monitoring of ICP and reducing blood pressure using intermittent or continuous intravenous medications to maintain cerebral perfusion pressure >60-80 mm Hg. (3) If systolic BP is >180 or MAP is >130 mm Hg and there is NOT evidence or suspicion of elevated ICP, then consider modest reduction of BP (target MAP of 110 mm Hg or target BP of 160/90 mm Hg) with BP checks every 15 minutes.

22. How would you manage elevated serum glucose in a patient with acute stroke? What about feeding? Persistent hyperglycemia during the first 24 hours after stroke is associated with poor outcomes, so patients with acute ischemic stroke and plasma glucose levels greater than 140 mg/dL should be given insulin; glucose levels should be monitored to avoid hypoglycemia. Normal saline should be used for intravenous fluid hydration rather than dextrose-containing fluids in order not to exacerbate hyperglycemia.Assessment of swallowing is recommended before patients who have experienced strokes start to eat or drink. Patients with dysphagic stroke should be offered enteral tube feedings via a nasogastric tube within the first 24 to 48 hours of admission; nasogastric feeding should be the chosen route within the first 2 or 3 weeks 23. List the 4 medications used to treat Alzheimers Dementia. What are their mechanisms of action and common side effects? Which is used in severe dementia Galantamine (Razadyne) Cholinesterase Inhibitor. Nausea and Diarrhea. Donepezil (Aricept) Cholinesterase Inhibitor. Nausea and Dizziness and Anorexia. Rivastigmine (Exalon) Cholinesterase Inhibitor. Nausea and Diarrhea and Anorexia. Memantine (Namenda) NMDA Inhibitor For Severe dementia. Hallucinations and anxiety are side effects. 24. Name at least 4 causes of non-Alzheimers dementia and the salient clinical Features/diagnostic criteria of each disorder Remember: Distinctions between cortical and subcortical dementia are evident during mild to moderate disease, but are less clear in later stages.. Some diseases that cause subcortical patterns of dementia lead to earlier gait, balance, and swallowing difficulties than occur with most causes of cortical dementia. The defining clinical signs of cortical dementia are aphasia (impaired language), apraxia (impaired movement programming), and agnosia (impaired recognition)the three As. Subcortical signs primarily include extrapyramidal syndromes and slowing of thought and movement (psychomotor slowing). Cortical signs characterize Alzheimer dementia and the frontotemporal lobar degeneration syndromes of primary nonfluent aphasia, semantic dementia, and frontotemporal dementia (with and without motoneuron disease). Subcortical dementias are primarily found in progressive supranuclear palsy, Parkinson disease with dementia, Huntington disease, and the small-vessel form of vascular dementia. Mixed patterns of dementia characterize cortical basal ganglionic degeneration and dementia with Lewy bodies.
Patients present with poor recent and short-term memory, difficulty naming and imprecise speech. After years patients may have more frontal-lobe symptoms (such as apathy) and late term complications include gait disorders

Alzheim er's - Most Common after the age of 60. There is a 4x increased risk with a family history.

Lew y Body Patients with visual hallucinations of little living things and mild Parkinsonism on
presentation. Patients usually have episodes of syncope and falls as well as excessive daytime somnolence. This is the most common cause of dementia in patients with Parkinson's disease. Psychosis is not uncommon

of insight.

Frontotem poral Dem entia Patient with personality changes and inappropriate behavior with lack Vascular Dem entia Patient with history of CVAs and/or cerebral hemorrhage proven by imaging
and onset of dementia within 3 months of the neurologic cause. Stepwise deterioration

CJD Rapidly Progressive course with startle myoclonus/cerebellar signs/akinesis and classic EEG (periodic sharp waves on a slow background) OR Elevated levels of 14-3-3 protein in CSF. P rogressive Supranuclear P alsy - Present like Parkinson's Disease, but have predominant gait
disorders. There is progressive loss of voluntary eye motion

Huntington's Disease - Chorea, personality changes, depression and psychosis beginning in the
30's. CT with atrophy of the caudate nucleus

AI DS - Dementia occurs in half of advanced cases

25. What are the secondary/medical causes of restless leg syndrome? How is RLS Treated? Iron Deficiency Anemia, ESRD, Withdrawal from sedatives. Treat with Dopamine Agonists. 26. Describe the presentation and exam of a patient with MS. List and briefly describe the three clinical courses of Multiple Sclerosis Any CNS symptom can be present in MS. Sensory symptoms, such as parasthesias are very common. Other findings include "useless hand" syndrome, a "tight band" sensation around the trunk/limb, optic neuritis (loss of vision with orbital pain), Lhermitte sign (spinal or limb paresthesias elicited by neck flexion), diurnal fatigue (typically worst in the early afternoon), and sensitivity to heat exposure or exercise (which may cause temporary worsening of fatigue or neurologic symptoms). Motor weakness, imbalance, bladder or bowel dysfunction (especially urinary urgency, frequency, and incontinence and constipation), and facial pain (trigeminal neuralgia) are other common symptoms. Exam can reveals afferent pupillary defects or internuclear ophthalmoplegia. Patients will develop symptoms separated both in time and anatomic location and quality i.e. optic neuritis that goes away and in a week has upper arm weakness then it goes away and then they get sensation loss in the leg.

R elapsing-R emitting: Weeks of dysfunction that improve Primary Progressive: Slowly Progressive Cervical Myelopathy. Secondary Progressive: Patients with Relapsing-Remitting MS that do not improve between
episodes and become disabled

27. Describe the treatment for Multiple Sclerosis. What can be used for acute flares? When do you treat acute flares? What about long-term management? What are the side effects and contraindications to the medications used for long-term management. What Chemotherapeutic Topoisomerase inhibitor used for breast cancer/AML/Lymphoma has been used to treat progressive MS and what is its major side effect? Steroids (IV Methylprednisolone then po prednisone) can be used for exacerbations when patient has visual disturbances or loss of function. If this does not help then plasma exchange can be used. Disease modifying agents include IFN-Betas (Side effect is a flu-like illness. They are contraindicated with depression and Liver Failure), glatiramer acetate (injection site reaction and non-cardiac chest pain) and Naralizumab (case reports of progressive multifocal leukoencephalopathy/ PML were reported ). All of these medications reduce relapse about 1/3rd. IFN can be combined with cyclophosphamide for severe cases Mitoxantrone can be used for progressive MS with cardiotoxicity as a side effect. 28. Describe the workup of a patient that presents with symptoms of MS. This includes not only tests to help diagnose MS but to rule out mimics if the clinical scenario warrants. MRI of Brain and Spine to look for Demyelination Brainstem Evoked Potential testing LP with myelin basic protein, oligoclonal bands or increased IgG index Send B12 (r/o SCD), RPR, HIV, ANA/ESR (Vasculitis) and Lyme Titers for DDx

29. Differentiate The Presentation of Myesthenia Gravis from Lambert-Eaton Syndrome. How do you diagnose each? What antibodies are present in each? What imaging must be done in MG? In LES? Both have progressive weakness and fatigue of extremities. Signs and symptoms in patients with myasthenia gravis include fatigable blurring of vision, binocular diplopia, ptosis, limb weakness, diplopia, slurred speech, dysphagia, and dyspnea. Findings on neurologic examination include ptosis, impaired ocular motility, and limb weakness that increases with repeated testing (fatigable weakness). Deep tendon reflexes and the sensory examination are normal. Lambert Eaton syndrome occurs in 5% of patients with Small cell lung cancer and other malignancies. Patients typically report progressive proximal limb weakness, and most have symptoms of dysautonomia, such as dry eyes, dry mouth, constipation, and erectile dysfunction. Lambert-Eaton myasthenic syndrome should be considered in any patient with findings of proximal limb weakness and absent deep tendon reflexes on neurologic examination. Below are the key differences between the two presentations: Myasthenia Gravis Normal Absent Worse Yes Lambert-Eaton Decreased Present Better No

Reflexes Autonomic Dz (dry mouth/impotence) Symptoms with repetitive motion CN III Involvement

Dx MG with EMG and decremental response of muscles to nerve stimulation and ACh Antibodies (positive in 85% of patients). There is a positive Tensilon Test (rapid relief of symptoms with administration of the medication) EMG with LES reveals that muscle action potentials increase with stimulation. Voltage-gated calcium channel antibodies are positive in LES. Imaging: CT for Thymoma in MG. Start out with CT C/A/P with LES and if negative, PET scan should be done.

30. How do you treat MG? More advanced MG? What about surgery? What is myasthenic crisis and how do you treat it? Treat MG with ACholinesterase such as pyridostigmine. More severe cases can be treated with steroids and immunosuppresives (azathrioprine, MMF, Cyclosporine). Thymectomy is indicated in patients with CT evidence of thymoma and may be considered in patients younger than 50 years of age without thymoma. Myasthenic Crisis is involvement of the respiratory muscles. It can present as wheezing or dyspnea and progresses to respiratory failure. Treat with IVIG or Plasmapheresis. Most patients need to be intubated. There is commonly a trigger present such as fever, aspiration, infection, thymoma, thyroid disease, medications

31. A 48 year old man is brought to you with a complaint of slurred speech, leg cramps and right foot drop. He has thenar atrophy, 2/5 strength in his legs and positive Babinski bilaterally. What is the likely diagnosis? What clinical/exam evidence is seen in the disease. What medication has shown to help the disease? What other measures increases survival? UMN+LMN = ALS. Patients with ALS typically report progressive painless weakness, atrophy, and fasciculations beginning in an arm or leg. Patients do not have sensory loss, pain, or impairment in bowel and bladder function. In addition to lower motoneuron signs of atrophy and fasciculations, upper motoneuron signs, such as hyperreflexia and extensor plantar responses, are also typically seen. Approximately 20% of patients have bulbar-onset ALS, characterized by slurred speech, difficulty swallowing, and emotional lability. As ALS progresses, patients develop weight loss and respiratory insufficiency. Cognitive impairment is not uncommon. Findings on neurologic examination include limb weakness, fasciculations, atrophy, brisk deep tendon reflexes, and extensor plantar responses. Signs of bulbar impairment (such as slurred speech), tongue atrophy, and fasciculations may also be evident. Can use riluzole (prolongs survival about 3-5 months) BiPAP increases survival 20 months

32. What are the cardinal manifestations of Parkinsons Disease? Describe them and how they present clinically. What are other features of patients clinically? Tremor (at rest) with pill-rolling Bradykinesia (Cannot initiate motor functions small writing and cannot button, Rigidity (Increased muscle tone) Postural Instability (stooped forward, stumbling gait, decreased arm swing) Patients also have staccato speech and lack of blinking as well as masked facies. Patients usually have autonomic disease including orthostasis, constipation and either urinary retention or incontinence. Patients have Akathisia (cant sit still). Signs suggesting an alternative condition include symmetric symptoms or signs, early falls, rapid progression, poor or waning response to levodopa, dementia, early autonomic failure, and ataxia. 33. Describe Treatment of Parkinsons. Make sure to include how to manage side effects of these medications, accounting for the "wearing off effect" and treatment of common comorbid conditions. In General: The mainstay of drug therapy is targeted toward dopamine replacement with levodopa or dopamine agonists. Drug treatment can be delayed until the patients symptoms interfere with employment or social activities. The choice of pharmacotherapy in early Parkinson disease remains somewhat controversial because of concerns about levodopainduced motor complications. Levodopa is the most effective medication used in the treatment of Parkinson disease but is associated with motor fluctuations, such as dyskinesias and a wearing-off effect, which refers to enhanced parkinsonian symptoms due to ineffective dopamine therapy. It is useful the think about treatment in the following manner:

Tremor Predominant:

Amantadine and Anticholinergics (Benztropine and Trihexyphenidyl) ropinirole), Amantadine, L-Dopa (with carbidopa) and MAOI

Non-Tremor Predominant and Younger than 70 : Dopamine Agonists (Pramipexole or Non-Tremor Predominant and Older than 70 : L-Dopa (with carbidopa) and MAOI
Patients with disabling dyskinesias on levodopa monotherapy may benefit from the addition of a dopamine agonist. Amantadine can also be used to reduce dyskinesias. In patients with wearing-off phenomena, the initial strategy is to increase either individual levodopa dosage or the frequency of levodopa administration. If dyskinesias then become problematic or the duration of levodopa effect becomes too short, coadministration of a dopamine agonist is often beneficial. Administration of entacapone or rasagiline has also been shown to reduce the wearing-off effect. Additionally, deep brain stimulation has been shown to improve motor function, reduce the wearing-off effect, and reduce dyskinesias. Patients with severe motor fluctuations despite optimal pharmacotherapy should be referred for consideration of deep brain stimulation. Also, remember that patients with Parkinson's disease commonly have depression and anxiety (even with early state disease) and psychosis in not uncommon with late stage disease. The first step in patients with anxiety/depression is to optimize dopaminergic therapy. SSRIs and SNRIs are first-line agents for those requiring drug treatment. in patients with persistent psychosis, reduction or elimination of medications should be considered. Cognitive-behavioral therapy should also be considered. Pharmacotherapy is occasionally

necessary in the treatment of psychosis. Cholinesterase inhibitors may be beneficial in some patients and are usually well tolerated. Quetiapine, an atypical antipsychotic medication that typically does not worsen motor function, is used by many experts as first-line therapy. Sleep disturbances, restless leg syndrome and rapid eye movement sleep behavior is not uncommon. Clonazepam before bedtime may be indicated if these events disrupt sleep or are dangerous to the patient. 34. What is multiple system atrophy. How does it present. What is the treatment It is a parkinsonian syndrome - consisting of parkinsonism, ataxia and autonomic nervous system disease. Parkinsonian symptoms, such as rigidity, bradykinesia, and postural instability, occur in conjunction with ataxia or symptoms of dysautonomia. Signs and symptoms of dysautonomia include erectile dysfunction, constipation, diarrhea, urinary incontinence, and orthostatic hypotension. A history of voice change or breathing difficulties should prompt ear, nose and throat evaluation to look for vocal cord abnormalities. Treatment of multiple system atrophy is symptomatic only. Carbidopa-levodopa should be administered to affected patients with significant parkinsonism; 25% of these patients will have an initial beneficial response to this drug. Orthostatic hypotension is treated with a combination of conservative and pharmacologic measures. Patients should be instructed to elevate the head of the bed, increase their salt intake, and use compression stockings. Medications used to treat orthostatic hypotension include midodrine, fludrocortisone, and pyridostigmine. 35. Describe Essential tremor and how it is treated. There is usually a Genetic Component (Autosomal Dominant fashion). Occurs with posture especially outstretched hands. Symptoms alleviated when the patient uses EtOH. Treat with propanolol or primadone if patient has severe symptoms. 36. Describe the clinical features of a patient with Progressive Supernuclear Palsy. How is it treated? Parkinsonism, Multiple falls, dysphagia, dysarthria, vertical gaze -> horizontal gaze palsy. Mild cognitive impairment progresses to dementia. Treatment of progressive supranuclear palsy is limited. A levodopa trial can be considered in patients with significant parkinsonism 37. List at least 6 causes of myopathy. What tests may you order to work-up a patient that presents with muscle weakness? Infection (HIV) Meds (Statins/Steroids/Niacin/Colchicine/AZT) Rhabdo (CK, Urine Myoglobin and Ua) PM/DM/IBM (Aldolase, CK, Jo-1, Mi-2) Thyroid Disease (TSH) Electrolytes (Calcium) Muscular Dystrophy (Becker/Duchenne) GBS/CIDP ALS EMG and Muscle Biopsy are needed if cannot dx one of these by other test

38. A 35 year old man with a past medical history of Diabetes Mellitus Type I Presents To the emergency department with a generalized tonic-clonic seizure. He is afebrile and has no other symptoms or complains. He uses an insulin pump which is working correctly.

What Lab workup should be done? CBC, LFTs, Tox Screen, CT/MRI and EEG. If this workup is negative, what is your treatment of choice? No tx for 1st seizure
39. Describe the patient that will get a critical illness polyneuropathy what clinical scenario would lead you to think of it? What ICU diagnoses are the most common causes? What are the symptoms/findings. What is spared? Patients with difficulty to wean from the vent with no other discernable causes should lead one to think about this diagnosis Patients with Sepsis are most commonly affected They have distal flaccid paralysis, decreased reflexes and distal sensory loss Cranial Nerves Spared 35. How does carpal tunnel syndrome present? List the medical causes of Carpal Tunnel Syndrome. How do you diagnose it? How does ulnar neuropathy present? How would you treat compression neuropathies, such as carpal tunnel or ulnar compression neuropathy. Carpal Tunnel syndrome presents with paresthesias and occasionally with pain or weakness involving the fingers. Examination may show sensory loss over the palmar surface of the first three digits and weakness of abduction and opposition of the thumb. The paresthesias are often worse at night or when holding a book or steering a car Medical causes include DM, Amyloid, Acromegaly, Hypothyroidism. Diagnose with EMG. Treat carpal tunnel syndrome with wrist splints and steroid injections. Surgery can be considered with severe symptoms or failure to respond to conservative treatment Ulnar neuropathy causes numbness of the fourth and fifth fingers and, when severe, may cause weakness of interosseous muscles. EMG should be performed to localize the site of ulnar nerve impairment and to document severity. The elbow is the most frequent site of compression. Patients are initially managed conservatively with a splint that can be used during sleep and instructions to avoid resting the elbow on furniture and other objects or to use an elbow pad. When conservative measures fail, surgical options include either decompression or translocation of the ulnar nerve. 36. A patient with a history of epilepsy presents to the emergency department with a seizure at home. He is on Keppra and has no drug allergies. In the emergency department he has another tonic-clonic seizure which is controlled with one dose of Lorazepam. He is then admitted to you for work-up of seizures. While on the floor patient has a seizure that does not abort with Ativan 2mg IV. His blood pressure is 200/92. What is your next step? What if this does not work? What if this 2nd step does not work? Treat for status: After Lorazepam (or Diazepam) -> Load with Dilantin. If Dilantin Does not work -> Load with Phenobarb If Phenobarb doesnt work -> Pentobarb/Propofol or Midazolam drip

37. List the symptoms of phenytoin toxicity. How is it treated? Hyper/Hyporeflexia, bradykinesia/ataxia, dizziness, tremors, nystagmus Multiple dose activated charcoal is used to treat. Dialysis does not help.

38. Describe the epidemiology of Meningiomas. What symptoms do patients present with? What is the treatment and when it is offered? How often do you image patients. What other malignancy is seen with meningioma in higher frequency than the general population? More common in women esp when pregnant (has progresterone receptor). Present with headache and focal neurologic symptoms, Can have seizures Surgery with Large or Symptomatic. If inoperable or if residual post-op-> Rads Image q3-6m for 1 yr then yearly for 5-10 years Patients with meningiomas have an increased risk of breast cancer 39. Describe the presentation of a patient with a glioma. How does imaging reflect the grade? What is the treatment for low grade? High grade? What is the median survival for low grade? High grade? If a patient with glioma presents with a pulmonary embolism can you anticoagulate them? Headache that wakes in the morning and worse with Valsalva. Patients usually have nausea and vomiting. 1/3 present with seizures. Low-Grade gliomas do not enhance with contrast but show a low attenuation. High Grade enhances with contrast and usually shows necrosis Low Grade: Surgery + Chemo/Rads High Grade: Chemo/Rads. Surgery only if mass effect/edema Median Survival: Low 8-10 years / High 1-2 years Yes You can safely anticoagulate. Primary brain cancers rarely bleed with anticoagulation 40. What cancers metastasize to the brain? The Meninges? What symptoms would lead you to believe that there is meningeal involvement? Lung, Breast, RCC, Melanoma, Thyroid. Leukemia/Lymphoma and solid organ (esp Breast/Lung) Meningeal involvement with headache, back pain, radicular symptoms and weakness. 41. Define the common malignant causes, clinical presentation and Diagnosis/Workup of the following paraneoplastic neurologic syndromes Paraneoplastic Encephalitis Opsoclonus Myoclonus Paraneoplastic Sensory Neuropathy Paraneoplastic Cerebellar Degeneration Paraneoplastic Encephalitis Usually with lung cancer. Usually limbic complaints including dementia/AMS and seizures. LP with pleocytosis and increased protein. Have antibodies in CSF (i.e Anti-Hu). Do CT C/A/P + mammogram or testicular U/S Opsoclonus Breast and SCLC. Ataxia, involuntary jerks and irregular eye movements/twitching. Dx is clinical. Workup for causes with CT and Mammogram

Paraneoplastic Sensory Neuropathy Usually Lung Cancer. Paresthesias, sensory ataxis, sensory loss. They have absent sensory potentials on Nerve conduction Studies. Do CT C/A/P + mammo or testicular U/S Cerebellar Degeneration Breast Cancer. Dysarthria and limb ataxia. Initial neuroimaging is normal but eventually get cerebellar atrophy. Patients have LP with pleocytosis and increased protein. 42. A 32 year old man is brought to the office by her sister for concerns of becoming aggressive over the past 2 months. The patient and her sister have noticed twitching of her body over the past month. The patient only has a history of depression, for which she takes amitryptyline. The family history is unknown since she the patient is adopted. On exam you notice brisk involuntary movements of her limbs and hands that move from one area to another What is the most likely diagnosis? Huntingtons What is the treatment? Only symptomatic for the chorea with amantadine + SSRI for Depression What is the differential diagnosis for the unpredictable movements of her body? DDx for Chorea includes: Huntington/Wilsons Drugs (Neuroleptic, Dopamine, Antichonergic Infections (Syndehams Chorea and Rheumatic Fever) AI Disease (Lupus esp with APLAb Positive) CVA 43. Describe Mononeuritis Multiplex. List some of the causes: Painful asymmetric asynchronous sensory and motor peripheral neuropathy involving isolated damage to at least 2 separate nerve areas. Multiple nerves in random areas of the body can be affected. Examination reveals preservation of reflexes and good strength except in regions more profoundly affected. Patients can get a syndrome with for example, left leg weakness which resolves then right wrist drop then Right Foot Drop etc etc. Causes: Diabetes, Vasculitis (PAN/Wegneners/TA), amyloidosis, direct tumor involvement, Autoimmune (RA, SLE, Sjogren, SScl), Paraneoplastic, Cryoglobulemia, Sarcoidois, Leprosy, HIV Although this list is extensive, most patients either have DM or rheumatologic diseases. 44. Describe the clinical presentation of a patient with carotid artery dissection. What is the treatment? Pain is the initial symptom of a spontaneous internal carotid artery dissection. Head, neck, or facial pain ipsilateral to the dissection is common. The headache is usually described as constant and severe. Unilateral facial or orbital pain is also common. Hypoageusia, or decreased taste sensation, may also be a presenting symptom. Amaurosis fugax and Ipsilateral Horners are also possible presenting symptoms/signs. Most ischemic cerebral symptoms arise from thromboembolic events; therefore, early institution of antithrombotic treatment provides the best outcome. Anticoagulation with intravenous heparin followed by warfarin has generally been accepted as medical management to prevent thromboembolic complications. Antiplatelet therapy has also been used when systemic anticoagulation is contraindicated. Patients with expanding or symptomatic dissecting

aneurysms and/or those who are refractory to medical therapy are candidates for angioplasty and stent placement. 45. Describe the patient that gets critical illness myopathy? What does the exam show? What lab test confirms the diagnosis and is followed daily for progression? Intubated patients that get steroids or NM blocking agents Patients get generalized and proximal flaccid paralysis, muscle atrophy Creatine Kinase Levels are high in these patients and can be trended for progression 46. Describe the presentation of a patient with myotonic dystrophy. What are common causes of mortality? What makes the inheritance pattern and presentation of the illness interesting? Symmetric distal muscle stiffness and weakness. Patients will have early cataracts, ptosis, temporal wasting and frontal balding. They will get cognitive dysfunction. Most have insulin resistance. AV block and diaphragmatic weakness can occur, which are common causes of mortality. The disease demonstrates anticipation in that it manifests at an early age in subsequent generations. 47. What clinical features differentiate peripheral from central causes of vertigo? What are some causes of central vertigo? Peripheral? Central: Patients will have constant vertigo, nystagmus can be horizontal or vertical and uni-or bidirectional. There is usually no hearing loss/tinnitus. Peripheral: Intermittant vertigo. Nystagmus is horizontal and unidirectional. Tinittus and hearing loss are usually present Central Causes: MS, Ischemic CVA esp Basilar location, Acoustic Neuromas Peripheral Causes: Menieres, BPPV, Labyrinthitis, Infection, Drug-Induced 48. How would you diagnose a patient with BPPV? What is the treatment? Patients typically describe vertigo lasting minutes in duration, with multiple episodes occurring over weeks to months. Tinnitus, ear pain, and hearing loss are absent, and nausea that is sufficiently severe or prolonged to cause vomiting is rare. The natural history of BPPV is to spontaneously remit over a period of several weeks. Recurrences are common. Otolith repositioning, commonly known as the Epley maneuver, has been shown helpful in resolving symptoms of BPPV. Variants of the maneuver can be taught to patients if the symptoms of BPPV return. If these maneuvers fail, medications may be considered in patients with frequently recurring episodes. Anticholinergic medications (scopolamine), antihistamines (meclizine or diphenhydramine), benzodiazepines, or phenothiazine may be useful until symptoms subside. 49. What is transient global amnesia? What are causes? How do you treat it? Intermittent transient loss of memory. Immediate recall ability is preserved, as is remote memory; however, patients experience striking loss of memory for recent events and an impaired ability to retain new information. Many patients are anxious or agitated and may repeatedly ask questions concerning transpiring events. Language function is intact, as is attention. Symptoms rarely persist past 24 hours.

Causes to be considered include TIA, migraine variant, and seizure (esp. temporal lobe). Medications, physical stress, and valsalva maneuver can be triggers Treat with reassurance. Refer to a neurologist for follow-up testing. 50. What is diabetic amyotrophy? Asymmetric muscle weakness and wasting in the lower extremities as a complication of diabetic neuropathy. Reflexes are usually decreased, and the quadriceps are commonly affected. It is partially reversible with glucose control. 51. Describe Cranial Nerve 7 and 6 Palsies. How do they present. What is the cause/treatment Bell's Palsy - weakness of ipsilateral facial muscles. There are multiple causes, but HHV-1 is commonly implicated. Paralysis of the upper and lower face distinguishes Bell palsy from stroke, which affects only lower facial muscles (sparing the forehead and eye) when associated with facial paralysis. Most patients experience excellent recovery, although as many as 30% of patients may have poor recovery. Treatment involves eye patching and lubrication to protect the cornea. A recently reported randomized, double-blind, placebo-controlled trial showed improved outcome in patients treated with prednisone within 72 hours of symptom onset. Evidence-based studies have not clearly demonstrated benefit with antiviral therapy. Patients with Abducens palsy usually present with binocular horizontal diplopia (double vision producing a side-by-side image with both eyes open) and esotropia in primary gaze. Patients also may present with a head-turn to minimize diplopia. Causes include neoplasm (although more common in younger patients), diabetes, stroke, and vasculitis. Workup includes screening for DM/HTN, MRI and ESR. Treatment depends on the cause, and use of patches/prisms 52. Describe TIA. Why is assessment of this condition so important. What is the workup? A focal neurologic deficit that resolves within 24 hours (this has sometimes been changed based on imaging, and now some state TIA is symptoms usually lasting less than 1 hour and without imaging evidence of infarction). Up to 20% of patients who have a TIA will have an acute stroke within 90 days, and approximately 50% of these strokes occur within the first 2 days. Diagnostic testing should include EKG, CBC, BMP, Lipids, Head CT and either CTA or MRI. One should also perform carotid U/S and TTE. A conventional angiogram may be necessary when the noninvasive assessment is inconclusive. Transesophageal echocardiography with testing for right-to-left shunting across the atrial septum is recommended for patients younger than 45 years when preliminary investigations do not disclose a cause of the TIA. 53. After a concussion, when can an athlete return to playing sports? Regardless of grade of concussion, all athletes require on-site evaluation and should not return to play during the event. There is some difference depending on society but in general any athlete with grade 2 (no LOC but mental status abnormalities) or grade 3 (LOC) concussion need neuro evaluation and should not return to play for at least a week.