MKSAP Neuro Flashcards
What AEDs increase risk of osteoporosis? Name 4. When should screening start?
Phenytoin, Phenobarbital, Carbamazepine, Valproic acid
Should undergo DEXA scan after 5 years of therapy
What disease is associated w/ exercise intolerance associated w/ myalgia, mild weakness, and myoglobinuria? How can these patients protect against exercise intolerance?
McArdle disease - glycogen storage disease V
They can protect against exercise intolerance by eating carbohydrate-rich diet
What is the medical research council muscle scale and when is it used?
Scale that evaluates muscle strength of each extremity x 3 movements; maximum score is 60, if <48 = ICU related weakness is diagnosed
What AEDs are preferred in pregnancy?
Levetiracetam and Lamotrigine (needs dose increase of 50%)
What 3 AChe inhibitors are used in mild-moderate Alzheimer’s? What are the contraindications to their use? Name 5.
- Donepezil, Rivastigmine, Galantamine
- Sick sinus syndrome, LBBB, uncontrolled asthma, angle-closure glaucoma, ulcer disease; caution with seizures if using donepezil
When do you do carotid endarterectomy?
> 80% stenosis in patients who have not had a stroke
>70% stenosis in females who have had a stroke; >50% in males who have had a stroke
What diagnostic test can be performed in patients w/ mild cognitive impairment to diagnose Alzheimers? What proteins are found?
- LP
2. Decreased amyloid-beta-42 peptide, and increased tau protein & p-tau levels
What monitoring is needed while on fingolimod?
Cardiac (bradycardia w/ first dose), eye exam (macular edema), liver enzymes, CBC (lymphopenia), yearly skin exam (basal cell carcinoma)
What disease presents w/ numbness, distal extremity weakness, unsteady gait, areflexia, and atrophy of distal extremity muscles and foreleg muscles?
Charcot-Marie-Tooth disease
What is St. John’s wort used for? What are some side effects? Name 5.
Depression
GI symptoms, dizziness, confusion, dry mouth, photosensitivity, sedation, urinary frequency, anorgasmia, drug interactions
What medication can improve functional recovery in severe TBI?
Amantadine
What presents as subacute severe pain -> resolution of pain -> progressive weakness and atrophy involving the shoulder girdle and upper extremity muscles?
Idiopathic brachial plexopathy/Neuralgic amyotrophy/Parsonage-Turner syndrome
It is often triggered by infection or surgery
How does reversible cerebral vasoconstriction syndrome clinically present? What are triggers? Name 5. How does it look on imaging? What is the treatment?
- Thunderclap headache
- May be triggered by exertion, valsava maneuvers, emotion, or showering/bathing; also vasoactive drugs (sympathomimetic agents, triptans, cocaine, cannabis), anti-depressants, pregnancy, head/neck surgery or trauma
- Mutlifocal constriction of intracranial vessels on MRA or CT angiography
- Calcium channel blockers (verapamil or nimodipine)
What are the two first line treatments for MS, and which is preferred in liver disease? What are the side effects? What is the FDA approved therapy for primary progressive MS, and what are the side effects? What is the FDA approved therapy for secondary progressive MS, and what are the side effects?
- Interferon beta (avoid in psychiatric disease and liver disease; can cause flu like sx, transaminitis) and glatiramer acetate (can cause injection site reactions, lipoatrophy)
- Ocrelizumab - can cause infections and infusion reactions; increases risk for PML and cancer
- Mitoxantrone - can cause cardiac toxicity and AML; also infection, nausea, oral sores, alopecia, menstrual irregularities, blue discoloration of urine
How does cavernous sinus thrombosis present? What is the most common cause?
Acute onset headache, proptosis, periorbital edema, painful ophthalmoplegia
Most commonly caused by contiguous spread of infection
How does carotid artery dissection present? Vertebral artery dissection?
- Orbital pain, partial Horner syndrome, ipsilateral signs of cerebral or retinal ischemia
- Occipital and neck pain; dysarthria, dysphagia, ataxia, and hemifield visual loss
What type of CNS tumor is a dural-based tumor w/ a smooth rounded shape with a “tail” tracking along the dura outside the brain parenchyma? What is the treatment?
Meningioma
Surgery if symptomatic, observation if small and asymptomatic; no chemotherapy
What is needed for staging of primary CNS lymphoma? What is the treatment?
Whole body PET or CT, bone marrow biopsy, and testicular U/S
IV methotrexate + rituximab; no surgery
How does the paraneoplastic syndrome associated with anti-GAD present? Name 4 findings. What cancers is it associated with?
Stiff person syndrome, type 1 DM, ataxia, brainstem encephalitis, parkinsonism, ophthalmoplegia
Thymoma, breast cancer
What conditions are associated with intracranial HTN? Name 5. What are the treatment options for idiopathic intracranial HTN? What are the potential side effects of these?
- Hypervitaminosis A, tetracyclines, retinoic acid, kidney failure, hypoparathyroidism, addison disease, use of estrogen and progesterone, pregnancy, steroid use or withdrawal
- Acetazolamide (preferred), Topiramate
- Paresthesia, kidney stones, taste perversion; weight loss in topiramate
How does intracranial hypotension present? What is the treatment?
- Orthostatic headache, thunderclap or subacute; may be associated w/ tinnitus, diplopia, neck pain, nausea, photophobia, and phonophobia
- Autologous epidural blood patch - blood is injected into epidural space
What cause of headache presents as diffuse non-nodular pachymeningeal enhancement on brain MRI?
Intracranial hypotension
Can also see cerebellar tonsillar descent, subdural fluid collections, decreased ventricular size, and engorgement of pituitary gland
What is the preferred treatment for trigeminal neuralgia and what are 3 main side effects? What are 3 alternative medications for treatment?
- Carbamazepine - hyponatremia, agranulocytosis, dizziness
2. Oxcarbazepine, Baclofen, Gabapentin, Lamotrigine
What are the contraindications for triptans?
Uncontrolled HTN, vascular disease (coronary, cerebral, or peripheral), or migraine w/ hemiplegic or brainstem auras
What two medications lack vasoconstrictor properties for migraine so may be used for acute migraine in patients w/ vascular disease, migraine w/ aura, and uncontrolled HTN?
Lasmiditan - highly selective 5-HT receptor agonist
Ubrogepant - calcitonin gene related peptide antagonist
What are 3 treatment options for status migrainosus?
Several days of steroids
IV dopamine antagonist + IV benadryl, IV ketorolac, and hydration
IV DHE + anti-emetics
What are the 5 meds that can be used in migraine prophylaxis? What are 4 second line agents?
- Propanolol, timolol, metoprolol, valproic acid, topiramate
- Atenolol, amitriptyline, venlafaxine, NSAIDs
What are erenumab, fremanezumab, galcanezumab, and eptinezumab used for?
Monoclonal antibodies used in migraine prophylaxis
How do cluster headaches present? What is the treatment?
- Cluster headache lasts 15 min to 3 hours, recurs 1-8 times daily over a span of weeks to months; months to years of remission; nocturnal attacks, may be precipitated by alcohol ingestion
- Oxygen, subcutaneous sumatriptan; Verapamil as long term prevention
How does short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) present?
Headache lasting 1 second to 10 minutes, may recur more than 100 times daily; typically no periods of remission
Treat w/ lamotrigine but mostly refractory to medical management
How does chronic paroxysmal hemicrania present? What is the treatment?
Headache lasting 2-30 minutes, can recur up to 40 times daily; typically no periods of remission
Indomethacin
What is the management for cough headache? What is the management for primary stabbing/ice pick headaches?
- Indomethacin; Brain MRI since up to half of patients can have a Chiari malformation
- Indomethacin
What are the indications for surgery/drainage in acute vs. chronic subdural hematomas? What is the indication for urgent evacuation of an epidural hematoma? At what size should a cerebellar hemorrhage be evacuated?
- Acute - hematoma thickness >10 mm, GCS <9, pupil asymmetry/fixation
- Chronic - hematoma thickness >10 mm, midline shift >5 mm, significant neurological compromise
- GCS <9, anisocoria, hematoma >30 mL in volume
- > 3 cm
What are the differences between absence seizure and focal seizure w/ altered awareness?
Absence seizure does not have aura, lasts <15 seconds, stops abruptly, no post-ictal state, can have multiple daily, and usually precipitated by hyperventilation
Focal seizure can have an aura, lasts >30 s, gradually terminates, has post-ical lethargy, can occur once weekly or monthly, not likely to be precipitated by hyperventilation
How can temporal lobe seizures present?
Rising feeling in stomach, deja vu, fear -> staring, arrest of speech or behavior, automatisms, altered smell/taste/hearing
What is the most common cause of medication-resistant adult-onset focal epilepsy?
Mesial temporal sclerosis w/ hippocampal atrophy
How does juvenile myoclonic epilepsy present?
Myoclonic seizures - may report dropping items from their hands; most have GTCS, 30% have absence seizures
Triggered by sleep deprivation, stress, and alcohol use
What are the 3 first line AEDs for focal seizures? 3 first line agents for generalized seizures?
- Lamotrigine, Levetiracetam, Oxcarbazepine
2. Lamotrigine, Levetiracetam, Valproate
What are some side effects of phenobarbital? Name 4.
Sedation, hepatotoxicity, rash, pancytopenia, osteoporosis
What are some side effects of phenytoin? Name 8.
Sedation, ataxia, tremor, diplopia, bradycardia, hypotension, rash, gingival hyperplasia, cerebellar atrophy, peripheral neuropathy, osteoporosis, pancytopenia, hepatotoxicity
What are some side effects of valproic acid? Name 6.
Sedation, ataxia, tremor, diplopia, weight gain, PCOS, reversible parkinsonism w/ dementia, pancytopenia, osteoporosis, hepatotoxicity, pancreatitis
What are some side effects of carbamazepine and oxcarbazepine? Name 5.
Sedation, tremor, diplopia, dizziness, hyponatremia, rash, pancytopenia, osteoporosis, hepatotoxicity
What are some side effects of lamotrigine? Name 4.
Sedation, ataxia, tremor, diplopia, headache, dizziness, rash
- Rapid titration associated with SJS, TEN, and DRESS
- Preferred in patients w/ depression or psychosis
- Can worsen myoclonus
What are some side effects of topiramate and zonisamide? Name 6.
Sedation, ataxia, tremor, diplopia, poor concentration, word finding difficulty, paresthesias, weight loss, anhidrosis, nephrolithiasis, acute angle glaucoma*, psychosis, cleft lip/palate in fetus
What are some side effects of levetiracetam? Name 3.
Sedation, irritability, psychosis, depression, thrombocytopenia (rare)
What are some side effects of lacosamide? Name 4.
Sedation, ataxia, tremor, diplopia, PR prolongation/AV block, atrial fibrillation
Which AED should be avoided in elderly patients? Patients with hepatic insufficiency? Patients w/ acidosis and nephrolithiasis (2)? What AEDs need to be dose reduced in CKD (3)?
- Valproic acid
- Valproic acid
- Topiramate, Zonisamide
- Gabapentin, Pregabalin, Levetiracetam
Aneurysms of what size in the posterior & anterior circulation can be managed conservatively?
<7 mm in posterior, <12 mm in anterior
What is the risk with ICA stenting vs. endarterectomy?
Peri-procedural stroke w/ stenting
Peri-procedueral MI w/ endarterectomy
What type of epilepsy is associated w/ nocturnal complex seizures that awaken people from sleep?
Frontal lobe epilepsy
Typically associated with underlying structural pathology
What test is indicated for patients <45 y.o. w/ ICH related to cocaine use?
Cerebral angiography b/c this case is associated w/ high incidence of vascular anomalies
Which BP drugs should be avoided in ICH?
Nitroglycerin or nitroprusside b/c it increases ICP
What is the difference between secondary progressive and primary progressive MS?
Secondary progressive begins as relapsing-remitting; relapses later become infrequent, but there is slowly progressive neurologic disability
Primary progressive presents in 5th or 6th decade; steady progression of disability
Alemtuzumab, Teriflunomide, Dimethyl fumarate, and Natalizumab are MS treatments. What are the main side effects?
Alemtuzumab - infusion reactions, infections, autoimmune thyroiditis, ITP, nephropathy, skin cancer
Teriflunomide - Lymphopenia, HTN, peripheral neuropathy, transaminitis
Dimethyl fumarate - lymphopenia, GI sx, PML
Natalizumab - PML, HA, chest discomfort; rare infusion reactions, hepatotoxicity
What medication can be used in MS to improve walking speed, leg strength, and gait? What is the main side effect and in whom should it be avoided?
- Dalfampridine
2. Seizures; avoid in those with kidney impairment
What medication can be used for pseudobulbar affect (uncontrolled fits of laughter or crying) in MS?
Dextromethorphan-quinidine
What medication is used for moderate to severe dementia?
Memantine
What medication can be used to treat Parkinson disease dementia?
Rivastigmine
What type of cognitive impairment presents w/ repeated falls, emotional incontinence (explosive crying, laughter), apathy, and severe cognitive slowing? What is the management for the cognitive impairment?
- Vascular cognitive impairment
2. Donepezil
What is found on MRI, EEG, and CSF in CJD?
MRI: increased intensity in DW sequence of basal ganglia and various cortical regions; “hockey stick sign” in posterior thalamus
EEG: periodic sharp wave complexes
CSF: 14-3-3 protein, and elevation of total tau protein
Abuse of what substance can cause a relative B12 deficiency and lead to subacute combined degeneration?
Nitrous oxide
What is the first line treatment for Parkinson disease in patients <65 y.o. vs. >65 y.o?
Pramipexole, Ropinirole, Cabergoline in patients <65 y.o.
Levodopa in older patients
What medication can be used to prevent dyskinesia in Parkinsons? What medication can improve neurogenic orthostatic hypotension? What medication is used for Parkinson psychosis? What medication is used for rapid relief of sx caused by sudden wearing off of Parkinson medication?
- Amantadine
- Droxidopa
- Pimavanserin
- Apomorphine
How does corticobasal degeneration (parkinson plus syndrome) present? Name 3 features.
Asymmetric parkinsonism, dystonia, myoclonus, cortical sensory deficits, cognitive deficits, and apraxia (impaired motor planning)
MRI shows asymmetric posterior parietal and frontal cortical atrophy
What can be used for symptomatic tx for Huntington disease?
Tetrabenazine, anti-psychotics, clonazepam, AEDs
What are treatment options for dystonia? Name 5.
Levodopa, BDZs, Baclofen, Anti-cholinergic drugs
Clozapine, Tetrabenazine, Zolpidem
What is uhthoff phenomenon?
Transient worsening of baseline neurologic sx w/ elevations of body temperature
Patients age <40 with essential tremor or dystonia should be screened for what?
Wilson disease - ceruloplasmin and 24 hour urine copper
What are 3 treatment options for essential tremor?
Propanolol, Primidone, Topiramate
What are some drugs that cause action tremor? Name at least 3 categories and 5 drugs.
Beta agonists, stimulants, AEDs, amitriptyline, fluoxetine, cyclosporine, tacrolimus, atorvastatin, steroids, amiodarone, procainamide, levothyroxine, verapamil
What syndrome presents w/ intention tremor, ataxia, parkinsonism, neuropathy, and dementia in older men w/ fam hx of intellectual disability in young males and premature ovarian failure in females?
Fragile X-associated tremor/ataxia syndrome
In what disease is oculomasticatory myorhythmia (rhythmic movements of eye convergence w/ chewing) found?
Whipple disease
What is the treatment for Tourette syndrome?
CBT, address psychiatric co-morbidities
If tics interfere w/ daily activities treat w/ meds - Clonidine (first line); Topiramate, Levetiracetam, Tetrabenazine, Guanfacine
What are some treatment options for tardive dyskinesia? Name 4.
Valbenazine - monoamine depleter; first medication to receive FDA approval for TD treatment
Tetrabenazine, Clonazepam, Anti-cholinergic agents, Amantadine, Botox
What type of tremor is seen both at rest and w/ action, is position dependent and has a null point at which tremor stops if there is a change in the position of the affected limb?
Dystonic tremor
What type of tremor has a coarse proximal tremor at rest that is worse w/ posturing (especially large amplitude), and is most severe during movements? What type of tremor increases in amplitude as the limb approaches the target?
- Rubral tremor - due to cerebellar outflow disorders (MS, stroke, TBI)
- Cerebellar tremor
What are some secondary causes of facial nerve palsy? Name 5.
Lyme disease, HIV, sarcoidosis, diabetes, vasculitis, malignancy
Which drug increases survival in ALS? Which drug slows functional decline?
- Riluzole
2. Edaravone
What imaging should all patients with myasthenia gravis have?
CT chest to screen for thymoma
What are the 3 main differences in lambert eaton when compared to MG?
Weakness improves w/ exercise, hyporeflexia and dysautonomia are present
What disease is associated w/ distal weakness, cataracts, frontal balding, arrhythmias, DM, thyroid disease, and mild cognitive impairment?
Myotonic dystrophy type 1
What lysosomal storage disorder has an adult onset form associated w/ proximal and respiratory muscle weakness? How is it diagnosed?
Pompe disease or acid maltase deficiency
Diagnose w/ alpha-glucosidase activity
What are some drugs that are contraindicated in myasthenia gravis? Name 5.
Aminoglycosides, FQNs, magnesium, beta blockers, calcium channel blockers, neuromuscular blocking agents, morphine, barbiturates, hydroxychloroquine
What is the first step in management for medically refractive epilepsy?
Video EEG monitoring to determine candidacy for epilepsy surgery
How does autoimmune limbic encephalitis present? What antibodies are targeted?
Hyponatremia, myoclonus, limbic encephalitis (amnesia, temporal lobe seizures, confusion)
Antibodies against voltage-gated potassium channel receptor (LGI1, CASPR2, contactin-2)
What is the BP goal post thrombolysis after ischemic stroke? What is the next step in management?
BP <180/105 mmHg
CT or MR angiography to determine candidacy for endovascular therapy
What is the leading cause of fatigue, headaches, and difficult to control HTN in patients w/ stroke?
Sleep-disordered breathing - diagnose with polysomnography
What are the 2 treatment options for idiopathic transverse myelitis?
Steroids Plasma exchange (or cyclophosphamide) if refractory to steroids
How does the miller fisher variant of GBS present and what antibodies are found?
Ataxia, areflexia, and ophthalmoplegia
Antibodies to GQ1b
Which meds can be used to control obsessive-compulsive behaviors in frontotemporal dementia?
SSRIs, TCAs
Dementia medications are not helpful
What is periodic limb movements of sleep and what is the management?
Stereotyped ankle, knee, and hip flexion during sleep
If not associated w/ a sleep disorder, no treatment is needed
What is the first step in treating psychosis in Parkinsons?
Discontinue dopamine agonist
How does multiple system atrophy present? Name 3 features.
Parkinonism, cerebellar ataxia, dysautonomia, early postural instability
For how long should anti-coagulation and anti-platelet therapy be held after deep ICH secondary to HTN?
30 days
What does POEMS stand for?
Polyneuropathy, organomegaly, endocrinopathy, monocloncal gammopathy, skin changes
What can be used to treat the behavioral and cognitive symptoms in Lewy body dementia?
Donepezil
What is the management of treatment-refractory depression associated with cognitive and functional decline?
Neuropsych testing
What disease presents w/ proximal muscle weakness, elevated CK, and biopsy w/ necrosis w/o inflammation? What antibody is found?
- Immune-mediated necrotizing myopathy
2. hmg-coA reductase
What monitoring is needed while on Alemtuzumab?
Monthly CBC, Cr, and U/A
Quarterly thyroid function tests
Yearly skin exam
What condition is considered in a patient with recurrent spontaneous lobar intracranial hemorrhages?
Cerebral amyloid angiopathy
What are the differences between parkinson disease and parkinson-plus syndromes in regards to progression, symmetry of neurologic signs, tremor, timing postural instability, timing of dysautonomia, and response to levodopa?
Parkinson plus has rapid progression, symmetric neurologic signs, no tremor, early postural instability and dysautonomia, and poor response to levodopa
Parkinson disease often has tremor, is slow, asymmetric neuro signs, late onset of postural instability and dysautonomia, and excellent response to levodopa
What are treatment options for cataplexy (sudden loss of muscle tone associated w/ laughing or intense emotions) in narcolepsy? Name 3.
Venlafaxine, fluoxetine, atomoxetine, sodium oxybate
What lobe does a seizure presenting w/ focal contralateral motor activity (clonic jerking) localize to? Focal contralateral pain, numbness, or paresthesias?
- Frontal lobe
2. Parietal lobe
What are some side effects of felbamate? Name 4.
Nausea, vomiting, diarrhea, aplastic anemia, hepatotoxicity
What are some side effects of tiagabine? Name 5.
Sedation, ataxia, tremor, diplopia, imbalance, headache, dizziness, acute confusion and encephalopathy
What are some side effects of perampanel? Name 3.
Dizziness, headache, sedation, homicidal ideation
What presents w/ days to weeks of mild confusion (despite being able to speak and walk), usually in patients w/ hx of generalized epilepsy but sometimes in healthy older adults?
Absence status epilepticus
EEG shows continuous generalized spike-and-wave discharges
What is included in the ABCD2 score for TIA?
Age >60, BP >140/90, clinical sx (focal weakness vs. speech impairment without weakness), duration (>60 min, or less), presence of diabetes
What needs to be performed after diagnosis of subarachnoid hemorrhage?
Catheter-based angiography for definitive diagnosis of aneurysms and other causes of SAH
What are some absolute exclusion criteria for tPA in patients w/ acute ischemic stroke?
Significant head trauma or prior stroke in past 3 months, non compressive site arterial puncture within 7 days, intracranial neoplasm or aneurysm, recent intracranial or spinal surgery, BP >185/110 despite treatment, active internal bleeding, active bleeding diathesis (plts <100, heparin within 48 hours + high PTT, anti-coagulation + INR >1.7, DOAC use within 48 hours), glucose <50, multilobar infarction with >1/3 of hemisphere involved
For how long should anti-platelets be held after stroke w/ hemorrhagic conversion?
7 days
What condition is behavioral-variant frontotemporal dementia associated with?
ALS
What medications can be used to lessen the wearing off phenomenon in Parkinsons?
Dopamine agonists (pramipexole, ropinirole, cabergoline), catechol-O-methyltransferase inhibitors (entacapone, tolcapone), or monamine oxidase B inhibitors (selegiline, rasagiline, safinamide)
What is the treatment for myasthenic crisis with prominent bulbar or generalized weakness?
IVIG or plasmapheresis before steroids
What condition has myoedema (muscle mounding after percussion) on exam?
Hypothyroid myopathy
What is the treatment for glioblastoma?
Resection if possible, followed by radiation and chemotherapy
Temozolomide, nitrosureas, bevacizumab
Which AEDs are preferred in CNS tumors, if AEDs are indicated?
Levetiracetam and lacosamide
What is a positive apnea test?
PCO2 >60 mmHg or >20 mmHg increase overbaseline, and no spontaneous respirations
Which AEDs cannot be used w/ OCPs due to decreased effectiveness?
Carbamazepine, oxcarbazepine, phenytoin, barbiturates, primidone, topiramate
What is the main difference between mild cognitive impairment and dementia?
Dementia causes occupational or social functioning
Mild cognitive impairment does not cause functional disability
What is the management for a patient age <60 with embolic stroke of unknown source and patent foramen ovale?
PFO closure
How do you differentiate dementia w/ lewy body from Alzheimer’s and other neurocognitive diseases?
REM sleep disorder - most common in dementia w/ lewy bodies
