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137 Cards in this Set

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What does the presence of Todd's paresis imply?
The seizure was partial in onset, then secondarily generalized.

When is imaging indicated for a seizure?
As a general rule, always get an MRI after any new-onset seizure.
What percentage of epilepsy patients achieve control with monotherapy?
70%
What is the first thing that should be given in status epilepticus, even before drugs?
100 mg IV thiamine with D5W infusion.
What should be administered if status epilepticus does not remit with IV lorazepam?
IV fosphenytoin
What should be administered if status epilepticus does not remit with IV fosphenytoin?
IV phenobarbital
What should be administered if status epilepticus does not remit with IV phenobarbital?
Induce coma with barbiturates, midazolam, propofol
Three classic toxicities: phenytoin
Gingival hyperplasia
Coasening of facial features
Ataxia
Three classic toxicities: carbamazepine
SIADH/hyponatremia
Agranulocytosis
Diplopia
Rare toxicity: vigabatrin
Irreversible optic nerve atrophy
Which AED: arrhythmias, hypotension
Phenytoin
Toxicities: valproate
Tremor
Weight gain
Hair loss
Hepatotoxicity
Thrombocytopenia
Classic toxicity: lamotrigine
Stevens-Johnson syndrome
Seizure features favoring temporal localization
Gustatory phenomena
Olfactory phenomena
Psychic phenomena (deja vu, jamais vu, derealization, etc)
Dx: patient with altered consciousness/awareness and automatic behaviors
Complex partial seizure
Classic features of a tonic-clonic seizure
Rigid tone --> symmetric myoclonus
Inconinence
Postictal confusion/somnolence lasting minutes to hours
EEG finding: absence seizure
3Hz spike-and-wave forms
MCC of seizures in childhood
Febrile seizure
Dx: teen with myoclonus upon awakening
Juvenile myoclonic epilepsy
Optimal treatment: juvenile myoclonic epilepsy
Valproate
Dx: seizure syndrome classically exacerbated by sleep deprivation
Juvenile myoclonic epilepsy
MCC of intractable complex partial seizures in adults
Mesial temporal sclerosis
Features of infantile spasm
Paroxysmal flexion movements with profoundly disorganized EEG pattern ("hypsarrhythmia")
Features of Landau-Kleffner syndrome
Loss of language function + abnormal EEG during sleep
Features of Lennox-Gastaut
Cognitive dysfunction, multiple seizures of different types, and 1-to-2 Hz generalized spike-wave forms on EEG
Treatment of choice: myoclonic seizures
Valproate
Treatment of choice: infantile spasm
Vigabatrin
Which medications MAY alter the course of Parkinson's disease?
MAOI-B (selegiline, rasagiline)
List non-classical Parkinson's features
Pain
Hypo-/anosmia
Sleep disorders
Orthostatic hypotension
Drugs that can cause essential tremor
SSRIs
TCAs
Beta agonists
Lithium
Amiodarone
Valproate
What are the two primary medications used for essential tremor?
Propranolol and primidone
What is the primary component of Lewy bodies?
Alpha-synuclein
Causes of drug-induced Parkinson's disease
Antipsychotics
Antiemetics
Some Ca2+ channel blockers
Amiodarone
Some immunosuppressants
List common DA agonists used in Parkinson's
Bromocriptine
Pramipexole
Ropinirole
Cabergoline
Patient diagnosed with Parkinson's doesn't respond to Sinemet: what is the next step?
Reconsider original diagnosis
What is the best pharmacologic approach to management of Parkinson's tremor?
Anticholinergics e.g. trihexyphenidyl
List some surgical approaches to Parkinson's management
Pallidotomy/thalamotomy
Deep brain stimulation of GPi
Which category of Parkinson's patients respond best to deep brain stimulation?
Patients that still derive benefit from L-dopa
Classic triad: Dementia with Lewy Bodies
1. Dementia
2. Bx abnormalities, hallucinations
3. Parkinsonism
Classic S/Sx: progressive supranuclear palsy
Parkinsonism
Extraocular muscle abnormalities
Prominent gait disturbances with frequent falls early on
Poor response to L-dopa
Marked dysphagia, dysarthria
Classic S/Sx: corticobasal degeneration
Classically begins with clumsiness, stiffness, or jerking of one arm, which then spreads to the ipsilateral leg, then the contralateral limbs
Parkinsonism
Alien hand syndrome
Classic S/Sx: multisystem atrophy
1. Parkinsonism
2. Cerebellar ataxia
3. Dysautonomia
4. Partial response to L-dopa
S/Sx: ataxia-telangiectasia
Gait ataxia in childhood, possible with parkinsonisms
Difficulty with saccades
Telangiectasia of the face
What is the most common presentation of juvenille Huntington's?
Parkinsonism
What drug can help with the chorea of Huntington's?
Tetrabenazine
MCC of subarachnoid hemorrhage
Berry aneurysm
Chronic disease most strongly associated with intraparenchymal hemorrhage
HTN
What stroke distribution can cause alexia without agraphia?
Dominant hemisphere PCA occlusion
In what type of patient would you expect a venous stroke?
Coagulopath
What is the most significant acute neurologic risk in stroke?
Increased ICP secondary to edema and/or hemorrhage
Patients with cardioembolic stroke should...
...be on warfarin.
Patients with TIA/stroke and significant carotid stenosis should...
...undergo endarterectomy.
All patients with TIA/stroke who are not on warfarin should...
...receive antiplatelet therapy.
LDL goal for TIA/stroke patients
<100
What is the main contraindication to anticoagulation after cardioembolic stroke?
Bacterial endocarditis
All stroke patients should be admitted and be put on telemetry: why?
Stroke can be arrhythmogenic; you may also uncover unknown afib or other arrhythmia as a cause of stroke
Target INR after cardioembolic stroke
2-3
Evidence suggests that which antiplatelet regimen has the greatest absolute risk reduction for second stroke?
ASA + dipyridamole
During NCS, amplitude corresponds to...
Number of fibers
During NCS, conduction velocity corresponds to...
...myelination.
Most common initial site of weakness in ALS
Arm
____ are a common and often very significant feature in all patients with ALS
Fasciculations
What is the classic pattern of spread in ALS?
UE ---> contralateral UE ---> ipsilateral LE ---> contralateral UE ---> bulbar muscles
Which muscle groups are spared in ALS?
Extraocular muscles, as well as bladder and bowel sphincters
MCC of death in ALS
Respiratory failure, aspiration pneumonia
Patients with ALS have a high incidence of cognitive deficits consistent with what process?
Frontotemporal dementia
What is the only agent shown to prolong survival in ALS?
Riluzole
MCC of radiculopathy
Degenerative spinal column disease (vertebral bodies, facet joints, or discs)
Which plexus is more commonly affected in autoimmune plexopathy?
Brachial
What intervention has been shown to improve outcomes in Bell's palsy?
10-day course of prednisolone
MCC of mononeuritis multiplex outside of diabetes
Vasculitis
DANG THERAPIST mnemonic
Diabetes
Alcholol
Nutritional
Guillain-Barre
Toxic
HEreditary
Recurrent
Amyloid
Porphyria
Infectious
Systemic
Tumors
____ is effective for CIDP, but not for AIDP.
Prednisone.
LEMS tends to involve ____, which are spared in myasthenia gravis.
Autonomics
Standard of care for myasthenia gravis
Pyridostigmine + an immunosuppressant
Common endocrine causes of myopathy
Hyperthyroid
Hypothyroid
Hyperparathyroid
Cushing's
Common medication-induced myopathies
Corticosteroids
Statins
Colchicine
Hydroxy/chloroquine
MCC toxic myopathy
Alcohol
MCC inflammatory myopathy in childhood
Dermatomyositis
MCC inflammatory myopathy age >50 years
Inclusion body myositis
Classic pattern of involvement: inclusion body myositis
Weakness of UE flexors and knee extensors, often asymmetric; dysphagia is also very common
Deranged cell line: dermatomyositis
Humoral immunity
Deranged cell line: polymyositis
Cell-mediated immunity
Classic histology: inclusion body myositis
Beta amyloid and hyperphosphorylated tau protein (the same as in Alzheimer's)
Which inflammatory myositis is associated with significantly elevated risk of malignancy?
Dermatomyositis
Treatment: inclusion body myositis
No known treatment; does not respond to steroids, immunosuppressants, etc.
Inclusion body myositis has inclusions found in what other two neurologic diseaes?
ALS and frontotemporal dementia
Any patient with rapid deterioration from a condition that has the potential to affect respiratory muscles should be monitored with...
Frequent bedside FVCs and NIFs
At what FVC is intubation indicated?
10-12mL/kg
What is it critical to evaluate for when assessing a patient with reported cognitive decline?
Depression.
Define "mild cognitive impairment."
An acquired and persistent deficit in one or more cognitive domains that is more severe than you would expect from aging alone but not severe enough to interfere with social and occupational capacities.
Age of onset in sporadic Alzheimer's disease correlates with...
...the number of ApoE4 alleles a person has.
Alzheimer's plaques: intracellular or extracellular?
Extracellular
Alzheimer's severity correlates better with tangle burden or plaque burden?
Tangle burden
What percentage of cases of dementia are due to Alzheimer's?
60%
Which lobes are most affected in Alzheimer's?
Temporal and parietal
Which forebrain nucleus is prominently affected in Alzheimer's?
Nucleus basalis of Meynert
Where are neurofibrillary tangles found within neurons?
Axon hillock
Most prominent symptom of Alzheimer's
Memory loss
Up to 40% of Alzheimer's patients develop...
...parkinsonisms.
Associated symptoms in Alzheimer's
Psychiatric issues
Myoclonus
Incontinence
Seizures
CSF evaluation in Alzheimer's
Low beta amyloid, high tau
MOA: memantine
NMDA antagonist
Four treatments for Alzheimer's
Memantine
Galantamine
Rivastigmine
Donepezil
Standard Alzheimer's treatment algorithm
Start with an AChE inhibitor at time of diagnosis, then add memantine when the dementia becomes moderately severe
Three key features distinguishing dementia with Lewy bodies from Alzheimer's
1. Early-onset parkinsonisms
2. Early-onset hallucinations
3. Fluctuations in cognition/mental status
Cognitive deficits: DLB vs. Alzheimer's
Compared to Alzheimer's, DLB patients tend to exhibit fewer memory deficits and more executive functioning deficits
Neurochemical abnormalities in DLB
A combination of Parkinson's and Alzheimer's: dopaminergic depletion from substantia nigra and cholinergic depletion from nucleus basalis of Meynert
Neuropathology: corticobasal degeneration
Accumulation of tau protein
Composition: Pick bodies
Tau protein
Classic findings: frontotemporal dementias
Disinhibited behaviors and language deficits ("semantic dementia")
Dx: rapid cognitive deterioration with frequent myoclonus
CJD
Diagnosis: narcolepsy
Two or most episodes of REM within 15 minutes + mean sleep latency of less than 8 minutes
Most common PNS manifestation of HIV
Distal, symmetric peripheral polyneuropathy
Most common CNS manifestation of HIV
AIDS dementia: progressive with pronounced apathy and social withdrawal
Work-up: HIV+ patient with headaches or mental status abnormality
MR head + LP
Work-up: HIV+ patient with multifocal brain lesions
Toxo IgG titer
MC manifestation: Neuro-lyme
Subacute or chronic meningitis weeks to months after innoculation, often with spontaneous remission
Tx: neuro-lyme
Two weeks IV ceftriaxone
MC CNS manifestation of tuberculosis
Basal meningitis with cranial nerve abnormalities
CSF in CNS tuberculosis
High opening pressure
High protein
Low glucose
Lymphocytic pleocytosis
Tx: CNS tuberculosis
Two months of INH, rifampin, ethambutol, and pyrazinamide, then another 7 months of just INH and rifampin
Which HSV: aseptic meningitis
HSV-2
Which HSV: adult encephalitis
HSV-1
Which HSV: neonatal encephalitis
HSV-2
Classic MRI: HSV encephalitis
Temporal lobe microhemorrhage
MCC: fatal encephalitis
HSV
Tx: zoster
Famciclovir or valacyclovir
Most common structure affected by MS
Spinal cord (50%)
"Lhermitte's phenomenon"
Electric sensation passing down the back and into the limbs upon neck flexion in MS patients
Most common symptom in MS
Weakness in one or more limbs (40%)
Histology: MS plaque
Perivascular collections of macrophages and CD4+ cells with marked focal demyelination and some axonal damage
MOA: natalizumab
Mab against α4 integrin
The only drug FDA approved for secondary-progressive MS
Mitoxantrone
Limiting toxicity: mitoxantrone
Cardiomyopathy
MC CNS manifestations of SLE
Neuropsychiatric: cognitive impairment, psychosis, altered consciousness
Classic neurologic manifestation of PAN
Mononeuritis multiplex