Neuro Flashcards
(131 cards)
seizures
seizures - excitatory activity in the cortex
cortical dysplasia - congenital, seizure focus
absence - …, simple automatisms present (eyelid fluttering, lip smacking), easily provoked by hyperventilation
- 3Hz spike on EEG
- NO post-ictal phase
- comorbidities - (inattentive) ADHD, anxiety
- treat ADHD with methylphenidate and atomxetine (non-stimulant) - YES provoked by hyperventilation
- treat - ethosuximide
focal seizures
- may generalize
- can have impairment of consciousness
- automatisms - involve both hemispheres (are seen in focal seizures with impairment of consciousness)
- NOT provoked by hyperventilation
temporal lobe epilepsy
- olfactory aura or deja vu
- affective sxs - rising sensation in stomach
- pie in the sky visual loss (left superior quadrant loss) will be on opposite side of lesion- Meyer loop
juvenile myoclonic epilepsy
- generalized seizure, most commonly myoclonic jerks after the first hour after awakening
Lennox- Gastaut - presents by age 5
- ID, severe seizures
- slow spike-wave pattern on EEG
Sturge-Weber syndrome
- seizures, ID, nevus in trigeminal territory (represents congenital unilateral cavernous hemangioma)
- other findings - hemianopia, hemiparesis, hemisensory disturbance
- ipsilateral glaucoma
- tramline intracranial calcifications
1) CT (urgent situation) or MRI (elective situation) should be performed in all pts with an apparent unprovoked seizure
- MRI is more sensitive in identifying most structural causes of epilepsy - temporal sclerosis, cortical dysplasia, vascular malformations, TBI, infarct, tumor, infection
2) EEG
hypocalcemia - severe will cause tetany and seizures
- but note in DiGeorge - this will occur in the newborn period only (overtime, compensatory parathyroid hyperplasia occurs…)
hypoglycemia
hyponatremia
Todd paralysis - transient limb weakness following partial seizure activity
- hemiplegia
(- pathophys is unknown - but though to involve neuronal exhaustion/inhibition in the postictal period)
status epilepticus - historically single seizure that lasts longer than 30 min
- recent study - brain that has seized for >5 min is at increased risk for developing permanent injury (due to excitatory cytotoxicity)
- cluster or seizures with pt normal recovering normal mental status in between
- increased risk of cortical laminar necrosis - cortical hyperintensity on diffusion-weighted imaging (=infarct)
- give lorazepamx2, thiamine, and glucose
- fosphenytoin (IV version)
- then can try phenobarb, other anti-epileptics - this is considered refractory status epilepticus
lamictal - good for prevention of primary generalized seizures AND partial seizures
dopamax
valproate - side effects include hair loss, tremor, TCP
- neural tube defects, dysmorphic facies
phenytoin
- drowsiness, diplopia, ataxia - and long-term bad side effects if used in young people
- gingival hyperplasia, SJS-TEN
- fetal hydantoin syndrome - facial and cardiac defects
tuberous sclerosis
intellectual disabilities, behavioral problems, epilepsy
adenoma sebaceum - appears between 5-10yrs of age
cardiac rhabdomyomas, renal angiomyolipomas, facial angiofibromas, hypopigmented macules (ash leaf spots)
narcolepsy
daytime sleepiness, hypnagogic hallucinations, sleep paralysis (inability to move soon after awakening), cataplexy
treat with sleep hygiene, scheduled naps, modafinil (promotes wakefulness)
note - sedating drugs (alcohol, benzos, antipsychotics) should be avoided in pts with narcolepsy
Tourettes
kids may have OCD
neurofibromatosis
type 1: AD
- cafe-au-lait macules
- neurofibromas - skin exam yearly (some lesions may undergo malignant transformation)
- lisch nodules (tan hamartomas of iris)
- other - optic gliomas, macrocephaly, feeding problems, short stature, learning disabilities, clustered freckles (axillary)
type 2:
bilateral acoustic neuromas, cataracts, meningiomas, ependymomas
Wilsons disease
AR
child and adolescents - liver disease (asymptomatic transaminitis to fulminant liver failure)
young adults - neuropsych disease (tremor, rigidity, depression, paranoia, catatonia)
ddx by low serum ceruloplasmin < 20 mg/dL + increased urinary Cu excretion
- Kayser-Fleischer rings
- atrophy of lenticular nucleus
can see Mallory hyaline bodies on liver bx (but generally, this is more suggestive of alcohol liver injury)
multiple sclerosis
Uhthoffs phenomenon - symptoms worsen with exposure to high temps
bilateral internuclear ophthalmoplegia
- only one eye is able to abduct - affected eye (ipsilateral) is unable to adduct to follow
- damage to heavily myelinated fibers of the MLF
if you suspect - get MRI (T2)
- LP for oligoclonal IgG bands afterwards, esp if dx is not clear
acute exacerbation - treated with glucocortiocids (IV methylprednisolone)
- plasmapheresis in pts refractory to steroids
- acute exacerbation will last day-weeks
optic neuritis - subacute painful vision loss (in 1 eye) with abnormal pupillary response to light in affected eye (APD, Marcus Gunn pupil)
- ddx by MRI of orbits and brain
- fundoscopy is normal as inflammation occurs behind optic nerve head
- common initial presentation of MS
segmental demyelination/inflammation = tranverse myelitis - can occur with MS
- subacute onset of flaccid paralysis (due to spinal shock), loss of ALL sensation below level of injury (typically occurs in thorax)
- get spinal MRI
CSF findings - mild CSF pleocytosis (increased WBC counts)
- protein is nl to mildly elevated
disease modifying agents - INFB, natalizumab (and other -mabs), glatiramer acetate, fingolimod, dimethyl fumarate
drugs you DONT use in the elderly
benzos - linked to confusion and falls, paradoxical agitation (will be seen w/i an hour of the dose)
Trinucleotide repeats
Friedreich ataxia
AR - due to GAA repeats –> abnormality in frataxin protein
- genetic counseling for future pregnancies
limb and gait ataxia (broad based gait), loss of reflexes, loss of vibratory and position sense
- most pts wheelchair bound by 25
- death by 30-35
CV - necrosis and degeneration of cardiac muscle fibers, may see abnormalities on EKG
- can have cardiac arrhythmia –> death
Huntingtons - AD
- atrophy of caudate and putamen, enlargement of lateral ventricles (box car ventricles)
- chorea, athetosis (slow, writing movements of hands and feet)
- mood disturbances
neuroblastoma
median age - 2yo
derived from neural crest cells
- firm, nodular mass located in abdomen usu - adrenals or retroperitoneal ganglia
- calcifications and hemorrhages seen on imaging
70% of pts will have metastatic dz when diagnosed - long bones, skull, bone marrow, liver, LNs, skin
urine HVA an VMA are elevated
Wilms tumor - from metanephros
syringomyelia
anterior commissure, then anterior gray horn (LMN damage) - damage involves crossing fibers of spinothalamic tract (pain and temp) and upper extremity motor fibers
- preservation of DCML (light touch, vibration, position sense)
associated with Arnold Chiari malformations (type 1), prior spinal cord injuries (whiplash injury), inflammation, infection, tumor
cervical spondylosis
pts > 40 - due to disc herniation
sxs - neck pain and stiffness, signs of radiculopathy and myelopathy
syncope
- neurocardiogenic* aka vasovagal - prolonged standing, emotional distress, painful stimuli
- prodrome - nausea, warmth, diaphoresis
- excess vagal tone - profound hypotension and bradycardia
situational - cough, micturition, defecation
orthostatic
- clues are that this doesnt occur when lying down
aortic stenosis, HCM, anomalous coronary arteries - syncope with exertion or exercise
- dyspnea, CP, fatigue on exertion
v. arrhythmias - hx of CAD, MI, cardiomyopathy, or decreased EF
sick sinus syndrome, brady, AV block - sinus pauses, increased PR or increased QRS duration
Torsades - hypoK, hypoMg, prolonged QT (meds)
Congenital long QT - fhx of sudden death, long QT, syncope with triggers (exercise, startle, sleeping)
TIAs that affect posterior circulation and brain stem can cause syncope - rare
can have seizure like activity during syncope - but this is considered nonepileptic in nature
vitamin issues/absorption issues
subacute combined degeneration
- degeneration of dorsal and lateral spinal tracts - B12 deficiency
- progressive symmetric sensory loss, impaired proprioception/vibration, sensory ataxia
- late - spastic muscle weakness
- can have UMN signs
- get serum MMA levels
d-lactic acidosis - occurs in pts with short bowel syndrome
- unabsorbed carbs, intestinal bacteria –> d-lactic acid –> absorbed
- usu asx but can get confusion, ataxia, and dysarthria with carb loading
anterior spinal cord syndrome
flaccid paralysis (due to spinal shock)
- loss of pain/temp below level of injury (STT affected)
- bilateral hemiparesis - LCST affected
- can have autonomic dysfunction
risk factors - aortic dissection, surgery, trauma (injury to anterior spinal artery, due to disc retropulsion, vertebral burst fracture)
get MRI
treatment
- decompression procedure
sellar mass
benign - pituitary adenoma, craniopharyngioma (Rathke’s pouch, kids), meningioma, pituicytoma
- craniopharyngioma - cystic structures with calcifications
malignant tumors - primary (lymphoma, etc.), mets (breast, lung)
glaucoma
open-angle - gradual loss of peripheral vision in both eyes –> tunnel vision
- increased cup:disc ratio
acute angle closure
- due to impaired drainage of aqueous humor into anterior chamber
- sudden onset pain and redness (conjunctival injection) of one eye, nonreactive mid-dilated pupil
- can see halos around lights
- spontaneous, triggered by meds (decongestants, antiemetics, anticholinergics like trihexyphenidyl used in Parkinsons, or tolterodine)
- tonometry –> gonioscopy (psrimatic lens to visualize iridocorneal angle)
- treat - pressure-lowering drops, laser/surg interventions
congenital glaucoma - sensitivity to light, excessive lacrimation
pseudotumor cerebri (IIH)
obese women, <45 yo, meds (tets, vitamin A excess, GH, steroids, OCPs)
headache (worse when lying flat), transient visual symptoms (can be vision loss), pulsatile tinnitus, diplopia
papilledema, visual field loss, sixth nerve palsy
MRI (to look for mass or hemorrhage) +/- MRV (to look for cerebral vein thrombosis)
- note empty sella is present in 70% of pts
- LP with opening pressure > 250 mm H2O
treat with acetazolamide (weight loss, stop offending meds)
if left untreated - risk of blindness
cerebral palsy
nonprogressive motor dysfunction
1) spastic
2) dyskinetic
3) ataxic
spastic diplegia - see in premies
- hypertonia, hyperreflexia in LE
- feet are down and in (equinovarus)
- clasp-knife rigidity
risk factors - prematurity (before 32 wks), IUGR, intrauterine infection, antepartum hemorrhage, placental pathology, multiple gestation, mat alcohol consumption, mat tobacco use
treat with - therapy, baclofen or botox for spasticity
comorbidities - ID, epilepsy, strabismus, scoliosis
ICP
causes - brain parenchyma, CSF, blood
headaches worse in the morning
papilledema - enlarged blind spot
- can cause momentary vision loss that varies according to head position
- can rapidly lead to permanent vision loss
reducing ICP - head elevation, IV mannitol, hyperventilation, removal of CSF, sedation (decreases metabolic demand, controls HTN)
- side note
- vent rate affects pCO2
- pO2 - affected by inhaled O2 or end-exp pressure
uncal herniation - CN3 palsy + ipsilateral posterior cerebral artery compression (contralateral homonymous hemianopsia)
- compression of reticular formation –> altered level of consciousness, coma
cushings reflex - HTN, bradycardia, respiratory depression
- concerning sign of brainstem compression
hydrocephalus
infants
- poor feeding, irritability, decreased activity, vomiting
- bulging fontanelle, prominent scalp veins, widely spaced sutures, rapidly increasing head circumference
get CT
VP shunt
vertigo
BPPV - calcium crystals in semicircular canals
- room spinning when turning head, nystagmus, nausea
infarct of medial vermis of cerebellum - vertigo, nystagmus
lateral cerebellar infarct - dizziness, ataxia, weakness, tendency to sway TOWARDs lesion
Menieres - excess endolymphatic fluid in inner ear
- triad of episodic dizziness, low-freq hearing loss, tinnitus
- can have significant vertigo that lasts for days
- treat with salt restriction and diuretics
vertebrobasilar insufficiency - vertigo + dysarthria, diplopia, numbness
AG tox:
ototoxicity
vestibulopathy - pts dont experience vertigo because there is no left/right imbalance
- both vestibular organs are affected equally
- head thrust test is abnormal - head moves away from target –> eyes move away from target and horizontal saccade back
oscillopsia - objects moving around in visual field
- gait disturbances
dementia
normal aging - mild cognitive impairment (no interference with daily activities) - dementia
pts with cognitive decline –> neurocognitive testing = MMSE
- MMSE <24 is dementia
- 20-24 = mild dementia
- 13-20 = moderate dementia
<12 = severe dementia
- THEN perform thyroid function and B12 levels
Alzheimers disease most common dementia in US) - linked to a loss of cholingeric neurons
- amyloid plaques
- memory loss first, insidious
- lang deficits, spatial disorientation, late personality changes, myoclonus, seizures, urinary incontinence
- psychotic features may appear in the middle of the disease course - delusions are commo
- life-expectancy of 3-8yrs after diagnosis
- dx requires 2 or more areas of cognitive defects
- CT will show atrophy - more prominent in parietal and temporal lobes, hippocampi (mesial temporal atrophy)
- treat with AchEI - donepezil, galantamine, rivastigmine
- memantine (NMDA antagonist) - approved for mod-severe dementia
Vascular dementia
- stroke event may be described as a fall
- stepwise decline
- early executive dysfunction
- cerebral infarct (hypodense), deep white matter changes (from chronic ischemia)
FTD (Picks)
- early personality changes, disinhibition (including disinhibition of primitive reflexes, so they may be present on exam)
- language changes
- late - mute, immobile, incontinent
Lewy body
- visual hallucinations (sees kids or animals), spont parkinsonism (symmetric and rigidity, whereas parkinsons will have unilateral effects), fluctuating cognition, visuospatial dysfunction (falls)
- memory loss is late
- eosinophilic intracytoplasmic inclusions - a-synuclein
- tx - cholinesterase inhibitors
- dont give typical antipsychotics - because these pts may exhibit neuroleptic hypersensitivity
NPH - wet, wobbly, wacky
- cause is diminished CSF absorption or obstructive hydrocephalus
- normal opening pressure on LP
- initially large volume LPs –> VP shunt
Prion (CJD) - sporadic, rapid progression, startle myoclonus, behavioral changes
- other features - akinetic mutism, cerebellar or visual disturbance, hypokinesia
- periodic sharp triphasic wave complexes on EEG and/or pos 14-3-3 CSF assay
- definitive ddx by brain bx findings (spongiform changes) or demonstrated PRNP gene mutations
- end-stage - people lose the ability to move and speak –> coma
- death w/i 1 yr of onset
pseudodementia - cognitive changes assoc with MDD
- concerning - weight loss and social isolation, sleep changes
polypharmacy, OSA, electrolyte disturbances, anemia
work-up - CBC, CMP, meds that they are on, TSH, B12 (treat anything below 400), MRI (get w/ and w/o contrast)
- others - CSF studies, ESR, PET, RPR, UA
- travel hx - Lyme disease for people who have traveled to the NE US
- AAN work-up - recommends neuroimaging, screening for depression, hypothyroidism, and B12 deficiency
side note - treatment options are poor for dementia
- and treatment does not change prognosis
avoid antipsychotics (atypical and typical), can give a tiny bit of haldol, mood stabilizers are good if pt becomes aggressive
myasthenia gravis
lambert eaton
dermatomyositis/polymyositis
all three are paraneoplastic syndromes
myasthenia:
presents earlier in women - 20s-30s, men 60s-80s
- antibody against nicotinic Ach receptor - lesion considered in the motor endplate
- px - ocular (diplopia), bulbar (dysarthria, dysphagia), symmetric proximal weakness
- myasthenia crisis - precipitated by surgery (esp thymectomy interestingly enough), anesthetics, infection, meds (Ags, b-blockers), pregnancy/childbirth
- sxs - generalized and oropharyngeal weakness, respiratory insufficiency/dyspnea
- during a crisis - hold pyridostigmine in order to reduce excess airway secretions and aspiration risk
- tx - intubate, plasmapheresis/IVIG and corticosteroids
- monitor vital capacity and max inspiratory force
ddx
- edrophonium/tensilon test (AchE inhibitor, immediately improves symptoms), ice pack tests (ice pack will relieve ptosis)
- AchR antibodies - highly specific
- CT scan - thymoma
treat
1) AchE inhibitors -
pyridostigmine
2) immunotherapy (cyclosporine), thymomectomy
Lambert-Eaton syndrome - diminished/absent DTRs
- symmetric proximal limb weakness, autonomic dysfunction
- assoc with small cell lung cancer
- treat with guanidine or 3,4-diaminopyridine (can add IVIG/immunosuppressants)
dermatomyositis/polymyositis
- symmetrical + proximal muscle weakness
- reflexes will be normal (other than in a diabetic.. confounds exam)
- other findings - ILD, esophageal dysmotility, Raynaud phenomenon
- polyarthritis
- skin findings in dermatomyositis - Gottrons papules (violaceous plaques, scaly, overlie MCP), heliotrope rash
- mediated by cytotoxic T lymphocytes
- dx - elevated CPK, aldolase, LDH (get EMG and bx in cases of uncertainty)
- tx - high-dose glucocorticoids + glucocorticoid sparing agent + screen for malignancy (age related)