Neuro Flashcards
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)
ALS
UMN and LMN disease - asymmetric limb weakness and bulbar dysfunction
- bulbar (medulla) dysfunction - dysfunction of CNs 9-12 (glossopharyngeal, vagus, accessory spinal, hypoglossal)
riluzole - glutamate (release) inhibitor, prolongs survival
- side effects - dizziness, N, elevated LFTs
death within 5 yrs
spinal cord injury
cord compression = medical emergency
- causes - spinal injury (disk herniation, compression fracture), malignancy (lymphoma, breast cancer mets), infection (epidural abscess)
- severe low back pain, worse in the recumbent position at night - due to distention of epidural venous plexus when lying down
- early - bilateral, LE weakness and absent DTRs
- late - UMN signs, paraplegia, bowel/bladder dysfunction
- reasons - descending CSTs affected (LE weakness, loss of rectal tone), ascending spinothalamic tract (sensation), descending autonomics (reticulospinal tract, urinary RT)
- emergency MRI of spine, IV glucocorticoids, rad-onc/surg consults
central cord syndrome
- hyperextension injury in elderly pts with pre-existing degenerative changes in c-spine (fall, whiplash)
- damage to central portion of anterior spinal cord - CST and LST
- weakness in upper extremities (because these correlate with the central part of the CST)
- can have loss of pain and temp in arms (due to damage to spinothalamic tract)
anterior ventral cord syndrome
- occlusion of ASA
- bilateral spastic motor paresis distal to lesion
Brown-sequard - hemisection of cord due to penetrating injury
- ipsilateral - weakness (LCST), spasticity, loss of vibration sense, and proprioception AT level of injury
- contralateral - loss of pain and temp, 2 levels below injury
- if this occurs in cervical region - can have ipsilateral Horner syndrome
(- compared to complete spinal transection - loss of everything, quadraplegia or paraplegia
posterior cord syndrome
- causes - MS, vertebral artery dissection
- bilat loss of vibratory and proprioceptive sensation, weakness, paresthesias
- urinary incontinence/RT
spinal cord infarct - abrupt symptoms
brain bleed
ICH - urgent non-contrast CT
- headache, AMS
Intraparenchymal hemorrhage
- BG (putaminal) hemorrhage - almost always involves internal capsule –> contralateral hemiparesis and hemianesthesia
- due to hypertensive vasculopathy - involves small penetrating branches –> formation of Charot-Bouchard aneurysms in deep brain structures –> rupture and bleed –> in BG, cerebellar nuclei/cortex, thalamus, and pons - AV malformation - most common cause of intraparenchymal bleed in kids
- symptoms - gradual onset
- symptoms include - ipsilateral hemitaxia (because corticopontocerebellar fibers decussate twice)
- spreading hematoma can lead to coma
- non-con CT and decompression imm
cerebral amyloid angiopathy - most common cause of lobar/cortical hemorrhage (occipital, parietal) in the elderly (age >60)
SAH - CT (and if needed–> get LP)
- due to berry aneurysms - will see blood collecting in brainstem and basal cisterns
- thunderclap headache, neuro deficits are UNcommon
- further evaluation with CT angio
SDH - tearing of bridging veins
- elderly, alcoholics (cerebral atrophy and increased fall risk)
- acute - slow bleed into subdural space, confusion is gradual (1-2d), impaired consciousness, increased ICP signs
- chronic - insidious, onset weeks after injury, somnolence, focal neuro deficits
epidural hematoma
- bleed doesnt cross suture lines
- sphenoid bone trauma –> tearing of middle meningeal artery
- uncal herniation - ispilateral CN3 palsy (down, out, and dilated due to loss of motor and PSNS), hemiparesis
- ddx by head CT
- urgent surgical evacuation for symptomatic pts
EMG
differentiates between nerve and muscle disease
stroke
greatest risk factor for both ischemic and hemorrhagic - HTN
- because it accelerates atherosclerotic process and promotes thrombi formation
- side note - cerebral amyloid angiopathy is the second most common cause of intracerebral hemorrhage (occurs in age >75)
if you suspect a stroke - get noncontrast CT head
cardioembolic stroke - gray-white junction
- if due bacterial endocarditis - you have septic embolism, treat with IV abx
- more sudden onset than atherosclerotic stroke
- embolic strokes are more likely to bleed than thrombotic strokes
ischemic stroke - hypodense region that will become evident >24hrs after event
- tpa window - 3.5-4hrs after sx onset (exclusion criteria is long, remember bleeding risk, prior stroke/head trauma in the last 3 mo, and recent intracranial/spinal surgery, BP > 185/110)
- stroke and not on ASA - add ASA imm
- stroke and on ASA - add clopidogrel (or dipyridamole)
- stroke + afib - long term anticoagulation (start outpatient)
ischemic stroke with hemorrhagic transformation - deterioration in pts neurologic status
- get emergent noncon CT –> urgent surgical decompression
intracerebral hemorrhage - focal neuro deficits and signs of increased ICP (vomiting and headache, bradycardia, reduced alertness)
- warfarin-associated intracerebral hemorrhage - give vitamin K and prothrombin complex concentrate (contains factors)
stroke in young
- PFO, IC dissection (trauma!, associated with Horners and varying degrees of hemiparesis), Ehlers-Danlos or Marfans, AVFs, moyamoya disease, drug abuse, hypercoagulable state
tx - lifestyle modification. Start asa, statin, and BP reduction.
Parkinsons
accumulation of a-synuclein in substantia nigra (BG) –> death of these neurons
2/4 for clinical ddx - resting tremor, rigidity, bradykinesia (hypokinetic, narrow based gait), postural instability
- symptoms present asymmetrically…
- other signs include mask-like facies
tremor - improves with activity (note essential tremor is the opposite)
- 4-5 Hz frequency
- starts on one hand before the other
- treat with trihexyphenidyl (anticholinergic)
rigidity - lead pipe or oscillating
postural instability - flexed axial posture, loss of balance during turning or stopping, loss of balance when pushed from stationary bipedal stance, frequent falls
drugs
- levodopa/carbidopa - side effect is somnolence and hallucinations
- entacapone - increases dopamine stimulation, side effect is dyskinesia (and other side effects of excess dopamine)
- amantidine - dopamine agonist, shown to delay dementia, side effects are ankle edema and livedo reticularis
- pramipexole - dopamine agonist (also used in restless leg syndrome)
- selegiline - MAOB inhibitor, side effect is insomnia and confusion
- note - increased dopamine –> nausea, orthostatic hypotension, dizziness, dyskinesia (long-term use), hallucinations (in order pts)
drug-induced parkinsonism - due to anticholinergics (benzotropine, diphenhydramine)
- side note - anticholingerics can improve tremor and rigidity but not bradykinesia
Shy-Drager syndrome - degenerative disorder
- parkinsonism
- autonomic dysfunction (orthostatic hypotension, impotence, incontinence, etc.)
- widespread neurologic signs (cerebellar, pyramidal, or LMN)
- treat by intravascular volume expansion (fludrocortisone, salt supplementation, a-agonists, compressive stockings)
conus medullaris
cauda equina
conus medullaris (UMN and LMN sxs)
- note - things that affect the spinal cord can cause UMN and LMN symptoms
- back pain with EARLY bladder and rectal dysfunction
- symmetric motor weakness, hyperreflexia
cauda equina - spinal roots (part of peripheral NS) compressed
- radicular pain, asymmetric LE weakness and hyporeflexia, saddle anesthesia, LATE bowel/bladder dysfunction
causes - cord compression due to mets, disc herniation/rupture, spinal stenosis, infection, tumor, hemorrhage, iatrogenic injury
management - emergency MRI, neurosurg eval
+/- IV glucocorticoids
- neurosurgical emergencies - due to risk of permanent loss of sphincter function
torticollis
focal dystonia of SCM
- dystonia - sustained muscle contraction –> abnormal movements
torticollis - congenital, idiopathic, trauma, local inflammation, drug-induced (antipsychotics, metoclopramide, prochlorperazine)
tremors
essential tremor, AD
- can affect UE, head, voice, etc.
- worsens with motion, arms extended, caffeine
- resolves during sleep, improves with alcohol consumption
- treat with propanolol (or primidone)
- orthostatic tremor is a variant of essential tremor - occurs in legs on standing, relieved by sitting down
Parkinsons - resting tremor of extremities
- basal ganglia dysfunction - progressive loss of dopaminergic neurons in basal ganglia
cerebellar (spinocerebellar ataxia, genetic condition) aka intention tremor - usu associated with ataxia (fall towards side of lesion), dysmetria, or gait disorder
- tremor increases as hand reaches target
physiologic
- low amplitude - 10-12 Hz, not visible normally
- onset with increased sympathetic activity (drugs, caffeine, anxiety)
- worsens with movement, can involve face and extremities
hemiballismus
due to damage of contralateral subthalamic nucleus
(- involved in the inhibition of movements)
thalamic stroke (aka lacunar stroke of thalamus)
- certain thalamic nuclei transmit sensory information from the contralateral side of the body and face
- stroke - contralateral (total) sensory loss
- can also have transient hemiparesis, athetosis, ballistic movements - because neighboring basal ganglia/internal capsule fibers are disrupted
- weeks to mo after stroke - thalamic pain syndrome (allodynia)
ID
fetal alcohol syndrome
1) small palpebral fissures
2) smooth philtrum
3) thin vermillion border
height, weight often < 10%
microcephaly + cognitive and behavioral disorders - social withdrawal, delays in motor an lang
treatment - early ddx is critical, therapy
DS is most common genetic cause of developmental delay
- flat facial profile, slanted fissures, small low-set ears, clinodactyly, etc.
Fragile X is the most common inherited reason for ID
- XD, CGG repeat
- long narrow face, prominent forehead and chin, large ears, macrocephaly, macroorchidism
- ADHD, autism
- female pts with the mutation may have normal intellect, may have mild ID
Infections
- CMV - ID, sensorineural deafness, blindness, jaundice, HSM, petechiae
- rubella - sensorineural deafness, cataracts, HSM, purpura
fetal phenytoin syndrome
hypoplastic fingers/nail and cleft lip/palate
give ppx vitamin K in first trimester - phenytoin may increase rate of fetal vitamin K degeneration
brain injury
cerebral contusion - heal without medical intervention
diffuse axonal injury - accel/decel injury
- coma
- head CT may show bleeds at grey-white junction
concussion - mild TBI, confusion, amnesia
- immediately after - rest for 1 day, reassess
- post-concussive syndrome - headache, confusion, amnesia, difficulty concentrating, vertigo, mood alteration, sleep disturbance, anxiety
- resolve few weeks to mo following TBI
Guillain-Barre
bugs - Campy (others are Herpes, Mycoplasma, H flu)
ascending polyneuropathy after respiratory tract or upper GI infection
demyelination of the peripheral motor nerves - neuropathic pain
flaccid paralysis, absent DTRs and nerve conduction velocities, severe back pain
- side note - absent DTRs and paresthesias = signs of NEUROpathy
- autonomic symptoms (ileus, BP, heart rate instability, orthostatic hypotension)
- respiratory compromise
CSF - elevated protein, normal cell count = albuminocytologic dissociation
+ use EMG for ddx
- infiltrating T lymphocytes and macrophages around nerver
plasmapheresis and IVIG
- and monitor autonomic and respiratory function
- assess pts pulmonary function by serial spirometry (FVC is gold std for assessing ventilation, decline in FVC indicates impending respiratory arrest –> tube)
typically - spontaneous recovery in a year
tick paralysis - neurotoxin in tick saliva –> paresthesias, fatigue, ascending muscle paralysis over hrs or days
- paralysis can be localized to or more pronounced in 1 leg/arm
- examine skin to remove tick –> will result in recovery
Wallenburg syndrome
lateral medullary infarct
- due to intracranial vertebral artery
- ddx on MRI
- give TPA
1) vertigo, falling to side of lesion, nystagmus, ipsilateral limb ataxia
2) abnormal facial sensation, loss of pain and temp in ipsilateral face and contralateral body
3) cranial 9 and 10 involvement - dysphagia, aspiration, hoarseness
(- contrast with lateral mid-pontine lesions - affect motor and principal sensory nuclei of trigeminal nerve)
4) autonomic dysfunction -
ipsilateral Horners syndrome, intractable hiccups, lack of autonomic respiration
medial medullary syndrome
branch occlusion of vertebral or ASA
contralateral paralysis or arm and leg
tongue deviation TOWARDS lesion
medial mid-pontine infarction
contralateral ataxia and hemiparesis of the entire body (face, trunk, limbs)
- variable loss of contralateral tactile and position sense
NM weakness
- UMN
- anterior horn
- peripheral nerves
- NMJ
- muscle fibers
UMN - leukodystrophies, vasculitis, brain mass in cortext, B12 deficiency (DCML)
anterior horn cells
- spinal muscular atrophy, ALS, paraneoplastic syndrome, polio
peripheral nerves
- hereditary primary motor sensory neuropathy
- GB
- diabetes
- amyloid neuropathy (myeloma)
- lead poisoning
NMJ
- MG, LES
- organophosphate poisoning
- botulism
muscle fibers
- muscular dystrophies (waddling gait, weak gluteal muscles)
- poliomyositis and dermatomyositis
- hypothyroidism (+ reduced DTRs)
- corticosteroids
- HIV myopathy
LOC
generalized seizures
- triggers - fever, hypoglycemia, sleep deprivation
- tongue biting, urinary incontinence, perioral cyanosis
- preceding aura, LOC, convulsions, post-ictal state
vasovagal syncope
- prolonged standing, stress
- presyncope (lightheadedness, pallor, diaphoresis, nausea)
- IMMEDIATE return to baseline
cardiogenic syncope (decreased CO in the setting of structural HD)
- exertion, dehydration
- sudden LOC (no prodrome), immediate return to baseline
v. s. breath-holding spells
- in 6 mo-6yrs
- cyanosis, pallor following an upsetting event or minor injury
- immediate recovery
HOCM (syncope on exertion), orthostatic hypotension (intravascular volume depletion, autonomic instability due b-blockers)
infective endocarditis
cardiac - valvular insufficiency, perivalvular abscess, conduction abnormalities, mycotic aneurysm
neuro - embolic stroke, cerebral hemorrhage, brain abscess, acute encephalopathy or meningoencephalitis
renal - renal infarct, glomerulonephritis, drug-induced interstitial nephritis (from therapy)
MSK - vertebral osteo, septic arthritis, MSK abscess
if you suspect - draw blood cultures, initiate broad-spectrum abx, echo
cerebellar lesion
broad-based gait, impaired heel-to-shin testing
posterior circulation issue
neurosyphilis
treponema pallidum - spirochetes directly damage the dorsal sensory roots
–> secondary degeneration of the dorsal columns
early - meningitis
late - dementia, tabes dorsalis (sensory ataxia, pos Romberg, lancinating pains, reduced DTRs), Argyll robertson pupils (accommodates but doesnt react), dysmetria (overshoot, undershoot of movements), neurogenic bladder
tertiary syphilis can cause uveitis
other features - decreased concentration, memory loss, dysarthria, tremors, irritability, headaches, personality changes and impaired judgement
treat with IV penicillin for 10-14d
PML
JC virus –> demyelinating disease
- affects immunocompromised - transplant pts
subacute AMS and multifocal neurologic deficits
drug overdoses
Wernickes encephalopathy - confusion (encephalopathy), horizontal nystagmus, gait ataxia
- due to thiamine deficiency
benzos - binds to GABA receptors
- slurred speech, ataxia, hypoT, depressed mental status
- give flumazenil
opioid OD - pinpoint pupils, respiratory depression
- naloxone
sleep
normal age-related sleep changes - decreased total sleep time, increased nighttime awakenings, phase advance (sleep earlier, wake up earlier)
headache
migraine - due to vasospasm
- often pos FHx
- unilateral, pulsatile and throbbing
- auras (can be paresthesias), photophobia, phonophobia, nausea
- basilar migraine - can have basilar aura sxs (vertigo, dysarthria, tinnitus, diplopia)
- abortive - triptan, NSAID, acetaminophen, antiemetics, ergots
- triptans - serotonin agonist –> vasoconstriction, decreases neurogenic inflammation - preventives - topiramate, divalproate, TCADs, b-blockers
- topiramate - can cause renal stones
- valproate - hepatotoxic
cluster
- M>F
- onset during sleep
- can occur in paroxysms
- behind one eye, excruciating, sharp and steady
- sweating, facial flushing, nasal congestion, lacrimation, pupil changes
- ppx with verapamil, Li
- acute attack can be aborted by 100% O2 (or subQ sumatriptan)
trigeminal neuralgia - maxillary and mandibular disorders
- tx - carbamazepine
tension
- stress, band-like pattern around head, muscle tenderness in head/neck/shoulders
- temporal and occipital regions
- use ibuprofen, aspirin, and caffeine to treat
kids - headaches are a common complaint
- most common = migraines - but will be bifrontal, shorter in duration, other sxs similar
- occipital headache is CONCERNING
- treat with supportive management –> triptans
- image if coordination difficulties, numb/tingling, focal neuro, headache that wakes you from sleep, increasing frequency
when would you image
- neuro findings - seizures, deficits, changes in consciousness
- change in nature
- onset age > 40, sudden onset, trauma, present on awakening
cancer pain, bony mets
mild - acetaminophen, NSAIDS (naproxen has a longer duration than ibu)
moderate - weak opioids and non-opioids
- codeine, hydrocodone, tramadol
severe - short-acting opioids
- morphine, hydromorphone
- calculate daily dose, convert to long-acting formulation (fentanyl patch, 72hr duration + oxycodone) PLUS short-acting opioids for breakthrough pain
glucocorticoid injections can be used as adjunctive therapy if there is incomplete improvement on opioid therapy
thalamic stroke
ischemia of sensory thalamic nuclei - contralateral loss of sensation and tingling
- delayed episodic, severe pain with ongoing paresthesias (Dejerine-Roussy syndrome)
