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NeuroPath (Movement disorders (akathisia (restlessness (neuroleptic use or…
NeuroPath
Movement disorders
akathisia
restlessness
neuroleptic use or PD
asterixis
flapping wrists - extension
Hep encephalitis, wilson disease, metabolic dz
athetosis
slow, snake-like writhing movements, esp fingers
basal ganglia
chorea
jerky, purposeless movements
basal ganglia
sydenham chorea
in acute rheumatic fever
dystonia
sustained involuntary contractions
writer's cramp, blepharospasm, torticollis
essential tremor
high frequency tremor with sustained posture worsens with movement or anxiety
familial. alcoholic which decrease tremor amplitude
nonselective BBlockers [propanolol], primidone
hemoballismus
sudden flailing of 1 arm and ipsilateral leg
contralateral subthalamic nucleus [lacunar stroke]
intention tremor
slow zigzag motion when pointing/extending to target
cerebellar dysfunction
myoclonus
sudden, brief, uncontrolled contraction
jerks, hiccups. common in metabolic[renal/liver failure]
resting tremor
uncontrolled movement of distal appendages. alleviated by intentional movement
substantia nigra
pill-rolling. PD
Spinal cord lesions
poliomyelitis and Wednig-Hoffman dz
congenital degeneration of anterior horns. "floppy baby" hypotonia, tongue fasciculations. ~7mo.
AR inheritance
poliomyelitis --> asymmetric weakness
Werdnig-Hoffman --> symmetric weakness
ALS - amyotrophic lateral sclerosis "Lou Gehrig disease"
combine UMN and LMN deficits without sensory or bowel/bladder deficits
can be caused by defect in superoxide dismutase 1
asymmetric limb weakness, fasciculations, eventual atrophy. treat: riluzole. FATAL
complete occlusion of anterior spinal artery
spares dorsal column and Lissauer tract. mid thoracic ASA is watershed area [above T8]
UMN deficit below lesion and LMN at level of lesion, loss of pain/temp below lesion
tabes dorsalis
tertiary syphilis. demyelination of dorsal columns and roots --> sensory ataxia [impaired proprioception and coordination]
assc. charcot joints, shooting pain, argyll-robertson pupils.
absent DTRs and + romberg sign
syringomyelia
syrinx expands and damages anterior white commissure of spinothalamic tract [2nd order neurons] --> bilateral loss of pain/temp sensation in cape-like distribution
seen in chair 1 malformation. can expand and affect other tracts
vit. B12 deficiency
subacute combined degeneration - demyelination of spinocerebellar tracts, corticospinal, and dorsal columns
ataxic gait, paresthesia, impaired position/vibration sense
cauda equina syndrome
unilateral sxs: radicular pain, absent knee/anl;e reflexes, loss of bladder/anal sphincter control. saddle anesthesia
tx: emergent surgery and steroids
compression of spinal roots from L2 and below, caused by intervertebral disk herniation or tumors
poliomyelitis
poliovirus - destruction of cells in anterior horn [LMN death
assymmetric weakness, hypotonia, flaccid paralysis, fasciculations, hyporeflexia, atrophy.
respiratory failure
CSF: high WBC [lymphocytic pleocytosis] slight increase in protein, no change in glucose.
Brown-Sequard syndrome
ipsilateral loss of sensation at level of lesion
ipsilateral LMN signs at level
ipsilateral UMN signs below level
ipsilateral loss of proprioception, vibration, light tough, tactile sense below level
Contralateral pain/temp/crude touch below level
if lesion above T1, may have ipsilateral horner from dame of oculosympathetic pathway
friedrich ataxia
AR trinucleotide repeat encoding frataxin [iron binding protein]
impairment in mitochondrial function --> degeneration of multiple spinal tracts
muscle weakness and loss of DTRs, vibratory sene, proprioception. Staggering gait, nystagmus, diabetes, hypertrophic cardiomyopathy.
presents in childhood with kyphoscoliosis
Neurodegenerative disorders
PD
loss of dopaminergic neurons of subs nivea pars compact. levy boils [alpha synuclein]
TRAPS - tremor, rigidity, akinesia, postural instability, shuffling gait
atrophy of caudate and putamen with ex vacuo ventriculomegaly. high dopa, low GABA, Ach. NMDA-Receptor binding and glutamate excitotoxicity --> neuronal death
HD
AD [CAG repeat disorder in chrome 4]. chorea, athetosis, aggression, depression, dementia.
CAG - caudate loses ACh and GABA
Alz
APP on chrom 21 - increased risk for down syndrome
ApoE2/4 or APP- decreased ACh --> cortical atrophy especially hippocampus. narrowing of gyro and widening sulk. can see plaques of B-amyloid plaques --> amyloid antipathy --> intracranial hemorrhage
Neurofibrillary tangles - number associated with degree of dementia
Frontotemporal dementia
early changes in personality/behaviro or aphasia. associated movement disorders - parkism, ALS-like UMN/LMN degeneration
frontotemporal lobe degeneration --> hyperphosphorylated tau or ubiquitinated TDP-43
Lewy body dementia
dementia and visual hallucinations --> parkism
intracellular lewy bodies in cortex
Vascular dementia
multiple arterial infarct or chronic ischemia. decline in cog ability with late onset memory impairment.
MRI/CT shows multiple cortical subcritical infarcts
CJD
weeks to months - prog dementia and myoclonus. periodic shark waves on EEG and high 14-3-3 protein in CSF.
Prions sheet --> B-pleated sheet resistant to proteases
idiopathic intracranial HTN [pseudotumor cerebri]
high ICP without cause on imaging. RFs female, obese, vitamin A excess, tetracycline, danazol,
papilledema, increased opening pressure on LP, provides HA relief
treat with weight loss, acetazolamide, topiramate.
stroke
5min hypoxia = irreversible damage
most vulnerable - hippo, neocortex, cerebellum, watershed areas
nonconformist CT to exclude hemorrhage --> tPA. CT detects ischemic changes in 6-24hr
MRI can detect ischemia 3-30min
timing
12-24hrs - eosinophilic cytoplasm with red neurons
24-72hrs - necrosis/neutrophils
3-5days - microglia
1-2weeks - reactive gloss and vascular proliferation
2wks - glial scar
Ischemic stroke
blockage --> ischemia --> liquefactive necrosis
thrombotic, embolic, hypoxic [hypoperfusion/hypoxemia]
treat - tPA if within 3-4.5hrs of onset without bleed. aspirin, clopidogrel, control BP, control glucose/lipids
TIA - reversible episode of focal near dysfunction without acute infarction on MRI. <15min resolution.
Stroke circulation effect
anterior
MCA
contralateral motor/sensory - limb/face
temporal lobe - wernicke and frontal lobe [broca]
wernicke aphasia - right superior quadrant visual field defect - temporal lobe involvement IF IN LEFT[DOMINANT] HEMISPHERE
Hemineglect if lesion affects non dominant [right] side
ACA
motor/sensory cortices of lower limbs
denticulate striate artery
striatum and internal capsule
contralateral paralysis and/or sensory loss
neglect/aphasia/visual field loss
common cause - hyaline arteriosclerosis 2/2 unmanaged HTN
posterior
anterior spinal artery
lateral corticospinal tract, medial lemniscus, caudal medulla [hypoglossal nerve
contralateral paralysis and decreased contralateral proprioception . ipsilateral hypoglossal dysfunction [licks the wound]
medial medullary syndrome - infarct of paramedic branches of ASA and/or vertebral arteries
posterior inferior cerebellar artery
lateral medulla [CN 9,10,11] vestibular nuclei, lateral spinothalamic tract, spinal trigeminal nucleus. sympathetic fibers, inferior cerebellar peduncle
dysphagia, hoarseness, no gag reflex. vom, vertigo, nystagmus. decreased pain and temp from contralateral body and ipsilateral face. ipsilateral horner syndrome, ataxia, dysmetria
Wallenberg [lateral medullary] syndrome - nucleus ambiguous effects specific to PICA lesions [oarseness and dysphagia]
anterior inferior cerebellar artery
lateral pons, facial nucleus, vestibular nuclei, spinothalamic tract, spinal trigeminal nucleus, sympathetic fibers, middle and inferior cerebellar peduncles.
paralysis of face - decreased lacrimation, salivation, taste from anterior 2/3 of tongue. vomiting, vertigo, nystagmus. decreased pain/temp/sensation from contralateral body and ipsilateral face. ipsilateral horner, ataxia, dysmetria
lateral pontine syndrome - facial droop means AICA's pooped.
Basilar artery
pons/medulal/lower midbrain. corticospinal, corticobulbar tracts. ocular cranial nerve nuclei, paramedian pontine reticular formation
RAS spared, therefore preserved consciousness. Quadriplegia, loss of voluntary facial mouth and tongue movements. loss of horizontal eye movements
"locked in syndrome"
posterior cerebral artery
occipital lobe
contralateral hemianopia with macular sparing
central post-stroke pain syndrome
thalamic lesions--> neuropathic pain
initial paresthesias --> allodynia and dysesthesia. 10% of stroke patients
Neurocutaneous
Sturge-Weber syndrome [encephalotrigeminal angiomatosis
congenital, non inherited developmental anomaly of GNAQ gene
affects small vessels
port-wine stain of face [nevus flammeus - CNV1/V2 distribution]
ipsilateral leptomeningeal angioma --> seizures, epilepsy
intellectual disability
episcleral hemangioma --> IOP high --> early onset glaucoma
Tuberous Sclerosis
TSC1/2 mutation, AD, variable expression
Hamartomas in CNS and skin
angiofibromas
MR
ash-leaf spots
cardiac rhabdomyoma
intellectual disability
renal angiomyolipoma
seizures
shagreen patches
increased incidence of supependymal giant cell astrocytomas and unfurl fibromas
Neurofibromatosis type 1 [von Recklinghausen disease]
NF1 tumor suppressor gene codes neurofibromin, negative regulator of RAS
AD, 100% penetrance
cafe au last spots
cutaneous neurofibromas
optic gliomas
pheochromocytoma
lisch nodules [pigmented iris hamartomas]
Neurofibromatosis type II
NF2 tumor suppressor gene, AD
bilateral acoustic schwannomas
juvenile cataracts
meningiomas
ependymomas
von Hippel-Lindau disease
deletion of VHL gene on chromosome 3. AD.
deve of numerous tumors
hemangioblastomas [high vascularity w/ hyper chromatic nuclei ] in retina , brain stem, cerebellum, spine
angiomatosis [cavernous hemangioma in skin, mucosa, organs]
lateral renal cell carcinomas
pheochromocyomas
cranial nerve lesions
5
jaw deviates toward lesion [unopposed force from pterygoid muscle]
10
uvula deviates away from side of lesion
11
weakness turning head to contralateral side. shoulder droop ipsilaterally [left SCM contracts to turn head to right]
12
LMN - tongue deviates toward lesion "lick your wounds"
7
UMN
destruction of motor cortex or connection between nucleus and cortex
contralateral paralysis of lower muscles with forehead sparing [bilateral UMN innervation]
LMN
destruction of facial nucleus or 7 tract
bells palsy - idiopathic, lyme, herpes simplex, zoster [ramsay hunt syndrome], sarcoidosis, tumors [parotid gland], diabetes,
ipsilateral paralysis of upper and lowe muscles, hyperacusis, loss of taste sensation to anterior tongue
Treat - steroids, acyclovir.
intracranial hemorrhage
epidural hematoma - middle meningeal artery 2/2 skull fracture --> biconvex blood collection not crossing suture lines
transtentorial herniation or CN3 palsy
subdural hematoma - rupture of bridging veins. acute or chronic [hypodense]. crescent shape crossing suture lines.
subarachnoid hemorrhage - trauma or rupture of aneurysm or AVM. worst headache of their life.
bloody or yellow spinal tap.
Nimodipine to prevent/reduce vasospasm that could cause ischemic infarct
high risk of communicating/ostructive hydrocephalus
intraparenchymal hemorrhage
Seizures
Partial - most common in medial temporal lobe. preceded by aura, can 2ndarily generalize
Simple
consciousness intact
Complex [impaired cons.]
Generalized
Absence [petit mal]
no post-ictal confusion
staring spells with preserved muscle tone, unresponsive to stimuli, short, provoked by hyperventilation
3Hz spike wave d/c
Ethosuximide, valproic acid [2nd line side effects - hair loss, tremor, thrombocytopenia]
myoclonic
quick, repetitious
tonic-clonic [grand mal]
alternating stiffening and movement
tonic
stiffening
atonic
"drop"
epilepsy
recurrent seizures, not including febrile
status epilepticus
continuous >5min or recurrent result in brain injury
Headaches
cluster
unilateral, repetitive. lacrimation and rhinorrhea. may present with Horner syndrome.
acute - sumatriptan and O2 pix: verapamil
tension
bilateral >30min. steady pain without photo/phonophobia or aura.
analgesics, NSAIDs, amitriptyline for chronic pain
migraine
unilateral 4-72hr. pulsating pain with Nausea, photo/phonophobia, may have aura. irritation of CN5, meninges, or blood vessels.
acute - NSAIDs, triptans, dihydroergotamine. pax: lifestyle changes, Bblockers, amitriptyline, topiramate, valproate.
trigeminal neuralgia
repetitive unilateral shooting pain in distribution of CN5 lasting <1min.
carbamazepine
Demyelenating/Dysmyelinating
MS
autoimmune inflammation and demyelination of CNS
present with optic neuritis, INO, hemiparesis, hemisensory sxs, GU dysfunction. worsen with increased body temp. Lhermitte phenomenon - neck flexion --> electric shock down spine
affects women in 20/30s white ppl.
charcot triad - SIN - scanning speech, intention tremor [incontenence INO], nystagmus
high IgG level and myelin basic protein in CSF.
oligoclonal bands diagnostic
MRI. will see periventricular plaques where there is loss of oligodendrocyte and reactive gloss with preservation of axons.
treat: slow down progression with [Binterferon, glatimer, natalizumab. treat flares with IV steroids. neurogenic bladder = cath, muscarinic antagonists, for spasticity - baclofen, GABA b receptor agonists, and for pain TCAs, anticonvulsants.
Acute inflammatory demyelinating polyradiculopathy
common subtype of Guillain-barre syndrome. autoimmune destroys schwann cells --> inflammation, demyelination of peripheral nerves and motor fibers.
symmetric ascending muscle weakness/paralysis, decreased tendon reflexes in lower extremities, autonomic dysregulation, sensory abn.
recover after weeks to months
respiratory support, plasmapheresis, IVIG. no steroids
high CSF protein with normal cell count, high protein --> papilledema
associated with campy and viral infections --> autoimmune attach peripheral myelin [molecular mimicry]
Other
acute disseminated [postinfectious] encephalomyelitis
multifocal inflamm and demyelination after infection/vaccination
rapidly progressive multifocal sxs and AMS
charcot-marie tooth disease
HMNS - hereditary motor and sensory neuropathy
hereditary nerve disorders from defective production of proteins in structure/function of peripheral nerves or myelin sheath.
AD pattern, foot deformities, LE weakness and sensory deficits
krabbe disease
AR lysosomal storage disease --> deficiency of galactocerebrosidase causes buildup of galactocerebroside and psychosine destroys myelin sheath.
peripheral neuropathy, dev delay, optic atrophy, globoid cells
metachromic leukodystrophy
AR lysosomal storage disease, common due to arylsulfatase A deficiency. buildup sulfatides --> impaired production/destruction of myelin sheath.
central&peripheral demyelination, ataxia, dementia
progressive multifocal leukoencephalopathy
destruction of oligodendrocytes. seen in 4% AIDA patients [ractivation of JC virus]. progressive, fatal.
associated with use of natalizumab and rituximab
Adrenoleukodystrophy
x-linked disorder
disrupts metabolism of very long chain fatty acids --> build up in nervous system, adrenal glands, testes. long term coma/death and adrenal gland crisis
Herniation syndromes
cingulate [subfalcine] under fall cerebri
compress ACA
transtentorial [central/downward]
caudal displacement of brain stem --> rupture paramedic basilar artery branches -->
duret hemorrhages
fatal
uncal
medial temporal lobe - ipsilateral CN3 [down/out],
ipsilateral PCA [contralateral homonymous
hemianopia with macular sparing],
contralateral crus cerebra [ipsilateral paresis]
cerebellar tonsillar herniation into foramen magnum
coma/death from compression of brain stem
Hydrocephalus [high CSF volt, ventricular dilation with/out high ICP
communicating
communicating
low CSF absorption by arachnoid granulations = high ICP, papilledema, herniation
normal pressure
elderly, idiopathic, CSF pressure elevated episodically. no increased in subarachnoid vol. ventricles expand --> distortion of corona radiate
SXS:
urinary incontinence, ataxia, cog dysfunction, magnetic gait!
Obstructive
structural blockage of CSF circulation
stenosis of aqueduct of Sylvius, colloid cyst blocking foramen of Monro, rumor
mimics
ex-vacuo ventriculomegaly
high CSF on imaging but decreased brain tissue and neuronal atrophy. normal ICP
Osmotic demyelination syndrome [central pontine myelinolysis]
acute paralysis, dysarthria, dysphagia, diplopia, loss of consciousness. can cause locked in syndrome. caused by rapid correction of hyponatremia. [hypernatremia results in cerebral edema/herniation
"from low to high, your pons will die" "from high to low, your brain will blow"
Aphasia inability to understand/speak/read/write [vs Dyarthria - motor inability to speak]
Repetition Impaired
Wernicke
fluent - no insight. superior temporal gyrus
Conduction
fluent with intact comprehension. caused by damage to arcuate fasciiculus
Global
confluent and impaired comprehension - all areas affected
Broca
inferior frontal gyrus of frontal lobe - insight intact
Repetition intact
transcortical motor
nonfluent, intact comprehension - frontral lobe around broca area, but broca is spared
transcortical sensory
fluent with impaired comprehension - affects temporal lobe but spares wernicke
Mixed
confluent and impaired comprehension - broca, wernicke, and arcuate fasciculus intact. surrounding watershed areas affected
Aneurysms
Saccular [berry]
at bifurcations in circle of willis - common in Acomm and ACA
subarachnoid hemorrhage or sxs via direct compression on surrounding structures
Acomp --> bilateral hemianopia [chiasm] if compressed. if rupture, ischemic - contralateral lower extremity hemiparesis and sensory deficits
PCom - ipsilateral CN3 palsy--> blown pupil [mydriasis], ptosis "down and out"
Charcot-Bouchard micro aneurysm
chronic HTN. affects small vessels [lenticulate striate arteries in basal ganglia/thalamus
primary tumors
Adult
oligodendroglioma
rare, slow growing. frontal lobes. "chicken-wire" capillary pattern
oligodendrocyte origin - fried egg cells, often calcified
meningioma
typically benign primary brain tumor near surfaces of brain in parasaggital region. extra-axial, dural attachment.
asx but may present with seizures or focal neural signs. tx: resect/radiosurgery
arachnoid cell origin. spindle cells in whorled pattern, psammoma bodies [laminated calcifications]
hemangioblastoma
cerebellar. VHL syndrome when found with retinal angiomas. EPO produced --> polycythemia.
closely arranged thin-walled capillaries with minimal intervening parenchyma
pituitary adenoma
non-functioning or hyper functioning. most common is prolactinoma, less common is somatotropin --> acromegaly, corticotrophins --> cushing's disease.
bitemporal hemianopia --> pressure at optic chiasm.
hyper/hypopituitarism caused by pituitary apoplexy
Schwannoma
cerebellopontine angle or along any peripheral nerve. CN8 in internal acoustic meatus --> vestibular schwannoma in NF2.
glioblastoma multiforme [grade 4 astrocytoma]
1yr median survival . can be butterfly glioma [cross corpus callous]
astrocyte origin, GFAP+. pseudopalisading pleomorphic tumor cells border central areas of necrosis and hemorrhage
Pediatric
Pilocytic [low grade] astrocytoma
well circ. posterior fossa. supratentorial. benign
Glial cell, GFAP +. cystic and solid
medulloblastoma
most common malignant tumor in kids. common in cerebellum. compress 4th ventricle --> noncommunicating hydrocephalus.
"drop-mets to the spinal cord
form pf PNET [primitive neuroectodermal tumor], homer-wright rosettes, small blue cells.
ependymoma
4th ventricle. hydrocephalus. poor prognosis.
perivascular rosettes. rod shaped blepharoplasty [basal ciliary bodies] near nucleus.
craniopharyngioma
most common childhood supratentorial tumor. cause bitemporal hemianopia [confued with pituitary adenoma]
derived from remnants of rathe pouch. calcification. cholesterol crystals found in motor oil-like fluid within tumor
Pinealoma
can cause parinaud syndrome [compression of tectum --> vertical gaze palsy. obstructive hydrocephalus, precocious puberty in males [B HCG production]
similar to germ cell tumors [test. seminoma]