NeuroPath (Spinal cord lesions (Brown-Sequard syndrome (if lesion above…
Spinal cord lesions
AR trinucleotide repeat encoding frataxin [iron binding protein]
impairment in mitochondrial function --> degeneration of multiple spinal tracts
presents in childhood with kyphoscoliosis
muscle weakness and loss of DTRs, vibratory sene, proprioception. Staggering gait, nystagmus, diabetes, hypertrophic cardiomyopathy.
if lesion above T1, may have ipsilateral horner from dame of oculosympathetic pathway
Contralateral pain/temp/crude touch below level
ipsilateral loss of proprioception, vibration, light tough, tactile sense below level
ipsilateral UMN signs below level
ipsilateral LMN signs at level
ipsilateral loss of sensation at level of lesion
poliovirus - destruction of cells in anterior horn [LMN death
CSF: high WBC [lymphocytic pleocytosis] slight increase in protein, no change in glucose.
assymmetric weakness, hypotonia, flaccid paralysis, fasciculations, hyporeflexia, atrophy.
cauda equina syndrome
compression of spinal roots from L2 and below, caused by intervertebral disk herniation or tumors
unilateral sxs: radicular pain, absent knee/anl;e reflexes, loss of bladder/anal sphincter control. saddle anesthesia
tx: emergent surgery and steroids
vit. B12 deficiency
subacute combined degeneration - demyelination of spinocerebellar tracts, corticospinal, and dorsal columns
ataxic gait, paresthesia, impaired position/vibration sense
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
tertiary syphilis. demyelination of dorsal columns and roots --> sensory ataxia [impaired proprioception and coordination]
absent DTRs and + romberg sign
assc. charcot joints, shooting pain, argyll-robertson pupils.
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
ALS - amyotrophic lateral sclerosis "Lou Gehrig disease"
combine UMN and LMN deficits without sensory or bowel/bladder deficits
asymmetric limb weakness, fasciculations, eventual atrophy. treat: riluzole. FATAL
can be caused by defect in superoxide dismutase 1
poliomyelitis and Wednig-Hoffman dz
congenital degeneration of anterior horns. "floppy baby" hypotonia, tongue fasciculations. ~7mo.
Werdnig-Hoffman --> symmetric weakness
poliomyelitis --> asymmetric weakness
uncontrolled movement of distal appendages. alleviated by intentional movement
sudden, brief, uncontrolled contraction
jerks, hiccups. common in metabolic[renal/liver failure]
slow zigzag motion when pointing/extending to target
sudden flailing of 1 arm and ipsilateral leg
contralateral subthalamic nucleus [lacunar stroke]
high frequency tremor with sustained posture worsens with movement or anxiety
familial. alcoholic which decrease tremor amplitude
nonselective BBlockers [propanolol], primidone
sustained involuntary contractions
writer's cramp, blepharospasm, torticollis
jerky, purposeless movements
in acute rheumatic fever
slow, snake-like writhing movements, esp fingers
flapping wrists - extension
Hep encephalitis, wilson disease, metabolic dz
neuroleptic use or PD
atrophy of caudate and putamen with ex vacuo ventriculomegaly. high dopa, low GABA, Ach. NMDA-Receptor binding and glutamate excitotoxicity --> neuronal death
AD [CAG repeat disorder in chrome 4]. chorea, athetosis, aggression, depression, dementia.
CAG - caudate loses ACh and GABA
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.
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
multiple arterial infarct or chronic ischemia. decline in cog ability with late onset memory impairment.
MRI/CT shows multiple cortical subcritical infarcts
Lewy body dementia
dementia and visual hallucinations --> parkism
intracellular lewy bodies in cortex
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
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
loss of dopaminergic neurons of subs nivea pars compact. levy boils [alpha synuclein]
TRAPS - tremor, rigidity, akinesia, postural instability, shuffling gait
Stroke circulation effect
central post-stroke pain syndrome
thalamic lesions--> neuropathic pain
initial paresthesias --> allodynia and dysesthesia. 10% of stroke patients
posterior cerebral artery
contralateral hemianopia with macular sparing
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"
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.
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 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
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
motor/sensory cortices of lower limbs
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
contralateral motor/sensory - limb/face
TIA - reversible episode of focal near dysfunction without acute infarction on MRI. <15min resolution.
treat - tPA if within 3-4.5hrs of onset without bleed. aspirin, clopidogrel, control BP, control glucose/lipids
thrombotic, embolic, hypoxic [hypoperfusion/hypoxemia]
blockage --> ischemia --> liquefactive necrosis
2wks - glial scar
1-2weeks - reactive gloss and vascular proliferation
3-5days - microglia
24-72hrs - necrosis/neutrophils
12-24hrs - eosinophilic cytoplasm with red neurons
nonconformist CT to exclude hemorrhage --> tPA. CT detects ischemic changes in 6-24hr
MRI can detect ischemia 3-30min
5min hypoxia = irreversible damage
most vulnerable - hippo, neocortex, cerebellum, watershed areas
von Hippel-Lindau disease
deve of numerous tumors
lateral renal cell carcinomas
angiomatosis [cavernous hemangioma in skin, mucosa, organs]
hemangioblastomas [high vascularity w/ hyper chromatic nuclei ] in retina , brain stem, cerebellum, spine
deletion of VHL gene on chromosome 3. AD.
Neurofibromatosis type II
NF2 tumor suppressor gene, AD
bilateral acoustic schwannomas
Neurofibromatosis type 1 [von Recklinghausen disease]
NF1 tumor suppressor gene codes neurofibromin, negative regulator of RAS
AD, 100% penetrance
lisch nodules [pigmented iris hamartomas]
cafe au last spots
TSC1/2 mutation, AD, variable expression
increased incidence of supependymal giant cell astrocytomas and unfurl fibromas
Hamartomas in CNS and skin
Sturge-Weber syndrome [encephalotrigeminal angiomatosis
congenital, non inherited developmental anomaly of GNAQ gene
affects small vessels
episcleral hemangioma --> IOP high --> early onset glaucoma
ipsilateral leptomeningeal angioma --> seizures, epilepsy
port-wine stain of face [nevus flammeus - CNV1/V2 distribution]
cranial nerve lesions
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.
destruction of motor cortex or connection between nucleus and cortex
contralateral paralysis of lower muscles with forehead sparing [bilateral UMN innervation]
LMN - tongue deviates toward lesion "lick your wounds"
weakness turning head to contralateral side. shoulder droop ipsilaterally [left SCM contracts to turn head to right]
uvula deviates away from side of lesion
jaw deviates toward lesion [unopposed force from pterygoid muscle]
disrupts metabolism of very long chain fatty acids --> build up in nervous system, adrenal glands, testes. long term coma/death and adrenal gland crisis
progressive multifocal leukoencephalopathy
destruction of oligodendrocytes. seen in 4% AIDA patients [ractivation of JC virus]. progressive, fatal.
associated with use of natalizumab and rituximab
AR lysosomal storage disease, common due to arylsulfatase A deficiency. buildup sulfatides --> impaired production/destruction of myelin sheath.
central&peripheral demyelination, ataxia, dementia
AR lysosomal storage disease --> deficiency of galactocerebrosidase causes buildup of galactocerebroside and psychosine destroys myelin sheath.
peripheral neuropathy, dev delay, optic atrophy, globoid cells
charcot-marie tooth disease
HMNS - hereditary motor and sensory neuropathy
AD pattern, foot deformities, LE weakness and sensory deficits
hereditary nerve disorders from defective production of proteins in structure/function of peripheral nerves or myelin sheath.
acute disseminated [postinfectious] encephalomyelitis
multifocal inflamm and demyelination after infection/vaccination
rapidly progressive multifocal sxs and AMS
Acute inflammatory demyelinating polyradiculopathy
common subtype of Guillain-barre syndrome. autoimmune destroys schwann cells --> inflammation, demyelination of peripheral nerves and motor fibers.
associated with campy and viral infections --> autoimmune attach peripheral myelin [molecular mimicry]
symmetric ascending muscle weakness/paralysis, decreased tendon reflexes in lower extremities, autonomic dysregulation, sensory abn.
high CSF protein with normal cell count, high protein --> papilledema
recover after weeks to months
respiratory support, plasmapheresis, IVIG. no steroids
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
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.
affects women in 20/30s white ppl.
repetitive unilateral shooting pain in distribution of CN5 lasting <1min.
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.
bilateral >30min. steady pain without photo/phonophobia or aura.
analgesics, NSAIDs, amitriptyline for chronic pain
unilateral, repetitive. lacrimation and rhinorrhea. may present with Horner syndrome.
acute - sumatriptan and O2 pix: verapamil
cerebellar tonsillar herniation into foramen magnum
coma/death from compression of brain stem
contralateral crus cerebra [ipsilateral paresis]
hemianopia with macular sparing],
ipsilateral PCA [contralateral homonymous
medial temporal lobe - ipsilateral CN3 [down/out],
caudal displacement of brain stem --> rupture paramedic basilar artery branches -->
cingulate [subfalcine] under fall cerebri
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
subdural hematoma - rupture of bridging veins. acute or chronic [hypodense]. crescent shape crossing suture lines.
epidural hematoma - middle meningeal artery 2/2 skull fracture --> biconvex blood collection not crossing suture lines
transtentorial herniation or CN3 palsy
continuous >5min or recurrent result in brain injury
recurrent seizures, not including febrile
tonic-clonic [grand mal]
alternating stiffening and movement
Absence [petit mal]
Ethosuximide, valproic acid [2nd line side effects - hair loss, tremor, thrombocytopenia]
3Hz spike wave d/c
staring spells with preserved muscle tone, unresponsive to stimuli, short, provoked by hyperventilation
no post-ictal confusion
Partial - most common in medial temporal lobe. preceded by aura, can 2ndarily generalize
Complex [impaired cons.]
Hydrocephalus [high CSF volt, ventricular dilation with/out high 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"
high CSF on imaging but decreased brain tissue and neuronal atrophy. normal ICP
structural blockage of CSF circulation
stenosis of aqueduct of Sylvius, colloid cyst blocking foramen of Monro, rumor
elderly, idiopathic, CSF pressure elevated episodically. no increased in subarachnoid vol. ventricles expand --> distortion of corona radiate
urinary incontinence, ataxia, cog dysfunction, magnetic gait!
low CSF absorption by arachnoid granulations = high ICP, papilledema, herniation
Charcot-Bouchard micro aneurysm
chronic HTN. affects small vessels [lenticulate striate arteries in basal ganglia/thalamus
at bifurcations in circle of willis - common in Acomm and ACA
subarachnoid hemorrhage or sxs via direct compression on surrounding structures
PCom - ipsilateral CN3 palsy--> blown pupil [mydriasis], ptosis "down and out"
Acomp --> bilateral hemianopia [chiasm] if compressed. if rupture, ischemic - contralateral lower extremity hemiparesis and sensory deficits
Aphasia inability to understand/speak/read/write [vs Dyarthria - motor inability to speak]
confluent and impaired comprehension - broca, wernicke, and arcuate fasciculus intact. surrounding watershed areas affected
fluent with impaired comprehension - affects temporal lobe but spares wernicke
nonfluent, intact comprehension - frontral lobe around broca area, but broca is spared
confluent and impaired comprehension - all areas affected
fluent with intact comprehension. caused by damage to arcuate fasciiculus
fluent - no insight. superior temporal gyrus
inferior frontal gyrus of frontal lobe - insight intact
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]
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
4th ventricle. hydrocephalus. poor prognosis.
perivascular rosettes. rod shaped blepharoplasty [basal ciliary bodies] near nucleus.
most common malignant tumor in kids. common in cerebellum. compress 4th ventricle --> noncommunicating hydrocephalus.
form pf PNET [primitive neuroectodermal tumor], homer-wright rosettes, small blue cells.
"drop-mets to the spinal cord
Pilocytic [low grade] astrocytoma
well circ. posterior fossa. supratentorial. benign
Glial cell, GFAP +. cystic and solid
cerebellopontine angle or along any peripheral nerve. CN8 in internal acoustic meatus --> vestibular schwannoma in NF2.
non-functioning or hyper functioning. most common is prolactinoma, less common is somatotropin --> acromegaly, corticotrophins --> cushing's disease.
hyper/hypopituitarism caused by pituitary apoplexy
bitemporal hemianopia --> pressure at optic chiasm.
cerebellar. VHL syndrome when found with retinal angiomas. EPO produced --> polycythemia.
closely arranged thin-walled capillaries with minimal intervening parenchyma
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]
rare, slow growing. frontal lobes. "chicken-wire" capillary pattern
oligodendrocyte origin - fried egg cells, often calcified
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