Pathoma Neurology Flashcards
PNS develops from?
neural crest
CNS develops from?
wall of neural tube
ventricles & spinal canal develop from?
lumen of neural tube
neural tube defects?
lack of folate PRIOR to conception
detected by elevated AFP in amniotic fluid or maternal blood
frog like appearance of fetus
anencephaly; lacks swallowing centers in brain - polyhydramnios
meningocele vs meningomyeleocele?
meningocele = meninges alone coming out; meningomyeleocele = meninges + spinal cord
what is spina bifida?
failure of posterior vertebral arch to close leading to vertebral defect, with cystic protrusion of underlying tissue through the defect
how does spina bifida occulta present?
dimple or patch of hair over vertebral defect
what is cerebral aqueduct stenosis?
congenital stenosis of the channel that drains cerebrospinal fluid from 3rd to 4th ventricle –> hydrocephalus
Where is CSF produced?
choroid plexus lining the ventricles
describe the flow of the CSF
from lateral ventricles to the 3rd ventricle via interventricular foramen of monro then to the 4th ventricle via cerebral aqueduct. The CSF then goes into the subarachnoid space via foramina of Magendie and Luschka
How does cerebral aqueduct stenosis present?
enlargin head circumferance due to the dilation of the ventricles (cerebral sutures are not fused)
massively dilated 4th ventricle w/absent cerebellum?
Dandy-Walker malformation; failure to develop cerebellar vermis
congenital extension of cerebellar tonsils thru foramen magnum?
Arnold-Chiari malformation; associated: hydrocephalus, meningimyeleocele and syringomyelia
what is syringomyelia
cystic dilation of the spinal cord from trauma or Arnold-Chiari leading to C8-T1 damage.
How does syringomyelia present?
loss of pain & temperature sensation in UE, sparing of position & fine touch due to involvement of anterior white commissure of spinothalamic tract with dorsal column sparing
syrinx expansion in syringomyelia can lead to what other symptoms?
can damage anterior horn (motor) & lateral horn (sympathetic to face, Horner’s syndrome)
sx of lower motor neuron dysfxn
anterior horn dysfxn; poliomyelitis, Werdnig-Hoffman dz
Poliomyelitis is the result of what neuro defects?
anterior horn; LMN defects
what are lower motor neuron signs?
flaccid paralysis with muscle atrophy, fsciculations, weakness with decreased muscle tone, impaired reflexes and negative Babinski
Werdnig-Hoffman has what defects?
inherited degeneration of anterior horn motor neurons, autosomal recessive; floppy baby”, death w/in a few years after birth”
ALS has what defects?
UMN & LMN degeneration
patient presenting with motor weakness in hands - what differentiates between ALS vs syringomyelia?
ALS is motor only; syringomyelia would also have pain/temp deficits
what are the upper motor neuron signs?
spastic paralysis with hyperreflexia, increased motor tone and positive Babinski due to damage to the lateral corticospinal tract
what is defect in familial ALS?
zinc-copper superoxide dismutase mutation (important mechnaism of removing free radicals)
difficulty walking, loss of vibratory sense & proprioception, mm weakness in LE, loss of DTR?
Friedrich’s ataxia; degenerative disorder of cerebellum
what are characteristics of Friedrich’s ataxia?
degenerative disorder of cerebellum & spinal cord tracts; autosomal recessive, trinuc GAA repeat in frataxin gene; HYPERTROPHIC cardiomyopathy
what gene is defective in Friedrich Ataxia? What does it do?
frataxin gene, necessary for mitochondrial iron regulation; increase risk of free radicals
what is inflamed in meningitis?
leptomeninges - the pia & arachnoid matter
MCC meningitis in neonates?
GBS, E coli, Listeria monocytogenes
MCC meningitis in children & teenagers?
Neisseria meningitidis; enters thru nasopharynx
MCC meningitis in adults & elderly?
Strep pneumo
MCC meningitis in unvaccinated?
Haemophilus influenza
MCC viral meningitis?
Coxsackievirus
Meningitis in immunocompromised?
think of fungi
Meningitis presentation
headache, nichal rigidity, fever; photophobia, vomiting and altered mental status may present
doing LP what layers are pierced?
Dura & Arachnoid, NOT Pia between L4 and L5
LP w/lymphocytes & low CSF glucose?
Fungal
LP w/lymphcytes & nl CSF glucose
Viral
LP w/neutrophils & low glucose
Bacterial; positive gram stain & culture
death from meningitis occurs because of:
herniation secondary to cerebral edema; seen w/bacterial b/c of pus production, etc
hydrocephalus, hearing loss, & seizures w/meningitis are from?
the healing process, fibrosis; primarily seen w/bacterial meningitis
majority of Cerebrovascular Dz?
ischemic (85%); hemorrhagic is ~15%
type of CVA caused by hypoglycemia?
global ischemia; see w/repeated episodes (insulinoma)
what are the major etiologies of global cerebral ischemia?
low perfusion, acute decrease in blood flow, chronic hypoxia, repeated hypoglycemia
highly vulnerable areas of the brain?
pyramidal layers of the cortex; layers 3, 5, & 6 leading to laminar necrosis
pyramidal neurons of hippocampus
purkinje layer of cerebellum
cortical lamenar necrosis
lines of necrosis; pyramidal layers 3, 5, & 6
focal neural deficits < 24 hours?
TIA
what causes a thrombotic stroke?
rupture of atherosclerotic plaque
where does atherosclerosis usually develop?
branch points, bifurcation of internal carotid and middle cerebral artery
what is the MC source of emboli in an embolic stroke?
left side of heart, atrial fibrillation
pale vs hemorrhagic necrosis following ischemia?
pale = thrombotic (ruptured plaque, prevents successful lysis, periphery of cortex); hemorrhagic = embolic (embolus comes in, causes ischemia, is lysed, periphery of cortex)
MC site of embolic stroke?
middle cerebral artery
what is a lacunar stroke?
results from hyaline arteriolosclerosis; this occurs w/HTN or diabetes
MC site of lacunar stroke?
lenticulostriate vessels (feeding deep structures of the brain) - lake like areas of infarction on gross inspection
order of histologic appearance following ischemic stroke?
eosinophillic change: red neurons (~12 hours) coagulative necrosis (24hrs): PMNs (1-3days), microglial cells (4-7days), granulation tissue (weeks 2-3) --> fluid-filled cystic space surrounded by gliosis (reactive astrocytes)
what is intracebral hemorrhage? What is it caused by? Complication of? MC site?
bleeding into brain parenchyma; due to ruptured Charco-Bouchard microaneurysms of lenticulostriate vessels; complication of HTN; basal ganglia MC site
How does an intracerebral hemorrhage present?
severe headach, nausea, vomiting coma
Charcot-Bouchard aneurysms?
caused by hyaline arteriosclerosis; can result in intracerebral hemorrhagic stroke
see a bleed on bottom of the brain?
subarrachnoid hemorrhage; really only cause
How does subarrachnoide hemorrhage present?
worst headach of my life with nuchal rigidity
What do you see on lumbar puncture with subarachnoid hemorrhages?
xanthochromia (yellow hue due to bilirubin breakdown)
MCC subarachnoid hemorrhage?
berry aneurysms, other cuases are AV malformation and anticoagulated state
MC location of berry aneurysms? Assocaited with?
cause subarachnoid hemorrhage; MC located in anterior circle of willis, specifically anterior communciating artery; associated w/marfan & ADPKD
histology of berry aneurysm?
thin walled, lack media layer