Neuro -Disease Flashcards
Von hippel Lindau presents w/ and increased risk of what
cavernous hemangionomas in the skin, mucosa, and organs;
hemangioblasoma retina, brain stem, cerebellum)
bilateral renal cell carcinoma risk
AD mutation of chromosome 3
Myasthenia gravis associations
Thymoma
Bronchogenic Carcinoma
Parkinsons w/ hallucinations and dementia
Location of lesion(3)
Lewy Body dementia
Lewy body - eosinophilic intracytoplamic inclusions
Located in
the substantial nigra
limbic cortex
subcortical nuclei
Werdnig Hoffman Disease
- presentation
LMN lesions only
due to setruciton of anterior horns ->flaccid paralysis
~polio
Lesions of the caudate nucleus and putamen
Huntingtons
- degeneration of GABAnergic neurons
- trinucleotide expansion of CAG on chromosome 4
Lewy Bodies found in (2)
Parkinsons
Lewy body dementia
Diffuse cortical atrophy sparing primary motor and sensory
Alzheimers
Selective frontal and temporal lobe atrophy
Picks
- silver staining cytoplasmic inclusions (pick bodies)
Pigmentes nodules on iris(Lisch nodules) and cafe au lair spots
-> risk of ?
Defect in ?
AD disease
Neurofibromatosis 1 or von Recklinhausen
optic path gliomas
sub q peripheral nerve nodules (neurofibromas)
Defect in tumor supressor NF1 on Chromosome 17
Low folate levels prior to conception leads to increase risk of what?
What marker may clue in
neural tube defects
elevated alpha fetoprotein in maternal blood and amniotic fluid
Maternal polyhydraminos may be due to this neuro defect
anecephaly(no skull or brain) - lack of cranial end to fuse
lack of swallowing mech
spina bifida occulta
dimple or patch of hair
simple failure of vertebral arch to close
Meningocele
failure of vertebral arch to close w/ protrusion of meninges
meningomyelocele
failure of vertebral arch to close w/ protrusion of meninges and spinal cord
3 congenital conditions that can lead to hydrocephalis
cerebral aqueduct stenosis
Dandy walker malformation
Arnold chiari malformation type II
cerebral aqueduct stenosis prevents what
drainage of CSF from the 3rd to the 4th ventricle -> accumulation in ventricle space
Foramen of monro
Drains lateral ventricles to 3rd ventricle
Foramina of magenie and Luschka
drians 4th ventricle to subarachnoid space
Massively dilated 4th ventricle congenitally called what?
Due to ?
Dandy walker malformation and it is due to failure of cerebellar vermis to form
- no separation of the cerebellum and thus the space is absent on CT
Congenital displacement of cerebellar vermis and tonsils ->
Associated w/ (2)
Arnold chiari malformation type II (type 1 asymptomatic)
- herniation of the cerebellum through the foramen magnum
- > obstruction of CSF flow and hydrocephalus
meningomyolcele (most)
syringomyelia
sensory loss of pain and temp w/ sparing go fine touch and position sense in the upper extremities
What is going on and where?
Causes(2)
syringomyelia -
- Usually C8 -T1, cape like distribution
- > preferential coring out the anterior white commissure where the sensation crosses and then ascends contra laterally in the spinothalamic tract
Caused by trauma or Arnold Chiari malformation
Syrinx expansion of syringtomyelia
2 locations
initally anterior white commissure
Anterior horn -> lower motor neuron defects
Lateral horn and hypothalamospinal tract (sympathetic)-> horner syndrome
ptosis
droopy eyelid
miosis
constricted pupil
anhidrosis
lack of sweating
fecal oral transmission bug leading to anterior horn difficulties
Upper or lower?
poliomyelitis
Lower motor neuron signs - flaccid paralysis fasciulations weakness impaired reflexes neg babinski
Lower motor neuron lesions (6 signs)
flaccid paralysis muscle atrophy fasciulations weakness impaired reflexes neg babinski
things go down
Upper motor neuron lesions(4 signs)
spastic paralysis
hyperreflexia
increased muscle tone
positive babinski
things go up
floppy baby inherited condition affects?
weednig hoffman disease
Auto Recessive disease; death in a few yrs
anterior horn -> LMN symptoms
Sporadic degeneration of the cortical spinal tract
amoyotrophic lateral sclerosis
(AML)
anterior horn degen-> LMN symptoms
Lateral Corticospinal Tract -> UMN symptoms
Atrophy and weakness of the hands w/out loss of sensory?
ALS
Zinc-Coppuer superoxide dismutase often implicated in
familial ALS
Presentation of ataxia and loss of vibratory sensation , proprioception, muscle weakness and loss of deep tendon reflexes
Due to ?
Friedriech ataxia
- degeneration of cerebellum and spinal cord
Autosomal recessive expansion of unstable tinucleotide repeat
Frataxin
Gene implicated in Fredriech ataxia -
- essential for mitochondrial iron regulation -> iron buildup
Friedreich ataxia associated w/ what extra neuro presentation
hypertorphic cardiomyopathy
wheelchair bound w/in few yrs
inflammation of the leptomeninges
meningitis
specifically the pia and the arachnoid
(not dura)
Meningitis in neonate (3)
Group b strep
E Coli
Listeria monocytogenes
Meningitis in childen and teens
Nesseria meningitidis
-nasopharynx -> blood path
Meningitis in adults and elderly
Streptococcus pneumoniae
Nonvaccinated infants and meningitis
H influenza
Most common viral meningitis
Coxsackie
HA, nuchal regidity and fever think
If photophobia?
Meningitis
may be viral
Can also have altered mental status and vomitting
Lumbar puncture done at
L4 and L5 - level of the iliac crest
Spinal cord ends at L2 - just caudal equina
CSF of bacterial meningitis
neutrophils
low CSF glucose (normally 2/3 of serum)
- more complications w/ pus and herniation -> death
CSF of viral meningitis
lymphocytes
normal CSF glucose
CSF of fungal meningitis
lymphocytes
decreased glucose
Sequela of meningitis
W/ healing and fibrosis if not dead
-hydrocephalus(obstruction), seizures(scarring) and hearing loss (nerve damage that exit)
Cerebrovascular disease -
2 causes and sub types w/in
6 total
ischemia (85%) - global -Focal --- >24 hr transient ischemic ---< 24 hr ischemic stroke hemorrhage(15%) - Subarachnoid - Intracerebral
Etiologies of global cerebral ischemia
4
low perfusion - atherosclerosis
acute decreased blood flow - shock
chronic hypoxia - anemia
repeated hypoglycemia
transient confusion and prompt recovery seen in this CVD
mild global ischemia - hypglycemia
Diffuse necrosis and survival -> vegetative state
Severe global ischemia -
laminar necrosis of the cerebral cortex
layer 3,5,6 necrosis w/ moderate global ischemia
-pyramidal neurons of the cerebral cortex
Long term memory affected w/ this CVD
Pyramidal neurons of hippocampus affected w/ moderate global ischemia
sensory perception w/ motor control affected w/ this CVD
moderate global ischemia of purkinje layer of the cerebellum
TIA
Transient ischemic attack 24 hrs
pale infarct seen w/
point often affected?
thrombotic stroke - ischemic
-rupture of atherosclerotic plaque
usually at branch points ( bifurcation of internal carotid or middle cerebral artery of circle of willis)
hemorrhagic infarct seen w/
point often affected
emboli stroke - ischemic
- maybe from the left atrium and atrial fibrillation
- emboli lysed and reprofused area
affects middle cerebral artery often
Hyaline arteriosclerosis secondary to HTN or DM leads to ?
affects?
lacunar strokes - ischemic
lenticulostriate vessels
- internal capsule - pute motor
- thalamus - pure sensory
Ischemic stroke leads to what type of necrosis
Histology?
liquefactive
red nueurons in 12 hrs, necrosis in 24, neutrophils invade followed by microglial cells (macrophages)
Gliosis (2-3 weeks)
Gliosis
healing stage of an ischemic stroke leading to a cystic space surrounded by microglial cells
Charcot bouchard microaneurisms analogous to
similar to lacunar strokes except there is weakening, aneurysm formation and rupture of lenticulostriate vessels
HTN predisposes again
Common site of intracerebral hemorage
basal ganglia fed by lenticulostriate vessels,
HTN risk
Presentation of severe HA, N/V, and eventual coma
Intracerebral hemorrhage -> bleeding into brain parenchyma
Xanthochromia w/ nucal regidity and HA
Subarachnoid Hemorrhage
Xanthochromia - yellow hue of CSF due to bilirubin breakdown on LP
Subarachnoid hemorrhage often occur where?
2 conditions the predispose?
Berry aneurism bleeds (lack media layer)
anterior circle of Willis branch points of the anterior communicating artery
Marfan and ADPCK
lens shaped lesion on CT
lesion of what vessels?
epidural hematoma
middle meningeal
Cresecent shaped lesion on CT
lesion of what vessels
subdural hematoma
bridging veins - stretched w/ cortical atrophy in the elderly and prone to tear
Lucid interval preceding neurologic signs
how long and associated w/ what
epidural hematoma
12-24 hrs before collapse
Herniation definition and 3 forms
displacement of brain tissue by mass effect or increased cranial pressure
Tonsillar herniation
Subfalcine herniation
Uncal herniation
tonsillar herniation is?
Compression of what?
cerebellar tonsils into the foramen magnum leading to cardio pulmonary arrest
brain stem
cingutate gyrus under the flax cerebra called?
Compression of what?
subfalcine herniation
compresses tha anterior cerebral artery ->infarction
eye rolling down and out w/ infarction of the occipital lobe (contralateral homonymous hemianopsia) and brain stem hemorrhage
Compression of cranial nerve III, posterior cerebral artery and paramedian artery
All due to Uncal herniation
Oligodendrocytes
Myelinate the CNS
Schwann Cells
myelin ate the PNS
deficiency or aeylsulfatase leads to?
Accumulation of what?
Leukodystrophy (accumulation of myelin)
auto recessive
Sulfatides cannot be degraded and accumulate in oligodendocyte lysosomes
Demylinating disorder
Krabbe disease is a deficiency in ?
Accumulation in?
Glactocerebrocidase
Accumulation of glactocerebrocide in macrophages
demylinating disorder
X linked defect that has impaired addition of coenzyme A to long chain fatty acids
Damage to 2 organs
Adrenoleukidystriophy
Adrenal glands and white matter of brain
Demylinating disorder
HLA DR2
Autoimmune destruction of CNS myeline and oligodendrocytes
MS is more commonly seen in in who where?
young adults , (20-30s), women
colder environements
What is internuclear opthalmoplegia and what is it associated w/
Lesion of the medial longitudinal fascicles leading to impaired coordination of CN3 and CN6 in eye movement (one does not move)
Seen in MS
Scanning speech is characteristic of
what is it?
MS
mimicking alcohol intoxication
Symptoms of MS (8)
Burred vision - one eye
Vertigo
Scanning speech
internuclear opthalmoplegia
Hemiparesis or unilateral loss of sensation
lower extremity weakness or loss of sensation
bowel, bladder, sexual dysfunction - sympathetics
Neurologic symptoms w/ periods of remission - multiple lesions separated by time and space
MS
Oligoclonal IG bands, increased lymphocytes and immunoglobulins on LP
MS
-also see myelin basic protein
Rx for acute and chronic MS
steroids
Chronic - interferon beta
MS MRI results
plaques - white matter demylination
Slowly progressing persistent infection of the brain?
due to
Characterized by
Subacute sclerosing PANencephalitis
Measles
Viral inclusions in the neurons AND oligodendorcytes
JC virus infects what neural structure causing?
Characterized by?
oligodendrocytes infected
causing Progressive multifocal leukoencephalopathy
Progressive multifocal leukoencephalopathy presentation
rapidly progressive nuerologic signs
-visual loss, weakness, dementia-> death
Rapid overcorrection of patient’s electrolytes can lead to ?
Which electrolyte in particular
central pontine myelinolysis
Na
Acute bilateral paralysis sets in a patient being treated for alcoholism or a liver transplant patient think?
central pontine myelinolysis
Rapid Na correction
Degeneration of cortex ->
Dementia
grey matter
Degeneration of the brainstem and basal ganglia
Movement disorders
Grey matter
What is the most common cause of Death in alzheimers
infection
Loss of learned motor skills and language, nut and bed ridden, no focal neuro deficits
What other symptoms
Alzheimers
also have slow onset memory loss short progressing to long term
Progressive disorientation
Increased risk of Alzeimers(2) - sporadic
Old age
Apo E Allele 4; Apo E Allele 2 protective
Early onset Alzheimers seen w/ (2)
Down syndrome (APP on 21) presenilin 1 and 2 mutations
Cerebral atrophy w/ narrowing of the gyri and widening of the sulci, dilated ventricles?
Also see what histological changes
Alzheimers
Abeta amylod
Amyloid precursor protein importance
coded on chromosome 21
- beta claevage vs normal alpha leads to accumulation
May deposit in vessels -> hemorrhage risk
hyperphosphoryated tau protein seen in ?
Make up what ?
Alzheimers
neurofibrillary tangles - tanges due to lack of organization of the microtubules
2nd most common cause of dementia?
Causal agents(3)
Vascular dementia.
HTN, Athersclerosis or vasculitis leading to multifocal infarction or injury
Round aggregates of tau protein
Pick bodies
in the neurons of the cortex of Pick’s Disease
Degenerative disease of the frontal and temporal cortex selectively
Symptoms(3)
Picks disease
Behavioral and language symptoms leading to dementia
Substantia nigra of the basal ganglia implicated in what disease?
what is lost?
Parkinsons
Lose dopaminergic neurons
Clinical features of Parkinsons(4)
TRAP
Tremor - pill rolling tremor at rest
rigidity - cogwheel rigidity
Akinesia/bradykinesia - slowing of voluntary movement- flat affect
Postural instability - shuffeling gate
Loss of pigmented neurons in the substantial nigra seen in what?
What are lewy bodies?
Parkinsons
Lewy bodies are round eosinophilic inclusions of alpha-synuclein - (LATE course vs Lewy body dementia)
Role of dopamine in Movement
D1 has a positive influence on positive movement in the cortex from the striatum of the basal ganglia
D2 inhibits the inhibition of the movement in the cortex
Both boost the signal
Dementia, hallucination ans parkinson like syndromes seen in less than a year is?
See what in histology?
Lewy body dementia
Cortical lewy bodies seen in cortex (Vs just the inner basal ganglia)
Huntington’s is due to loss of what where?
Lose GABAergic neurons in the caudate nucleus of the basal ganglia
The genetic component of Huntington(3)
AD disorder
trinucleotide repeat of CAG
expansion during spermatogenesis -> anticipation
Presentation of Huntingtons(2)
Chorea - loss of inhibition -> random movement
Athetosis - slow involuntary snake like movement of fingers
Dementia
depression
- Age 40
Elderly patient w/ urinary incontenince, gait instability and dementia
caused by?
Normal pressure hydrocephalus
- increase CSF -> dilated ventricles w/ arachnoid granulations not draining as well
- Wakyness due to stretched corona radiate next to the ventricles
What improves normal pressure hydrocephalus?
Presentation?
Lumbar puncture improves symptoms
Ventriculoperitoneal shunting Rx
Wet wobbly and wacky
PrP c
normal prion protein in CNS neuron
alpha helical conformation
PRP sc
beta pleated conformation -> encephalopathy
does not degrade and conerts normal protein to pathologic protein
Spongiform encephalopathy is due to? (3)
sporadic (CJD)
inherited (familial)
transmitted (mad cow)
Damage to neurons and glial cells w/ intracellular vaculoles
Spngiform encephopathy
Rapidly progressive dementia w. ataxia and starle myoclonus
See what on EEG
Creutzfeldt Jakob disease
most common form of spongiform encephalopathy
Periodic sharp waves
Familial encephalopathy characterized by(2)
Severe insomnia
exaggerated startle response
well circumscribed lesion found at the grey white border of the brain
METS
Usually lung breast and kidney
Most common brain tumors of adults (3)
Seen usually
glioblastoma multiforme - astrocyte derived
meningioma
schwannoma
Supratentorium
Most common brain tumors of kids (3)
Seen usually
pilocytic astrocytoma
ependymoma
medulloblastoma - neuroectoderm derived
Seen infratentorium
Cerebral tumor that crosses the corpus collosum - butterfly lesion?
Histology you see
Glioblastoma multiforme
See necrosis w/ pseudopalisading and endothelial proliferation
MOST COMMON in ADULTS - Malignent
GFAP positive tumor cells(2)
Glioblastoma multiforme - adults
Pilocytic astrocytoma - kids
positive due to presence of intermediate filament in glial cells
Cerebral lesion presenting w/ seizures and a round mass attached to the dura
Preferentially seen in?
Meningioma
Seen in women
Histology shows whorled appearance in this benign tumor of arachnid cells
also see what on histo
Meningioma
psammoma bodies
Presentation of hearing loss and tints w/ a S100 marker
Schwannoma - benign
Frequently affects CN8 at the cerebrellopontine angle
Schawnnomas are seen bilateral in
Neurofibromatosis type 2
Calcified white matter in the front lobe presenting w/ siezures
Oligodendroglioma
Malignant tumor
Fried egg appearance on biopsy -
Oligodendroglioma
cystic lesion w/ mural nodule seen in a kid
pilocytic astrocytoma
Benign tumor of cerebellum
Rsenthal fibers are what?
thick eosinophilic processes of astrocytes
also are GFAP positive
small round blue cells -> hormer wright rosette
original cell type?
Medulloblastoma - malignant tumor seen in kids
Neuroectoderm derived
Drop METS refers to
medullablastoma which spreads to CSF and cauda equine due to rapid growth
poor prognosis
tumor along the 4th ventricle leading to hydrocephalus
Histology see?
ependymoma - malignant tumor in kids
perivascular psudorosettes
Cariopharyngiomas come from
May present w/
epithelial remnants of Rathke’s pouch
Supratentoral mass in a child or adult
Bitemporal hemianopsia due to (2)
pituitary adenoma
craniopharyngioms - especially if a kid
Calcifcation on imaging of this benign but highly recurring tumor
craniopharyngioma
unilateral facial drooping involving the forehead
bells palsy
Weber syndrome which nerve?
contrallateral hemiparesis and eye motor nerve palsy w/ CN3
Medial pontine syndrome which nerve?
Contralateral hemiparesis and sensation and abducens
Posterior infereior cerebellar arter infarct affects which nerves
glossopharyngeal, vagus and spinal accessory
Symptoms of Central Pontine myelinilysis(6)
acute paralysis dysarthias dysphagias diplopia LOC locked in syndrome
- overly rapid correction of Na
- also see increased signal density in the pons
Corticospinal tract desiccates
above the medullary pyramids in the caudal medulla before descending
Spinothalmic tract desiccates
early and crosses in anterior white cosmissure before ascending
Dorsal thalamic pathway desiccates?
later in the caudal medulla
after becoming the medial lemniscal
Lesion of the R vagus nerve or nuclei -> uvula deviates?
deviates to the left. (pulled to the right)
Right side is not working
- could have issues w/ the left corticobulbar tract or
soft palate portion of the left motor cortex
Lesion of the L hypoglosseal nuclei or nerve the young deviates
Pushed to the right side
-Licking your wound
-Could also have issues with the Right corticobulbar tract and higher up the tounge portion of the right motor cortex
Artery that supplies the medial lemniscus and medullary pyramids
Anterior spinal artery
Stroke differs from Bells palsy how
Stroke you have eyebrow function
- contralateral paralysis of the lower face - UMN lesion
- the ipsilateral side of the lesion compensates for the upper portion of the face
bells palsy is lower in the tract and thus you have ipsilateral loss of upper and lower face
6 diseases you see bells palsy
AIDS
Lymes
Herpes simplex/zoster
less common-
Sarcoidosis,
tumors
Diabetes
midline CN nuclei in the brainstem
12 divides equally
- 3 - mid brain
- 4 - mid brain
- 6 - pons
- 12 - medulla
lateral CN nuclei in the brainstem
5 - pons 7 - pons 9 - medulla 10? 11 - spinal cord/medulla
berry aneurysms in the circle of will associated w/ (3)
ADPCKD
Ehlers Danlos
Weber Syndrome is due to a lesion in
Level of lesion
See(2):
CN (1)
Paramedian branches of posterior cerebral artery
Middle lesion (12/3) at the midbrain level
CN 3 involvement - ophtalmoplegia, ptosis, dilation and down and out
Cerebral peduncle lesion -> contralateral spastic paralysis(lesion occurs before motor cortex info coming down hits the cerebral peduncles)
Causes of locked in syndrome (2)
pontine lesions
hyponatremia rapid correction
basalir artery rupture
Medial inferior pontine syndrome lesion in
Symptoms?(4)
CN (1)
Basilar artery - paramedial branches
contralateral spastic hemiparesis -corticospinal
contralateral loss of light/touch and kinesthetic - ML
Paralysis of gaze to side of lesion (CN6 and PPRF)
Ipsilateral paralysis of Lateral rectus (CN6)
lateral inferior pontine syndrome lesion in
Symptoms(7)
CN (3)
Anterior inferior cerebellar Artery (AICA)
- Lateral lesion and a lower pontine, rule of 4
ipsilateral facial nerve palsy - CN 7
ipsilateral loss of taste anterior 2/3 - CN7
ipsilateral deafness, Nystagmus, N/V - CN8
Ipsilateral limb and gait ataxia - middle and inferior cerebral peduncles
ipsilateral loss of pain and temp from face - spinal trigeminal (CN5) tract
Contralateral loss of pain/temp body - spinothalmic
Ipsilateral horner
Lateral Superior pontine syndrome lesion
Symptoms(7)
CN - 2
Anterior inferior cerebellar artery (AICA)
lateral and upper pontine level
Ipsilateral loss of taste - anterior 2/3 -CN 7
ipsilateral limb and gait ataxia damage to middle and inferior cerebella peduncle
ipsilateral loss of pain/temp to face - spinal trigeminal nucleus (5) and nerve fiber
ipsilateral loss of light touch and vibration from face - Main sensory trigeminal nucleus
ipsilateral jaw weakness and deviation of jaw towards lesion - trigeminal motor nut
contralateral loss of pain/temp from body - spinothalamic
ipsilateral horner - descending symp
medial medullary syndrome lesion
Symptoms(3)
CN - 1
anterior spinal artery (ASA)
-medial lesion of medulla
Contralateral spastic hemiparesis - corticopinal
contralateral tactile and kinesthetic defects
-ML
Tounge deviates towards the lesion (CN12) - middle divisible by 12
Wallenberg syndrome lesion
Symptoms(6)
CN - 3
AKA Lateral medullary syndrome
Posterior inferior cerebellar artery (PICA)
Loss of pain/temp contralateral body - spinothalmic
loss of pain/temp ipsilateral face - trigeminal thalamic
hoarsness/swallowing. loss of gag - CN 9 and 10
Ipsilateral horners
Vertigo, nystagmus, N/V (Some CN 8 nuc in medulla)
ipsilateral cerebellar deficits - inferior cerebellar peduncle
Presentation of internuclear opthalmoplegia (2)
Lesion is where?
Nystagmus of affected eye lesion and pact of adduction in lateral gaze movement
Convergence is normal
Lesion is in the medial longitudinal fasiculus
Causes of MLF syndrome (2)
Stroke >50 years old
MS < 50 years old - HIGHLY myelinated
lack of communication w/ CN6 and CN III* so they do not work together
most common site of berry aneurisms
anterior communicating artery
Assiated risk factors in berry aneurisms in addition to congenital defects (4)
Race - blacks
HTN
Smoking
Advanced age
Complications of a epidural hematoma (2)
Presentation on CT
transtentorial herniation w/ CNIII palsy
lucid period -> coma
CT shows biconvex (lens hyper dense blood collection
CT bone looks ?
Blood?
Order what when ruling out ischemic vs hemorragic stroke?
White
Hyperdense whitish
CT w/out contrast