Neuro -Disease Flashcards
(419 cards)
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