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neuropathology 4-15


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name two diseases that affect the CEREBRAL CORTEX
1) Alzheimer's disease 2) Pick's disease
name two diseases that affect BASAL GANGLIA & BRAIN STEM:
1) Huntington's disease 2) Parkinson disease
in the spinal cord:

name 3 degenerative MOTOR NEURON diseases

Name 3 degenerative SPINOCEREBELLAR diseases
1)ALS = amyotrophic lateral sclerosis 2)Werdnig-Hoffmann disease 3) Polio

1) Olivo-ponto-cerebellar atrophy 2) Friedreich's ataxia 3) subacute combined degeneration [B12 deficiency]
what is the most common cause of elderly dementia?
name two pathological findings characteristic of Alzheimer's
1) senile plaques = intracytoplasmic inclusion bodies 2) neurofibrillary tangles (abnormally phosphorylated tau protein)
what is the 2nd most common cause of dementia in elderly?
multi-infarct dementia in elderly\
How can Alzheimer also affect intracranial vasculature?
amyloid angiopathy --> intracranial hemorrhage
The familial form of Alzheimer's is associated with what chromosomes and (name the allele's name in 2 of the 4)
chromosome 1, 14, 19 (APO-E4 allele), 21 (p-App gene)
What is pathognemomic with Pick's disease upon histology?
Pick bodies = intra-cytoplasmic inclusion bodies
What cortical areas does Pick's disease affect
Frontal and temporal lobes (remember; sharp, atrophic appearance of gross specimen)
What is the inheritance pattern of Huntington's
Auto Dominant
What are some symptoms of Huntingtons?
1) chorea 2) dementia
Huntington's is due to atrophy of _____ what?
atrophy of caudate nucleus = loss of GABA-nergic neurons
what is the genetic abnormality of Huntington's?
1) Chr. 4 - expansion of CAG repeats
Parkinson disease is associated with what pathology findings?
1) Lewy bodies 2) depigmentation of substantia nigra (loss of dopaminergic neurons)
rare cases of Parkinson's have been linked to what contaminant of certain illicit drugs?
MPTP = contaminant to street drug
Parkinson's can make you feel in a "TRAP" = ?
T = tremor at rest; R = cogwheel rigidity; A = akinesia ; Postural instability
ALS = Lou Gehrig's disease (the Iron Horse of the Yankees, hero of Joe DiMaggio) = what signs is ALS associated with?
Both UMN and LMN deficits
Werdnig-Hoffman disease - presents as birth as?
1) floppy baby syndrome 2) note tongue fasciculations as well (also seen in ALS)
for Polio, what kind of signs to you see?
predominantly LMN deficits.
name 4 types of cranial related hemorrhages (think of layers that could possibly be involved)
1) epidural hemorrhage 2) subdural h 3) subarachnoid h 4) parenchymal h
what is a common site of epidural hematoma/hemorrhage
1) rupture of MMA: middle meningeal artery, often 2ndary to fracture of temporal bone
what does CT show for epidural h?
biconcave disc NOT crossing suture lines
what else do you see in epidural hematoma?
lucid interval
How do subdural h. most often occur?
rupture of bridging veins (think, elderly being jolted in roller coaster)
subdural hematoma is venous bleeding so how would this influence symptomatic findings?
venous = less pressure = delayed onset of symptoms
what types of people do you see subdural h.?
1) elderly, 2) alcoholics 3) blunt trauma/sudden change in velocity injuries
for SUBDURALs what do you see upon CT?
1) crescent-shaped hemorrhage instead 2) YES IT does cross the suture lines
subarachnoid hemorrhage is often seen where (name 2 types
rupture of 1) aneurysm (usually Berry aneurysm) or an 2)AVM
symptoms of subarachnoid hemorrhage?
the worst headache of my life
since its subarachnoid (hemorrhage), what do you see on what test?
bloody or xanthochromic CSF on spinal tap
name 4 risk factors for parenchymal hematoma
1) HTN 2) amyloid angiopathy 3) diabetes mellitus 4) tumor
most common site for Berry aneurysms
bifurcation of the anterior communicating artery (Circle of Willis)
berry aneurysms are associated with which genetic diseases
adult polycystic disease, Ehlers-Danlos s., and marfan's
Classic triad of Multiple Sclerosis
SIN: Scanning speech, intention tremor, nystagmus (affect woman 20-30s)
Prevalence of MS
increase prevalence w/ increased distance from the equator
Clinical s/s of MS, labs, pathology.
periventricular plaques, preservation of axons, loss of oligodentrocytes, reactive astrocystic gliosis, increased protein in csf(IgG) in CSF, relapsing course, optic neuritis, MLF syndrome, hemiparesis, bladder/bowel incontinence)
Progressive multifocal leukoencephalopathy (PML) is associated w/
JC virus and seen in 2-4 % of AIDS pts (reactivation or latent infect)
pathogenesis of Guillian-Barre syndrome
Inflammation and demyelination of peripheral nerves and motor fibers of the ventral roots (sensory effec less severe than motor)
s/s of Guillian-Barre syndrome, lab findings
symmetrical ascending muscle weakness beginning in the distal lower extremities. Autonomic fxn may be severely affected (eg. Cardiac irregularities, HTN, or hypoTN) Findings: inc. csf protein w/ normal cell count ("albumino-cytologic dissociation") elevated protein may lead to papilledema
association between G-B syndrome and⬦
herpesvirus or camplobacter jejuni infection, inoculations, and stress but no definitive link to a pathogen
organism causing polio and mechanism of action
poliovirus, transmitted via fecal oral route and enters blood stream then into CNS where it causes destruction of the anterior horn of S.C. leading in turn to LMN destruction
s/s of polio
malaise, HA, fever, nausea, abd. Pain, sore throats, signs of LMN signs
lab findings of polio
csf w/ lymphocytic pleocytosis w/ slight elevation of protein, virus recovered from stool or throat
describe Broca's (expressive) aphasia
confluent aphasia w/ intact comprehension broca's is broken speech
describe Wernicke's (aphasia)
Fluent aphasia w/ impaired concentration Wernick's ="What?" W area located in superior temperal gyrus
describe two types of partial seizures
1.simple partial (awareness is intact)--motor, sensory, autonomic, psychic 2.Complex partial (impaired awareness)
describe types of Generalized seizure ( diffuse)
1.absence- blank stare (petit mal) 2.myoclonic- quick repetitive jerks 3.tonic-clonic- alternating stiffening and mvmt (grand mal) 4. Tonic- stiffening 5.atonic-"drop" seizures
what are the causes of seizures in children?
genetic, infection, trauma, congenital, metabolic
what are the causes of seizures in adults?
tumors, trauma, stroke, infection
what are the causes of seizures in elderly?
stroke, tumor, trauma, metabolic, infection
what is Horner's syndrome?
sympathectomy of face (lesion above T1) 1.Ptosis 2.anhidrosis and flushing of affected side 3.miosis [PAM is horny]
Horner's syndrome is associated w/ what type of tumor?
pancoast tumor
what is syringomyelia?
enlargement of the central canal of the S.C. Crossing fibers of spinothalamic tract are damaged.
what are the s/s of syringomyelia?
b/l loss of pain and temp sensation in the upper extremities w/ the touch sensation. Most common at C8-T1
syringomyelia commonly seen in what pts?
pts w/ Arnold-Chiari malformation
Tabes dorsalis what infection is tabes dorsalis associated w/?
teriary syphilis
Tabes dorsalis what happens in the CNS?
degeneration of dorsal columns and dorsal root
Tabes dorsalis dorsalis also is associated with what s/s?
charcot's joint, shooting (lightning) pain, Argyll Robertson pupils, and absence of DTRs

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