Path - Demyelinating, Neurodegenerative, etc. Flashcards
what disease process often follows a viral infection or sometimes a viral immunization?
acute disseminated encephalomyelitis (ADEM)
what disease process almost always follows an upper respiratory infection?
acute necrotizing hemorrhagic encephalomyelitis (ANHE)
list the inclusions seen in Huntington’s, Alzheimer’s, and parkinsons’
HD: expanded polyglutamine repeats
Alz: beta amyloid
PD: alpha-synuclein
describe the morphological hallmark of Alzheimer’s
global cortical atrophy starting in the frontal and temporal lobes –> then parietal lobes –> then occipital lobes
congo red stain will stain amyloid, and can be indicative of what dz processes
- alzheimers’s
- cerebral amyloid angiopathy
compare morphology of pick disease and AD
pick: only frontal lobes are affected
AD: global
what is the demographic for progressive supranuclear palsy
men (2x more likely)
5th-7th decade
compare adult and juvenile Parkinson disease in terms of inheritance and protein
adult: autosomal dominant, alpha-synuclein
juvenile: autosomal recessive, alpha-synuclein and parkin
morphology of brain of pt with HD
brain is small
- decreased body of corpus callosum
- decreased head of caudate nucleus
- decreased globus pallidus
what is anticipation and how does it affect patients with HD
repeat expansions during spermatogenesis lead to earlier onset fo the disease (juvenile form)
- inherited through the father
describe the pathogenesis of HD
there is loss of medium spiny striatal neurons that leads to dysregulation of basal ganglia circuitry that modulates motor output
failure to remove cells with DNA damage is characteristic of what dz process
ataxia telangiectasia
SOD1 (superoxide dismutase) mutation is associated with what dz process
ALS
bunina bodies are associated with what dz process
ALS
decreased side of anterior horn neurons is associated with what disease process
ALS
protein and location for Alzheimer
protein: beta amyloid and tau
location: extracellular neurons
protein and location for progressive supra nuclear palsy
Protein: Tau
location: neurons and glia
protein and location for Parkinson
Protein: alpha-synuclein
location: neurons
protein and location for ALS
Protein: TDP-43, SOD-1
location: neurons
what gene and enzyme are affected in Tay-Sachs
gene: HEXA
enzyme: hexosaminidase A (can’t degrade GM2 gangliosides)
age of presentation of Tay-sachs
about 1 year old, then death around ages 2-3
cherry red spots in maculae is indicative of what dz
tay sachs
in mitochondrial encephalopathies, what is the most commonly affected tissue, and what is the second
first: muscle
second: CNS
sx of kearn-sayre syndrome
cerebellar ataxia
progressive external ophthalmoplegia
pigmentary retinopathy
cardiac conduction defects
progressive inability to move the eyes and eyebrows
histologic hallmarks of kearn-sayre syndrome
spongiform change in the gray and white matter and neuronal loss most evidently in the cerebellym
progressive inability to move the eyes and eyebrows indicates what dz
kearn-sayre syndrome
what causes beriberi dz
thiamine deficiency (B1)
pseudolaminar necrosis of the cortex could be indicative what of what problem
hypoglycemia
