Term
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Definition
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Term
|
Definition
global atrophy - most pronounced in frontal, temporal, parietal lobes
narrowed gyri, sulci widened
widening of ventricular spaces |
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Term
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Definition
L: normal (see large pyramid cells; neurons)
R: tangle and plaques of AD (tangles involve cell body and proximal axon) |
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Term
| histopathologic hallmarks of AD |
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Definition
neuritic plaques, neurofibrillary tangles
neuronal loss in hippocampus and neocortex |
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Term
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Definition
neuritic plaque of AD
occur OUTSIDE the cell body
core of amyloid-beta surrounded by halo of abnormal astrocytes |
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Term
| dementia with Lewy bodies (DLB) |
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Definition
account for ~30% of all dementias
mild global atrophy, may see gross depigmentation of SN |
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Term
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Definition
DLB cingulate gyrus
some atrophy |
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Term
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Definition
DLB hippocampus (entorhinal cortex)
neuron loss and gliosis in hippocampus |
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Term
| histopathologic hallmarks of DLB |
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Definition
loss of substantia nigra neurons
loss of neurons in the basal nucleus of Meynert
presence of Lewy bodies in the cerebral cortex, most notable in:
-anterior cingulate gyrus
-entorhinal and insular cortices
-amygdala |
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Term
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Definition
Lewy bodies
cytoplasmic protein aggregates in neurons - alpha-synuclein protein
push neuronal nucleus off to the side |
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Term
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Definition
Pick disease - relatively rare type of senile dementia
grossly characterized by extreme atrophy of frontal and temporal lobes |
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Term
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Definition
L: Pick disease; Pick bodies
R: normal
dentate gyrus of hippocampus |
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Term
| histopathology of Pick disease |
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Definition
severe neuronal loss and gliosis of affects areas
presence of Pick bodies
ballooned neurons |
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Term
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Definition
| tau immunostain of Pick bodies in dentate gyrus |
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Term
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Definition
L: spongiform change in cortex of Creutzfeld-Jakob disease
R: normal brain |
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Term
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Definition
rapidly progressive dementia caused by transmissible protein "prion-protein"
may occur in familial forms
no noticeable atrophy |
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Term
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Definition
spongiform change in cortex - vacuolization of the neuropil
neuronal loss and gliosis may be mild, but can be significant in some cases |
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Term
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Definition
L: normal brain, see head of caudate bulging into ventricle
R: Huntington Disease, marked loss of caudate neurons |
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Term
Huntington Disease (HD)
gross |
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Definition
a familial degenerative movement disorder
gross pathology shows marked atrophy of the caudate nucleus and at times milder atrophy of putamen |
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Term
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Definition
L: gliosis of striatum
R: normal |
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Term
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Definition
| marked loss of small neurons in the caudate nucleus as well as severe gliosis |
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Term
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Definition
L: depigmented substantia nigra of Parkinson disease ("brown eyeliner")
R: normal SN ("black eyeliner") |
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Term
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Definition
movement disorder showing pallor of the substantia nigra and locus coeruleus grossly
nucleus basalis of Meynert also involved |
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Term
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Definition
L: loss of pigmented neurons in SN of PD patient
R: normal SN with large, pigmented neurons |
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Term
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Definition
loss of pigmented neurons and gliosis in SN and LC
loss of neurons and gliosis in nucelus basalis of Meynert
*characteristic Lewy body* - diagnostic for the disease |
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Term
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Definition
gliosis of subtantia nigra with Lewy body (arrow)
Parkinson |
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Term
| progressive supranuclear palsy (PSP) |
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Definition
progressive degenerative disorder involving mainly subcortical nuclei
most severely involved nuclei: globus palliidus, subthalamic nucleus, sunstantia nigra, red nucleus, periaqueductal gray area, inferior olivary nucleus, dentate nucleus of cerebellum |
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Term
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Definition
severe neuronal loss and gliosis
presence of tangles (light microscopically identical to those of AD) present in the subcortical nuclei (no plaques) |
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Term
| subthalamic nuclei tangles of PSP |
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Definition
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Term
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Definition
L: normal pons
R: loss of white matter tract to contralateral cerebellar hemisphere, marpken atrophy of pon; MSA |
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Term
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Definition
top: normal cerebellum
bottom: MSA cerebellum, loss of input and output fibers in cerebellum |
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Term
| multiple system atrophy (MSA) |
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Definition
| progressive neurodegenerative movement disorder clinically characterized by profound ataxia |
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Term
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Definition
atrophy of inferior olivary nucleus
loss of neurons in pontine nuclei
atrophy of cerebellar cortex |
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Term
| MSA involves multiple systems with varying severity |
|
Definition
olivopontocerebellar system - atrophy and loss of neurons
striatonigral system - degeneration of SN and basal ganglia
autonomic system - loss of autonomic neurons in the intermediolateral column, vagus nucleus and Edinger-Westphal nucleus |
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Term
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Definition
ALS
tabes dorsalis
Friedrich ataxia
syringomyelia |
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Term
| amyotrophic lateral sclerosis (ALS) |
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Definition
spinal cord disease that affects both the UMN and LMN
degeneration of spinal cord tracts (cortico-spinal)
loss of anterior horn cells and bulbar motor neurons
degeneration of peripheral motor nerves and neurogenic muscular atrophy |
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Term
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Definition
ALS spinal cord section
1: normal ascending dorsal column fibers
2: normal dorsal root (incoming sensory fibers)
3: pallor of lateral corticospinal tract from loss of myelin with the loss of UMN
4: atrophic ventral root with loss of myelin of exiting LMN |
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Term
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Definition
Friedrich ataxia
involvement of ascending and descending tracts |
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Term
|
Definition
familial form of ataxia with onset usually under the age of 20
peripheral and central nervous systems involved
-posterior columns
-dorsal root
-spinocerebellar tract
-corticospinal tract
-other brain involvement |
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Term
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Definition
Friedrich Ataxia
top: atrophic dorsal nerve roots, lack myelin fibers
bottom: anterior nerve roots preserved |
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Term
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Definition
Tabes dorsalis
axon loss in posterior columns |
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Term
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Definition
part of the neurosyphillis complex and involved degeneration of the posterior columns (sensory ascending tracts)
causes sensory ataxia |
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Term
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Definition
syringomyelia
asymmetrical cavitary lesion
NO involvement of central canal |
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Term
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Definition
tubular cavitation (syrinx) of the spinal cord with glial rim, quite expensive over a number of segments of cord
usually begins during 2md-3rd decade and is slowly progressive |
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Term
| possible syringomyelia etiology |
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Definition
developmental anomaly
trauma
tumor in cord |
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