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Flashcards in XXIII - The Nervous System Deck (209)
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Morphology: In later stages of this disease, all muscles eventually become almost totally replaced by fat and connective tissue

Duchenne Muscle Dystrophy (TOPNOTCH)


What is the histologic pathology that is usually seen in Duchene muscle dystrophy and is rare in Becker Muscle Dystrophy?

Enlarged, rounded, hyaline fibers that have lost their normal cross striation, believed to be hypercontracted fibers (TOPNOTCH)


Morphology: Ring fiber ( subsarcolemmal band of cytoplasm that appears distinct from the center of the fiber) and Sarcoplasmic mass

Myotonic Dystrophy (TOPNOTCH)


Morphology: Disuse changes with Type 2 fiber atrophy, post synaptic membrane is simplified, with loss of AChRs from the region of the synapse. Immune complexes as well as the MAC of the complement cascade can be found along the post synaptic membrane as well

Myasthenia Gravis (TOPNOTCH)


Electron Microscopy: tubular and filamentous inclusions are seen in the cytoplasm and the nucleus, and they are composed of B-amyloid or hyperphosphorylated tau.

Inclusion body myositis (TOPNOTCH)


Grotton lesions (scaling erythematous eruption or dusky red patches over the knuckles, elbows, and knees) are seen in what disease entity?

Dermatomyositis (TOPNOTCH)


Morphology: the most consistent pathologic findings in skeletal muscle are aggregates of abnormal mitochondria that stain with modified Gomori Trichrome and fiber appears irregular on cross section "ragged red fibers" and on electron microscope, they contain "parking lot inclusions"

Mitochondrial Myopathies (Oxidative phosphorylation Disease) (TOPNOTCH)


This is general reaction of the motor unit which is a result of primary destruction of the axon, with secondary disintegration of its myelin sheath.

Axonal degeneration (TOPNOTCH)


This general reaction of the motor unit occurs when there is dysfunction of the Schwann cell or damage to the myelin sheath; there is no primary abnormality of the axon

Segmental demyelination (TOPNOTCH)


Morphology: endoneurial arterioles show thickening, hyalinization, and intense PAS positivity in their walls and extensive reduplicaton of the basement membrane.

Peripheral Neuropathy in DM Type 2 (TOPNOTCH)


Of all the dystrophies, this type is the only one that shows pathologic changes in the intrafusal fibers of muscle spindles, with fiber splitting, necrosis and regeneration

Myotonic Dystrophy (TOPNOTCH)


Morphology: the principal morphologic characteristic is accumulation of lipid within myocytes. The myofibrils are separated by vacuoles that stain with oil red O or Sudan black

Lipid Myopathies (TOPNOTCH)


Lambert Eaton Myasthenic Syndrome is a paraneoplastic process which commonly develops in what type of carcinoma?

Small cell carcinoma of the lungs (TOPNOTCH)


The most important histopathological indicator of CNS injury, regardless of etiology



Lafora bodies

Myoclonic Epilepsy(TOPNOTCH)


Gross morphology: gyri are flattened, intervening sulci are narrowed, and the ventricular cavities are compressed

Generalized cerebral edema(TOPNOTCH)


A syndrome of sudden, deep intracerebral hemorrhage that follows even minor head trauma by an interval of 1-2 weeks

Spat-apoplexie (Delayed post traumatic hemorrhage) (TOPNOTCH)


Subdural hematomas most often become manifest approximately how many hours after the injury?

48 hours(TOPNOTCH)


The most widely accepted explanation for diffuse axonal injury

mechanical forces damage the integrity of the axon at the node of Ranvier, with subsequent alterations in axoplasmic flow(TOPNOTCH)


Morphology: wide asymmetric distribution of axonal swellings that appear within hours of the injury and are best demonstrated with silver impregnation techniques or with immunoperoxidase stains for AB protein

Diffuse axonal injury(TOPNOTCH)


How long does it take for the clot to lyse in subdural hematomas?

1 week(TOPNOTCH)


How long does it take for fibroblasts to grow from the dural surfaces into the hematoma after subdural hematoma/

2 weeks(TOPNOTCH)


How long does it take for the development hyalinized connectice tissue in SDH

1-3 months(TOPNOTCH)


Gross morphology: brain is swollen, gyri are widened, and the sulci are narrowed

Global cerebral ischemia(TOPNOTCH)


In the setting of global ischemia, early histological changes are seen after how many hours after the insult?

12-24 hours(TOPNOTCH)


In the setting of global ischemia, subacute changes are seen after how many hours after the insult?

24 hours to 2 weeks(TOPNOTCH)


In the setting of global cerebral ischemia, repair is seen after approximately how many weeks?

2 weeks(TOPNOTCH)


In the setting of global ischemia, this is characterized by eventual removal of all necrotic tissue, loss of normally organized CNS structure, and gliosis



In the setting of global ischemia, this is characterized by necrosis of tissue, influx of macrophages, vascular proliferation and reactive gliosis

Subacute changes(TOPNOTCH)


In the setting of global ischemiaThis is characterized by acute neuronal cell damage

Early change(TOPNOTCH)