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Pathology F10

Other Neurologic Disorders

QuestionAnswer
Amyotrophic Lateral Sclerosis (ALS) -aka Lou Gerhig's Disease -most common motor neuron disease (upper & lower are affected) -short life expectancy (FATAL disease- within 4 yrs of diagnosis) -10% of cases are hereditary
Pathology of ALS -motor functioning is completely eliminated -sensory nerves are entirely preserved (therefore sensory and cognitive functioning are still there)
Guillian-Barre Syndrome -aka Acute inflammatory demylinating polyneuropathy (AIDP) -symmetrical
Pathology of Guillian-Barre Syndrome -immune system damages myelin on peripheral nn -progressive ascending paralysis of peripheral nn -begins at the feet & ascends via dermatomal/myotomal patterns -once deltoid involvement, get to emergency room IMMEDIATELY as the diaphragm is next to go
Symptoms of Guillian-Barre Syndrome -can progress so quickly that you go to bed fine & the next morning you can't move -symptoms may reverse -once the body begins remyelinating but may not have full recovery -reversal is descending (opposite) -very few die
Guillian-Barre may be triggered by: -infection with campylobacter (a type of bacteria often found in undercooked food, especially poultry) *most common -anesthesia -Epstein-Barr virus/Mono -Hodgkin's disease -HIV (immunocompromised) -rarely rabies or influenza immun
Polio -virus attacks ant horn cells in SC -random attacks= random location of m weaknesses -lower motor neuron disease
Giant Motor Units associated with Polio -innervation to mm are lost, and in the recovery, adjacent motor neurons send out additional innervation to the orphaned mm (C5 doesn't just innervate the delts, it innervates the whole arm) -in post-polio syndrome= giant neurons are lost=> more weaknes
Polio Signs & Symptoms -fever -sore throat -headache -vomitting -fatigue -back pain or stiffness -neck pain or stiffness -pain or stiffness in arms or legs -muscle spasms or tenderness -polio meningitis *last 2-10 days; after these are gone, the result is muscle weakn
Huntington Disease -caused by an expansion repeat (CAG)mutation in gene coding for the protein huntingtin on chromosome 4
Signs of Huntington -initial signs of chorea are flickers in fingers and ticlike grimaces of the face -eventually they become higher amplitude dancelike movements -begin to disrupt voluntary actions of the extremities & interfere with gait -speech becomes dysrhythmic
Early signs of Huntington -personality changes (irritability, anger, depression, loss of interest) -decreased cognitive abilities (diff making decisions, learning new info, answering ??, remembering imp pts) -mild balance probs -clumsiness -invol face mvmnts (grimacing)
Later signs of HD -sudden jerky, invol mvmnts (chorea) throughout body -severe probs w/ balance & coordination -jerky, rapid eye mvmnt -hesitant, halting or slurring -swallowing probs -dementia **life expectancy=10 yrs after diagnosis
Dystonia -involuntary twisting mvmnt characterized by sustained mm contraction -cocontraction occurs/freezing episodes -2/3 of cases are idiopathic -can be focal (as in hands-seems as if overuse issue) 30-50 yrs old
Tardive dyskinesia -dystonia caused by meds (psychotrophics- for mental illnesses) -disorder of mvmnt; grimacing, slow kind of mvmnt
Created by: CWestrick