NEUROMUSCULAR DISEASES

Disease / Overview / Epidemiology / Clinical Presentation / Prognosis
Multiple Sclerosis / - Auto-immune disease
- Multiple scarring of demyelinated areas of CNS
- Common sites: Pyramidal tracts, Post column, Optic nerve, Brainstem, Cerebellar peduncles, Large veins. / - 85% are 15-50 yrs old (avg- 30)
- Womenmen (2:1)
- WhiteBlack,Eskimo,Asian
- Temperate climates higher risk
- Geographic location in 1st 15 yrs of life. / Fatigue
Walking problems
Bowel & bladder problems
Pain & other sensations
Visual disturbances
Cognitive problems
Tremors / - Unpredictable
- Initial: return to normal fxn b/w attack
- Progresses: progressive loss of fxn w/ less improvement b/w attacks.
- ¼ of people require assistance for ADLS
Amyotrophic Lateral Sclerosis (ALS) / - Motor Neuron Disease
- Ant. horn cells & motor cranial nuclei
- Degenerative, terminal disease
- Onset: UE > LE > bulbar
- NO cure; Unknown cause / - 1-2/100,000 people
- men > women (2:1)
- Onset: 50s (2/3 b/w 50-70)
- 90-95% sporadic ALS
- 5-10% genetic ALS / Asymmetrical wkness
Cramping/muscle twitching
Pyramidal tract dysfunction
Dysphagia
Dysarthria
Cognition remains intact / - Rapid progressive wkness
- Respiratory failure
- Death: 3-5yrs (50% survive over 4)
- Mechanical vent increases survival
- Initial: bulbar & resp wkness  life expectancy is shortened.
Guillain-Barre Syndrome (GBS) / Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)
- Acute nerve inflammation causing demyelinization & muscle wkness/paralysis & sensory loss.
- No pre-disposing factors are known / - 1:100,000 (men > women)
- White > Black
- Young adults
- 5th – 8th decades
- Immune-mediated disorder
- More common in JapanChina / - ASCENDING paralysis
- Rapid, symmetrical (24-72hrs)
- Max paralysis 1-3 wks
- Numbness, altered sensation
- Blurred vision, palpitations, difficulty move face muscles, muscle contractions / - 50% minor deficits
- 15% persistent residual effects in fxn
- 80% ambulatory in 6 months
- 5% die (resp, cardiac, etc)
- W/in 2-3 wks: remyelinization begins
- Strength returns in DESCENDING pattern.
Post Polio Syndrome (PPS) / - Affect 10-40 yrs after Polio
- No known cause but may be 2o to overuse of muscles from past when rehabbing w/ Polio
- Metabolic overload on the limited capacity of the neuromuscular system
- Neuronal loss w/ normal aging / - Incidence not known 2o lack record
- ~600,000 living w/ past Polio
- 20-40% have PPS Sx / Unaccustomed fatigue
New wkness in muscles
Pain in muscles/joints
Sleeping problems
Breathing difficulties
Swallowing problems
Fxn’al decline in activity / - Very slowly progressing condition
- Long periods of stability
- Decreased ability to tolerate cold temp
Parkinson’s Disease / - Progressive degeneration of dopamine producing cells of BG
- Loss of dopamine in striatum / - Over 65: 1/1,000
- Over 80: 4/100 (25%)
- ~ 1 million in America / Rigidity
Mask-like face
Bradykinesia
Resting tremor
Dementia ~ 50%
ETC…. / - More than 35% fall every year w/ 18% having Fxs.
- Dementia (30-93%)
- Slowly progressive w/ long subclinical period (20-30yrs)
Alzheimer Disease / - Atrophied Cerebrum
- Most common type of Dementia in elders
- 4th leading cause of death in elders
- Possible Risk Factor: Genetic, level of edu, females, history of head injury, heavy metals/toxins, positive family Hx. / - 65: 10% 85: 50%
- 75 y/o & older: Women>Men (3:1) / Loss of memory & intellectual
Restlessness & wandering
Decrease word finding
Depression
Impaired judgment
General wkness/fatigue
No Dx until autopsy or brain biopsy / - Slowly progressive disease
- 8-10 yrs
- Meds to help treat but NO cure
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