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primary lateral sclerosis (PLS)
Epidemiology:
1) rare
2) onset usually occurs after age 50
Pathology:
1) exclusively upper motor neuron disease
Genetics: not hereditary
Clinical manifestations:
1) progressive muscle weakness
2) difficulty with balance
3) weakness & stiffness in the legs
4) clumsiness
5) spasticity in the hands, feet, or legs
6) foot dragging
7) dysarthria due to involvement of the facial muscles
8) usually begins in the legs, but it may also start in the tongue or the hands
9) progresses gradually over a number of years, or even decades
10) no amyotrophy that occurs in amyotrophic lateral sclerosis (ALS)
Differential diagnosis:
-> amyotrophic lateral sclerosis (ALS)
Management:
1) treatment is symptomatic
2) baclofen & tizanidine may reduce spasticity
3) quinine or phenytoin may decrease cramps
4) Physical therapy often helps prevent joint immobility
5) Speech therapy may be useful for those with involvement of the facial muscles.
6) prognosis
a) no cure, but not fatal
b) patients rarely lose the ability to walk
Related
amyotrophic lateral sclerosis (ALS); Lou Gerig's disease
General
motor neuron disease
References
- Asbury, McKhann, McDonald. Diseases of the Nervous
System. Clinical Neurobiology. Saunders 1992
- NINDS Primary Lateral Sclerosis Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Primary-Lateral-Sclerosis-Information-Page