an ALS Hypothesis

dag.stenberg at helsinki.nospam.fi dag.stenberg at helsinki.nospam.fi
Mon Jan 31 09:34:14 EST 2000


John H. <johnhkm at netsprintXXXX.net.au> wrote:
> I thought it may have been a SOD problem, but haven't managed to have a
> close look at it yet. Isn't there a familial tendency here also?

> Would someone more knowledgeable than the current authors in this thread
> care to enlighten? I know something about apoptosis etc but ALS .... .

Fast attempt at partial answer:
- ALS = amyotrophic lateral sclerosis in a degenreative disease
afflicting both upper (brain, especially cortex) and lower (spinal cord 
plus lower brain stem) motor neurons selectively. 
- It leads to progressive paralysis and muscle atrophy because of the 
degeneration of lower motoneurons, and to spasticity due to the
degeneration of upper motor neurons.
- a synonym is Lou Gehrig's disease. A famous living patient is Steven
Hawkings, a most famous scientist and public figure.
- paralysis and atrophy usually start in limbs, not always symmetrically
- the last to be afflicted is brain stem: swallowing, respiration etc.
- sometimes concomitant symptoms, not clearly motor, show as emotional
instability, proneness to laughter, crying etc. 
- the disease may start between 30 and 60 years of age
- it progresses in some cases until death (from respiratory paralysis)
but often stops from unknown reason, perhaps only paralyzing the legs,
making the patient wheel-chair-bound.
- no treatment yet, only symptomatic help
This about the clinical picture.

- in about 5-10% of patients there seems to be a familial predisposition
- in these there has been shown mutations in the SOD1 gene
- the article KPC wrote about is about a mouse model of lower motor
neuron degeneration
- in this article mitochondrial involvement is shown


-- I hope this is all right, have to run....
-- John, I have stored your question about the Lanfumey paper on 5HT1A.
I will get back to it, but a certain urgent project is in its final
stage.

Dag Stenberg




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