Re: Amyotrophic lateral sclerosis
Re: Amyotrophic lateral sclerosis
Posted by CCF Neurology MD on September 20, 1997 at 22:16:28:
In Reply to: Amyotrophic lateral sclerosis posted by Leon L. Claypool on September 19, 1997 at 22:00:13:
: : Patient: Vo Thi Lan - Age: 46 - Sex: Female - Height: 5'5" - Weight: 79 lbs.
: I - History of disease:
: Occured 2 years ago with the following symptoms:
: Headache, trembling and little activity of upper and lower limbs, myotonic dystrophy and muscular twist on the upper limbs and the upper body. Weakness of upper and lower limbs. Atrophy of arm and shoulder muscles, difficulty walking. Dysphonia and dysphagia.
: II - Antecedent of disease:
: Lives in city in Central Vietnam. Twenty years of teaching in rural area. Mother has arterial hypertension. Uncle had cerebrovascular accident.
: III - Examination:
: Pulse: 80 to 90 and regular - Temperature 36.5 to 37.2 C - Blood pressure 130/80 - 140/90mmHg
: 1. Nervous system:
: a. Upper limbs: Regular myotonic dystrophy of uppers limbs, relaxing muscles, tendon reflex decreased and the following muscular twist and atrophy - Atrophy of thenar and hypothenar muscles (flat hands). Atrphy of interphalangeal muscles (two last interphalangeal bones). Atrophy of wrist flexor muscles (very abductive hands). Atrophy of shoulder muscles.
: b. Lower limbs: Regular spasm myotonic dystrophy of the lower limbs. Rigidity of walking, tendon reflex increased, Babinsky is positive.
: c. Dysphonia and dysphagia.
: d. Normal sensation, no sphincter trouble, no psychiatric trouble.
: 2. Other organs: Normal
: IV - Diagnosis: Amyotrophic lateral sclerosis.
: V - Treatment: Prednisolone, Stugeron, Papaverine, Vitamins, Valium and Physiotherapy.
: VI - Result: No response to therapy, progressively worse.
: PLEASE HELP!
=Dear Leon: Although "myotonic dystrophy" is not a feature of amyotrophic lateral sclerosis (ALS), the rest of your description may indeed be consist with the disorder, unfortunately. A precise diagnosis, however, with appropriate tests including an EMG examination, is mandatory. If Ms. Lan has not already had one, I strongly suggest that she undergo a detailed neurological evaluation at a reputed academic neurology department in Vietnam. If she can travel, coming to the US for such an evaluation would be ideal. ALS is unfortunately a relentlessly progressive neurodegenerative disease which ultimately leads to paralysis and wasting of muscles of the limbs, trunk, respiration, and swallowing.The average patient with ALS survives for less than 3-4 years after diagnosis unless he or she is maintained on artificial breathing and other advanced support measures. A minority of patients, however, may survive much longer.
Although there is no curative or dramatically effective medication for the treatment of ALS, some recently developed medications (riluzole, for instance) show a very modest beneficial effect. Several others are promising and are in advanced phases of clinical trial.
None of the medications Ms. Lan is on currently alters the course of ALS. Prednisone may, in fact, be deleterious if Ms. Lan indeed has ALS.
We are sorry to learn of Ms. Lan's problem; unfortunately, there is little help we can offer. This information is provided for general medical education purposes only. Please consult your physician for diagnostci and treatment options of your specific medical condition.
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