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To examine animal exposure in ALS patients, a case-control study was undertaken on 40 ALS patients and 40 closely matched controls. Exposure to pets of any kind and to small dogs in the period from birth until 10 years before onset of ALS symptoms was significantly increased. Affected men showed a trend toward increased exposure to neurologically ill pets, but there was no significant difference when male and female cases were grouped. No differences between ALS cases and controls were observed in exposure to any other single animal species. There may be a link between ALS and exposure to house pets.

A double-blind, placebo-controlled trial of single doses of thyrotropin releasing hormone (TRH) was performed on 12 patients with amyotrophic lateral sclerosis. Each patient was given subcutaneous injections of TRH 150 mg or placebo, and IV infusions of TRH 500 mg or placebo at 72- to 96-hour intervals. Eight motor and functional ratings were scored at regular intervals after each injection. Side effects were seen in all patients and were obvious to patients and examiners, making true blinding impossible. Nevertheless, statistically significant improvement was seen only in dynametric strength 1 hour after subcutaneous injection (p less than 0.05). Significant improvement occurred, in one patient only, on subjective speech testing during IV infusion of TRH. In none of six other ratings was there a significant difference between TRH and placebo. Subjective improvement was noted by 11 of 12 patients.

ALS is the most common of the various MNDs, which also include the clinical entities of PBP, PMA, and PLS. Mean age of onset of ALS is 57 years, and there is a sex predilection for men in a ratio of 1.5:1. Area of first symptom is in the lower extremity is 36 per cent of cases, in the upper extremity in 32 per cent, and 25 per cent of patients have a bulbar onset. Motor cranial nerves in the lower brain stem from cranial nerve nuclei or corticobulbar tract degeneration are affected. This results in symptoms of speech and swallowing difficulty and emotional lability in up to 60 per cent of cases. One hundred per cent of cases have motor weakness, over 90 per cent have muscle atrophy and fasciculations, and 47 per cent have spasticity. There seems to be a pattern of progression of ALS signs and symptoms based on area of onset with LLE involvement tending to follow RLE weakness, LUE weakness following RUE onset, and RUE involvement following next in patients whose onset is bulbar. Significant numbers of ALS patients had sparing of involvement of parts of the body for follow-up times approaching 3 years. Although the majority of patients experience a deterioration that is significantly linear, seven of 28 patients or 25 per cent achieved a plateau lasting a minimum of 9 months. Survival in our series was 4.08 years for all forms of MND.(ABSTRACT TRUNCATED AT 250 WORDS)