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The introduction of palliative therapies in amyotrophic lateral sclerosis (ALS) will alter the epidemiology of ALS as it is known now. Although incidence rates will remain unchanged in the near future, prevalence rates will likely increase dramatically. Better understanding of the age-specific presentation of motor neuron diseases worldwide will shed light on the vexing questions concerning the variable incidence rates in some countries and apparent incidence gradients in North America and Europe.

The general, systemic, and specific neuromuscular adaptation to physical exercise and training in healthy individuals is reviewed with application to individuals with neuromuscular disease in general and ALS in particular. A pilot study utilizing patients with ALS suggests the cardiopulmonary response to acute exercise is similar to normal individuals. However, a case study of a training response suggests the success of neuromuscular adaptation will be dependent upon the degree of motor neuron involvement.

Three measures of lower extremity function were compared in a homogenous population of ALS patients. Isokinetic dynamometry was shown to be a sensitive tool for change in strength over time. It demonstrated positive correlations with gait velocity as well as other behavioral measures. Manual muscle tests were relatively insensitive and no more reliable than isokinetics. Isokinetics are a useful adjunct in the assessment of ALS.

Focal, small-to-moderate and transient improvement occurred in the muscle strength and function of patients with ALS who received TRH in dose-response and screening studies. In a small pilot study of 12 patients, 3 months administration of TRH at 10 mg per kg on alternate days resulted in localized increased strength of jaw muscles as well as significant improvement in lower extremity function. Aerobic exercise capacity was particularly improved in patients with ALS following administration of TRH. Autonomic effects of TRH on heart rate, respiration, and blood pressure were not serious and attenuated slightly over the course of the study.

PML is a progressive opportunistic infection of the CNS caused by a commonly occurring virus that may become manifest in the context of reduced host resistance in persons of any age. The authors review the disease and its causes, including a discussion of treatment attempts and differential diagnoses.

Heart rate, respiratory rate, systolic and diastolic blood pressure, and oral and skin temperature changes following intravenous administration of TRH occur at lower infusion rates in patients with ALS and bulbar involvement than in patients with ALS without bulbar involvement. This autonomic sensitivity to TRH infusion is characteristic of a more advanced stage of ALS with difficulties in speaking and swallowing. Patients who will be receiving neuropeptides, such as TRH, must be carefully studied to determine whether subclinical bulbar involvement is present. Such patients may require a lower initial dose or dose rate adjustment as well as continual monitoring during neuropeptide administration.