Early Controversies Over Athetosis: II. Treatment

BACKGROUND: Athetosis has been controversial since it was first described by William Hammond in 1871; many aspects of Hammond’s career were equally controversial. METHODS: Primary sources have been used to review treatment controversies in the 50-year period following the initial description of athetosis. RESULTS: The treatments used most commonly employed available pharmaceutical agents and modalities (e.g., galvanism). Initial anecdotal reports of success were seldom confirmed with subsequent experience. Several novel invasive therapies were also developed and promoted, all of which damaged or destroyed either upper or lower motor neuron pathways, and were also often associated with high mortality rates. In general, these therapies substituted paresis for abnormal spontaneous movements. These included peripheral nerve stretching, excision of a portion of the precentral gyrus, rhizotomy, nerve “transplantation” (i.e., neurotomy and nerve-to-nerve anastomoses), and “muscle group isolation” (i.e., alcohol neurolysis). There was no agreement on the appropriateness of such high-risk procedures, particularly given the intentional generation of further neurological morbidity. DISCUSSION: Pharmaceutical agents and modalities initially employed for athetosis had little a priori evidence-based justification and no biologically plausible theoretical framework to guide empiric treatment selection. Subsequently, all the invasive procedures employed were directed at lessening or removing the manifestations, rather than the underlying cause, of the abnormal central nervous system “irritation,” usually by imposing paresis or paralysis. Factors contributing to the disparity in outcomes between favorable initial reports and the often-disappointing results of later studies included reliance on anecdotal reports or small uncontrolled case series, placebo effects, biased observation, misdiagnosis, and biased reporting.