Early Controversies Over Athetosis: II. Treatment

Douglas J Lanska

doi:10.5334/tohm.163

Abstract

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.

Highlights

IntroductionAthetosis is an involuntary movement disorder characterized by slow, smooth, sinuous, writhing movements, involving the hands.[1,2,3,4,5,6,7,8,9] Since its description in 1871 by American neurologist William Alexander Hammond (1828–1900) (Figure 1), and subsequent elaboration by Hammond and his son Graeme Monroe Hammond (1858–1944),[2,3,4,5,6,7,9,10,11,12,13,14,15] the disorder has been a source of controversy,[1] as were many aspects of Hammond’s career, either as US Army Surgeon General during the Civil War or later as a civilian neurologist in New York.[9,16,17,18,19,20,21,22,23,24,25] Hammond struggled to establish athetosis as a distinct clinicopathological entity, and had successfully predicted the striatal pathology in his initial case (albeit somewhat serendipitously), athetosis was considered by many late 19th and 20th century neurologists as a form of post-hemiplegic chorea or part of a continuum between chorea and dystonia.[9,26,27,28,29] European neurologists, and the French in particular, initially ignored or discounted the concept.[1]
There was no agreement over how athetosis should be treated, with many anecdotal reports of benefit, which were not confirmed by subsequent experience
Nervines [a nerve tonic], narcotics, electricity, courses of treatment with cold water, massage, and hypnosis have been employed without successful results [original emphasis].’’36 Somewhat later, in 1908, Austrian neurologist Lothar von Frankl-Hochwart (1862–1914) concluded, without enthusiasm, that such non-specific agents should be tried, but that there was rarely any evident benefit:[39]

Summary

Introduction

Athetosis is an involuntary movement disorder characterized by slow, smooth, sinuous, writhing movements, involving the hands.[1,2,3,4,5,6,7,8,9] Since its description in 1871 by American neurologist William Alexander Hammond (1828–1900) (Figure 1), and subsequent elaboration by Hammond and his son Graeme Monroe Hammond (1858–1944),[2,3,4,5,6,7,9,10,11,12,13,14,15] the disorder has been a source of controversy,[1] as were many aspects of Hammond’s career, either as US Army Surgeon General during the Civil War or later as a civilian neurologist in New York.[9,16,17,18,19,20,21,22,23,24,25] Hammond struggled to establish athetosis as a distinct clinicopathological entity, and had successfully predicted the striatal pathology in his initial case (albeit somewhat serendipitously), athetosis was considered by many late 19th and 20th century neurologists as a form of post-hemiplegic chorea or part of a continuum between chorea and dystonia.[9,26,27,28,29] European neurologists, and the French in particular, initially ignored or discounted the concept.[1]. The purpose of the present article is to review some of the controversies concerning treatment of athetosis in the 50-year period following its initial description. There was no agreement over how athetosis should be treated, with many anecdotal reports of benefit, which were not confirmed by subsequent experience. Methods: Primary sources have been used to review treatment controversies in the 50-year period following the initial description of athetosis. Several novel invasive therapies were developed and promoted, all of which damaged or destroyed either upper or lower motor neuron pathways, and were often associated with high mortality rates. These therapies substituted paresis for abnormal spontaneous movements. 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

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