A contribution to the pathology of paramyoclonus multiplex (Friedreich's type) / by J. Ramsay Hunt.

  • Hunt, James Ramsay, 1872-1937.
Date:
1903
Catalogue details

Licence: In copyright

Credit: A contribution to the pathology of paramyoclonus multiplex (Friedreich's type) / by J. Ramsay Hunt. Source: Wellcome Collection.

Provider: This material has been provided by The Royal College of Surgeons of England. The original may be consulted at The Royal College of Surgeons of England.

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    ordinated movements of cerebral origin. The knee-jerks were greatly exaggerated. The gross motor power suffered no im- pairment. The electrical reactions were normal. This was essen- tially true of the psychical and sensory functions. At times pain- ful flexor spasms of the thigh occurred. There were absolutely no stigmata of hysteria. The peculiar features of this affection, viz., the spontaneous contraction of individual muscles not singly under the control of the will, and the absence of synergetic muscular action in the affected members, were so at variance with the known character- istics of muscular contractions of cerebral origin as to render some other explanation probable. Friedreich, after a careful con- sideration of all the possible sources, as the brain, spinal cord, nerves and muscles, proposed the hypothesis which referred the symptoms to an excessive irritability of the anterior horn cells of the cord. This theory was in harmony with the special clinical features of the case, i.e., the single muscle contraction, the ab- sence of synergetic action, the great exaggeration of the knee- jerks, and their diminution as the motor symptoms subsided, the great susceptibility of the contractions to peripheral stimuli, and their cessation on voluntary innervation of the part. In the course of a few years the accuracy of Friedreich’s ob- servation was confirmed on many sides by notable observers. Among the earlier recorded cases were those of Lowenfeld,2 Remak,3 Seeligmiiller,4 Francotte,5 Homen,6 Allen Starr,7 Bech- terew8 and Marie.9 It was but natural that Friedreich’s descrip- tion, based on one case, should undergo considerable enlargement and some variation, as the literature was enriched by other ob- servers, and that even new forms and types should be added. Thus, in 1886, Seeligmiiller described a congenital case; Unver- richt10 in 1891 added a family form associated with epilepsy, and Gucci11 an hereditary form in 1893. Some of the more im- portant semeiological deviations from the original Friedreich type which have been subsequently described are as follows: That the muscular contractions are not limited to the proximal seg- ments of the extremities, but that all the voluntary muscles may be involved with the sole exception of those moving the eyeballs. Voluntary movement has not in all cases seemed to soothe or in- terrupt the contractions ; indeed, the contrary effect has been noted.