[Benign forms of multiple sclerosis : a revaluation (author's transl)]. / Les formes bénignes de la sclérose en plaques. Réévaluation.

The authors studied 38 cases of benign forms of MS, defined as those allowing a normal or nearly normal social, occupational, and family life over a "long" period of tens of years, for at least 15 years (mean : 28 years, range : 15 to 68 years).--Forms with rare relapses with long intervals between them (18 cases), and a mean period of 14 years (5 to 30) between the first and second episode.--Recurrent forms (17 cases) with frequent attacks (one or two a year), including 8 cases with no further relapses after an average period of 10 years, and 9 cases with continuation at the same rhythm for 15 to 23 years.--Secondarily progressive forms belonging to both types--8 in the first and 3 in the second--characterized by the late onset (mean : 25 years, range : 15 to 47 years) of a progressive paraplegia.--Slow forms (3 cases) with an early but only slowly evolving progressive phase, which fall into a border line category of benign forms. Independently of the mean age of onset (26 years); the predominence of females (2.2); and the symptomatology of the first attack (40 p. 100 involve the cranial nerves, and 40 p. 100 have pyramidal signs), the two essential characteristics of benign forms are the remarkable regression of relapses (rare of frequent) and the absence of a progressive phase. Once the 10-year point has been passed without permanent disability the prognosis is good, but with the reserve that the disease can become worse at a later stage.

[Familial olivo-ponto-cerebellar atrophy with myoclonus. Limits of cerebellar myoclonic dyssynergia (Ramsay-Hunt syndrome)]. / Atrophie olivo-ponto-cérébelleuse familiale avec myoclonies. Les limites de la dyssynergie cérébelleuse myoclonique (Syndrome de Ramsay-Hunt)

The case is described of a woman of 26 suffering (like her mother, a brother and a sister) from a progressively degenerating cerebellar syndrome, at first considered to be hereditary cerebellar ataxia, but which, after action myoclonus appeared, was diagnosed as dyssynergia cerebellaris myoclonica (D.C.M.). Anatomical verification however revealed a syndrome of olivo-ponto-cerebellar atrophy comprising massive demyelinisation of the white matter of the cerebellum and of the cerebellopontine fibres; atrophy of the pontine nuclei; the cerebellar cortex itself was severely affected; moderate nigral lesions; marked spinal lesions of the cerebellospinal fasciculi and of the posterior columns; lesions of the anterior horns and of the bulbar nuclei; cortical chromatolysis. The fact that the dentate system remained unaffected, also noted in some cases of olivo-ponto-cerebellar atrophy with myoclonus, whilst in a number of other cases the lesion remains clinically silent, emphasises the difficulty in establishing an accurate correlation between myoclonus and dentate nucleus. Discussion of the nosological limits of D.C.M.: confirmed cases generally displayed lesions of the dentate system and hereditary degenerative spino-cerebellar lesions. The same clinical symptoms can be observed in cases which do not come under this classification--or even under that of degenerative conditions of the cerebellar system--and D.C.M. appears to be only a syndrome, the Ramsay-Hunt syndrome.