Significance of Extensor Plantar Responses in Muscular Dystrophy.

The absence of primary disease of the central nervous system is usually regarded as characteristic of the muscular dystrophies. However, there have been occasional reports of the presence of signs, generally accepted as indicating organic disease of the central nervous system, in otherwise typical examples of muscular dystrophy (Bell, 1943); these signs include extensor plantar responses which have been recorded on several occasions (Kollarits, 1906; Crouzon, 1911; Leri, Girot and Basch, 1923: Sjovall, 1936; Levison, 1951: Hassan and Mostafa, 1961). A report is here given of a family in which each of four male siblings showed evidence of classical pseudohypertrophic muscular dystrophy and also bilateral extensor plantar responses; the significance of this association is discussed.