Sjögren-Larsson syndrome (Karl Gustaf Torsten Sjögren)
A form of severe mental deficiency (idiocy or imbecility) endemic in Sweden. No patients reported with low or normal intelligence. Other characteristics include congenital ichtyosis or ichtyosiform erythroderma, and spastic paralysis. Associated disorders may be sparse and brittle hair, short stature, pigmentary retinal degeneration, seizures, hypohidrosis, dysplasia of dental enamel, kyphosis, hypertelorism, and metaphyseal dysplasia with small irregular epiphyses.
No sex predilection. Consanguinity between parents; high incidence in siblings. Inheritance is autosomal recessive.
Rud’s syndrome is entered as a separate entity under Einar Rud, Danish physician, born 1892.
- Vicente Pardo-Castello, H. Faz:
Archives of Dermatology, Chicago, 1932, 26: 915.
- K. G. T. Sjögren, T. K. Larsson:
Oligophrenie in combination with congenital ichtyosis and spastic disorders. A clinical and genetic study.
Acta psychiatrica et neurologica scandinavica, Copenhagen, 1957, 32, supplement 113: 9-105.