Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + At a Glance Download Section PDF Listen ++ Inherited syndrome with mental retardation, cerebellar ataxia, nystagmus, strabismus, and brachydactyly. + Synonym Download Section PDF Listen ++ Brachydactyly-Nystagmus-Cerebellar Ataxia Syndrome. + Incidence and Genetic Inheritance Download Section PDF Listen ++ Only a few cases have been reported (four generations in one family). Transmitted as an autosomal dominant trait. + Clinical Aspects Download Section PDF Listen ++ Mental retardation, nystagmus, strabismus, and shortening of the metacarpal and metatarsal bones. The posterior column ataxia is characterized by reduced sensory action potentials in nerve conduction tests and ataxia secondary to cerebeliar atrophy. + Anesthetic Considerations Download Section PDF Listen ++ No reports exist about this syndrome in the context of anesthesia. Mental retardation can affect the cooperation of the patient, and sedative premedication and/or the presence of the primary care-giver for induction of anesthesia may be helpful. + References Download Section PDF Listen ++Biemond A: Brachydactylie, nystagmus en cerebellaire ataxis als familiar syndrome. Ned Tijdschr Geneesk 78:1423, 1934. ++Nachmanoff DB, Segal RA, Dawson DM, et al: Hereditary ataxia with sensory neuronopathy: Biemond's ataxia. Neurology 48:273, 1997. [PubMed: 9008534]