++
Inherited syndrome with mental retardation, cerebellar
ataxia, nystagmus, strabismus, and brachydactyly.
++
Brachydactyly-Nystagmus-Cerebellar Ataxia Syndrome.
++
Only a few cases have been
reported (four generations in one family). Transmitted as an autosomal
dominant trait.
++
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.
++
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.
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]