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At a glance

Rare genetic disorder with blindness, deafness, developmental delay, and spasticity.

Synonyms

Nyssen-Van Bogaert-Meyer Syndrome; Opticocochleodentate Degeneration.

Incidence

Less than 20 cases reported.

History

First described by Belgian neurologist Rene Nyssen and neuropathologist Ludo van Bogaert in 1934.

Genetic inheritance

Most likely an autosomal recessive transmitted disease.

Clinical aspects

Dentate nucleus and medial lemniscal structures degenerate, and the patients present in infancy with muscular hypotonia or atonia, which changes in childhood to rigidity and spasticity quadriplegia. Patients are mentally retarded. Blindness occurs secondary to optic nerve atrophy and sensorineural deafness secondary to cochlear degeneration. Death usually occurs before age 10 years.

Anesthetic considerations

Communication with these patients may be difficult (blind, deaf, mentally retarded) and behavioral compliance decreased. Consequently, anxiolytic premedication may be helpful. However, presence of the primary caregiver at the bedside during induction of anesthesia may be even more helpful. Chest radiograph should be obtained preoperatively because recurrent pneumonias secondary to aspiration are common.

Pharmacological implications

Succinylcholine should not be used to avoid a hyperkalemic response.

References

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Larnaout  A, Ben Hamida  M, Hentati  F: A clinicopathological observation of Nyssen-van Bogaert syndrome with second motor neuron degeneration: Two distinct clinical entities. Acta Neurol Scand 98:452, 1998.  [PubMed: 9875626]
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Meyer  JE: Über eine kombinierte Systemerkrankung in Klein-Mittelund Endhirn. Arch Psychiat Nervenkr 182:731, 1949.
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Nyssen  R, van Bogaert  L: La dégénérescence systématisée opticocochl éo-dentelée. Rev Neurol 2:321, 1934.

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