Rare genetic disorder with blindness, deafness,
developmental delay, and spasticity.
Nyssen-Van Bogaert-Meyer Syndrome; Opticocochleodentate
Fewer than 20 cases reported;
most likely an autosomal recessive transmitted disease.
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.
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.
Succinylcholine should not be used to
avoid a hyperkalemic response.
Meyer JE: Über eine kombinierte Systemerkrankung in Klein-Mittel
und Endhirn. Arch Psychiat Nervenkr 182:731, 1949.
Nyssen R, van Bogaert L: La dégénérescence systématisée
optico-cochléo-dentelée. Rev Neurol 2:321, 1934.