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Congenital sensory neuropathy
Author(s) -
Pinckers A.,
Bosch A. A. I. van't Pad,
Aandekerk A. L.,
Cruysberg J. R. M.
Publication year - 1988
Publication title -
acta ophthalmologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.534
H-Index - 87
eISSN - 1755-3768
pISSN - 1755-375X
DOI - 10.1111/j.1755-3768.1988.tb04599.x
Subject(s) - anisocoria , medicine , cornea , sensory system , sensory neuropathy , keratitis , ophthalmology , surgery , sensory nerve , pupil , neuroscience , psychology
. The authors examined a patient presenting with congenital sensory neuropathy with selective loss of small myelinated nerve fibres. The appearance of (bilaterial) keratitis or corneal ulceration in early childhood is strongly suggestive of congenital corneal anaesthesia. Concomitant symptoms such as anisocoria, abnormal pupillary reaction, diminished tear production and disturbed sensibility to pain and temperature point to a generalized disease: one of the hereditary sensory and autonomic neuropathies. In order to establish a definite diagnosis, elaborate neurological examination, including ultrastructural study of a musclenerve biopsy, is required. Tarsorrhaphy, therapeutic flushfitting PMMA scleral lenses and hydrophilic HEMA contact lenses are advocated, in order to protect the cornea. The results with high‐water‐content hydrophilic contact lenses are promising, those of keratoplasty limited.