Congenital sensory neuropathy
作者:
A. Pinckers,
A. A. I. van't Pad Bosch,
A. L. Aandekerk,
J. R. M. Cruysberg,
期刊:
Acta Ophthalmologica
(WILEY Available online 1988)
卷期:
Volume 66,
issue 3
页码: 293-298
ISSN:1755-375X
年代: 1988
DOI:10.1111/j.1755-3768.1988.tb04599.x
出版商: Blackwell Publishing Ltd
关键词: corneal anaesthesia;indifference to pain;sensory neuropathy;congenital;hydrophilic contact lenses
数据来源: WILEY
摘要:
Abstract.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 limi
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