Gt. Plant et al., AN EPIDEMIC OF OPTIC NEUROPATHY IN TANZANIA - CHARACTERIZATION OF THEVISUAL DISORDER AND ASSOCIATED PERIPHERAL NEUROPATHY, Journal of the neurological sciences, 145(2), 1997, pp. 127-140
An epidemic of subacute bilateral visual failure has affected large nu
mbers of teenagers and young adult Africans in coastal Tanzania since
1988, Previous work had indicated that many patients had sensory sympt
oms, but the nature of the neurological involvement was uncertain, The
primary objective of this study was to characterize the accompanying
neurological disorder, Furthermore, the nature of the visual loss was
uncertain from previous reports as both retinopathy and optic neuropat
hy had been suggested, Full ophthalmic and neurological examinations w
ere carried out at the Muhimbili University Hospital in Dar es Salaam,
Nerve conduction studies, pattern electro-retinograms and cortical vi
sually evoked responses and colour contrast sensitivity tests were car
ried out, Thirty eight young Africans and 12 controls were included in
the study, The characteristic fundus picture was symmetrical temporal
optic atrophy, and thinning of the caeco-central nerve fibre layer, F
luorescein angiography was normal. The electrophysiological and colour
contrast sensitivity tests confirmed optic neuropathy in the majority
of cases but primary retinal involvement was indicated in some. Neuro
logical examination and nerve conduction measurements showed evidence
of a peripheral neuropathy in 47% of the patients. The peripheral neur
opathy is likely to have involved large fibres (from the nerve conduct
ion studies) but the symptoms suggest small fibre loss also, 42% had d
eveloped hearing loss, Urinary thiocyanate levels were uniformly low,
Serum was negative for antibodies to HTLV-1. DNA analysis from three c
ases was negative for three known mutations associated with Leber's he
reditary optic atrophy (11778, 3460 and 14484). This entity, occurring
predominantly in a young age group, does not correspond closely to ot
her tropical neurological syndromes previously described from East Afr
ica although it is clinically very similar to Strachan's syndrome (ori
ginally described in the Caribbean and more recently in prisoners of w
ar) and also to an epidemic of optic and peripheral neuropathy that ha
s recently occurred in Cuba, The aetiology has not yet been determined
, A micronutrient deficiency is likely but has not been established. (
C) 1997 Elsevier Science B.V.