Clinical course of a cohort in the Cuban epidemic optic and peripheral neuropathy.
Nearly 51,000 Cubans were afflicted during an outbreak of an optic neuropathy (ON) and peripheral neuropathy (PN) between 1991 and 1993. We re-examined 14 of 20 affected individuals 16 months after an initial evaluation. The optic features were painless symmetric vision loss with poor visual acuity, color vision loss, central or cecocentral scotoma, optic disc pallor, and nerve fiber layer drop-out. The neurologic symptoms included stocking-glove sensory changes, hearing loss, leg cramps, sensory ataxia, hyperactive or absent reflexes, and complaints of memory loss. Two of 11 ON probands tested harbored Leber's hereditary optic neuropathy (LHON)-associated mitochondrial DNA mutations. All patients had received multivitamin therapy. We performed comparisons using the paired two-tailed t test. On re-examination, 12 of 14 patients demonstrated improvement. One patient remained unchanged. One woman with the nt-3460 mtDNA mutation showed a decline in vision. In patients not harboring mtDNA mutations, overall visual acuity, color vision, and peripheral neuropathy manifestations improved significantly (p < 0.001 for each manifestation). Most of the patients with Cuban ON and PN improved on multivitamin therapy. The significance of the mtDNA mutations is unclear. In the 2 LHON patients, manifestation of the disease may have been precipitated by nutritional deficiency. Patients with poor recovery or further deterioration should be evaluated for other factors, including poor vitamin therapy compliance and alternative diagnoses.
Mojon
D S
DS
Department of Ophthalmology, Columbia University College of Physicians and Surgeons, New York, NY, USA.
Kaufmann
P
P
Odel
J G
JG
Lincoff
N S
NS
Márquez-Fernandez
M
M
Santiesteban
R
R
Fuentes-Pelier
D
D
Hirano
M
M
D000328:Adult; D015331:Cohort Studies; D003118:Color Perception / Q000187:drug effects; D003462:Cuba; D004272:DNA, Mitochondrial / Q000235:genetics; D004196:Disease Outbreaks; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009154:Mutation; D009460:Neurologic Examination; D015418:Optic Atrophies, Hereditary / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology; D009901:Optic Nerve Diseases / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology*; D010523:Peripheral Nervous System Diseases / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology*; D016896:Treatment Outcome; D014792:Visual Acuity / Q000187:drug effects; D014815:Vitamins / Q000627:therapeutic use
D004272:DNA, Mitochondrial; D014815:Vitamins