Brain barrier dysfunction in Cuban epidemic optic neuropathy.

BACKGROUND AND PURPOSE: There are practically no references to cerebrospinal fluid (CSF) studies in tropical or nutritional neuropathies. In the present paper we present the results of CSF studies in patients with Cuban Epidemic Optic Neuropathy (CEON) during epidemic and endemic periods, with an appraisal as to the contribution of brain barriers' function in the pathophysiology of this disease. METHODS: Two hundred and five patients with CEON were studied during the epidemic period (1992-1993) and 12 patients outside the outbreak (1995-1997). CSF protein determination and electrophoresis were carried out, as well as serum and CSF albumin and immunoglobulin G (IgG) quantitation for calculating IgG and Q(alb) indexes, in order to evaluate intrathecal IgG synthesis and the permeability of the blood-CSF barrier (B-CSF B). RESULTS: One fourth of the patients had increased permeability of the B-CSF B, but damage was more frequent between 16 and 60 days from onset of disease, disappearing after 120 days. B-CSF B dysfunction was more prevalent in patients with severe neurological impairment, although it was not related to the severity of ophthalmological damage. The group of patients studied outside of the outbreak (endemic period) showed similar results. DISCUSSION: The possible association of increased permeability of the B-CSF B with oxidative stress, which lies on the basis of this epidemic outbreak, is discussed.

[Epidemic neuropathy: proposal and arguments to rename Strachan disease as Strachan and Madan disease]. / Neuropatía epidémica: proposición y argumentacion para renombrar a la enfermedad de Strachan, como de Strachan y Madan.

INTRODUCTION: Strachan's disease is a condition which mainly affects the nervous system. It is characterized by optic, auditory and peripheral neuropathies and lesions of the skin and mucous membranes. In 1955 Miller Fisher gave it this name, since the clinical condition described by Henry Strachan in Jamaica during the nineteenth century was similar to that seen in Canadian prisoners-of-war in Japanese concentration camps during the Second World War. DEVELOPMENT: Since there are similarities between these clinical disorders and the major neuropathic epidemic seen recently in Cuba, we have reviewed and compared the endemic and epidemic conditions of similar characteristics seen in Cuba during the nineteenth and twentieth centuries. We also make a detailed review of a similar condition described in 1898-1900 by Doctors Madan, López and Santos Fernández, during the last Cuban War of Independence. This seems to be one of the earliest descriptions of the disorder. We also consider the so-called Strachan's syndrome or disease, and descriptions from the same period of tobacco-alcohol amblyopia and beriberi. These conditions seem to have been very similar to the so-called optical and peripheral forms of the current Cuban epidemic. It is concluded that the clinical characteristics of the recent Cuban neuropathic epidemic, at least in the optical form, were seen to be endemic during the nineteenth century. In many cases this was considered to be alcoholic amblyopia or some other obscure neuropathy which became epidemic during periods of severe economic depression. CONCLUSION: Madan gave a full description of the disorder at the same time as Strachan did. In 1898 he also suggested its true cause and died trying to relieve it. We therefore consider that Strachan's syndrome should be renamed the Strachan-Madan syndrome.