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International audience; Few studies report a protective role of childhood solar exposure to multiple sclerosis. Our objective was to confirm the protective role of childhood solar exposure in multiple sclerosis in Cuba, Martinique and Sicily. This was a matched case- control study, and cases met Poser criteria for clinically, laboratory (definite, probable) multiple sclerosis. Controls were resident population, without neurological disorder, living close to cases (within 100 km), matched for sex, age (+/-5 years), residence before age 15. We recruited 551 subjects during a 1-year period (193 cases, Cuba n = 95, Sicily n = 50, Martinique n = 48; 358 controls). Some (89%) met definite clinical multiple sclerosis criteria (relapsing remitting form (with and without sequel) (74%), secondary progressive (21%), primary progressive (5%)). Odds ratios in a uni-variate analysis were: family history of multiple sclerosis (5.1) and autoimmune disorder (4.0); wearing shirt (3.5), hat (2.7), pants (2.4); sun exposure causing sunburn (1.8); sun exposure duration (1 h more/day; weekends 0.91, weekdays 0.86); bare-chested (0.6); water sports (0.2). Independent factors in the multivariate analysis were family history of multiple sclerosis (4.8 (1.50-15.10)), wearing pants under sunlight (1.9 (1.10-3.20)), sun exposure duration (1 h more/ day, weekdays 0.90 (0.85-0.98), weekends 0.93 (0.87-0.99)), water sports (0.23 (0.13-0.40)). We conclude that outdoor leisure activities in addition to sun exposure reports are associated with a reduced multiple sclerosis risk, with evidence of dose response.

Data on epidemiology of neuromyelitis optica (NMO) remained scarce in the last century, but the recent development of diagnostic criteria now enables inclusion of both monophasic and relapsing NMO in epidemiologic studies. Given the rarity of NMO, multicentric studies are needed to confirm a presumed higher frequency in women and in populations of black/Asian ancestry. The Caribbean basin is a suitable area for collecting a large NMO cohort and to assess the prevalence, incidence, and mortality of this disorder.This population-based survey of the NMO spectrum in the French West Indies (FWI) and Cuba included 151 cases.Ninety-eight patients (female/male ratio: 9.8) had NMO. Age of onset in NMO patients was 30.9 years. Mean annual incidence of NMO in the French West Indies for the period July 2002 to June 2007 was 0.20/100,000 inhabitants (IC 95% 0.05-0.35). Incidence rates were steady in the FWI during the 1992 to 2007 period. Decreasing mortality in the FWI during the 1992 to 2007 period explained the increasing prevalence which was 4.20/100,000 inhabitants (IC 95% 3.7-5.7) in June 2007. The prevalence of NMO in Cuba on November302004 was 0.52/100,000 inhabitants. (IC 95% 0.39-0.67). Prevalence rates did not differ significantly by ethnic group in Cuba, however, black Cubans exhibited the highest prevalence.Epidemiologic studies on NMO in each population are needed to determine whether aggressive therapies can reduce the mortality of this devastating disorder.In the Caribbean basin, NMO involves almost exclusively young women; the epidemiologic data confirm its predilection for populations of African ancestry. In the FWI, recent and aggressive therapy has lowered mortality but with an increase in the prevalence of NMO.

Few neurological diseases are linked with anti-glutamic acid decarboxylase antibodies (GAD-ab); stiff man syndrome is an example. Cerebellar ataxia is a new feature of this expanding spectrum. No therapeutic trial is yet available in these diseases. We here report on four patients suffering from cerebellar ataxia linked with GAD-ab and review the data in the literature on this recently described syndrome.We conducted an open trial with monthly pulsed steroids. Steroid pulses were given six months followed with placebo for another six months. Main clinical and biological parameters were monitored monthly (International Cooperative Cerebellar Ataxia Rating Scale [ICARS] and GAD-ab).The clinical response was found limited and inconstant. Transient decline in GAD-ab level was noted in two patients. Moreover, GAD-ab level was found highly variable and did not correlate with clinical parameters.Cerebellar ataxia with GAD-ab is an increasingly described syndrome. Outcome can be severe, leading to definitive cerebellar atrophy. Diagnosis is supported by high level of serum GAD-ab with intrathecal secretion. Experimental data have suggested a direct excitotoxic effect of GAD-ab on Purkinje cells. Response to various treatments is often disappointing. Improvement has been obtained with veinoglobulins in individual patients. A weak clinical and biological response was associated with monthly steroid pulses.

Neuromyelitis optica (Devic's disease, NMO) is an inflammatory disease of the central nervous system preferentially involving the spinal cord and optic nerves in either a monophasic or relapsing-remitting course. B-cell induced pathogenesis was recently described for NMO. Rituximab is a chimeric monoclonal antibody directed against the CD20 antigen, which causes depletion of B-cells. Rituximab might be effective in treatment of NMO. We report the two-year clinicoradiological and biological follow-up data from a patient included in July 2005 in a prospective trial of rituximab for severe NMO refractory to immunosuppressant therapy. Blood B-cell depletion after rituximab induction was maintained for 10 months. Seven attacks occurred during a two-year follow-up. EDSS increased from 7.0 to 8.0. Left visual acuity decreased from 20/20 degrees to 20/60 degrees. Spinal cord atrophy worsened.

Leptospirosis is an anthropozoonosis, which affect 100000 people by year worldwide.Leptospirosis and IgA deficiency were revealed in a young man by acute anterior uveitis, after canyoning practice in the French West Indies.Uveitis should be added to the ocular phenotype of leptospirosis.

Neuromyelitis optica (NMO) is a rare inflammatory and demyelinating disorder of the central nervous system, restricted to optical nerves and spinal cord. The main neuroradiological aspects, now summarized into a complete set of diagnosis criteria, are a normal cerebral MRI at onset and longitudinal involvement of the spinal cord concerning more than 3 vertebral segments. The clinical course and frequency of typical lesions remain unknown.We here report neuroradiological data from patients suffering from NMO.Brain and spinal cord MRI were systematically reviewed for 32 afro-Caribbean patients.A typical longitudinal spinal lesion was seen in 44.7 percent with or without edema; a lesion involving less than 3 vertebral segments in 26.3 percent and no lesion in 21.1 percent. Longitudinal study of a few bouts suggested a progressive normalisation of spinal cord appearance. Atrophy was negatively correlated with immunosuppressive treatment. Cerebral lesions usually absent at onset were correlated to the follow-up. In a non-recursive condition, patients completed diagnostic criteria for encephalic and spinal lesions in 82.8 percent and 48.1 percent.Radiology of spinal bouts showed multiple aspects besides the typical form. The notion of multiple bouts must be added to the spinal criteria to achieve good sensitivity. A typical extensive spinal lesion is usual in the follow-up, but seen after less then half of the bouts. Requiring such a lesion would delay the diagnosis.

Cerebellar ataxia with antiglutamic acid decarboxylase antibodies (GAD-ab) is an exceptional newly recognized autoimmune disorder. The cerebellar ataxia may occur in isolation or be associated with stiff man syndrome another rare GAD-Ab induced disorder of central nervous system.A 38-year-old woman with a past history of Graves disease presented with insidious cerebellar symptoms including ataxic gait, dysmetria, dysarthria, and oscillopsia. A thorough survey of markers of paraneoplastic cerebellar ataxia and collagen diseases was negative. Her serum contained high level of GAD-ab (647.2 U/ml) and MRI evidenced pure cerebellar atrophy leading to diagnosis of autoimmune cerebellar ataxia. Under corticosteroids, cerebellar symptoms partially improved, but serum GAD-ab titre dramatically decreased.Testing for GAD-ab may be indicated in patients with idiopathic cerebellar ataxia, particularly mature women with organ-specific autoimmune diseases. Corticosteroids must be started to prevent irreversible cerebellar atrophy.

Few data are available regarding stroke among young black patients. We have conducted, in Martinique, a prospective study among young Afro-Caribbeans aged 15 to 45 Years, from June 1994 through May 1999. We identified 60 cerebral infarcts (CI) and 20 cases of intracerebral hemorrhage (ICH). Annual incidence and case fatality rate at 30 days were 7.6/100000/Year (95p.cent confidence interval, 3.13 to 11.35) and 8.3p.cent respectively for CI, and 2.42/100000/Year (95p.cent confidence interval, 0.06 to 4.7) and 25p.cent for ICH. Hypertension was the most prevalent stroke risk factor: 35p.cent in CI and 68p.cent in ICH. According to the TOAST criteria, a probable cause of ischemic stroke was identified in 61.3p.cent. Atherosclerosis, lacunar infarcts and cardioembolism were the main causes of CI (13.3p.cent for each etiology). Spontaneous cervical arterial dissection was found in only 6.7p.cent. Hypertensive ICH (60p.cent) was the most common subtype of ICH. In Martinique, high frequencies of ICH and lacunar infarcts are characteristic of stroke in young Afro-Carribeans. These suggest the role of the high prevalence of hypertension in the black population. We confirm the heterogeneity of stroke etiologies and the low prevalence of cervical arterial dissection in black people.

The optical disease observed concurrent with multiple sclerosis is poorly known in blacks because of the rare occurrence of this pathology in the black population. The few studies currently available suggest more severe problems in black subjects than in Caucasians. Martinique has subjects who are genetically close or even identical but who acquired multiple sclerosis in two very different endemic areas: some in Martinique and others in France.We performed an ophthalmological transversal descriptive study in a population of 57 black multiple sclerosis patients living in Martinique, selected according to the Poser criteria. Patients were divided into two groups: an M group (26 cases) made up of patients who had never left Martinique or the West Indies and an FM group (31 patients) made up of patients who had lived at least one year in France between the ages of 5 and 15.At least one episode of retrobulbar optic neuritis was observed in 16 cases (61.5%) in the M group and in 11 cases (31.5%) in the FM group. The number of eyes with either one episode or more of retrobulbar optic neuritis or papillitis was higher in the M group, 26 cases (50%) than in the FM group, 14 cases (22.6%). Multiple sclerosis began by a disease of the optic nerve in 12 cases (46%) in the M group and in 7 cases (22.6%) in the FM group. The mean visual acuity is 20/30 in the M group and 20/20 in the FM group. The mean value of the p 100 wave was 131 ms in the M group and 113 ms in the FM group.Multiple sclerosis observed in the black population living in Martinique (M group) is characterized by frequent and severe visual problems. The visual phenotype of the FM group is quite similar to the visual phenotype of Caucasians. More than ethnic and genetic factors, the tropical area of acquisition should have an influence on the visual phenotype. For unknown reasons, the optical problems appear severe when multiple sclerosis is acquired in a low endemic area.

We investigated the adhesion of blood mononuclear cells (MNC) isolated from patients with HTLV-1-associated myelopathy (HAM/TSP). MNC from HAM/TSP patients were significantly more adherent to activated endothelial monolayers than MNC from non-HAM/TSP (controls and HTLV-1 carriers) subjects. Blocking studies demonstrated that the adhesion molecules VLA-4 (CD49d), ICAM-1 (CD54), and L-selectin (CD62L) all contributed to increased binding. However, anti-ICAM-1 antibody was the most efficient in inhibiting binding HAM/TSP patients MNC to activated endothelial cells. Expression on MNC of molecules involved in adhesion was also studied by flow cytometry in HAM/TSP patients, HTLV-1 carriers, and healthy control subjects after two days culture without any mitogen. In HAM/TSP patients, L-selectin expression on CD4+ and CD8+ subsets was lower than in controls; interestingly, HAM/TSP patients had lower percentage of CD4+ subset expressing L-selectin than HTLV-1 carriers. The percentage of CD4+ and CD8+ cells expressing VLA-4 was found to be similar to controls in both HAM/TSP patients and HTLV-1 carriers. Following two days in culture without mitogen, the percentage of T cells expressing ICAM-1 increased in HAM/TSP and carriers, but not in controls. This study provides information regarding trans-endothelial migration of MNC across the blood brain barrier in HAM/TSP and suggests ICAM-1 and its counterpart molecule LAF-1 are involved in massive infiltration of lymphocytes observed in the spinal cord.

We conducted a retrospective study in 20 cases of idiopathic intracranial hypertension (IIH) referred to the ophthalmology out-patients department at the Centre Hospitalier Universitaire of Fort de France. The objectives were to characterize symptomatology, severity, disease course and to discuss treatment.We performed a complete ophthalmological and neurological examination, calculated the body mass index (BMI) and evaluated visual field. Each patient underwent a CT scan with injection and a lumbar punction with measurement of cerebrospinal fluid pressure. We also specified the treatment chosen and its duration, and the number of recurrences.This study included 20 cases, 19 women and 1 man, with a mean follow-up of 28.6 months. The average BMI was of 32.2 kg/m2. Fifteen patients out of 20 were obese (75%), all of them women, so 79% of the women were obese. Sixteen patients had headache (80%), 15 a loss of visual acuity (75%) and 5 transient visual loss (25%). For all the clinical examination showed bilateral papilloedema. Visual field disturbances were observed in 11 patients (73.3%). Papilloedema disappeared after medical treatment of a mean 2.4 months duration for each access. In 9 cases of 18 (50%) recurrences were observed after gradual treatment with-drawal.The long-term treatment, the side effects, the possible severity of the ocular disease, the difficulties of dietary restriction and the high rate of recurrence, would, for us, indicate a need to reconsider surgical treatment. We propose to perform lumboperitoneal shunt or optic nerve sheath decompression if medical treatment fails at the first onset of IIH or in case of severe visual loss, pregnancy or recurrence.

A case of acute disseminated encephalomyelitis (ADEM) preceding the features of cutaneous lupus is reported.To suggest that ADEM and cutaneous lupus are pathophysiologically related.Neurological complications of systemic lupus erythematosus are common. However, demyelinative central nervous system manifestations are rare, and restricted to neuromyelitis optica (NMO). NMO is thought to be a partial form of ADEM.A clinical, neuroradiological and immunological study was performed in this case.ADEM was diagnosed in a young black female. Three years later, she developed a cutaneous lupus without hypocomplementemia.A relationship between ADEM and cutaneous lupus is suggested. The normal serum complement level in our patient might be a predisposing factor for the development of ADEM.

A 24-year-old woman developed hyperemesis gravidarum, complicated by Wernicke's encephalopathy and central pontine myelinolysis. The first-was due to a deficiency of thiamine, and was hastened by intravenous dextrose without thiamine supplements. However, no changes in serum sodium or phosphorus values were observed. In this case, the pathophysiology of central pontine myelinolysis remained obscure.

The objective of this study is to describe the clinical and immunological aspects observed in patients with both "tropical spastic paraparesis/HTLV-I associated myelopathy" (TSP/HAM) and ocular dryness. In 10 such patients clinical and biological examinations completed with a biopsy of secondary salivary glands were performed to assess the etiology of the ocular dryness. According to the Chisholm's scale, 70% of the patients had a biopsy grade 3 or grade 4. Polyclonal hypergammaglobulinemia was found in 90% of patients and lymphocytic alveolitis in 80%. Three patients had past medical history of chronic uveltis. All the findings were compatible with Sjögren's syndrome, however no characteristic immunological disorders were found. Antinuclear antibodies and rheumatoid factor proved negative in all cases.

The purpose of this study was to confirm that ophthalmological features seen in patients in Martinique, French West Indies, could be linked to infection by HTLV-I. The authors studied 93 HTLV-I infected patients divided into 70 patients with HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) and 23 asymptomatic HTLV-I carriers. They did a complete ophthalmological examination with an assessment of lacrymal secretion by means of three tests: Shirmer 1, break-up time and rose Bengal. Some patients had a biopsy of secondary salivary glands. When possible, detection of HTLV-I antibodies was carried out in the aqueous humor. In 45 of the 93 patients (48.4%) the presence of dry keratoconjunctivitis was recorded. In 22 of these 45 cases, a biopsy of the secondary salivary glands showed the presence of lymphoplasmocytoid infiltrations comparable to the glandular changes that occur with Gougerot-Sjögren syndrome. Among the 93 patients, 15 cases of uveitis were noted (16.1%) with 13 cases of anterior uveitis and 11 cases of vitritis. The inflammation was bilateral in 9 cases (9/15 = 60%). Two cases of cotton wool spots, 3 cases of abnormalities in the distribution of the retinal pigment and 7 cases of corneal lesions were also noted. Higher levels of anti-HTLV-I antibodies were detected in the aqueous humor of 3 patients with uveitis. The coexistence of dry eye (keratoconjunctivitis), uveitis and retinal microangiopathy in patients who are suffering from HAM/TSP could suggest the involvement of an autoimmune or immunological mechanism in the physiopathology of the illness.

A 34 year old woman with AIDS presented with adynamia and obsessive-compulsive behaviour. CT scan revealed bilateral toxoplasmic lesions involving basal ganglia. The neuropsychological picture resolved under specific antitoxoplasmic treatment. However, relapse of toxoplasmosis occurred in associating with the same behavioral changes. This is the first case of such a syndrome due to infectious lesions, and in which improvement through transient, was observed.

The most common neurological picture associated with HTLV-1 infection is a slowly progressive spastic paraplegia often involving nerve and muscle inflammation. We report here six cases of inflammatory vasculitis of the nervous system observed among HTLV-1 infected patients.HTLV-1 infection was diagnosed in 6 female patients, mean age 58 years (range 40-77 years) using ELISA, Western blot or polymerase chain reaction.All 6 patients presented a pyramidal syndrome which was associated with peripheral neuropathy in 3, myositis in 1 and a sicca syndrome in 3. Two groups of patients could be identified. In 3 patients, the neurological picture was typical of HTLV infection; pathology examination of biopsy material revealed necrozing vasculitis. In 3 other patients, the clinical course was particularly rapid, highly suggestive of anterior spinal artery infarction, associated, in 2, with a major inflammatory syndrome.These 6 cases demonstrate that vasculitis can be associated with HTLV-1 infection as has been observed in HIV-infected patients. In certain cases, clinical signs of vasculitis may be lacking while in others they may dominate the clinical manifestations.

We present the results of ophthalmologic examinations of a consecutive series of 30 patients with HTLV-I associated myelopathy. This is the first ophthalmologic prospective study reported outside the Japanese endemic area. Twenty-one of the patients (70%) had kerato-conjunctivitis sicca in addition to accessory salivary gland lymphocytary infiltration in 7. Two cases of uveo-papillitis, 1 case of cotton-wool spots and 3 cases of retinochoroidal degeneration were also observed. Moreover, an aliquot of the aqueous humour was collected from the anterior chamber in 5 patients and showed high titer of HTLV-I antibodies for the 2 patients with uveo-papillitis. We propose different hypotheses to explain the physiopathologic characteristics of these features.

To date, 271 cases of HTLV1-associated paraplegia have been observed in Martinique (French West Indies). The clinical picture consisted mostly in a spastic paraparesis or paraplegia with sphincter disturbances (80%) and lower limbs pains (60%). The severity of the disease appeared variable: after a mean disease duration of 6.5 years, 40% of the patients could walk without help, 35% used a single crutch, and 25% used a couple of crutches or were confined to a wheelchair. A variable neuromuscular component was observed in 70 cases (25.4%). In 38 cases, the peripheral signs (SIGNS) or the myositis were only mild. In contrast, 25 patients presented with severe amyotrophy evoking amyotrophic lateral sclerosis, and 7 other had features of dermatopolymyositis. Lastly, an extra-neural spreading of the disease was extremely frequent, including lymphocytic alveolitis (76%), sicca syndrome (69%) and more rarely uveitis, arthritis or vasculitis.