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Objective: Non-viral opportunistic infections involving the central nervous system in AIDS patients most commonly result from toxoplasmic encephalitis (TE). Combination pyremethamin (pyr)/sulfadiazine (sulf) is the mainstay treatment for TE, but many patients experience severe adverse events occasionally requiring discontinuation of this antitoxoplasmic medication. This investigation assessed the effects of an open, prospective trial of alternative trimethroprim/sulfamethoxazole: cotrimoxazole (CTX) therapy for TE in AIDS patients., Patients and Methods: The subjects were 18 AIDS patients with a first presumptive attack of TE (Group 1) and 9 relapsing patients, including 6 out of Group 1 (Group 2). We gave CTX as a therapy at the dose of 960 mg four times a day for 48 hours, then 960 mg three times a day for two weeks, followed by 960 mg twice daily until computed tomography showed complete disappearance of active TE lesions. Life-long maintenance therapy consisted to CTX 960 mg daily., Results: Group 1: Seventeen patients improved clinically and achieved complete resolution on computed tomography scars over a mean period of 33 days (range: 21-56). Only one patient was withdrawn from the study at day 18 due to a severe skin rash. Neither serious hematologic nor liver toxicity were observed. Under maintenance therapy, 7 patients relapsed after an average duration of 15.5 months. Relapses were precipitated either by poor compliance (5/7) or erronenous CTX protocol (2/7). Group 2: There were 15 relapses affecting 9 patients who were treated successfully with CTX. CTX was discontinued in one relapsing patient who experienced a Stevens-Johnson syndrome on day 13. This patient had previously experienced cutaneous intolerance to sulfadiazine., Conclusion: A relative low dose regimen of CTX appears to be strongly efficient and safe treatment for toxoplasmic encephalitis in AIDS. Such a study is of particular interest for developing countries where TE is highly prevalent, given the wide availability of CTX which could be proposed as an economic first line therapy.

Study Design: A case of symptomatic ossification of ligamenta flava in a black man from Martinique (French West Indies) is reported., Objectives: To show that ossification of ligamenta flava may be observed in racial groups other than Japanese people and that the postoperative prognosis of symptomatic ossification of ligamenta flava is not always excellent., Summary of Background Data: Ossification of ligamenta flava causing slowly progressive myelopathy or radiculopathy is rare. It usually occurs in the lower thoracic spine. Ossification of ligamenta flava has mainly been described as occurring in Japanese people and very rarely in Caucasians. Diagnosis is based on a computed tomographic scan or magnetic resonance imaging, and postoperative prognosis is usually good., Methods: Low thoracic ossification of ligamenta flava was diagnosed in a black man from Martinique, based on the computed tomographic scan data and on the histopathologic examination of the removed tissue. The patient was clinically evaluated before and 1 year after the operation. A postoperative computed tomographic scan was performed. A magnetic resonance image was not available in this case., Results: The patient exhibited severe subacute myelopathy. After decompression, the neurologic recovery was incomplete. A postoperative computed tomographic scan showed complete excision of ossification of ligamenta flava and decompression of the spinal cord., Conclusions: Ossification of ligamenta flava may occur in black people. An incomplete postoperative recovery may be observed in such cases of unusual subacute compressive myelopathy.

A prospective incidence study was carried out in the French Caribbean island of Martinique between May 1st 1994 and April 31st 1995. incidence was 80.6 (77.7 when standardized with 1990 U.S. population). This incidence was higher than that observed in the Swiss canton of Geneva where the same methodology was used. The individualized risk factors of first provoked and unprovoked seizures in Martinique were alcoholism, head trauma and cerebro-vascular accidents.

We report a case of bilateral acute retinal necrosis (ARN) following an acute optic neuromyelitis (AONM) in an immunodepressed patient (T CD4 lymphocyte count under 50/mm3) suffering from acquired immunodeficiency syndrome (AIDS). Despite the medical treatment the evolution led to blindness by bilateral total retinal detachment. The neuro-ophthalmological features occurred prior to the retinal manifestation, and the acute optic neuromyelitis occurred after a spreading zoster. The varicella-zoster virus (VZV) seemed to be involved because of recurring cutaneous zoster, spreading of this zoster just before the AONM, previous reports showing a link between VZV and AONM, and VZV and ARN. However, our patient had first an AONM responding well to corticosteroid therapy following one month later by an ARN leading to blindness despite the antiviral treatments received as soon as possible. There is a chronical viremia+ in immunodepressed patients with recurring and spreading zoster. The rupture of the hemato-encephalic barrier observed in AONM could facilitate the invasion of the eye by the virus, leading to an ARN. This hypothesis could explain the two complications due to the VZV, the AONM and the ARN, the first one is of dysimmunitary origin and the second one could probably result of a direct viral attack of the retina. This should incite to treat as soon as possible each retrobulbar optic neuritis in patients with AIDS, especially if past history of zoster.

Study Design: A case of acute disseminated encephalomyelitis (ADEM) preceding the features of cutaneous lupus is reported., Objectives: To suggest that ADEM and cutaneous lupus are pathophysiologically related., Summary of the Background Data: 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., Methods: A clinical, neuroradiological and immunological study was performed in this case., Results: ADEM was diagnosed in a young black female. Three years later, she developed a cutaneous lupus without hypocomplementemia., Conclusion: 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.

Purpose: 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., Methods: 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., Results: 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., Conclusion: 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 20-year-old man developed over three weeks a sensory and painful neuropathy associated with diffuse alopecia. There was motor weakness, and superficial and deep hypoesthesia of the inferior limbs. Deep tendon reflexes were normal. Electrophysiological study mainly showed axonal motor neuropathy. This patient was admitted six weeks after the first symptoms. The clinical picture suggested thallium poisoning, which was confirmed by thallium concentrations in plasma, urine, hair and nails. After search, thallium was identified in a rat poison.

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.

A 37 year-old man had headaches for 10 days, then a single tonic-clonic seizure and coma due to an extensive cerebral venous thrombosis. In spite of full-dose heparin treatment for 7 days, the clinical picture worsened along with increasing edema on CT-Scan. Direct thrombolytic treatment was then performed using transvenous catheterization and instillation of Urokinase (2.6 MU over 4 days). A near complete repermeabilization of the sinuses was obtained and the patient improved dramatically in a few days. The only adverse effect of Urokinase was hematuria. Based on our experience and review of the literature which includes 26 previous cases, direct thrombolytic therapy appears to be a relatively safe procedure. This treatment should be considered in a patient with extensive dural sinus thrombosis which fails to respond to heparin treatment.

X linked subcortical laminar heterotopia and lissencephaly syndrome (XSCLH/ LIS) is an intriguing disorder of cortical development, which causes classical lissencephaly with severe mental retardation and epilepsy in hemizygous males, and subcortical laminar heterotopia (SCLH) associated with milder mental retardation and epilepsy in heterozygous females. Here we report an exclusion mapping study carried out in three unrelated previously described families in which males are affected with lissencephaly and females with SCLH, using 38 microsatellite markers evenly distributed on the X chromosome. Most of the X chromosome was excluded and potential intervals of assignment in Xq22.3-q23 or in Xq27 are reported. Although the number of informative meioses did not allow a decision between these two loci, it is worth noting that the former interval is compatible with the mapping of a breakpoint involved in a de novo X;autosomal balanced translocation 46,XX,t(X;2)(q22;p25) previously described in a female with classical lissencephaly. In addition, haplotype inheritance in two families showed a grandpaternal origin of the mutation and suggested in one family the presence of mosaicism in germline cells of normal transmitting males.

We report a new syndrome that we call "recurrent optic neuromyelitis with endocrinopathies" in eight Antillean women from Martinique and Guadeloupe Ocular involvement was either monocular or binocular, whereas myelopathy was acute or subacute. In seven patients, myelopathic symptoms recurred, and in six patients, visual problems recurred. Spinal cord involvement was a consistent band-like pseudo-syringomyelic dissociated sensory loss. All eight patients had endocrinopathies consisting of amenorrhea, galactorrhea, diabetes insipidus, hypothyroidism, or hyperphagia. Spinal cord MRI revealed cavitation-like images. Various immunosuppressant treatments had little effect on the uniformly deteriorating course, ending in blindness and paraplegia. Six patients died within 5 years of onset, and an autopsy in one patient showed multiple demyelinizing lesions of the spinal cord with thickened blood vessels walls without evidence of inflammation. These cases appear to constitute a syndrome distinct from MS and from classic Devic's syndrome, not only because of the association with endocrinopathies but because of the stereotypy of the recurrences, the absence of MRI lesions in the cerebral white matter, and the unusual image of cavitation of the spinal cord. The syndrome is also distinct from HTLV-I-associated paraparesis, which is endemic in the West Indies.

A case of optic neuropathy and central nervous system disease developed in association with primary Sjögren's syndrome is described. Initial visual loss was partially reversible with intra veinous steroid therapy but four months later she underwent a controlateral visual loss and showed for the first time clinical manifestation of sicca syndrome. Fluorescein angiography showed signs of ischemic choroidopathy. Laboratory studies and histological findings on minor salivary gland biopsy was suggestive of primary Sjögren's syndrome. Attempts to taper oral steroid therapy resulted in worsening neurological disease and immunosuppressive therapy by azathioprine was successfully added to her regimen. Because of therapeutics and prognosis consequences, primary Sjögren's syndrome should be considered in differential diagnosis of optic neuropathy.

Autosomal dominant cerebellar ataxias (ADCAs) are a group of neurodegenerative disorders that are clinically and genetically heterogeneous. We report here a genetic linkage study, with five chromosome 12q markers, of three Martinican families with ADCA type 1, for which the spinocerebellar ataxia 1 (SCA1) locus was excluded. Linkage to the SCA2 locus was demonstrated with a maximal lead score of 6.64 at theta = 0.00 with marker D12S354. Recombinational events observed by haplotype reconstruction demonstrated that the SCA2 locus is located in an approximately 7-cM interval flanked by D12S105 and D12S79. Using the z(max)-1 method, multipoint analysis further reduced the candidate interval for SCA2 to a region of 5 cM. Two families shared a common haplotype at loci spanning 7 cM, which suggests a founder effect, whereas a different haplotype segregated with the disease in the third family. Finally, a mean anticipation of 12+/-14 years was found in parent-child couples, with no parental sex effect, suggesting that the disease might be caused by an expanded and unstable triplet repeat.

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.

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.

Autosomal dominant cerebellar ataxia type I was diagnosed in three unrelated families from Martinique (French West Indies), and linkage to the locus for spinocerebellar ataxia 2 (SCA2) was established. Neuropathological findings in two patients were those of olivopontocerebellar atrophy without oligodendroglial cytoplasmic inclusions. Cerebellar ataxia was associated with hyporeflexia in 68% of 31 examined patients, with slowed and/or limited eye movements in 65% and with dementia in 29%. No patients had optic atrophy, pigmentary retinal degeneration, spasticity or parkinsonism. Mean age at onset was 33 +/- 16 years, and onset before the age of 20 years was correlated with a more rapid and severe course of the disease. Movement disorders, oculomotor disturbances, sphincter disturbances and cognitive impairment were significantly more frequent in early than in late onset patients. This explains why the phenotype was strikingly different in one family, in which mean age at onset was much earlier. Comparison with previously described SCA2 families indicated similarities, such as reduced saccade velocity, supranuclear ophthalmoplegia and decreased reflexes, although phenotypic heterogeneity remains the outstanding feature of this disorder.

Human T-cell leukemia virus type I (HTLV-I) is associated with a large spectrum of clinical manifestation including adult T-cell leukemia (ATL) and tropical spastic paraparesis or HTLV-I-associated myelopathy (TSP/HAM). In most cases, however, infected patients remain asymptomatic. The participation of the immune system in the pathogenesis of TSP/HAM has been suggested. In this study the IgG antibody response of HTLV-I-infected individuals has been investigated using both ELISA with a panel of nuclear and cytoplasmic proteins and peptides known to be recognized by antibodies from patients with various systemic autoimmune diseases, and immunoprecipitation of ribonucleoproteins from HeLa cell extracts. The results were compared with the reactivity of sera from individuals with non-HTLV-I-related neurological diseases and healthy blood donors. Raised levels of autoantibodies reacting with several nuclear and cytoplasmic antigens were found in TSP/HAM and ATL patients. In asymptomatic HTLV-I-seropositive individuals, both the prevalence and level of IgG antibodies were lower and directed only against a restricted set of antigens. The mechanism of induction of these antibodies still remains obscure. However, the results show that a significant autoimmune response exists in these patients and it may contribute to the pathogenesis of the disease.

Myositis linked to HTLV-1 is unfrequent. Over a period of 8 years, 14 patients with inflammatory myopathy were diagnosed in Martinique. Seven were seropositive for HTLV 1 antibody; the clinical and pathological data of whom are presented herein. Five patients presented with polymyositis, two with dermatomyositis. All seven patients had extra-muscular clinical features including neuropathy (4/7) and myelopathy (6/7), resulting in a quite peculiar clinical picture. Muscle biopsy showed a neurogenic process combined with myositic changes in 3/7 patients. Corticotherapy led to dramatic improvement in only one case, but with no sustained effect. HTLV 1 may be considered the etiological agent of this form of dermato-polymyositis, characterized by a clearly distinctive clinico-pathological picture, and a poor response to corticotherapy. As in the case of tropical spastic paraparesis/HTLV 1 associated myelopathy, careful assessment of non-steroidal therapy is now warranted.

Envenomation by the Bothrops lanceolatus, a snake found only in Martinique, leads to swelling and pain, and occasionally to systemic signs and/or coagulopathy. Severe thromboses at some distance from the site of the bite may appear within 48 hr. Uncertainties as to the actual development of thrombotic complications in patients appearing to be suffering from moderate poisoning and as to the availability and the toxicity of a monospecific antivenom (AVS) initially led us to reserve antivenom for the most severe cases, and to use anticoagulants to prevent thromboses in all patients. This approach was modified after we observed serious thromboses in patients with moderate poisoning. Of 50 adult snake bite cases hospitalized between June 1991 and August 1994, 11 developed serious thrombotic complications at 36 /+- 27 hr (mean +/- SD) (range 12-96) following envenomation, despite early preventive anticoagulant therapy. Those included pulmonary embolism (two cases), cerebral infarction (six cases), myocardial infarction (one case), and cerebral and myocardial infarctions (two cases). Sixteen patients were not treated with AVS: 10 of these recovered without complications and six developed systemic thrombosis causing permanent disability in three cases. Thirty were treated with an intravenous infusion of 2-6 vials of AVS given 2-48 hr after the bite. Of these, three died of cerebral infarction that developed before the initiation of serotherapy. All others recovered. Among patients treated with AVS, three presented with mild anaphylactic reactions, while one developed serum sickness that responded to steroids. These data indicate that preventive anticoagulant therapy is of limited efficacy in Martinique.(ABSTRACT TRUNCATED AT 250 WORDS)

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 main neurological manifestation due to Human T-Cell Lymphotropic Virus type (HTLV1) infection is a chronic spastic paraparesis called HTLV1-associated paraparesis (HAP). Since 1985, we observed in Martinique (French West Indies) 276 cases of HAP. Among them, 70 patients fulfilled clinical, electrophysiological or histological criteria of associated peripheral nervous system involvement (42/70), myositis (8/70), or both (20/70). Muscle biopsy revealed neurogenic atrophy of muscle fibers or myositic changes in 41/70. Neuromuscular involvement was only mild in 19/70. On the other hand, 25 patients presented with a syndrome mimicking amyotrophic lateral sclerosis, and 7 other patients with features of polymyositis. This study shows that neurological manifestations associated to HTLV1 infection may be more complex than the well-known chronic spastic paraparesis, since 25.4% of the HAP Martinican patients exhibit neuropathic or myositic features.

A retrospective study was conducted in Martinique to identify patients with polyarthritis and positive serologic tests for the human T-cell lymphotropic virus Type I (HTLV-1). Patients with metabolic or bacterial causes of polyarthritis were excluded. We found 17 cases of HTLV-1-positive polyarthritis (6.7% of the total of polyarthritis patients followed in our department); there were 14 females and three males, and all the patients were West-Indian blacks. Mean age at diagnosis was 50 years. Five patients also had tropical spastic paraparesis. The polyarthritis was the inaugural manifestation of T-cell leukemia in one patient. Four patients had received blood transfusions. Fever, myalgia, and/or skin lesions were present at onset of the polyarthritis in seven cases. All 17 patients had peripheral, bilateral, symmetric polyarthritis; the most commonly involved sites were the hands (17/17) and knees (14/17). Three patients had rheumatoid factor and five had antinuclear antibody. Ten patients met at least four American Rheumatism Association criteria for rheumatoid arthritis; they accounted for 6.7% (10/150) of all polyarthritis patients managed in the same hospital department during the same period. We compared these ten patients with 20 HTLV-1-negative rheumatoid arthritis patients matched on gender, ethnic origin, and disease duration. There were no significant differences between the two groups for any of the parameters studied.

Cogan's syndrome consists of non syphilitic interstitial keratitis associated with vestibuloauditory dysfunction. This syndrome is rare and very few cases have been reported since Cogan's first characterization in 1945. We present the case of a 24-year-oldman with a history of recurrent access of arthralgia and glomerulonephritis. Bilateral corneal disease consists of peripheral whitish patchy opacities in deep stroma. The patient also shows bilateral severe deafness, fever, meningeal and tetrapyramidal syndrome. Ocular involvement responds well to corticosteroids therapy but hearing loss is severe and often irreversible.

Spinocerebellar ataxia 2 (SCA2) is one of the loci for the clinically and genetically heterogeneous group of autosomal dominant type I cerebellar ataxias. After initial linkage to chromosome 12q in Cuban families, SCA2 was shown to be the gene responsible for the disease in Italian, Tunisian, French-Canadian, Austrian-Canadian and Martinican kindreds with dominant ataxia, and the candidate interval was reduced to 6.4 cM between markers D12S84 and D12S79. Comparison of patients from families of different geographical origins clearly demonstrates the clinical interfamilial variability of the clinical signs which reaches statistical significance for the frequency of extrapyramidal rigidity, postural tremor and dementia. The most striking difference between the 29 Martinican SCA2 patients and those with SCA1 on chromosome 6p or SCA3/MJD on chromosome 14q is the greater frequency of hyporeflexia in the former. A mean 12.5 year anticipation is observed, with a more rapid clinical course of the disease in successive generations, indicating that an expanded trinucleotide repeat probably constitutes the underlying molecular mechanism.

Objectives: 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., Methods: 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., Results: 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., Conclusion: 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.

The humoral immune response in sera from 30 human T cell lymphotropic virus type I (HTLV-I)-positive individuals from Martinique in the French West Indies was studied. The subjects were subdivided into those suffering from TSP/HAM and those being asymptomatic. In general, TSP/HAM patient sera seemed to contain more virus-specific antibodies than did the sera from the asymptomatic subjects. Three of the 13 TSP/HAM sera and 1 of the 17 asymptomatic sera contained HTLV-I-specific IgM antibodies, whereas 6 and 5 sera, respectively, contained IgA antibodies. By correlating the ability of patient sera to inhibit HTLV-I-induced syncytia with their antibody reactivity in ELISA to 42 synthetic peptides, together corresponding to the entire envelope glycoprotein of HTLV-I, a number of putative neutralizing domains were identified. Eight synthetic peptides representing the regions with the highest coefficient of correlation between neutralizing titer and ELISA reactivity were employed to specifically adsorb potentially neutralizing antibodies, and were also used directly, without sera, in the syncytium-neutralizing test. By those techniques, three novel and two previously described domains that seemed to contain neutralizing epitopes were identified. Two of the novel neutralizing sites resided in the external glycoprotein (gp46) and were contained within amino acids 53-75 and 287-311, respectively, and one was located in the transmembrane glycoprotein (gp21) within amino acids 346-368. Our findings may have implications for the rational design of subunit vaccines for prevention of and/or alteration of the clinical outcome of HTLV-I-related diseases.

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.

The human T-lymphotropic retrovirus type I (HTLV-1), isolated in 1980, has been shown to be responsible for two distinct systemic diseases: adult T-cell leukemia and HTLV associated myelopathy (HAM). Recently, an ever increasing number of publications have described other disorders associated with HAM. We report a case of uveitis-optic disc neuritis which, to our knowledge, had not been previously described. The fact that both uvea and optic disc were inflamed would suggest that immune-mediated reactions are involved in the pathogenesis of HAM.

A 33 year-old male homosexual infected with human immunodeficiency virus type I developed an asymmetrical and painful neuropathy in the lower limbs. Neuro-muscular biopsy showed a necrotizing vasculitis. There were no clinical features indicative of systemic vasculitis. Prednisone therapy dramatically improved the neuropathy, without adverse effects. Although rare, necrotizing arteritis must be considered in HIV-1 patients with neuropathy, especially in case of mononeuropathy multiplex and when immunodepression is mild or absent, since a successful corticosteroid therapy can be prescribed.

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.

A 63-year-old woman developed gradual slowness, recurrent multifocal deficits, severe constitutional symptoms and hypopituitarism which progressed to death over 2.5 months. Elevation in lactico dehydrogenases was the main biological abnormality. Necropsy showed an intravascular malignant proliferation which proved to be a lymphoma of B-lineage. In order to better define diagnostic criteria, we reviewed previously reported cases of intravascular lymphomatosis of the central nervous system. A strategy for establishing the diagnosis is proposed.

Twenty nine cases of central nervous system infection due to Monosporium apiospermum have been reported. Six of them occurred after an aspiration pneumonia following a near drowning. The case of a 53 year old man is reported: M apiospermum was isolated from cerebrospinal fluid, sputum, urine and from the mud of the ditch where the patient had fallen. Though the treatment used recommended doses of intravenous, intrathecal and intraventricular amphotericin B, it was ineffective and the patient died 97 days after the near drowning. Necropsy showed that the ventricular infectious process was more prominent distant from the antifungal ventricular source.

There are few reports of amyloidosis diagnosed by deliberate biopsy of accessory salivary glands. Usually, a biopsy performed for dry mouth syndrome reveals an unsuspected amyloidosis. We report the case of 2 patients with lambda-type light chain monoclonal gammapathy complicated by generalized amyloidosis and in whom biopsy of the accessory salivary glands showed signs of amyloidosis. In the first patient accessory salivary gland biopsy was performed because these glands were enlarged, and the monoclonal dysglobulinaemia was subsequently diagnosed by serum immunoelectrophoresis. In the second patient with nephrotic syndrome, renal biopsy could not be carried out owing to the presence of a renal malformation; amyloidosis was confirmed by periumbilical fat aspiration, and a systematic biopsy of accessory salivary glands also showed evidence of amyloidosis. Biopsy of accessory salivary glands seems to be a particularly simple and safe method to detect generalized amyloidosis in patients with chronic inflammatory disease or monoclonal dysglobulinaemia.

6 p. 100 of a population of 129 heart transplant recipients presented isolated convulsions under cyclosporin (Ci) immunosuppressive treatment. These seizures were unexplained by acute causes, infectious or cerebro-vascular. Seizures started generally a few days after Ci therapy onset, and led often to severe convulsive states, with sometimes a status epilepticus. For most of them, Ci serum levels were within, or just above, the recommended therapeutic range. Ischemic brain damage that occurred prior to surgery and Ci therapy, could be a contributing factor to Ci-induced seizures in this population of heart transplant recipients.