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D Smadja

Publications and source records attributed to D Smadja.

At least 55 records · Page 3Linked to original sources
PubMed1997

[Bilateral optic neuropathy disclosing primary Gougerot-Sjögren syndrome].

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.

Aged↗
PubMed1997

[Gayet-Wernicke encephalopathy and centropontine myelionolysis induced by hyperemesis gravidarum].

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.

Adult↗
PubMed1997

[Peripheral neuropathy caused by thallium poisoning].

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.

Adult↗
PubMed1996

Autosomal dominant cerebellar ataxia type I in Martinique (French West Indies): genetic analysis of three unrelated SCA2 families.

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.

Adolescent↗
PubMed1996

Ocular manifestations in patients with HTLV-I associated infection--a clinical study of 93 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.

Adult↗
PubMed1996

[Sjögren's syndrome and HTLV-I myelopathy].

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.

Adult↗
PubMed1995

IgG autoantibody response in HTLV-I-infected patients.

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.

Adult↗
PubMed1995

Autosomal dominant cerebellar ataxia type I in Martinique (French West Indies). Clinical and neuropathological analysis of 53 patients from three unrelated SCA2 families.

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.

Adolescent↗
PubMed1995

Clinical characteristics of HTLV-1 associated dermato-polymyositis. Seven cases from Martinique.

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.

Adult↗
PubMed1995

Prevention of thromboses in human patients with Bothrops lanceolatus envenoming in Martinique: failure of anticoagulants and efficacy of a monospecific antivenom. Research Group on Snake Bites in Martinique.

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)

Adolescent↗
PubMed1995

[Loss of psychic auto-activation. Obsessive-compulsive behavior. Toxoplasmic abscess of the basal ganglia].

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.

AIDS-Related Opportunistic Infections↗
PubMed1995

Autosomal dominant cerebellar ataxia type I linked to chromosome 12q (SCA2: spinocerebellar ataxia type 2).

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.

Adolescent↗
PubMed1995

[Involvements of the peripheral nervous system and skeletal muscles in HTLV1-related paraplegia. Study of 70 cases seen in Martinique].

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.

Electromyography↗
PubMed1995

[Interstitial keratitis and Cogan syndrome].

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.

Adult↗
PubMed1995

Polyarthritis in HTLV-1-infected patients. A review of 17 cases.

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.

Adult↗