Publications (6)20.07 Total impact
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Article: A new mutation of the lamin A/C gene leading to autosomal dominant axonal neuropathy, muscular dystrophy, cardiac disease, and leuconychia.
Journal of Medical Genetics 04/2004; 41(3):e29. · 6.36 Impact Factor -
Article: Chronic inflammatory demyelinating polyneuropathy associated with dysglobulinemia: a peripheral nerve biopsy study in 18 cases.
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ABSTRACT: The possible occurrence of chronic inflammatory demyelinating polyneuropathy (CIDP) in association with an identified dysglobulinemic status is recognized and a causal relationship between the two has been suggested. We had the opportunity to study 18 patients presenting with CIDP and dysglobulinemia. This was an IgG monoclonal gammopathy (IgG MG) in 8 cases, an IgM monoclonal gammopathy (IgM MG) in 8, an IgG-IgM biclonal gammopathy in 1 case and an IgM monoclonal cryoglobulinemia in another. A peripheral nerve biopsy specimen was available for all patients and the morphological findings in these specimens in the cases of CIDP with IgG MG or cryoglobulin did not differ from those without, whereas characteristic features were observed in the cases of CIDP with IgM MG and anti-myelin associated glycoprotein activity.Acta Neuropathologica 08/2000; 100(1):63-8. · 9.32 Impact Factor -
Article: [Case of superficial hemosiderosis of the central nervous system treated with trientine].
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ABSTRACT: A 58-year-old woman, with recurrent headaches, exhibited cerebellar alaxic gait, anosmia, deafness and a pyramidal syndrome, with a progressive onset. In cerebrospinal fluid there was erythrocytes and siderophages. MRI on T2-weighted images revealed a marginal hypo-intensity, leading to the diagnostic of superficial siderosis of the central nervous system. None haemorragic lesion was found. The patient was given Trientine. Unfortunately she worsened on later examinations.Revue Neurologique 05/1998; 154(3):243-5. · 0.49 Impact Factor -
Article: [Multineuritis in essential hypereosinophilia syndrome].
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ABSTRACT: A 72-old-man with a dramatic weight loss, exhibited a mononeuritis multiplex of subacute onset, and myalgia. There was hypoesthesia and weakness on median and ulnar nerves bilaterally, and weakness on left femoral nerve. Electrodiagnostical studies showed an axonal pattern of neuropathy. There was a considerable hypereosinophilia on blood count without any aetiologia found on exhaustive explorations. The idiopathic hypereosinophilic syndrome with a mononeuritis multiplex was suggested. Histological findings were an acute myelino-axonal degeneration with endoneural oedema, without vasculitis nor eosinophil cell on nerve-biopsy. Dramatic improvement occurred with corticosteroids and immunosuppressive treatment.Revue Neurologique 01/1998; 153(12):785-9. · 0.49 Impact Factor -
Article: Peripheral neuropathy associated with monoclonal IgG of undetermined significance: clinical, electrophysiologic, pathologic and therapeutic study of 14 cases.
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ABSTRACT: Fourteen patients with peripheral neuropathy and monoclonal IgG of undetermined significance are reported with a retrospective study of the clinical features, electrophysiologic and sural nerve biopsy findings. There were two groups. Five patients had a relapsing chronic sensorimotor polyneuropathy with clinical (5/5), electrophysiologic (4/5) and pathologic (5/5) features compatible with chronic inflammatory demyelinating polyneuropathies (CIDP). The nine others had a slowly progressive sensory (5/9) (SPNP) or sensorimotor (4/9) (SMPNP) axonal polyneuropathy. Four patients of the first group were treated with intravenous human immunoglobulin (400 mg/kg/day for five days) with significant clinical improvement. The motor conduction velocities and distal latencies of two of these patients improved following treatment, thus matching the clinical improvement. Our results on peripheral nerve biopsies confirm the differentiation of patients with CIDP from those with SMPNP and SPNP. There was no specific immunologic serologic reactivity in any of the cases.Journal of the Peripheral Nervous System 02/1996; 1(2):139-48. · 2.80 Impact Factor -
Article: [Localized muscular hypertrophy in a familial form of sarcoidosis].
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ABSTRACT: Muscular hypertrophy, rarely reported in muscular sarcoidosis, is usually associated with the myositic, myopathic and nodular types. We report a case with an isolated localised muscular hypertrophy simulating a tumor formation developed after repetitive efforts, in a familial context of sarcoidosis. MRI shows muscular high intensity lesions in the thighs and calves, and after treatment, central areas of low intensity signal. Muscular biopsy confirms diagnosis of sarcoidosis. Gallium-67 scintigraphy shows systemic extension of disease. This case highlights the usefulness of recent imaging techniques in diagnostic strategy and follow up.La Revue de Médecine Interne 02/1995; 16(11):843-6. · 0.61 Impact Factor
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Institutions
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1996
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University of Bordeaux
Bordeaux, Aquitaine, France
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