I Klein

Publications (2)3.03 Total impact

• Article: [Susac syndrome].

  T Papo, I Klein, K Sacré, S Doan, B Bodaghi, F Aubart-Cohen
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  ABSTRACT: Susac syndrome is a mysterious vasculopathy affecting brain, retina and inner ear in young women. Main features of the disease are increasingly recognized: subacute encephalopathy often mimicking psychosis and frequently heralded with unusual ophthalmic migraine; frequent subclinical meningitis; brain MRI with multiple and bilateral white and gray matter nuclei lesions, with prominent involvement of corpus callosum; bilateral involvement of central retina artery branches, not only with occlusions but also with peculiar leakage of fluorescein through arteriolar walls on late stages of angiography; non-specific bilateral cochleovestibular symptoms with audiogram showing perception hypoacousia that predominates on low frequencies. Outcome, prognosis, pathogenesis and a rational basis for treatment are discussed in this review. A key message for the clinician should be to perform brain MRI, audiogram and retinal angiography whatever the mode of entry, in order not to miss one (or two) features of this syndrome triad.
  La Revue de Médecine Interne 12/2011; 33(2):94-8. · 0.61 Impact Factor
• Article: Aseptic meningitis and ischaemic stroke in Fabry disease.

  O Lidove, M-P Chauveheid, C Caillaud, R Froissart, L Benoist, S Alamowitch, S Doan, R Szalat, N Baumann, J-F Alexandra, P Lavallée, I Klein, F Vuillemet, F Sedel, K Sacré, Y Samson, E Roullet, T Papo
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  ABSTRACT: Fabry disease (OMIM 301 500) is an X-linked lysosomal storage disease. Neurological symptoms in Fabry disease mainly include stroke, acroparesthesia, cranial nerve palsies and autonomic dysfunction. We report on aseptic meningitis in Fabry patients. Clinical analysis, brain magnetic resonance imaging, cerebrospinal fluid analysis, treatment and outcome data were analysed in three cases of meningitis associated with Fabry disease. Mean age at meningitis onset was 26.6 (24-28) years. Headache was present in all cases and fever in two cases. Meningitis was always diagnosed before Fabry disease. A familial history of Fabry disease was present in two cases. Non-neurological symptoms caused by Fabry disease were present in all cases. All patients also suffered stroke and sensorineural hearing loss. Cerebrospinal fluid (CSF) analysis showed pleocytosis (mean, 36; range: 8-76 cells/mm(3)) and a high protein level (mean, 63; range, 47-70 mg/dl). C-reactive protein blood levels and erythrocyte sedimentation rate were raised. Diagnosis was assessed by low alpha-galactosidase A dosage and/or gene mutation analysis in all cases. All patients were treated with enzyme replacement therapy (ERT). In two cases, lumbar puncture was repeatedly performed and there was no normalisation of CSF under ERT alone, at 9 and 24 months of follow-up, respectively. One patient who suffered intracranial hypertension was treated efficiently with steroids, associated with azathioprine. The fact that Fabry disease could be an auto-inflammatory disorder is discussed. Fabry disease may cause aseptic meningitis.
  International Journal of Clinical Practice 11/2009; 63(11):1663-7. · 2.41 Impact Factor