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ABSTRACT: Klippel-Trenaunay syndrome (KTS) is a rare phakomatosis characterized by cutaneous hemangiomata, venous varicosities and bone and soft tissue hypertrophy also of the affected limb. Central nervous system involvement is rare, arising from a malformation or from coagulation disorders. We report the case of a patient presenting a KTS with stroke. The biological assessment revealed antithrombin III deficiency. Although rare, antithrombin III deficiency should be kept in mind in KTS patients with neurological involvement.
Revue Neurologique 11/2008; 164(10):855-8. · 0.49 Impact Factor
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Journal of Neuroradiology 05/2008; 35(4):244-5. · 1.21 Impact Factor
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ABSTRACT: Seizures are common in hyperglycemia and are often the first manifestation particularly in non-ketotic hyperglycemia (NKH). Published reports emphasize partial motor seizures almost exclusively. Here we present the clinical, biological and electrophysiological features of 22 consecutive patients with NKH who developed focal seizures and were treated in our department. Neurological exam was normal in 15 cases. When abnormal, it showed postictal obnubilation or diabetic polyneuropathy. Interictal EEG (obtained in 17 patients) showed focal or generalised slowing in 65% of cases, and ictal EEG (six patients) showed rapid spikes most often unilateral. CTs were normal in 77% of cases, and showed age-compatible cortico-subcortical atrophy in the others. All patients were alert, with glucose values between 13.6 and 55 mmoles/l and osmolarity values increased in all cases to a mild or moderate extent (266-309.20 mosm/l). Three out of 22 patients (14%) presented with motor epilepsy partialis continua. In 11/22 cases (50%), diabetes mellitus had not been diagnosed previously. Seizures associated with NKH were resistant to anticonvulsant treatment but responded well to insulin therapy and rehydration. They subsided completely in an average of 4 days, and only one patient had to be transiently transferred to ICU. We conclude on the importance of an early diagnosis of this condition to prevent malignant evolution of the epileptic syndrome into a state of hyperosmolarity and coma associated with a much higher mortality.
Neurophysiologie Clinique/Clinical Neurophysiology 11/2004; 34(3-4):183-7. · 1.98 Impact Factor
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ABSTRACT: Epilepsy is 5 to 7 times more frequent among patients with myasthenia than in the general population. Myasthenia gravis is often associated with other affectsion, most of immunological origin. However, hypotheses explaining the association are still uncertain. We report a case of association in a woman who had epilepsy since the age of 9 years and developed myasthenia at the age of 23 years after having discontinued here anti-epileptic treatment for two years.
Revue Neurologique 02/2004; 160(1):93-5. · 0.49 Impact Factor
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ABSTRACT: The presenting symptomatology in a case of fronto-callosal glioma were affective indifference, severe disorders of attention and dynamic aphasia with marked reduction in spoken expression. Spontaneous and induced writing were abundant and incoercible. Their meticulous production and formal correction contrasted with its semantic incoherence. This behavior is comparable with that of the compulsive activity that may result from pallidal lesions or bilateral frontal lesions. The term graphomania is proposed to distinguish this behavior from echographia and hypergraphism.
Revue Neurologique 02/1988; 144(3):158-64. · 0.49 Impact Factor
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ABSTRACT: Klippel–Trenaunay syndrome (KTS) is a rare phakomatosis characterized by cutaneous hemangiomata, venous varicosities and bone and soft tissue hypertrophy also of the affected limb. Central nervous system involvement is rare, arising from a malformation or from coagulation disorders. We report the case of a patient presenting a KTS with stroke. The biological assessment revealed antithrombin III deficiency. Although rare, antithrombin III deficiency should be kept in mind in KTS patients with neurological involvement.
Revue Neurologique 164(10):855-858. · 0.49 Impact Factor
