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OBJECTIVE To investigate the course of diffusion changes in Wilson disease (WD) and to evaluate their clinical and radiologic correlates. METHODS MRI with fluid-attenuated inversion recovery (FLAIR) and diffusion weighted images (DWI) were performed in 13 symptomatic patients with WD who had typical neurologic manifestations of the disease (sWD patients)(More)
In France, the prevalence of stroke and the level of disability of stroke survivors are little known. The aim of this study was to evaluate functional limitations in adults at home and in institutions, with and without self-reported stroke. A survey named "the Disability Health survey" was carried out in people's homes (DHH) and in institutions (DHI).(More)
Cerebellar syndromes and radiologic cerebellar atrophy after hyperpyrexia have occasionally been reported, mostly in neuroleptic malignant syndromes, but neuropathologic studies are extremely rare. We studied 3 patients (a 74-year-old woman, a 63-year-old man, and an 80-year-old man) who had heat stroke during heat waves in France. One patient had(More)
Fifteen patients were observed between 1987 and 1990: there were six with angiographically confirmed vertebral artery dissection, and 9 with carotid artery dissection. Results showed concordance of MRI and angiographic findings, in all cases but one. The dissected portion consistently showed a semilunar hyperintensity narrowing the residual eccentric signal(More)
Cortical vein thrombosis without sinus involvement is rarely diagnosed, although it may commonly be overlooked. We report four cases of cerebral venous thrombosis limited to the cortical veins. The diagnosis was made on surgical intervention in one patient and by angiography in three patients. Together with a survey of the published cases, the clinical and(More)
A woman, who presented with clinical and radiological signs of a right temporal mass suggestive of a brain tumour, was found to have granulomatous angiitis associated with cerebral amyloid angiopathy; the diagnosis was confirmed by biopsy. She is still well 13 years after excision of the lesion. The association of granulomatous angiitis and cerebral amyloid(More)
BACKGROUND AND PURPOSE The clinical forms of Wilson's disease (WD) neurological manifestations can be divided into three movement disorder syndromes: a) dystonic, b) ataxic, c) parkinsonian syndrome. These syndromes in WD seldom occur in isolation. Clinical rating scales such as the Unified Parkinson;s Disease Rating Scale (UPDRS), the International(More)
Heavy metals and trace elements play an important role in relation to the physiology and pathology of the nervous system. Neurologic diseases related to disorders of metabolism of copper and iron are reviewed. Copper disorders are divided into two classes: ATP7A- or ATP7B-related inherited copper transport disorders (Menkes disease, occipital horn syndrome,(More)
Wilson's disease (WD) with extensive cortico-subcortical lesions represents a rare neuropathological subgroup, the pathogenesis of which is not clearly determined. We report two new cases with identical lesions. In the families of each of the patient, there were mutations in the ATPase7B gene, especially in the family of proband 1, and in the first cousin(More)