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Ataxia with ocular motor apraxia type 1 (AOA1) is an autosomal recessive cerebellar ataxia (ARCA) associated with oculomotor apraxia, hypoalbuminaemia and hypercholesterolaemia. The gene APTX, which encodes aprataxin, has been identified recently. We studied a large series of 158 families with non-Friedreich progressive ARCA. We identified 14 patients (nine(More)
One case of a strictly unilateral left rolandic mu rhythm, revealing a metastatic tumor of the concerned region, is reported. The clinical feature consisted of partial Jacksonian motor seizures involving the upper limb and the face on the right side. As the tumoral process extended, the mu rhythm turned into a 6 c/sec theta rhythm, similarly located and(More)
BACKGROUND Familial adult myoclonic epilepsy (FAME) is defined by autosomal dominant inheritance, adult onset of myoclonus of the extremities, infrequent epileptic seizures, nonprogressive course, abnormality of polyspikes and waves on examination by EEG and photosensitivity, giant somatosensory evoked potentials, enhancement of C reflex, and premyoclonus(More)
Collet-Sicard syndrome is a rare condition, defined as unilateral palsy of the last four cranial nerves. It differs from Villaret syndrome because of absence of sympathetic involvement. Collet-Sicard syndrome is most often caused by skull tumors, carotid artery dissections or head and neck trauma. We report the case of a 57-year-old man who presented palsy(More)
Various neurological pictures attributed to human toxocariasis are sparsely reported in the literature. A case of toxocaral infection presenting as meningoradiculitis is described herewith. Etiological diagnosis was oriented by the finding of an eosinophilia in the spinal fluid, then confirmed by the result of the immunodiagnosis of toxocariasis from both(More)
Only a few cases of cavernomas induced by radiation treatment, 78 patients, have been reported in the literature. The prevalence may be underestimated. Cavernomas occur several years after radiotherapy for brain neoplasia. Medulloblastoma, glioma and acute lymphoblastic lymphoma are commonly diagnosed and treated in childhood, generally in males. We report(More)
Cavernomas are vascular malformations mostly observed in the central nervous system. They occur in sporadic and familial forms. Familial forms are characterized by the presence of multiple lesions, an autosomal dominant pattern of inheritance and possible de novo lesions. We report two sporadic cases whose follow-up showed the appearance of new lesions.
In the majority of cases, severe stroke is accompanied by difficulty in swallowing and an altered state of consciousness requiring artificial nutrition and hydration. Because of their artificial nature, nutrition and hydration are considered by law as treatment rather basic care. Withdrawal of these treatments is dictated by the refusal of unreasonable(More)
INTRODUCTION Miller-Fisher syndrome (MFS) is a rare auto-immune post-infectious syndrome, characterized by an ataxia, an ophthalmoplegia and a generalized areflexia. It is considered as a clinical variant of Guillain-Barré syndrome (GBS). MFS is correlated with the presence of anti-GQ1b antibodies, elevated cerebrospinal fluid (CSF) protein levels, presence(More)