[Partial non-convulsive status epilepsy in multiple sclerosis]. / Sclérose en plaques et état de mal épileptique partiel non convulsif.

This report describes the observations of two patients with a several years' history of multiple sclerosis who presented sudden neurologic impairment. The symptomatology was suggestive of a non-convulsive partial status epilepsy. The clinical presentation was a paroxysmal dysphasic phenomenon in the first case without any consciousness impairment, associated with slight right hemiparesis. Electroencephalographic investigations revealed asymmetrical patterns, left-sided slow waves and periodic lateralized epileptiform discharges (PLEDs). Antiepileptic treatments were partially effective and intravenous steroids were needed for complete recovery. For the second patient, clinical presentation was acute psychiatric symptoms with disorientation, alternating manic symptomatology and mutism. Electroencephalography showed left fronto-central rhythmic continuous slow wave and spike wave activity. Intravenous antiepileptic treatment quickly improved the symptomatology. These observations draw attention to the fact that an epileptic cause should not be ruled out when a patient with multiple sclerosis presents sudden neurologic or psychiatric impairment. An early diagnosis allows immediate antiepileptic treatment. Intravenous steroids can be added to stop seizures.

[Causes of paralysis of the hypoglossal nerve. Apropos of 32 cases]. / Causes des paralysies du nerf grand hypoglosse. A propos de 32 cas.

Thirty-two consecutive cases of hypoglossal nerve palsy (excluding syringomyelia and amyotrophic lateral sclerosis) collected between 1971 and 1987 were reviewed. The XIIth nerve palsy was clinically isolated in 8 cases, associated with other cranial nerve palsies in 16 cases and with long tracts involvement in 8 cases. Seventeen cases were related to tumours. Malignant tumours were predominant, especially middle and posterior fossa bone metastases. Carcinomatous meningitis and brainstem glioma were also found, as well as lymphoproliferative disorders and benign tumours such as chemodectoma and neurinoma. A vascular origin was established in 6 cases, related to vertebrobasilar infarct, truncular ischaemia and internal carotid dissection. The paralysis was consecutive to head or neck trauma in 4 cases and to various inflammatory processes in 4 other cases. The last case was caused by Chiari's malformation. To our knowledge, this is the first aetiological review of XIIth nerve palsy in the literature.

[Cerebellar imitation synkineses]. / Syncinésies d'imitation cérébelleuses.

Two patients with autosomal dominant pure cortical cerebellar atrophy, belonging to the same family, exhibited imitation synkineses of hands and feet when the contralateral extremity was moved. The phenomenon was observed particularly when alternate movements of one hand were performed, but it also existed when flexion-extension movements of one foot took place. The induced synkinetic movements were mainly observed on the right side in one patient and exclusively observed on the right side in the other one. At electromyography, the imitation synkinesis took place about 200 milliseconds after the first inducing movement, but tended to be simultaneous with the following ones. Imitation synkinesis appeared to be shared by other cerebellar conditions: 8 cases of sporadic pure cerebellar atrophy and 2 cases of post-surgical injury of the anterior lobe vermis. In the 2 genetic cases, there was no pyramidal sign nor sensitive disturbance, the somesthesic evoked potentials being normal. Thus, the imitation synkinesis was considered as having a cerebellar origin. The cerebellar imitation synkinesis might be provoked by the lack of a physiologic cerebellar inhibition located in the paleo- and/or neo-cerebellum. The predominance of imitation synkineses on the right side suggests that cerebellar inhibition is stronger for the dominant side, in order to liberate it from archaic synkineses.

Confirmation of linkage of Friedreich ataxia to chromosome 9 and identification of a new closely linked marker.

A linkage analysis with chromosome 9 markers was performed in 33 families with Friedreich ataxia (FA). Linkage with D9S15, previously established by S. Chamberlain et al. (1988, Nature London 334:248-249) was confirmed in our sample (z(theta) = 6.82 at theta = 0.02) while INFB (interferon-beta gene) shows looser linkage. An additional marker, D9S5, was also shown to be closely linked to FA (z(theta) = 5.77 at theta = 0.00).

[Giant thrombosed aneurysm of the left vertebral artery developing in the fourth ventricle]. / Anévrysme géant thrombosé de l'artère vertébrale gauche développé dans le quatrième ventricule.

A case of giant, thrombosed, non haemorrhagic aneurysm of the distal portion of the left vertebral artery is reported. The patient came to medical attention with an acute cervical pain after a minimal cervical traumatism and a diagnosis of torticollis from rheumatologic cause was made. In fact, a few weeks before, he had suffered three episodes of right homonymous hemianopsia. Subsequently, hiccup, vomiting, orthostatic dizziness with postural hypotension appeared, suggesting a medullary lesion. CT scan showed a round, heterogeneous high-density lesion near the fourth ventricle. Angiography was normal. MRI showed an oval mass in the fourth ventricle, between the medulla and the cerebellum. Surgery found an aneurysm of the end of the left vertebral artery.