[Withdrawal of artificial nutrition and hydration in severe stroke: medical, legal and ethical considerations].

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 obstinacy enshrined in law and is justified by the risk of severe disability and very poor quality of life.

[Advance directives shedding light on ethics].

An advance directive is an essential document for providing a better understanding of an end-of-life situation. It is not imposed on the doctor but must nevertheless be taken into account in the decision-making. The application of the directive invites a time of reflection on ethics and discussion among peers.

[When consciousness goes].

Some neurodegenerative diseases gradually affect the patient's consciousness. While the patient may be declared incompetent, decisions concerning treatment must take into account his or her wishes, however weakly they may be expressed. A real ethical challenge for all the caregivers who must ensure the person's dignity is maintained and refrain from demonstrating therapeutic obstinacy.

[Collet-Sicard syndrome after carotid artery dissection].

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.

[Radiation-induced cavernoma: two cases].

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.

[Recurrent Miller-Fisher syndrome].

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 of mostly sensitive electrophysiological abnormalities and for some authors central involvement with increased signal intensity of brainstem and cerebellum on MRI.

Cerebellar ataxia with oculomotor apraxia type 1: clinical and genetic studies.

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.

[Toxocara canis meningoradiculitis].

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 serum and spinal fluid.

Absence of linkage to 8q24 in a European family with familial adult myoclonic epilepsy (FAME).

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 spike detected by means of the jerk-locked averaging EEG method. These findings were also observed in patients with benign adult familial myoclonic epilepsy (BAFME) and patients with familial cortical tremor. FAME and BAFME have been described only in Japan.

Appearance of new lesions in two nonfamilial cerebral cavernoma patients.

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.

[Neurological manifestation of infectious endocarditis: 14 cases].

Fourteen cases of infective endocarditis revealed by neurological manifestations are reported: 8 strokes (one transient ischemic attack, one regressing and 6 completed strokes), 2 intracranial hematomas (one due to ruptured mycotic aneurysm), 2 toxic encephalopathies, one grand mal seizure, one suppurative meningitis. Most of them were native valve endocarditis, and streptococcus was the most frequently responsible bacteria. The outcome was characterized by a high mortality (6 cases) and morbidity (4 cases).

[Arachnoid cysts and subdural hematomas].

Arachnoid cysts of the middle cerebral fossa is a not uncommon lesion which can occur in young subjects, sometimes after minimal head trauma. Subdural hematomas and sometimes intracystic hemorrhage may develop. We report a personal series of seven cases seen in young subjects (6-24 years).

[Pneumocephalus complicating osteoma of the frontal sinus].

Osteomas of the paranasal sinuses are benign, often asymptomatic, tumors which progress very slowly. Endocranial development of an osteoma can breach the dura mata, allowing air to enter the cranium producing pneumocephalia which leads to severe neurological deficiencies. Pneumocephalia is an exceptional complication of osteoma.

[Cerebral venous angiomas. 12 personal cases and review of the literature].

Cerebral venous angiomas (CVAs) are made up of veins with abnormal structure: thick walls, lumens dilated of irregular calibre that converge radially towards a wide draining vein. The arteries are normal. The veins are separated by a normal nervous tissue.

[Lambert-Eaton and Schwartz-Bartter syndromes and peripheral neuropathies associated with bronchial carcinoma: apropos of a case].

A case of Eaton-Lambert myasthenic syndrome associated with inappropriate secretion of antidiuretic hormone is reported. This case included a demyelinizing peripheral neuropathy and was related to a small-cell carcinoma of the lung. Twelve similar cases appeared in the literature, most of them associated with small-cell carcinoma or undifferentiated lung tumors.

[Diagnosis of moderate stenoses of the origin of the internal carotid artery using the continuous emission Doppler technic: semeiologic study].

The authors propose two signs for detecting moderate (about 50% reduction of the arterial lumen) stenosis at the origin of the internal carotid (IC) using the Doppler technique: (1) the 'arch-pattern,' drawn on the systolic and diastolic blood flow velocity enveloping curve when slowly translating the probe from the common carotid (CC) to the end of the accessible part of the IC; (2) The 'proximo-distal carotidian ratio,' the latter being pathological if below 1. A large scale confrontation with angiography is still necessary to validate them.

[Apropos of a case of thyrotoxic periodic paralysis (TPP): electromyographic study (EMG)].

The authors report on a case of thyrotoxic periodic paralysis characterized by attacks of flaccid tetraplegia with hypokalemia in a patient with Basedow disease, the cure of which led to the disappearance of the neurological symptomatology. The EMG investigations, performed in the course of a spontaneous paralytic attack, showed the same abnormalities as in the few similar published cases: discordance between the rich interferential recordings and the weakness of the movements; low amplitude of the electrodetection tracings; reduced amplitude of the evoked muscle action potentials on nerve stimulation. These myogenic patterns are related to the aspect of the muscle biopsy which, when performed during the paralytic phase, shows a vacuolization of the muscular fibers.

[Chorea and Vaquez's disease. Apropos of a case associated with extrapyramidal tremor].

After reporting a case, the authors recall the description of two fairly uncommon complications of polycythemia vera, chorea and extrapyramidal tremor, whose course is closely related to that of the hematological disease. Clinical and physiopathological data are discussed.

[A case of unilateral and lesional mu rhythm--clinical and encephalographic development].

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 reactive, then into a larger and more active slow focus.

[Cleido-cranial dysostosis with malformation of the cervico-occipital junction].

A 66 years old man with cleido-cranial dysostosis suffered from a progressive ataxic gait for 45 years. Physical examination discovered cerebellar and pyramidal signs and altered deep sensibility in lower limbs. Roentgenograms showed skeletal dysostosis and a severe malformation of the cervico-occipital junction.

[Emergency disobliteration of the carotid prepolygonal segment. Personal experience of 76 cases].

The authors report 76 cases of carotid obstruction, mainly localized at the origin of the carotid artery, that have been operated upon in emergency. Two types of lesions are considered: occlusion and very tight stenosis. The principle of early surgery in cases of acute occlusion is very controverse.