[Withdrawal of artificial nutrition and hydration in severe stroke: medical, legal and ethical considerations]. / Aspects médicaux, législatifs et éthiques de l'arrêt de la nutrition et de l'hydratation artificielles dans l'accident vasculaire cérébral grave.

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. It is usually the last among other withholding and withdrawal decisions which have already been made during the long course of the disease. Reaching a collegial consensus on a controversial decision such as artificial nutrition and hydration withdrawal is a difficult and complex process. The reluctance for such decisions is mainly due to the symbolic value of food and hydration, to the fear of "dying badly" while suffering from hunger and thirst, and to the difficult distinction between this medical act and euthanasia. The only way to overcome such reluctance is to ensure flawless accompaniment, associating sedation and appropriate comfort care with a clear explanation (with relatives but also caregivers) of the rationale and implications of this type of decision. All teams dealing with this type of situation must have thoroughly thought through the medical, legal and ethical considerations involved in making this difficult decision.

[Collet-Sicard syndrome after carotid artery dissection]. / Syndrome de Collet-Sicard après dissection carotidienne.

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 of the left lower cranial nerves IX-XII linked to carotid artery dissection after trivial neck injury.

[Radiation-induced cavernoma: two cases]. / Cavernomes radio-induits. A propos de deux cas.

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 new cases of cavernomas induced by radiation treatment. The first case was a 55-year-old man given radiation and chemotherapy for frontal astrocytoma at the age of 46. The second concerned a 30-year-old woman treated by radiation and surgery for brainstem medulloblastoma at the age of four. Epidemiological and pathogenic features of radiation-induced cavernoma are discussed.

[Recurrent Miller-Fisher syndrome]. / Syndrome de Miller-Fisher récidivant.

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. Recurrent MFS is extremely rare with only 21 cases since the first description in 1970. CASE REPORT: A 54-year-old women presented MFS with two episodes in 19 years. Clinically, the first episode was a "classical" MFS, and the second an extensive MFS with tetraparesis and respiratory failure. CSF protein levels and cerebral MRI were normal. Anti-GQ1b antibodies were strongly positive and anti-GM1, anti-GM2 antibodies were slightly positive, campylobacter jejuni serology was negative. Electromyography showed isolated sensory abnormalities in median nerves territory. CONCLUSION: We report a new case of recurrent MFS with unusual clinical, biological and electrophysiological features.

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. The genes responsible for FAME and BAFME were mapped in the same genetic interval in 8q22.3-q24.1 OBJECTIVE: To study clinical and genetic characteristics of a European family with FAME. METHODS: A four-generation European kindred presenting with FAME, including 18 members, is described. Clinical analysis was performed on 15 living subjects and electrophysiologic study on 5 patients. Linkage analysis was performed with fluorescent microsatellites encompassing the FAME/BAFME locus (8q23.3-q24.1). RESULTS: Ten living and three deceased relatives had the clinical characteristics of FAME. Mean age at onset of the 10 living patients was 41 years (range, 30-60 years). Eight of the 13 affected subjects had generalized tonic-clonic seizures. Electrophysiologic studies confirmed the diagnosis of FAME in the five patients studied. The pattern of inheritance was consistent with an autosomal dominant inheritance. The locus responsible for FAME/BAFME was excluded. CONCLUSION: Observation of a European family extends the occurrence of familial adult myoclonic epilepsy to non-Japanese patients. Exclusion of linkage of this family to the locus for familial adult myoclonic epilepsy/benign adult familial myoclonic epilepsy established the genetic heterogeneity of this disorder.

[Lambert-Eaton and Schwartz-Bartter syndromes and peripheral neuropathies associated with bronchial carcinoma: apropos of a case]. / Syndromes de Lambert-Eaton et de Schwartz-Bartter et neuropathie périphérique associés à un carcinome bronchique: à propos d'un cas.

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. Etiologic and diagnostic aspects of these syndromes are discussed. When isolated, their causes are various, including the classic context of the paraneoplastic syndromes. Their association is highly suggestive of a lung carcinoma and must enable to make an early diagnosis by use of all possible means of detection.

[Cerebral venous angiomas. 12 personal cases and review of the literature]. / Les angiomes veineux encéphaliques. 12 cas personnels et revue de la littérature.

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. The malformation is thought to develop as a compensatory venous drainage consecutive to the occlusion, or lack of development, of one or several transcerebral veins during the formation of the mature venous system. Together with arteriovenous angiomas, capillary telangiectasias and cavernous angiomas, CVAs belong to the vascular angiomatous malformations, also called hamartomas. The present study is based on 12 cases collected between 1984 and 1989, all explored by CT and angiography, and by MRI in 2 cases. The diagnosis therefore was neuroradiological, except in 1 case where it was obtained by neuropathological examination. The malformation was supratentorial in 10 cases and cerebellar in 2 cases. Most CVAs were discovered in patients whose symptoms could hardly be attributed to these malformations and consequently were termed asymptomatic (6 cases). Two cases were found in subjects with generalized epileptic seizures without clear-cut relationship with the angioma; 4 cases were revealed by haemorrhages: subarachnoidal haemorrhage in 2 cases and supratentorial intraparenchymatous haematoma in 1 case. These 3 cases had a spontaneously favourable outcome after a follow-up of several years. One patient with a cerebellar hematoma died postoperatively of edematous infarction of the cerebellum. One of these patients had two symmetrical CVAs, one in each cerebral hemisphere (multiple venous angiomas), and in another patient the CVA was probably associated with a cavernous angioma.(ABSTRACT TRUNCATED AT 250 WORDS)

[Pneumocephalus complicating osteoma of the frontal sinus]. / Pneumocéphalie compliquant un ostéome du sinus frontal: 2 cas.

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. We found 40 cases reported in the literature. Brain CT scan easily gives the diagnosis. Surgical exeresis with closure of the breach gave very satisfactory results in our 2 cases.

[Arachnoid cysts and subdural hematomas]. / Kystes arachnoïdiens et hématomes sous-duraux.

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). Clinical presentation was not specific, the complication usually being revealed by signs of intracranial hypertension. The pathogenesis of subdural hematoma is discussed. Magnetic resonance imaging is the most useful diagnostic tool, providing excellent tissue specificity, although CT scan is often used to visualize a subdural hematoma and subsequent arachnoid cyst. Treatment relies on surgery to empty the subdural hematoma and remove compression. There has been no real consensus on treatment modalities. Long-term prognosis is good in most cases.

[Toxocara canis meningoradiculitis]. / Méningo-radiculite à toxocara canis.

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.

[Neurological manifestation of infectious endocarditis: 14 cases]. / Endocardite infectieuse à révélation neurologique: 14 cas.

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). This emphasizes the usefulness of preventing antibiotherapy in patients with known predisposing factors and the necessity for these patients to be admitted in neurological intensive care units.

[Cleido-cranial dysostosis with malformation of the cervico-occipital junction]. / Maladie de Pierre Marie et Sainton avec malformation de la charnière cervico-occipitale.

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. The disease was transmitted with a dominant autosomic inheritance. The patient died from cardiac and respiratory distress. Post mortem examination confirmed radiologic anomalies: basilar invagination with a low-situated brain stem. The paucity of neurological complications of cleido-cranial dysostosis is emphasized. This is apparently the first reported case with clinical signs of cervico-occipital malformation.

[Emergency disobliteration of the carotid prepolygonal segment. Personal experience of 76 cases]. / La désobstruction d'urgence de l'axe carotidien dans son segment pré-polygonal. Expérience personnelle à partir de 76 observations.

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. According to the facts reported (38 cases) results are rewarding when operative decision is taken under precise conditions judged as favorable: short lapse of time since occlusive onset, absence of intracranial hypertension, absence of comatose state and no sign of brain oedema. Some patients under go surgery in the priviledged condition of being already in a medico-surgical unit (post operative, post angiographic, embolic occlusions). Majority present the most common hemiplegic attack. However difficulty resides in the inefficient mode of transport to hospital and lack of highly specialized units on admission. A very tight stenosis (at extreme a pseudo-occlusive stenosis) with clinical recurrent deficit, modified Loppler's recording and hemodynamic repercussion at angiography is an operative emergency. When results of early surgery on 38 cases of stenosis are compared to those of late operation in 14 similar cases early decision becomes compulsory once tight stenosis of the carotid artery is identified and operative criteria respected. In the light of this report that "wait and see attitude" needs be somewhat revised when confronted to the dramatic ictal hemiplegia.

[Chorea and Vaquez's disease. Apropos of a case associated with extrapyramidal tremor]. / Chorée et maladie de Vaquez. A propos d'un cas associé a un tremblement extrapyramidal.

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]. / A propos d'un cas de rythme mu unilatéral et lésionnel--évolution clinique et électroencéphalographique.

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. This focus transistorily decreased under X-ray treatment. The particular aspects of this observation are discussed in the light of the literature data.

[Apropos of a case of thyrotoxic periodic paralysis (TPP): electromyographic study (EMG)]. / A propos d'un cas de paralysie periodique thyrotoxique (ppt): étude électromyographique (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. The pathophysiological underlying mechanisms of the thyrotoxic periodic paralysis still remain hypothetic.

[Diagnosis of moderate stenoses of the origin of the internal carotid artery using the continuous emission Doppler technic: semeiologic study]. / Le diagnostic des sténoses moderées de l'origine de la carotide interne en Doppler à émission continue: recherche seméiologique.

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.

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. We report two sporadic cases whose follow-up showed the appearance of new lesions.