Narcolepsy associated with arteriovenous malformation of the diencephalon.

Narcolepsy associated with localized brain lesions is rare, and few reports of well-documented cases have been published. We describe the case of a 20-year-old male (HLA DQw1 negative) who fulfilled clinical and polygraphic criteria of symptomatic narcolepsy. Narcolepsy in this patient was associated with an arteriovenous malformation involving the structures around the third ventricle. Clinical symptoms improved after embolization and radiosurgery. These findings support the hypothesis that lesions in the vicinity of the third ventricle can cause symptomatic narcolepsy.

[Neurologic manifestations of basal cell nevomatosis. A case]. / Manifestations neurologiques de la naevomatose baso-cellulaire. Un cas.

The naevoid basal cell syndrome, also called Gorlin's syndrome, is a dysembryoplasia akin to the phakomatoses and which may affect the skin, the bones, the nervous system, the eyes, the endocrine glands and the genitalia. A case with numerous tumours and malformations is presented, together with a review of the literature.

[Benign paralysis of the last 4 cranial nerves. Arguments in favor of an ischemic mechanism]. / Paralysie bénigne des quatre derniers nerfs crâniens. Arguments en faveur d'un mécanisme ischémique.

The last four cranial nerves are fed by the ascending pharyngeal artery, a branch of the external carotid artery. The fact that paralysis of these nerves may occur immediately after arteriography of the external carotid artery demonstrates that ischaemic truncular neuropathies do exist. The deficit is sudden and usually regressive. Ischaemia may account for regressive paralysis of these nerves reported in diabetes, herpes zoster and after some traumas. Apparently idiopathic and benign paralysis might be due to the same mechanism. All 4 nerves may be affected, or only the IXth, Xth and XIth nerves, or the XIth nerve may be spared, being fed by two arteries. Apart from the obvious case of arteriographic accident, the diagnosis of paralysis of ischaemic origin can only be made after other causes, notably compression, have been excluded, since direct evidence of arterial obstruction is exceptionally obtainable; regression at follow-up is a major argument in favour of ischaemia.

[Acute parasellar compression syndrome disclosing hypophyseal adenoma. 3 cases]. / Syndrome de compression parasellaire brutale révélateur d'adénome hypophysaire. Trois observations.

Three cases of acute necrosis of a hypophyseal adenoma are reported with a review of the medical literature. The clinical presentation as an acute parasellar compression syndrome was the result of aseptic meningitis in 2 cases and of cavernous sinus thrombosis in the other; the usual symptoms of headaches, oculomotor paralysis and impairment of consciousness were observed. The diagnosis was suspected on clinical grounds, by the radiological changes of the pituitary fossa and confirmed by computerised axial tomography. All three patients had a favourable outcome. Surgery was only required in one case with threatening visual complications. Dissociated anterior pituitary deficiency persisted in all cases; two patients also had diabetes insipidus. In one case, the pituitary necrosis stabilised an acromegaly for a two year period.

[Dissection of the vertebral arteries after manipulation of the cervical spine. Apropos of a case]. / Dissection des artères vertébrales après manipulation du rachis cervical. A propos d'un cas.

Dissecting aneurysm of the vertebral arteries following chiropractic manipulation of the spine. A thirty-five years old man had a Wallenberg Syndrome following chiropractic manipulation of the spine. Plain films and hypocycloidal tomography showed a foramen arcuale on both side. Arteriography lead to the diagnosis of dissecting aneurysm of the vertebral arteries. The favourable course point to the value of the posterior communicating arteries and the spinal artery as collateral pathways of the vertebro-basilar circulation.

Benign intracranial hypertension and disorders of CSF absorption.

Sixteen patients suffering from benign intracranial hypertension were studied by a continuous measurement of intraventricular pressure, a simultaneous recording of intraventricular and sagittal sinus pressures, and a measurement of the cerebrospinal fluid (CSF) resistance to drainage. Isotope cisternography was performed and the patency of the dural sinuses verified by direct sinography or phlebography or both. The same procedure was used to study 6 other patients suffering from disorders leading to the same type of intracranial hypertension. In 16, our results confirm a defect in CSF absorption mechanisms linked to an abolition of the pressure gradient between CSF and sagittal sinus in 6 patients, as well as an important increase in CSF resistance to drainage in 10 others. Despite this defect, the CSF circulation was normal in most patients (10 of 12) as demonstrated by isotope cisternography.

Primary empty sella turcica in children. Report of two familial cases.

The authors describe cranio-facial deformities found in two sisters and associated with spinal anomalies, short stature and delayed skeletal maturation. The principal radiological features were an enlarged J-shaped sella turcica and intrasellar cisternal herniation. Enlargement of optic foramina and internal acoustic canals were also present. These asymptomatic cases of "empty sella" seem to be part of a general dysplastic syndrome rather than a local disease.

Benign intracranial hypertension. A clinical evaluation of the CSF absorption mechanisms.

Fifteen patients suffering from Benign Intracranial Hypertension due to various causes were investigated by measuring the CSF resistance to flow, and the pressure difference between the CSF and the sagittal sinus. Isotope cisternography was also performed, and the patency of the dural sinuses was checked by both sinography and phlebography. In twelve the results confirm a defect in the resorption mechanism, the nature of which proved to be venous in six, and linked to impairment of subarachnoid villi in six others. Three patients could not be classified. The significance of normal isotope cisternography is discussed.

Intracranial pressure with intracerebral hemorrhages.

Intracranial pressure was recorded continuously during an average of 15 days in 17 patients suffering from primary intracerebral hemorrhage. In 12 cases the highest pressures were recorded just after the stroke; then the intracranial pressure decreased and became normal in an average of 20-30 days. Other patterns of evolution were less often observed: a rapid and lethal elevation of pressure in one case, a constantly low pressure in two, and a stagnant evolution with moderate hypertension in two others. Secondarily developing intracranial hypertension was never observed during the monitoring period. Evacuation of the clots was performed in six patients. This only slightly shortened the course of the increased intracranial pressure. It is concluded that intracerebral hematoma appears as an expanding lesion only during the time of its formation. The prognosis depends more upon the destructions by the hemorrhage than upon the increased pressure. Nevertheless, true hypertension is possible. Knowledge of intracranial pressure in the course of intracerebral hemorrhage is important in deciding whether the treatment is to be surgical or conservative. Measurements of the intracranial pressure in our practice has reduced the number of interventions, with identical or slightly improved results.

[Resistance in cerebrospinal fluid flow in normal pressure hydrocephalus]. / La résistance à l'écoulement du liquide céphalo-rachidien dans l'hydrocéphalie à pression normale

C.S.F. resistance to flow (Rf) was measured in 24 patients by the mean of a constant infusion manometric test, and the results were compared with those of pneumo-encephalography and isotope cisternography. It is concluded that Rf is constantly elevated in patients with normal pressure hydrocephalus, is at least as reliable as the two other tests and therefore provides a really valuable aid to the identification of the patients to be shunted.