Li-Fraumeni syndrome: multiple distinct brain tumours in two brothers.

Li-Fraumeni syndrome is a rare autosomal dominant cancer-prone condition characterized by the occurrence of a large set of different types of cancer in a patient and their family. A germline disease-causing mutation of the gene encoding the p53 protein is associated with the syndrome. We report on a family in which segregation of a TP53 mutation in two generations was associated with two brain tumours, a leiomyosarcoma and a thyroid carcinoma in four male patients.

Continuous intrathecal infusion of a solution containing two drugs: calculation methods.

The calculation of the parameters of the pump is complicated for a mixture of two drugs in continuous intrathecal therapy with implantable and programmable pumps. It is important to determine the best proportion of drug solutions to obtain the desired clinical results and also to adapt their proportion to obtain the maximal volume of solution so that the time between two fillings is maximized. This paper proposes mathematical formulas that make easier and faster the calculation of the flow of the pump, the volume of each drug solution to be added to the pump, the concentration of each drug in the mixture, and the duration of the infusion.

Calculation methods for the continuous intrathecal infusion of a solution containing two or three drugs.

The continuous intrathecal infusion of drugs with implantable and programmable pumps is commonly used for the treatment of otherwise intractable pain and spasticity. There is now a consensus that it is possible to use mixtures of two or even three drugs in selected cases. The calculation of the parameters of the pump is easy for a single drug, but it becomes more complicated for a combination of two or three drugs.

[Isolated idiopathic amyloid tumor of the sacrum. An important differential diagnosis].

Primary amyloidosis may present as a pseudotumoral mass. Often associated with myeloma or paraproteinemia, it may also be isolated, without preexisting or associated disease. Isolated pseudotumoral bone involvement is rare and especially in the spine.

Unusual occurrence of a pseudo-aneurysm of the middle cerebral artery in a patient with fibromuscular dysplasia.

Our patient is the first reported case of pseudo-aneurysm due to the rupture of an intracranial artery in the context of fibromuscular dysplasia (FMD). As we assume that our case is probably not unique, we conclude that this diagnosis may be sometimes overlooked for lack of confirmation either by surgery or autopsy. The retrospective study of the arteriograms suggests some clues that should be taken into account to foresee the existence of an intracranial pseudo-aneurysm in order to avoid unexpected peroperative difficulties: 1.

[Epidural metastases at the spinal level. Clinical study of 82 cases].

This is a retrospective review of 82 cases of spinal epidural metastasis. A warning vertebral or radicular pain was present in 85% of the cases. In 33% of the patients, who complained only of pain without any neurological deficit, a complete myelographic block was already present.

Gram-negative bacillary meningitis in neurosurgical patients.

The authors report 34 cases of Gram-negative bacillary meningitis related to traumatic cranial lesions or neurosurgery observed between 1973 and 1980 at two neurosurgical units (Institut J. Bordet, Brussels, and Inselspital, Bern). As a typical nosocomial infection, meningitis developed after prolonged hospitalization in most patients, and was mainly due to highly resistant organisms, such as P.

[Normotensive hydrocephalus. Retrospective clinical study of 47 cases].

The clinical observation of 47 patients, undergoing surgery for "normotensive hydrocephalus" during the period January 1975 to January 1981 have been analysed retrospectively. The condition of half the patients improved following insertion of a cerebrospinal fluid bypass. Complications of varying degrees of severity were noted in 21 percent of cases.

[Intraventricular hematomas. Treatment with ventricular drainage combined with intracranial pressure monitoring (author's transl)].

Twelve patients with intraventricular hematomas were treated only with continuous ventricular drainage combined with intracranial pressure monitoring. Half of the patients showed a favourable clinical response. The main interest of ventricular drainage lies in the treatment of acute hydrocephaly which is usually associated.

Non traumatic cerebellar hematomas.

Five cases of spontaneous cerebellar hematomas are reported. In four cases the diagnosis was made by computerized tomography. The clinical diagnosis being difficult, surgical treatment was often applied too late and the diagnosis sometimes never made.

Effects of 1% and 2% enflurane on intracranial pressure in man.

We have studied the effects of enflurane on intracranial pressure (ICP) in 9 neurosurgical patients, whose ICP was continuously monitored for therapeutic or diagnostic purposes. The control ICP was under 15 Torr in 5 cases, under 20 Torr in 3 cases and of 25 Torr in one case. In order to achieve stable ventilatory conditions, the patients were kept under controlled ventilation, breathing a mixture of 60 to 70% nitrous oxide and oxygen.

[Solitary vertebral osteochondroma with spinal cord compression (author's transl)].

Solitary vertebral osteochondromas with spinal cord compression are very rare. Here we report a case of an osteochondroma of the 5th cervical vertebra protruding into the spinal canal. The therapeutic approach is discussed in relation to the natural evolution and the favourable prognosis of these lesions.

[The surgery of peripheral nerves. New perspectives in treatment by the microsurgical techniques (author's transl)].

The author describes the different techniques of reconstructive surgery of peripheral nerves and the failures that marked these trials until the advent of microsurgical techniques. After stressing the importance of the biology and physiopathology of a traumatized nerve and its stem cells, the author detailes the regeneration stages of the nerve cell when ideal conditions of axonal alignment are established by microtechniques. Considering the different notions of biology and pathology in relation to the time delay between the traumatism and referral to the surgeon, the author insists on the absolute necessity of operating during the period of full regeneration of the nerve cell, i.