[Lhermitte-Duclos disease. Granulomolecular hypertrophy of the cerebellum]. / Maladie de Lhermitte-Duclos. Hypertrophie granulo-moléculaire du cervelet.

A case of Lhermitte-Duclos disease is reported. The onset is unusually sudden in a 16 years-old girl. However cerebellar lesions and hydrocephalus are of long duration as showed the skull base anomalies. This slow evolution is the rule in many cases previously reported, which suggests that the disease is due to a dysplasic disorder, perhaps one of cellular differentiation of the cerebellar cortex.

[Intracranial ependymomas. Experience with 86 cases]. / Les épendymomes intracrâniens. Expérience de 86 cas.

The authors report a series of 86 intracranial ependymomas. The origin of the tumor was supratentorial in 53 cases, infratentorial in 33. All patients underwent surgery and most of them had also radiation therapy. The operative mortality rate was 25%. Five year survival rate was 31%. Better prognosis was recorded for low grade tumors. The location of the tumor, total or incomplete surgical excision had no influence concerning long-term survival. The five year survival rate for the 28 children in the series was 42%, against 19% for adults. Local recurrence occurred in 39% of patients, and spinal metastases were recorded in only 2 cases. The authors emphasize the need for systematic radiation therapy, with different protocols according to age of patient, location and histological type of tumor.

[Distal intracranial arterial aneurysms. Apropos of a case of giant aneurysm of the right angular gyrus artery]. / Les anévrysmes artériels intracrâniens distaux. A propos d'un cas d'anévrysme géant de l'artère du pli courbe.

A 57 year old man presented with a giant, fusiform, partially thrombosed aneurysm, located distally to the right angular gyrus artery, and revealed by a subarachnoïd hemorrhage. There was no history of head trauma, endocarditis or systemic infection, and physical examination was normal. The treatment consisted in simple trapping of the aneurysm, responsible for a transient ischemic parietal syndrome. After a short review of the pathogenesis of peripheral aneurysm of the C.N.S., and previously reported cases of distally located aneurysms of the middle cerebral artery, only one similar case was found concerning a posterior temporal artery aneurysm involving to a giant serpentine aneurysm. The case presented is believed to be a segmentary form of cerebral arterial dolicho ectasia; relations between arterial ectasia or fusiform aneurysms and serpentine aneurysms are discussed.

[Lhermitte-Duclos disease. Apropos of a case]. / Maladie de Lhermitte-Duclos. A propos d'une observation.

The authors report a case of dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease). This woman's first neurosurgical admission was in 1970 at the age of 15 years. Hydrocephalus related to aqueduct-stenosis was diagnosed and a ventriculo atrial shunt was inserted. Ten years later, she underwent partial resection of a third ventricle tumor which was identified as a pilocytic astrocytoma. In 1986, computerized tomography revealed a non-enhancing left cerebellar mass lesion. Surgical excision was performed, and pathological examination disclosed dysplastic gangliocytoma of the cerebellum.

False positive-false negative CT scan in late epileptic seizure: a meningioma-glioblastoma association.

A false-negative finding on initial CT is reported in a case of supratentorial glioma. This observation was peculiar because the first CT revealed a meningioma which might initially have been related to the clinical symptoms. The term false positive-false negative CT is proposed. The reasons for such CT failures are discussed. The accuracy of clues as to the localization of the glioma provided by EEG is emphasized.

Cerebellar medulloblastoma: results of multidisciplinary treatment. Report of 120 cases.

The authors report a series of 120 cerebellar medulloblastomas. All patients were operated on between 1953 and 1982. Among them, 88 completed the treatment with radiotherapy, and 32 had additional chemotherapy. The operative mortality was 22.5%. The 5-year survival rate was 30% in the whole series. Recurrences occurred in 35 patients, associated with supratentorial or spinal metastasis in 60% of cases.

[Late posttraumatic syringomyelic syndromes. Pathogenetic theories apropos of 3 cases]. / Syndromes syringomyéliques post-traumatiques tardifs. Théories pathogéniques à propos de trois observations.

Three patients with a post-traumatic spinal cord cavity are described: clinical signs are identical to those of syringomyelia, but pain is more frequent. Diagnosis is made easier by magnetic resonance imaging (MRI) and a syringoperitoneal shunt is proposed for treatment. Nevertheless, if results are good, they seem worse than in idiopathic syringomyelia. The main pathogenic theories are discussed: the rupture and coalescence of microcysts developed at a distance of 1 or 2 mm from the transected spinal cord, subsequently leads to syrinx formation; haemorrhagic or ischemic post-traumatic areas could also lead to syrinx formation. Hydrodynamic factors could be expected and make the pathogenesis not far from syringomyelic one.

[Spinal cord compression caused by dorsal disk hernia. Complication of Scheuermann's disease]. / Compression médullaire par hernie discale dorsale. Une complication de la maladie de Scheuermann.

Association of Scheuermann's disease with a thoracic herniated disc compressing the spinal cord is rare but is clearly demonstrated in the case reported here. This confirms the non-fortuitous nature of this association also suggested by the analysis of published cases. The interest of spinal cord angiography and of surgery through a postero-lateral approach are emphasized.

Traumatic extradural hematomas in infancy and childhood. Experience with 144 cases.

The authors report a series of 144 children with traumatic extradural hematomas operated on at the Lille Department of Neurosurgery between 1969 and 1982. The patients are divided into different groups according to age, and clinical findings were recorded for each age group. The overall mortality rate was 9%. The authors demonstrate that prognosis is related to age, neurological status at time of surgery, and duration of postoperative coma.

[61 years' survival of Koch's bacillus in a closed focus. Disclosure in a case of spinal cord compression caused by sequelae of Pott's disease]. / Soixante et un ans de survie d'un bacille de Koch dans un foyer clos. Découverte lors d'une compression médullaire par séquelles de mal de Pott.

A 68 year-old woman presented with a spinal cord compression from an angular kyphosis due to Pott's disease at age 7. Clinical, radiological and pathological studies of the vertebral lesions did not demonstrate an evolutive lesion. However, culture yielded some viable Koch's bacilli. This bacteriologic confirmation 61 years later exemplifies the importance of host immunity reactions, that may prevent the development of KB, and the remarkable capacity of survival of the bacillus.

[Cerebral abscesses disclosing pulmonary angioma. Rendu-Osler disease]. / Abcès cérébraux révélateurs d'un angiome pulmonaire. Maladie de Rendu-Osler.

A 50-year old male patient presented with two successive cerebral abscesses over a 9-month period. Rendu-Osler-Weber disease was diagnosed on the basis of recurrent epistaxis and telangiectasis of the mucosae and skin. In addition, many members of the patient's family had a history of epistaxis and one had a cerebral angioma of unusual location. Our patient's chest was normal on physical and radiological examination, but pulmonary angiography disclosed a small angioma. Removal of the angioma prevented further cerebral infections. The prevalence of pulmonary arterio-venous fistulae in Rendu-Osler-Weber disease and their clinical manifestations (especially in the central nervous system) are reviewed, together with the value of family studies and the therapeutic approach.

[Peritoneal csf pseudocysts. Rare complication of ventriculo-peritoneal shunts]. / Pseudo-kystes péritonéaux de L.C.R.. Complication rare des dérivations ventriculo-péritonéales.

The authors report 6 cases of abdominal CSF pseudo-cysts as a complication of ventriculo-peritoneal shunts. The clinical picture and diagnosis confirmation by radiology and echography are reviewed. Histogenesis and pathogenesis are discussed.

[Regressing median peduncular infarction]. / Infarctus pédonculaire médian d'évolution régressive.

A case of a medial and caudal infarction of the midbrain in a 56 years old woman is reported. The clinical syndrome included a rapidly recovering sleepiness, an ophthalmoplegia related to damage to the caudal part of the oculomotor nuclear complex, a slow extrapyramidal dysarthria and a severe ataxia. The latter was characterized by the prevalent involvement of posture control, resulting from an axial hypotonia and the lack of balance reflexes. The CT scan showed a low density area in the central part of the midbrain. Electrophysiological investigations were carried out to study the impairment of the sensory and auditory tracts in the brainstem. In spite of the gravity of initial signs, recovery was good and the patient led a normal life 8 months later.

[Craniopharyngioma in the same family]. / Craniopharyngiome dans une même fratrie.

Within 2 years, two sisters, aged 23 and 33, were operated on for a craniopharyngioma. With regard to this exceptional feature, relation between neuroectodermal affections and craniopharyngiomas is discussed, and genetic determination of the occurrence of those tumors is evoked.

Use of acrylic prosthesis in cervical spine pathology (report of 45 cases).

The authors report on their experience with the use of a reinforced acrylic prosthesis in cervical spine pathology. The study of their own 45 cases enables the advantages and disadvantages of the technique to be demonstrated. A discussion of possible improvements in the technique and its indication completes the report.