In vitro karyotypic and immunophenotypic characterisation of primitive neuroectodermal tumours: similarities to malignant gliomas.

Monoclonal antibody (Mab) mediated immunotherapy of brain tumours requires the identification of tumour-restricted cell surface antigens. We have characterised four primitive neuroectodermal tumours, which included pineoblastoma, medulloblastoma and ependymoblastoma cultures, that demonstrated in vitro evidence of malignant behaviour (anchorage-independent growth and nu/nu xenograft tumour formation). The cytogenetic findings ranged from normal G-banded and Q-banded karyotypes through mixed near-diploid/hyperdiploid. These cultures resembled the cell surface immunophenotypic spectrum of malignant gliomas. They were distinguished from normal glia in vitro by the expression of restricted fetal mesenchymal, neuronal, myoblastic, melanocytic, epidermal, chondrocytic, lymphoid and epithelial antigens. Certain antigens appeared sufficiently represented among central nervous system (CNS) neoplasms to afford potential targets for Mab-mediated immunotherapy.

M4 protocol for cerebellar medulloblastoma: supratentorial radiotherapy may not be avoided.

The main goal of the M4 protocol was to evaluate the efficacy of treatment excluding supratentorial radiation in patients with newly diagnosed medulloblastoma. All patients underwent surgical resection and received postoperative chemotherapy. Chemotherapy was adapted to the initial staging and prognostic factors (Group A: good-risk; Group B: poor-risk). Chemotherapy was started early after surgery, and consisted of two courses of the "eight drug in one day" regimen and two courses of high dose methotrexate. Radiotherapy was delayed until 5 (Group B) to 7 (Group A) weeks after the first course of chemotherapy. Radiotherapy was administered only to the posterior fossa and the spinal axis. Only 3/16 patients (18%) are alive and disease-free with a mean follow up of 6 years. The site of progression was supratentorial in 9 out of 13 patients and three patients had spinal and/or cerebrospinal fluid relapses. Only one patient had isolated posterior fossa relapse. The mean time to relapse was 484 days. We conclude that the chemotherapy regimens used in the M4 protocol do not allow the reduction of irradiation fields in patients with cerebellar medulloblastoma. In spite of long-term side effects on neurocognitive functions, supratentorial radiotherapy should remain a major component of medulloblastoma treatment.

Medulloblastoma in adults: survival and prognostic factors.

The authors report a series of 30 adults with medulloblastoma treated after surgery between 1975 and 1990. Half the patients presented a classical medulloblastoma and the other half presented desmoplastic medulloblastoma. Brainstem infiltration was noted in 3 patients, cerebellar peduncle infiltration in 6 cases and 4 patients had cerebrospinal fluid (CSF) involvement. Tumor resection was achieved in all patients except 1, and in 5 surgery was incomplete. All patients but 2 received postoperative cerebral radiotherapy. Six patients did not receive chemotherapy before radiotherapy, 7 patients received CCNU-Vincristine concomitantly with and after irradiation, 1 patient was given a CHOP regimen, and the remaining 16 patients received the 8 drugs in a 1-day (8/1) regimen before irradiation. The median follow-up time was 104 months. The 5- and 10-year overall survival rates were 58.5% and 41%, respectively. Two toxic deaths occurred under the 8/1 regimen, and no better survival was observed for this group of patients in comparison with those receiving other regimens. Persistence of malignant cells in the CSF, brainstem involvement, cerebellar peduncle infiltration, non-radical surgery and a postoperative performance status (PS) of more than 2 were significantly correlated with an adverse outcome for overall survival in adult patients with medulloblastoma.

Non-metastatic childhood ependymomas.

PURPOSE: Intracranial ependymomas of childhood are relatively infrequent. There are significant disagreements concerning optimal postoperative treatment as well as the patterns of relapse following treatment. The purpose of this retrospective study was the analysis of the recurrence pattern and therefore the implication on the extent of the radiotherapy fields. Data from 37 patients referred within 19 years were used for this study. PATIENTS AND METHODS: From April 1975 to July 1993, 37 children aged 1-14 years were referred for postoperative treatment of an intracranial ependymoma. Twenty-eight children received postoperative radiation therapy and 26 patients received chemotherapy. The median follow-up is 6 years (range 2 months to 19 years). RESULTS: Overall survival and event free survival at 5 and 10 years were 40%. Eighteen children relapsed. Relapses occurred from 1.5 months to 3.6 years post treatment. Relapses were distant in four cases and local in 14. Age, sex, extent of primary resection, chemotherapy and type of radiation therapy did not influence the outcome. Children with poorly differentiated tumors who did not receive postoperative radiation therapy had a higher relapse rate but this difference is not statistically significant. CONCLUSIONS: Despite doses of radiation > or = 50 Gy the majority of recurrences were local. Our results, despite the small number of patients are in accordance to those previously published, suggest that prophylactic craniospinal irradiation is superfluous. Better means of achieved local control are required, such as three-dimensional conformal radiation therapy with dose-escalation study or hyperfractionation regimen.

Stereotactic radiosurgery for arteriovenous malformations of the brain using a standard linear accelerator: the Lyon experience.

Radiosurgery (RS) was initiated in Lyon in October 1989. The technique was adapted from that described by Lutz and Saunders in Boston (BRW stereotactic frame). Irradiation is delivered with 18-MV photons produced by a LINAC. From December 1989 to December 1992, 41 patients with arteriovenous malformations were treated by RS; the median age was 33 years. The largest lesion diameter was 11.2-38.5 mm. Fifteen to 20 Gy were delivered on the 70% isodose line. Angiography was performed at 2 years post-treatment in 32 patients demonstrating an overall complete thrombosis rate of 81.3%. This incidence was significantly correlated with the Spetzler and Martin grade before RS (P = 0.0055). Two patients (4.9%) experienced haemorrhage after radiosurgical treatment and one died from an intracerebral-intraventricular haemorrhage. Four patients (9.7%) experienced permanent radiation-induced neurological complications.

Auditory early- and middle-latency evoked potentials in patients with quadrigeminal plate tumors.

Both early auditory evoked potentials (BAEPs) and middle-latency auditory evoked potentials (MLAEPs), were recorded in nine patients suffering from a quadrigeminal plate tumor. These recordings were performed before surgery in six cases and after surgery in three cases. The results of these examinations were correlated with impairments of the midbrain auditory pathways as shown by magnetic resonance imaging. BAEPs and MLAEPs were abnormal in five of nine cases and eight of nine cases, respectively. The two examinations yielded normal results in only one case. These data show that the functional evaluation of the midbrain should not be limited to the recording of BAEPs, routinely performed for brain stem functional evaluation, but should also include recording of MLAEPs, although the technique is a little more delicate. In the five patients with abnormal BAEPs, I-V conduction time was increased unilaterally (three patients) and bilaterally (two patients), and the I/V amplitude ratio was abnormal in two patients. In one of these two patients, isolated destruction of the right inferior colliculus was responsible for an abnormality affecting Wave V of the BAEP that was visible only after left ear stimulation. The most frequently observed MLAEP abnormality was a delay in the peaking of the Pa component, assumed to be of cortical origin. Therefore, a limited impairment of the midbrain may delay the peaking latency of Pa. Unilateral hypovoltage of Na-Pa was also observed. In only one case were Na and Pa components unilaterally abolished, in a patient suffering from a postoperative lesion extending from the right inferior colliculus to the right medial geniculate body.(ABSTRACT TRUNCATED AT 250 WORDS)

Treatment of cerebrospinal fluid rhinorrhea by percutaneous lumboperitoneal shunting: review of 15 cases.

Fifteen patients with recalcitrant cerebrospinal fluid (CSF) fistula underwent the insertion of a lumboperitoneal shunt. The shunt consists of a two-piece Silastic tube and has been used in a population of 150 patients with communicating hydrocephalus, persistent postoperative meningocele, and benign intracranial hypertension. The spinal catheter is introduced subcutaneously and no flushing device is used. We studied three groups: 9 patients had a history of head trauma, and 7 of these had undergone one or several ineffective direct approaches to the dural leak. Four patients presented with a presumably congenital fistula. Two patients had persistent rhinorrhea due to previous intracranial procedures. Indium-111 cisternography was performed in 10 patients before lumboperitonel (LP) shunting and failed in 2 of those to document the site of leakage. Twelve patients showed cessation of rhinorrhea after LP shunting. In 4 of these, shunt-related complications responded to shunt removal with no further recurrence of rhinorrhea. Two patients underwent revision of the shunt. In 3 patients, the LP shunt failed to control the CSF leak and further intracranial procedures were indicated. The LP shunt provides an attractive and technically simple solution when direct methods of treatment have failed. Additionally, LP shunting should be considered as a primary mode of treatment in elderly patients or when impairment of CSF dynamics is documented by radionuclide cisternography and computed tomographic scanning. When an LP shunt is ineffective, shunt function should be checked by isotopic studies before additional surgery is performed.

Sarcoidosis of the pineal gland. Case report.

The authors report a case of sarcoidosis of the pineal gland with no other organ manifestation of the disease. The tumor was successfully removed by a direct surgical approach.

Intra-operative embolization of cerebral arteriovenous malformations by means of isobutylcyanoacrylate (experience in 20 cases).

20 cases of cerebral arteriovenous malformations (AVM's) are reported, treated by direct intraoperative embolization. A 50% mixture of butyl-2-cyanoacrylate (IBC) and ethyl mono-iodostearate (Duroliopaque) was used. The clinical history of the patients was a long history of seizures in 10 cases, a haemorrhage in 9 cases, and a motor deficit in 1 case. In the immediate post-operative course, 1 patient died, 3 patients had a serious post-operative haemorrhage, 6 patients presented with a transient post-operative deficit, and in 10 patients the post-operative course was uneventful. During the following years, a late haemorrhage occurred after incomplete AVM eradication in 3 patients, leading to AVM resection. The AVM eradication was eventually complete in 3 cases with embolization only, and in 7 cases with embolization and AVM resection. The overall management of AVM in all 20 patients was: embolization only in 13 cases, embolization and resection in 7 cases. The long term results are: operative death 1, late death 3, long lasting deficit 1; uneventful 15. The authors discuss the technique of embolization, the effect of IBC on the vessel walls, the evolution of their general management of AVM's after this experience. Eventually the intra-operative embolization may be helpful after incomplete intra vascular embolization, to facilitate the AVM eradication.

The operation of arthrodesis for malformations of the occipito-atloid joint, according to Gilles Bertrand.

Malformations of the occipito-vertebral hinge destabilize the bones and injure the neural structures of the bulbo-spinal junction and of the cervico-spinal cord. The Authors have found the Gilles Bertrand intra-articular C/1-C/2 bilateral arthrodesis to be an extremely efficient surgical procedure for stabilizing the occipito-atlo-axial region, producing a relative lowering of the odontoid and relieving pressure in the occipito-atloid region. Excellent post-operative results derive from the relatively easy and non-destabilizing operative procedure. No deaths have been recorded in connection with this operation. This contrasts with the traditional occipito-atloid pressure-relieving procedure (either with or without opening of the dura mater), a procedure which, in addition to not stabilizing the upper cervical spine, sometimes results in the patient's death.

[Para-aortic paraganglioma found by arteriography. Apropos of a case and review of the literature]. / Paragangliome para-aortique découvert en artériographie. A propos d'un cas et revue de la littérature.

A 53 years old man had an angiography for suspected renovascular hypertension (arteritis, renal insufficiency, duplex scanning). It showed a narrow right renal artery streched by a 45 mm mass arising from the adrenal. The computed tomography showed the tumor and the nuclear magnetic resonance imaging indicated a pheochromocytoma. The patient had no complain of headaches, palpitations or sweating. Biochemistry was normal except for a slight serum creatinin elevation and a non significant urinary noradrenaline level. A diagnostic of non functioning pheochromocytoma was made. The therapeutics consisted in a surgical ablation of the tumor and the right kidney (non functioning) and the patient became normotensive thereafter without treatment. The histologic feature was an aortico-sympathetic paraganglia, the adrenal was normal. Paraganglias are arising from the paraganglion system including chemodectoma and glomus jugulare tumor. Non functioning retroperitoneal paraganglias are uncommon: less than 50 in the literature between 1902 and 1992.

[Chronic pulmonary heart disease during ventriculo-atrial shunts in children]. / Coeur pulmonaire chronique au cours des dérivations ventriculo-atriales chez l'enfant

This grave complication is a major cause of mortality in ventriculo-atrial shunts in children with hydrocephalus. It occurs at a much higher rate than with long-term indwelling intracavitary pacemakers, which suggests that the shunt procedure is responsible for either chronic infection or the introduction of cerebral thromboplastin.

[Is valproic acid teratogenic?]. / L'acide valproïque est-il tératogène?

In more than 7 000 cases of neonatal malformations processed up to 1982 in a regional computer register 71 infants had been born from epileptic mothers. A significantly higher than expected number of spina bifida aperta were observed in this group, related to the administration to the mother of the anti-epileptic agent valproic acid. A pathogenic hypothesis and a possible preventive procedure for this type of malformation are discussed.

[Non-syringomelic medullary syndrome with Arnold-Chiari malformation (author's transl)]. / Syndromes médullaires non syringomyéliques et difformité de Chiari.

The authors present 15 observations of a medullary syndrome of the non-syringomelic type, in patients with Arnold-Chiari's malformation (type I; 13 times - type II; twice), verified surgically, and without severe malformations of the occipito-vertebral articulation. Brown-Sequard's disease was present in 6 cases, subacute combined degeneration in 2 cases, and pyramidal lesions were very predominant in two other cases. The last 5 cases had cordonal signs associated with signs of disturbance of the anterior horns in the medulla, affecting all four limbs, and more especially the lower limbs. In four cases the medullary cavity was exposed, and in two cases extended along the whole length of the cord up to the terminal cone. The literature is reviewed and diagnostic and pathogenic problems raised by these myelopathies are discussed. They merit systematic investigation.

[Tethered conus syndrome or fixed spinal cord syndrome. Lumbosacral lipomas]. / Syndrome de traction du cône terminal ou syndrome de la moelle attachée. Lipomes lombo-sacrés.

Fifty-eight cases presenting a tethered conus with spina bifida occulta are analysed. Twenty-three patients had a lipoma, the others had various malformations of the cord. Twenty patients had no symptom or sign, 38 had neurological symptoms and signs. Only 2 had no skin malformation. The aim of surgery is to reduce the volume of the lipoma, untether the cord, and reconstruct the posterior protection of the cord. Twenty-six patients were cured or improved, 12 patients were stabilized, 19 remained asymptomatic, 1 was worsened.

Results of surgical treatment for cervicobrachial neuralgia. A retrospective study of 122 patients with long-term follow-up.

Surgical nerve root decompression is rarely needed in the treatment of cervicobrachial neuralgia. In patients with prolonged and resistant pain or neurological deficits, or when signs of associated spinal cord suffering have been found, various surgical procedures can be used, but is most cases the disco-arthro-radicular conflict can be solved by the anterior approach with or without bone graft. This review of long-term résults in a series of 122 patients operated upon and re-examined more than 3 years later shows that the radicular symptoms were relieved in 95% of the cases. Evaluation of anatomical results did not reveal any instability or aggravation of discarthrosis at the site of surgery. However, in 30% of the cases disc degeneration was increased in the over- and underlying levels but without recurrence of clinical symptoms; 4% of the patients in this series were reoperated upon for this aggravated or de novo disc disease. The authors underline the importance of a thorough radioanatomical examination not only to decide whether or not a "soft hernia" or an arthrotic hypertrophy should be operated, but also to evaluate the extent of the decompressive operation to be performed.

MRI in Sturge-Weber syndrome.

Five children (3 girls, 2 boys, aged from 1 to 18 years) presenting with Sturge-Weber syndrome were explored by MRI with contrast injection in 2 cases. The respective positions of CT and MRI are discussed: CT is the method of choice to display leptomeningeal calcifications. MRI seems to be more accurate than CT to determine the location of the lesions and to demonstrate the enhancement of the angioma after contrast injection.

[Percutaneous injection of methyl-metacrylate in osteoporosis and severe vertebral osteolysis (Galibert's technic)]. / Injection percutanée de méthyl-métacrylate dans le traitement de l'ostéoporose et ostéolyse vertébrale grave (technique de P. Galibert).

The authors present their preliminary experiences using methyl-metacrylate by percutaneous injection for the treatment of osteoporosis and osteolysis of the vertebral body and for vertebral angiomas. The injection of cement strengthens the vertebral body and prevents its collapse. The mixture used consist of 4.80 g of powder with 5 cm3 of monomer liquid. The technique is performed with radiological control under neuroleptanalgesia and a local anesthesia before insertion of the needle. This technique gives good results for the treatment of vertebral pain caused by osteoporosis or osteolysis.

[Monitoring cerebrospinal fluid shunts by thermography]. / Contrôle des dérivations du liquide céphalo-rachidien par la thermographie.

The authors have used a thermographic system to evaluate cerebrospinal fluid (CSF) shunt patency. Thermographic detection of the catheter rests on the gradient that exists between the temperature of the CSF-containing tube and that of the nearby skin. The equipment consists of an infra-red sensitive camera connected to a television screen. No cooling or warming of the reservoir was performed. The entire length of the superficial part of the shunt could be explored. The present study concerns 36 thermographic examinations in 31 patients. The CSF-containing catheter was usually warmer than the skin, but in some circumstances its upper part appeared to be cooler than the skin of the head and its lower part warmer than the skin of the neck. The thermographic results are in agreement with the clinical findings and the course of the disease. The method, which is harmless, simple and easily reproducible, seems to be useful for the determination of CSF shunt patency.

[Lumbo-peritoneal CSF shunt (author's transl)]. / La dérivation lombo-péritonéale.

In a preliminary technical report, the authors describe a procedure for lumbo-peritoneal shunt consisting of the percutaneous introduction of a sub-arachnoid proximal catheter. There would appear to be many advantages in the treatment of communicating hydrocephalus when compared with classical shunt methods taking the CSF from the ventricle.