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A new family is reported of a Bickers-Adams-Edwards syndrome. This family has been studied up to three generations. Two female carriers are known. Among the six male children who are affected, four are severely mentally retarded, have spasticity of the legs, and survived with a mild macrocephaly, and two show a more severe and rapid progression of head enlargement. A partial aqueductal stenosis, with remarkable ventricular dilatation, has been demonstrated by pneumoencephalography in three boys. A deformity of the thumbs links these six children together. One of them has been treated by a ventriculoperitoneal shunt, when 18 months old, without any improvement in the neurological condition. The mental deficiency is much more severe than could be expected from the degree of hydrocephalus, at least as estimated clinically by the macrocephaly. Hydrocephalus is precocious, and the ventricular dilatation very advanced when seen by PEG studies. Recognition of the female carriers is not possible.

The authors describe a patient with ischaemic neurological incidents due to bilateral hypoplasia of the carotids. Because the use of the superficial temporal artery or the occipital artery was impossible, the extra-intracranial anastomosis was performed by a direct venous graft between the external carotid artery and the posterior temporal artery. The authors describe and illustrate the operation, emphasizing the particular difficulties due to differences in diameter of the arteries and the graft. Because postoperative control angiography demonstrated perfect permeability of the anastomosis, this technique is suggested for when a normal branch of sufficient diameter of the external carotid artery cannot be used.

Les auteurs presentent un cas de meningiome olfactif absolument typique par sa semiologie et son mode evolutif. L'arteriographie pre-operatoire a fait decouvrir un anevrysme latent de la communicante anterieure. Cette association doit etre consideree comme fortuite, alors que la coexistence de malformations vasculaires cerebrales et de certaines varietes de tumeurs plaide en faveur de l'origine dysembryoplasique de celles-ci. L'intervention a permis l'ablation du meningiome et l'exclusion de l'anevrysme, ce temps vasculaire ayant ete facilite par une voie d'abord particulierement elargie. Die Autoren berichten uber einen Fall eines absolut typischen Olfaktoriusmeningeoms hinsichtlich der Vorgeschichte und der Entwicklung. Die praoperative Angiographie zeigte unerwartet ein Aneurysma der Art. communicans anterior. Dieses Zusammentreffen ist beachtenswert, da zerebrale vaskulare Misbildungen und gewisse Tumorarten dysontogenetischen Ursprungs sein konnen. Der Eingriff erlaubte die Entfernung des Meningeoms und den Verschlus des Aneurysma. Dieser vaskulare Teil des Eingriffs war besonders einfach durch den vorher geschaffenen grosen Zugangsweg. The authors present a case of olfactory grove meningioma which was absolutely typical in its history and pattern of development. The preoperative angiogram revealed an unsuspected aneurysm of the anterior communicating artery. This association ought to be considered as fortuitous, while the coexistence of cerebral vascular malformation and of certain types of tumour argues in favour of a dysplasic origin of these. The operation allowed removal of the meningioma and cure of the aneurysm, this vascular part of the operation having been made particularly easy by the very roomy approach which had been produced. Los autores presentan un caso de meningioma del olfatorio absolutamente tipico por su semiologia y su forma de evolucion. La arteriografia preoperatoria permitio descubrir un aneurisma latente de la comunicante anterior. Esta asociacion debe considerarse como fortuita, ya que la coexistencia de malformaciones vasculares cerebrales y de ciertas variedades de tumores, hablan a favor de un origen disembrioplastico de las mismas. La intervencion permitio la ablacion del meningioma y la exlusion del aneurisma. Esto ultimo se facilito mediante una via de penetracion particularmente amplia.

Report of a case of so called "benign" intracranial hypertension whose main characteristics are: the etiology attributed to a thrombosis of the venous transverse sinus in the course of a polycythemia; the clinical signs with a reinforcement during the course of the illness of a previous epilepsy; the prognosis with specially heavy visual impairment questioning the prefix "benign" applied to this condition.

We present a multicentric randomized double-blind study of the curative effect of intravenous Nimodipine on the post hemorrhagic vasospasm after aneurysmal rupture. On 122 validated cases out of 188 analyzed patients, the study demonstrates a significative effect on the mortality and severe morbidity related to vasospasm: the reduction of the risk is appreciated to 72%. The major effect is obtained on the mortality risk. A selective effect can be demonstrate on the isolated vasospasm.

Stereotaxic angiography offers many advantages over conventional techniques for the diagnosis of cerebral tumours: three dimensional analysis of the vascular pedicles supplying thetumours; precise identification of the structures with which certain cortical vascular loops are connected; early detection of small pathologic displacements; pre-planning of a precise surgical procedure, and finally a radiographic control of each intervention under the same circumstances.

The authors report six cases of tumours limited to the cervical inter-vertebral foramen. The patients presented with cervico-brachial neuralgias occasionally accompanied by radicular sensitivo-motor impairment. Radiographs and tomographs of the cervical spine demonstrated enlargement of the intervertebral foramen with bone proliferation depending on the etiology and provided in easy diagnosis in all the cases. A myelogram showed the absence of extension into the spinal canal thus allowing distinction from the group of dumbell tumors. In the six cases vertebral angiography gave information regarding the vascularisation and relationships of the tumour. The histological diagnosis was different in each case : neurofibroma, chordoma, osteochondroma, osteoma, osteoblastoma and an aneurysmal bone cyst. The same surgical approach was used in the six cases. Neither a laminectomy with facettectomy, nor a classical antero-lateral approach allow for a satisfactory exposition of the tumor. On the contrary a lateral interscalenic approach including dissection of the brachial plexus lead to a trouble free excision. This approach was precised by anatomical dissections.

Intra-carotid administration of antineoplastic agents (ACNU, BCNU and CISPLATIN) is being explored as an alternative route of drug administration for the treatment of malignant gliomas, in several centres [3, 4, 14–19].

After a critical analysis of the criteria usually used for arguing in favor of the hydrodynamic determinism of the syringohydromyelic malformations, the authors report convincing data according to which such dysmorphic status would be the result of abnormalities in the genetic programs of the morphogenesis of the central canal.

The authors report seven cases of cerebral aneurysms complicating bacterial endocarditis. The evolution was good in three cases, two of which without sequelae. The bacteries involved were staphylococcus, streptococcus, and enterobacter. The cardiopathy was mitral in four cases. Two patients presented an isolated focal neurological impairment, while the other five presented a severe coma either isolated (one case) or associated with focal neurological deficits (four cases). An intracerebral hematoma was diagnosed in five cases (four died). Only one patient was not operated. The surgical treatment was as follows: one carotid ligation (good result), two evacuations of intracerebral hematomas (both died), two evacuations of intracerebral hematomas with clipping of the aneurysms (one died, one had a fair result in despite of residual hemiplegia), one clipping of aneurysm (good result). Repeated cerebral angiographies should be systematically performed in cases of bacterial endocarditis in order to disclose and follow up cerebral aneurysms which may occur in this condition. Except in cases of emergency, surgery should be differed, in particular, in cases of proximal or multiple aneurysms. Aneurysms of the peripheral cortical arteries are more accessible to treatment and may be operated in the acute phase.

The natural history of malignant gliomas is compared to the mean survival duration after surgery alone and/or surgery associated with radiotherapy and chemotherapy. In the state of the art a combined treatment by surgery and roentgentherapy offers the best prognosis but does not allow for more than 35% of expectation of life at the end of the first year. According to the severity of the general prognosis the present authors advocate a strict care for the functional integrity. An improved knowledge in the biology of cerebral gliomas justifies further consideration in the surgical treatment preparing the expected effects of radiotherapy and chemotherapy.

Five cases of brain-stem hematoma are described. The cause of these hematomas was identified as "cryptic angioma" (1 cavernous angioma, 1 telangiectasia, 3 arteriovenous malformations). So, they are so-called "secondary hematoma", as opposed to brain-stem hematoma in relation with hypertension. Such secondary hematomas are reported in the literature: 37 operated on cases and 22 untreated cases were found. The clinical picture does not seem to be typical. The presentation appears to be either with the acute onset of a stroke, or with a subacute onset including relapsing symptoms. A progressive deterioration suggesting a pontine glioma or mimicking demyelination is not rare. The CT scanner appearance is often characteristic showing a high density area in the brain-stem which enhanced after injection of contrast medium with an aspect of "halo". Angiography is usually negative. The natural history of brain-stem hematoma due to rupture of a cryptic angioma is not well documented, but it seems that prognosis is very poor. So, the authors insist on surgical evacuation which is effective and safe allowing the diagnosis of brain-stem hematoma and in some cases the identification of the malformation.

The pathogenesis of syringomyelia is still under discussion. The authors advocate the role of the pressure of the spinal C.S.F. whose flow can be directed from the perimedullary space into the cavity. This force is responsible for an hydrodynamic dissection of the cord, even in cases of foraminal syringomyelia. Therefore it is not efficient to draw the fluid from the syrinx towards the subarachnoïd space. The authors suggest a syringoperitoneal shunt. The present report is only a technical note, according to the poor number of cases operated by this way (4 patients) and to a too-short follow-up (2 years for the first case).

The authors report a new case of carotid body tumour operated on, in a 26 year old woman. Recalling the main clinical and histological characteristics of these tumours, they emphasize the importance of pre-operative assessment and the decisive value of arteriography. Modern techniques of surgery of the carotid bodies permit one to face the risk of damage during removal. They re-emphasize the necessity of surgical treatment able to ensure a cure. Finally, emphasizeing the special gravity of advanced tumours (group III), they condemn a conservative attitude and recommended operation as early as possible.

The respective role of surgical ablation versus palliative methods (i.e. shunting followed by irradiation) in the treatment of tumors of pineal region is still questionnable and must take into account the variability of their pathology. Thus, knowledge of tumoral type in each case is the first step to therapeutic discussion. The stereotactic methods allow a faithful biopsy, easy to interpret and without major risks.

The authors emphasize the value of extra-intracranial anastomosis prior to occlusion of the cervical segment of the main cerebral arteries, and report a case of carotid-cavernous sinus fistula successfully treated by combined extra-intracranial anastomosis and intra-luminal occlusion of the carotid artery with a balloon catheter.

During the 1975 Oxford Congress, we proposed the use of stereotactic neuroradiology for the evaluation of pineal tumours and from then, we adopted the following strategy: Stereotactic biopsy in all cases, except for highly vascularized tumours. Radiotherapy (external or interstitial) for radiosensitive tumours as germinomas, gliomas, pineo blastomas... Direct surgical approach for radioresistant tumours as teratomas, pineocytomas, meningiomas... Chemotherapy was associated in certain definite histological types (embryonal carcinoma).

An experimental study about 36 nervous grafts on laboratory rats is reported. This study emphasizes the interest of microsurgical technique and, further more, the role of the number of stitches improving the results of each anastomosis and, thus, the quality of neural regeneration. In each animal, after sectioning tibial nerve, a graft (sciatic nerve same side) is interposed and sutured in three different ways; in a first lot of animals 6 stitches at the level of each anastomosis; in a 2nd lot: 2 stitches and in a 3d one a single stitch. The animals are tested at the end of the 20th week, with an electrophysiological control before being sacrified. The pathological controls take in account the number of regenerated nervous fibers below the graft. Our study confirm the superiority of results in the 6 stitches lot. The authors emphasize the role played by a watertight suture to prevent an anarchic growth of regenerating axones outside the graft.

The treatment of tumours of the pineal region is the subject of keen controversy between advocates of surgical excision and those of radiotherapy with ventricular shunting. The proponents of these extremes often seem to ignore the wide pathological variety of tumours of this site. Attempted excision of a germinoma invading the walls of the 3rd ventricle is as futile and dangerous as irradiation of a teratoma. Stereotaxic biopsy provides precise pathological diagnosis and thus allows more specific management; the procedure is safe and no serious complications were experienced in 25 cases.

Since Winslow identified it in 1732, the cavernous sinus has given rise to numerous discussions about its description, its embryology, its dynamic, and even its own designation: cavernous sinus, cavernous area, parasellar region…

The authors report the pathological and clinical findings in a case of histiocytosis X in a child. A rapidly progressive neurological syndrome of brain stem damage, occurred 5 years after the onset. Blockage of the 3rd ventricle led to surgical exploration of the posterior fossa. Lesions of the brain stem and cerebellum, in addition to the granuloma of the hypothalamus, are described. The neurological symptoms in histiocytosis X often suggest a space-occupying lesion but usually occur in the later prolonged forms in the adult. Association of thesaurismosis due to P.V.P., of therapeutic origin, produced numerous clinical skin and joint signs.

A new family is reported of a Bickers-Adams-Edwards syndrome. This family has been studied up to three generations. Two female carriers are known. Among the six male children who are affected, four are severely mentally retarded, have spasticity of the legs, and survived with a mild macrocephaly, and two show a more severe and rapid progression of head enlargement. A partial aqueductal stenosis, with remarkable ventricular dilatation, has been demonstrated by pneumoence-phalography in three boys. A deformity of the thumbs links these six children together. One of them has been treated by a ventriculoperitoneal shunt, when 18 months old, without any improvement in the neurological condition. The mental deficiency is much more severe than could be expected from the degree of hydrocephalus, at least as estimated clinically by the macrocephaly. Hydrocephalus is precocious, and the ventricular dilatation very advanced when seen by PEG studies. Recognition of the female carriers is not possible.

The book's contents are as follows: Part I: Oncogenesis. Part II: Neuropathology. Part III: Tumoral Immunobiology and Oncobiology. Part IV: Biological and Diagnostic Imaging. Part V: Clinico-Pathological Studies. Part VI: Neurosurgical Procedures and Radiotherapy Trends. Part VII: Chemotherapy and Immunotherapy.

This young man was operated on twice for thoracic spinal cord compression. He had been on corticosteroid therapy for the last 2 years subsequent to a renal transplant. The only anomaly discovered during the operation was a large quantity of extradural fat that did not present the characteristics of lipoma. The remarkable postoperative clinical improvement suggested that the fat deposit was responsible for the spinal cord compression.