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Dysembryoplastic neuroepithelial tumour (DNT), a non-evolutive intracranial cortical lesion, is generally associated with epileptic seizures principally among youths. A case of a DNT which presented with uncommon clinical features, characterized by severe intracranial hypertension and progressive blindness warranting emergency surgery, is documented. In addition to the classical radiological and neuropathological features characteristic of DNTs there was a large haemorrhagic cystic haematoma as a result of repeated dissections and/or ruptures of the abnormal vessels in areas, explaining some of the atypical clinical symptoms. Therefore the need for a regular, careful clinical and radiological follow-up of cases with cystic DNTs is strongly recommended.

Children aged 0-15 years hospitalized in Geneva for head trauma during the last quarter of a century are reviewed. More than half of the severely injured children were not from Geneva area. New methods of management have been introduced progressively. The cases are divided in four successive time periods and classified according to their pathology. A continuous improvement in mortality is obvious, decreasing from 29.4% to 2.2%, but climbing again to 15.2% in the last period, probably due to more severe pathologies. For the Geneva area the mortality decreased progressively from 10.4/100,000 to 3.5/100,000 annually, due to better organization and management, but also to a drop in the incidence of severe cases from 35.5-13.5/100,000 per year. A decrease in the number of traffic accidents is responsible for this. However, the number of handicapped children has not changed.

Plexiform neurofibromas of the orbit, sometimes extending to the temporal region and the face, are considered to be a rare but devastating and disfiguring complication of neurofibromatosis type 1. The first symptoms appear in infancy and the involvement of the orbit and the face is present in nearly all children after the age of 5. The disease is unilateral in most cases but can exceptionally involve both sides of the face. Progressive deformation of the orbital frame due to the expanding plexiform neurofibroma and buphthalmos occurs in a large proportion of cases. The associated sphenoidal dysplasia, which is thought to be, according to the most recent hypothesis, genetically determined, will inescapably increase the burden to the orbital content, cause pulsating proptosis and will endanger noble structures, finally resulting in loss of vision. Using the Jackson classification, the authors report their personal series of 22 cases (19 operated). Until now, there has been no effective medical treatment for plexiform neurofibroma and surgery remains the standard care for these patients. Controversies remain about the timing of the first operation and today most multidisciplinary teams involving plastic, maxillofacial, ophthalmologic, and neurosurgeons favor early intervention to try to minimize the secondary deformation of the orbital and facial skeleton. A number of cases of plexiform neurofibromas are illustrated within the three Jackson groups and treatment results of the rare elephantiasis neuromatosa cases are presented. Special techniques such as preoperative embolization of heavily vascularized plexiform neurofibroma are also discussed.

Phacomatoses, or neurocutaneous disorders, are a group of congenital and hereditary diseases characterized by developmental lesions of the neuroectoderm, leading to pathologies affecting the skin and the central nervous system. There is a wide range of pathologies affecting individuals at different moments of life. The genetics is variable: while neurofibromatosis 1 and 2, tuberous sclerosis and von Hippel-Lindau disease are all inherited as autosomal dominant traits, Sturge-Weber syndrome is sporadic. Other neurocutaneous disorders can be inherited as autosomal recessive traits (i.e., ataxia-telangiectasia), X-linked (i.e., incontinentia pigmenti) or explained by mosaicism (i.e., hypomelanosis of Ito, McCune-Albright syndrome). In this review, we discuss the major types of neurocutaneous disorders most frequently encountered by the neurosurgeon and followed beyond childhood. They include neurofibromatosis types 1 and 2, tuberous sclerosis, Sturge-Weber syndrome and von Hippel-Lindau disease. In each case, a review of the literature, including diagnosis, genetics and treatment will be presented. The lifespan of the disease with the implications for neurosurgeons will be emphasized. A review of cases, including both pediatric and adult patients, seen in neurosurgical practices in the Lille, France and Lausanne, Switzerland hospitals between 1961 and 2007 is presented to illustrate the pathologies seen in different age-groups. Because the genes mutated in most phacomatoses are involved in development and are activated following a timed schedule, the phenotype of these diseases evolves with age. The implication of the neurosurgeon varies depending on the patient's age and pathology. While neurosurgeons tend to see pediatric patients affected with neurofibromatosis type 1, tuberous sclerosis and Sturge-Weber syndrome, there will be a majority of adult patients with von Hippel-Lindau disease or neurofibromatosis type 2.

Failure to thrive is frequently seen in breastfed infants. The most common diagnosis is insufficiency of breast milk in an otherwise healthy child. However, several differential diagnoses need to be considered. Progressive feeding difficulties and failure to thrive can be the first manifestation of group B streptococcal ventriculitis. This rare disease does not present with acute symptoms of sepsis or meningitis but evolves insidiously with no fever. Diagnosis is therefore often delayed and made only when intracranial hypertension develops. Cerebrospinal fluid (CSF) culture confirming the group B streptococcal infection and cerebral imaging are the necessary investigations for diagnosis. To our knowledge, only 10 cases have been previously reported.

In 1992, the American academy of paediatrics has recommended that infants be placed on their backs to sleep, because prone sleeping has been correlated with sudden infant death syndrome. Following this article, medical paediatric community has documented an exponential increase in the diagnosis of posterior cranial deformities, which were considered as the consequence of unrelieved pressure onto the occiput during infant sleep. These last 15 years, management of posterior positional plagiocephaly has evolved but is still not standardized; it varies according to local specificities, and medical or parental preferences. Treatment of deformational plagiocephaly includes preventive counseling, repositioning adjustments and exercises, physiotherapy, osteopathy, treatment by dynamic cranial orthosis. On extremely rare occasions, corrective surgery is proposed. This article aims at reviewing the epidemiologic, diagnostic, and various therapeutic options of posterior positional plagiocephaly.

Since the recommendation that infants sleep in the supine position, there has been an increase in cases of posterior positional plagiocephaly. Even though this condition is a purely cosmetic problem, if it is severe it may affect the child psychologically. Positioning may help in mild or moderate cases, but more active treatment may be necessary in severe cases.A prospective study of 260 children treated by dynamic orthotic cranioplasty for posterior positional plagiocephaly was conducted in Lausanne from 1995 to 2001. Construction of these cranial remodelling helmets is decribed in detail.The treatment lasted 3 months on average, was effective, well tolerated, and had zero morbidity. The ideal period for initiating this therapy is between the ages of 4 and 6 months.The remodelling helmet is a convincing option which can be recommended in infants with posterior positional plagiocephaly whose skull deformity is not satisfactorily corrected by physiotherapy. It should always be used before surgery is considered for patients with recognised positional plagiocephaly in the first year of life.

Occipital condyle fractures (OCF) are seldom described in children, with only 14 cases reported in the literature. We report the observation of a 13-year-old child in whom such a fracture was diagnosed. A review of the paediatric cases allowed us to identify the clinical features of OCF in children. The average age was found to be 10 years, and the causative traumatism was most often related to road traffic or sporting accidents with associated head injury. The level of consciousness was depressed in 67% of the cases, and in 53%, an injury to the cranial nerves, brain stem or high spinal cord was present. Neck pain with reduction of head mobility was reported in 27% of the cases. Treatment was generally conservative, i.e. a hard collar or halo vest for an average of 8 weeks. The course was favourable with respect to osseous consolidation, mobility and pain. On the other hand, the neurological deficits improved, but sequelae remained. Early diagnosis and treatment is mandatory since the secondary displacement of fractured fragments can injure the cranial nerves in a delayed fashion or even be fatal due to compression of the brain stem.

We report the case of a 61-year-old woman, who developed progressive paraparesia over a period of 8 months. Conventional X-rays of the thoracic spine showed an intra-spinal calcified lesion at T10. On CT-scan and MRI, the lesion appeared anterior to the cord, thus making a posterior approach hazardous. Total resection of this calcified meningioma was achieved through a right transthoracic transcorporeal approach, under close monitoring of the somatosensory evoked potentials. Despite a delayed pseudomeningocele formation requiring an additional thoracotomy, outcome after 7 years is excellent with no residual neurological deficit. No recurrence was seen on a CT-scan performed two years after the surgery. Calcified anterior meningiomas of the spine are rare lesions. Surgical outcome has been unfavorable for a long time in relation with posterior or postero-lateral approaches. Although anterior transthoracic procedures are routinely performed for extradural spinal lesions, this approach is rarely used for intradural lesions. A calcified anterior spinal thoracic meningioma should be managed like the more frequent calcified thoracic disk hernia, despite the increased risk of cerebrospinal fluid effusion requiring subsequent repair.

A retrospective study of 75 patients operated for pituitary prolactin-secreting adenomas between 1972 and 1992 is presented. 57 were women, 18 males. The major symptom was amenorrhea for women and impotence for men. Prolactinemia is correlated to the size of adenoma and thus permits a prediction of surgical results. Most of the patients with a prolactinemia under 300 ng/ml were cured by surgery alone. Surgical treatment alone at the-term follow-up cure 87% of the micro-adenomas, 17% of the enclosed adenomas, and none of the invasive adenomas. In this study there is only 7% of true recurrence. According to the high cure rate and low frequency recurrence after transphenoidal surgery for micro-adenomas we suggest this approach as the first choice treatment. On the other hand the best treatment for macro-prolactinomas is medicamentous.

In the treatment of frontoethmoidal cephaloceles (FEC), three aims should be pursued: (1) to provide good exposure for a safe neurosurgical correction, (2) to obtain the best cosmetic result with minimal scarring, and (3) to inflict on the child the least surgical trauma. Between 1986 and 1991, 6 children were operated on in our unit for FEC. In this study, we compare the advantages of the transcranial surgical approach versus the transfacial surgical approach. Because most of the facial anomalies presented by these patients are represented by modifications in the position and shape of the medial orbital walls, as well as elongation of the nose, we feel more comfortable using the transcranial approach. It permits safer translocation of the medial orbital walls and recreation of the dorsum of the nose with a calvarial bone graft, and provides wide access for neurosurgical correction. In our experience, the most difficult part of the operation remains correction of the length of the nose. The transfacial approach, with planned skin resection, may thus represent an advantage in these cases. Therefore, our purpose is not to oppose these two surgical procedures, but often to combine them for safer treatment and better cosmetic results.

Published cases of cervical dysraphic lesions are rare. Their estimated proportion is about 5% of the entire group of spinal dysraphism. Four cases are reported, representing 2 types of lesion: 2 meningoceles with a simple fibrous band fixing the posterior aspect of the cord to the skin and 2 meningomyelocystoceles with other associated anomalies (hydromyelia, hydrocephalus, Chiari II, Peter's ocular anomaly). The clinical presentation is that of a soft posterior cervical mass without marked neurological impairement. The surgical treatment is simple, consisting in the resection of the lesion, intradural exploration to untighten the cord if fixed by a fibrous band, and drainage of the hydrocephalus. Neurological outcome is generally good immediately but may need sometimes secondary surgical exploration.

Whether hemifacial spasm (HFS) is due to axono-axonal ephaptic transmission or to facial nucleus abnormal hyperexcitability remains controversial. The neurophysiological hallmark of HFS is the delayed response (DR). This response has an indirect pathway and thus a long latency. It is evoked A) as a distant response in muscles innervated by a facial branch other than the one stimulated, and B) in muscles innervated by the branch stimulated. In this work, 99 single all-or-none DRs of 24 cases of cryptogenic HFS were studied by threshold stimulation of a branch, or of the trunk, of the facial nerve. A) Eighty-eight distant DRs were studied. Fifty-four of them were frequently evoked as double discharges (DDs), or sometimes as multiple discharges, with a 3 to 7 ms interval. A collision technique, using paired stimuli, showed that the second discharge of 12 out of 20 DDs was accompanied by a back-wave due to a proximal ectopic re-excitation on the axon, or to the back-firing of an alpha cell (F-response), or to both. F-waves in HFS were more frequent than in normals or in other facial pathologies. B) Eleven DRs were recorded in muscles innervated by the facial branch stimulated. Some of them persisted when, using a stronger stimulus, the same all-or-none potential was also evoked as a direct response. In other cases both the direct response and the DR were evoked with identical stimulation threshold. The direct-indirect response interval of these 11 DRs was shorter than the normal M-F interval. These findings suggest that, in the case of HFS, axons are interconnected by uni- and bidirectional ephapses. Self-sustained repetitive firing in such a group of axons apparently results from re-excitations occurring both at the ephapse site and at the cellular level (F-responses). Spasm develops when several groups fire together. The changing excitability of the alpha cells modulates the importance of the phenomenon.

The authors reports their experience of 5 cases of intra- and peri-aqueductal tumors. With the exception of 1 case, a subependymoma of the aqueduct discovered at autopsy in a 77 year old woman, the 4 other patients (2 females and 2 males) are relatively young (respectively 14, 23, 14 and 26) at the time when they presented with signs of chronically raised intracranial pressure due to a triventricular hydrocephalus. One patient presented with a partial Parinaud's sign, but the focal and long tract signs were conspicuously absent in the other patients. The diagnosis of a space occupying lesion in the tectum mesencephali or in the aqueduct could only be ascertained with the recent use of M.R.I. The radiological work-up of the cases suggest that theses lesions, presumably of glial origin, are very slowly growing tumors. Four patients have been treated for their symptomatic hydrocephalus, but no treatment of the tumor has been proposed, as the clinical state and the images remain remarkably stable. (Mean duration of follow up from the time of shunting = 3.7 years, extremes = 1.5-8 years). A review of the literature shows that not more than 48 cases of tumors of the aqueduct have been reported. Because of the small number of such observations, there are, until now, no precise informations on the management of such cases to decide if the patient will benefit of an open or stereotactic biopsy associated with radiotherapy or if one can rely on serial clinical and radiological examinations. Further information on the long term follow up is needed.

A case of dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is reported, discovered on a routine autopsy. Clinical and anatomopathological data of the case are compared with previously reported cases. Review of the various proposed pathogenic hypotheses.

An internuclear ophthalmoplegia was diagnosed and recorded electro-oculographically in a patient with pontomedullary listeriosis. The etiological differential diagnosis in case of internuclear ophthalmoplegia is discussed. It is emphasized that listeria monocytogenes encephalitis should be considered because this pathology may be electively located in the pontomedullary area and because of the encreasing frequency of human listeriosis.

Four patients with rapidly progressive tetraparesis in relation with skin or joint infection and septicemia are reviewed. Clinical signs of medullary compression was present in all cases, confirmed by neuroradiological examinations. However, all surgical approaches failed to demonstrate clear evidence of compression. Within three weeks, the neurological picture of severe tetraparesis had an excellent clinical evolution in all cases. One of the patients died after developing pulmonary complications: necropsy did not show any signs of compression. The difficulty of differential diagnosis between an infectious compressive surgical pathology and an inflammatory disease (acute transverse myelitis type) is emphasized, with review of literature.

Between 1978 and 1984, the University Hospital of Geneva (Hôpital Cantonal Universitaire) received 46 head injured patients who "talked and died" after their brain insult. Only 14 of them had associated systemic lesions whilst two thirds of the series had an isolated head injury. Detailed review of the case records shows that death was preventable in a quarter of the cases. For 28 patients (60%), the factors contributing to death are avoidable or, at least, their management should be greatly improved. The diagnosis was confirmed by autopsy in 31 patients (67%).

Closed injury with lesions of the great vessels of the neck are rare and usually due to high energy mechanisms with severe secondary deficit. 2 cases are reported, concerning young patients with relatively mild injury, neurological deficit not developing before 6 months after the traumatism in the first case and 8 months in the second. At this stage, the neurological signs were immediately severe, with cerebral infarction, leading to death in the first case. Pathological lesion of lesion of pseudo-anevrismal type, localised at the right carotid and subclavian artery bifurcation, was evidenced in the first case. In the second case, a bilateral intimal lesion of the carotid arteries at C1 level is illustrated on angiography. The mechanism of these lesions as well as the delayed manifestation of neurological deficits are discussed.