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Introduction: When a child walks in the clinic with a unilateral frontal flattening, it is usually associated in our minds with unilateral coronal synostosis. While the latter might be the most common cause of anterior plagiocephaly, it is not the only one. A patent coronal suture will force us to consider other etiologies, such as deformational plagiocephaly, or synostosis of another suture. To understand the mechanisms underlying this malformation, the development and growth of the skull base must be considered., Materials and Methods: There have been few reports in the literature of isolated frontosphenoidal suture fusion, and we would like to report a series of five cases, as the recognition of this entity is important for its treatment., Conclusion: Frontosphenoidal synostosis must be searched in the absence of a coronal synostosis in a child with anterior unilateral plagiocephaly, and treated surgically.

The PTEN hamartoma tumor syndrome, manifestations of which include Cowden disease and Bannayan-Riley-Ruvalcaba syndrome, is caused by various mutations of the PTEN gene located at 10q23. Its major criteria are macrocephaly and a propensity to develop breast and thyroid cancers as well as endometrial carcinoma. Minor diagnostic criteria include hamartomatous intestinal polyps, lipomas, fibrocystic disease of the breasts, and fibromas. Mutations of PTEN can also be found in patients with Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum). The authors report the case of a 17-year-old girl who had a severe cyanotic cardiac malformation for which surgery was not advised and a heterozygous missense mutation (c.406T>C) in exon 5 of PTEN resulting in the substitution of cysteine for arginine (p.Cysl36Arg) in the protein, which was also found in her mother and sister. The patient presented in the pediatric emergency department with severe spastic paraparesis. A magnetic resonance imaging study of the spine showed vertebral hemangiomas at multiple levels, but stenosis and compression were maximal at level T5-6. An emergency T5-6 laminectomy was performed. The decompression was extremely hemorrhagic because the rapid onset of paraparesis necessitated prompt treatment, and there was no time to perform preoperative embolization. The patient's postoperative course was uneventful with gradual recovery. This represents the first report of an association of a PTEN mutation and multiple vertebral angiomas. The authors did not treat the remaining angiomas because surgical treatment was contraindicated without previous embolization, which in itself would present considerable risk in this patient with congenital cyanotic heart disease.

Complex central nervous system (CNS) malformations frequently coexist with other developmental abnormalities, but whether the associated defects share a common genetic basis is often unclear. We describe five individuals who share phenotypically related CNS malformations and in some cases urinary tract defects, and also haploinsufficiency for the NFIA transcription factor gene due to chromosomal translocation or deletion. Two individuals have balanced translocations that disrupt NFIA. A third individual and two half-siblings in an unrelated family have interstitial microdeletions that include NFIA. All five individuals exhibit similar CNS malformations consisting of a thin, hypoplastic, or absent corpus callosum, and hydrocephalus or ventriculomegaly. The majority of these individuals also exhibit Chiari type I malformation, tethered spinal cord, and urinary tract defects that include vesicoureteral reflux. Other genes are also broken or deleted in all five individuals, and may contribute to the phenotype. However, the only common genetic defect is NFIA haploinsufficiency. In addition, previous analyses of Nfia(-/-) knockout mice indicate that Nfia deficiency also results in hydrocephalus and agenesis of the corpus callosum. Further investigation of the mouse Nfia(+/-) and Nfia(-/-) phenotypes now reveals that, at reduced penetrance, Nfia is also required in a dosage-sensitive manner for ureteral and renal development. Nfia is expressed in the developing ureter and metanephric mesenchyme, and Nfia(+/-) and Nfia(-/-) mice exhibit abnormalities of the ureteropelvic and ureterovesical junctions, as well as bifid and megaureter. Collectively, the mouse Nfia mutant phenotype and the common features among these five human cases indicate that NFIA haploinsufficiency contributes to a novel human CNS malformation syndrome that can also include ureteral and renal defects., Competing Interests: Competing interests. The authors have declared that no competing interests exist.

Introduction: Few series compare endoscopic third ventriculostomies (ETV) and ventriculoperitoneal shunts (VPS). To avoid the complications after a shunt insertion, there is an increased tendency to perform a third ventriculostomy. We reviewed all pediatric patients operated in the French-speaking part of Switzerland for a newly diagnosed obstructive hydrocephalus since 1992 and compared the outcome of patients who benefited from ETV to the outcome of patients who benefited from VPS. There were 24 ETV and 31 VPS., Discussion: At 5 years of follow-up, the failure rate of ETV was 26%, as compared to 42% for the VPS group. This trend is also found in the pediatric series published since 1990 (27 peer-reviewed articles analyzed)., Conclusion: In accordance to this trend, although a statistical difference cannot be assessed, we believe that ETV should be the procedure of choice in pediatric obstructive hydrocephalus.

We report on an 11-year-old female with a history of cervicobrachialgia and progressive weakness of the right arm. Cervical spine MRI showed an enhancing heterogeneous intradural mass occupying the right C6-C7 foramen. She underwent a right C6-C7 foraminotomy with a complete macroscopic removal of the lesion. Pathological examination revealed a synovial sarcoma. Treatment was completed by chemotherapy and proton radiotherapy, and the girl remained free of symptoms for 3 years. After appearance of new symptoms, a local recurrence was confirmed, and despite aggressive treatment with salvage chemotherapy and radiotherapy, the disease progressed beyond medical control, and the child died, 6 years after diagnosis. Early recognition of this rare entity compared to its more benign differential diagnosis is crucial, as an aggressive management is needed., ((c) 2007 S. Karger AG, Basel.)

Introduction: Growing skull fractures are a rare complication of head injuries (Ersahin et al. in Neurosurg Rev 23:139-144, 2000; Hayashi et al. in Childs Nerv Syst 13:349-351, 1997; Ramamurthi and Kalyanaraman in Neurosurgery 32:427-430, 1970; Zegers et al. in Eur J Pediatr 162:556-557, 2003). Although early diagnosis and prompt treatment are important to prevent the underlying progressive brain damage, the clinical presentation and the morphological investigations are rarely specific or sensitive shortly after the trauma., Discussion: The authors present three cases of growing skull fractures: the use of ultrasonography (US) via the fracture line contributed to early diagnosis and prompt treatment in two cases. US was not performed in the third case, and this delayed management. Treatment consisted of a watertight duraplasty with a free flap of pericranium without cranioplasty. US via the fracture line appears to be a sensitive and reliable method of detecting the dural tears in the early stages of growing skull fractures., Conclusion: Duraplasty alone with a flap of pericranium remains the simplest and least expensive method of treatment. Cranioplasty is not necessary in young children.

Introduction: Meningeal solitary fibrous tumour is a relatively recent pathological entity that has rarely been described in children. With radiological techniques, it cannot be distinguished from meningiomas, and the diagnosis has to be confirmed histologically., Case Report: We discuss the possible histogenesis of this tumour and the need for recognizing this lesion as a separate entity. We report the case of a 12-year-old boy who developed a meningeal solitary fibrous tumour; the main clinical symptoms were progressive headaches for a long period and recent transient hemiparesis., Conclusion: This child presents an uneventful evolution without additional therapy 3.5 years after surgery.

Object: The authors conducted a study to evaluate and compare prospectively the implantation of either an empty carbon fiber composite frame cage (CFCFC) or an iliac crest autograft after anterior cervical discectomy (ACD) for cervical disc herniation with monoradiculopathy., Methods: Thirty-six consecutive patients with one-level radiculopathy due to single-level cervical disc herniation were treated by ACD, and implantation of either an empty CFCFC (24 patients) or an iliac crest autograft (12 patients). Radiological and clinical assessments were performed preoperatively, immediately postoperatively, and at 3, 6, and 12 months postoperatively. Fusion at the 12-month follow-up examination was demonstrated in 96% of the patients in the cage group and in 100% of those in the autograft group. The mean anterior intervertebral body height was 3.7 mm preoperatively and 3.9 mm at 12 months in the CFCFC, and 4.1 and 3.8 mm, respectively, in the autograft group. In cage-treated patients, neck pain, as measured using the visual analog scale (VAS) (Score 0 = minimum; 10 = maximum) decreased from 6.4 preoperatively to 2.0 at 12 months, and radicular pain decreased from 8.4 preoperatively to 1.5 at 12 months. In the autograft group, neck pain changed from a mean preoperative VAS score of 7.2 to 2.5 at 12 months, and radicular pain decreased from a preoperative mean of 7.8 to 1.4 at 12 months. Analysis of the 12-Item Short Form Health Survey domains and the Oswestry Disability Index scores indicated a significant improvement in both the Physical and Mental Component Summary domains in both groups., Conclusions: Implantation of an empty CFCFC or a tricortical iliac crest autograft after ACD are safe and reliable options for the treatment of cervical disc herniation causing one-sided radiculopathy. Both procedures produced equally satisfying clinical and radiological results, leading to a high fusion rate and maintaining intervertebral height. Implantation of an empty CFCFC has the advantages of avoiding any donor site morbidity and requiring a significantly shorter operative time.

Background: The purpose was to review our experience with the surgical management of brainstem cavernomas (BSCs) and especially the impact of the surgical timing on the clinical outcome., Method: We retrospectively reviewed 22 patients harboring a BSC, who underwent 23 procedures., Findings: Surgery was carried out during the early stage after the last haemorrhage, with a mean delay of 21.6 days (range 4-90 days). Sixteen procedures were performed after a first bleeding event while seven after multiple bleedings. Complete resection was achieved in 19 patients (86.4%). Early after surgery, 12 patients (52.2%) improved neurologically, 5 (21.7%) were stable and 6 (26.1%) worsened. New postoperative deficits were noted after 9 procedures (39.1%). Statistically significant factors for postoperative aggravation were: late surgery (P = 0.046) and multiple bleedings (P = 0.043). No patient operated on within the first 19 days after bleeding did worsen (n = 11), as opposed to 6 out of 12 who did when operated on later. After a mean follow-up of 44.9 months, 20 patients (90.9%) were improved, 1 patient (4.6%) was worse and 1 patient was lost to follow-up (4.6%), after reoperation for rebleeding of a previously completely resected cavernoma. Late morbidity was reduced to 8.6%. The mean Glasgow Outcome Scale (GOS) at the end of the follow-up period was 4.24, compared to a mean preoperative GOS of 3.22 (P<0.001). Complete neurological recovery of motor deficits, sensory disturbances, cranial nerves (CNs), internuclear ophtalmoplegia and cerebellar dysfunction were respectively 41.7%, 38.5%, 52.6%, 60.0% and 58.3%. Among the most affected CNs: CN 3, CN 5 and CN 7 were more prone to completely recover, respectively in 60.0%, 70.0% and 69.2%., Conclusions: Surgical removal of BSCs is feasible in experienced hands with acceptable morbidity and good outcome. Early surgery and single bleeding were associated with better surgical results.

Background: The authors present their current attitude towards management of craniopharyngiomas in children. Radical surgery cannot be performed when one is not sure about its potential danger to the visual pathways and the hypothalamus., Methods: Most of the surgeries that have been performed in our institution via an intracranial approach were incomplete and followed by radiotherapy, in the last 10 years with stereotactic conformational radiotherapy., Discussion: Considering our past and present experience concerning the transphenoidal approach for treatment of craniopharyngiomas, we make a plea for the consideration of this approach in children whenever feasible (around 25% of the cases in children older than 5 years). Craniopharyngiomas that can be removed by this approach represent a milder form of the disease and the results concerning the visual, hypothalamic functions and quality of life are significantly better than that obtained via an intracranial approach. When the craniopharyngioma cannot be removed by this approach because of tight hypothalamic adherences, the technique of cystosphenoidostomy with a custom-made shunt plus adjuvant stereotactic conformational radiotherapy is an alternative for its total removal.

Object: This study was designed to determine whether the frequency of shunt-dependent hydrocephalus in patients suffering from aneurysmal subarachnoid hemorrhage (SAH) differs when comparing surgical clip application with endovascular obliteration of ruptured aneurysms., Methods: In this prospective nonrandomized study, 245 patients with aneurysmal SAH treated using either surgical clip application or endovascular coil embolization were studied at our institution between September 1997 and March 2003. One hundred eighty patients underwent clip application and 65 had coil embolization. In those patients who underwent clip application of anterior circulation aneurysms, the lamina terminalis was systematically fenestrated. The occurrence of acute, asymptomatic, and shunt-dependent hydrocephalus was analyzed in both treatment groups. A subgroup analysis of patients with good clinical grade (World Federation of Neurosurgical Societies [WFNS] Grades I-III) and better Fisher Grade (1-3) and of patients with Fisher Grade 4 hemorrhage was performed. Acute hydrocephalus was observed in 19% of surgical cases and 46% of endovascular ones. The occurrence of asymptomatic hydrocephalus was similar in both treatment groups (p = 0.4). Shunt-dependent hydrocephalus occurred in 14% of surgical cases and 19% of endovascular cases. This difference did not reach statistical significance (p = 0.53). Logistic regression models controlling for patient age, WFNS grade, Fisher grade, and acute hydrocephalus in patients with good clinical grade and better Fisher grade revealed no significant difference in the rate of shunt-dependent hydrocephalus in both therapy groups (odds ratio [OR] 0.8, 95% confidence interval [CI] 0.2-2.65). Results of similar models indicated that among patients with intraventricular hemorrhage (IVH), surgical clip application carried a lower risk of shunt-dependent hydrocephalus (OR 0.32, 95% CI 0.14-0.75) compared with that for endovascular embolization., Conclusions: Shunt-dependent hydrocephalus was comparable in the two treatment groups, even in patients with better clinical and radiological grades on admission. Only patients in the endovascular therapy group who had experienced IVH showed a higher likelihood of shunt-dependent hydrocephalus.

Objects: Tectocerebellar dysraphism was first described by Padget and Lindenberg, together with occipital encephaloceles, cerebellar midline defects, tectal beaking, posterior kinked and displaced brainstem, and inverted cerebellum. We present a unique case of lipoencephalocele associated with tectocerebellar dysraphia and discuss the etiologies of both pathologies. The importance of the developmental comprehension of posterior fossa malformations is stressed., Case Report: A 9-month-old girl in good health was referred for a huge occipital mass. On local examination, the patient had a rounded mass about 10 x 12 cm in size, covered by alopecic skin, with a small eccentric dimple without any discharge. Magnetic resonance imaging revealed a lipoencephalocele with an intra- and an extracranial part, associated with tectocerebellar dysraphia with no aspect of inverted cerebellum as originally described in the tectocerebellar dysraphia. Resection of the extracranial part of the lipoma was performed for esthetic reasons. The intracranial part remained in situ., Conclusion: The association of lipoencephalocele and tectocerebellar dysraphia is extremely rare. The functional prognosis seems to be less severe for this combination than for the isolated tectocerebellar dysraphia. This association favors the dysraphic theory for the embryogenesis of the lipoma. It seems safe to restrict the surgery of this bizarre malformation to its external part., (Copyright 2004 Springer-Verlag)

Object: An increasing number of asymptomatic children are diagnosed with occult spinal dysraphism, raising the question of their optimal management. Urodynamic study (UDS) is the most reliable method of detecting neuro-urological abnormalities in these children. The rate of postoperative retethering ranges from 10 to 20% and is not always immediately clinically significant. The aim of this prospective study was to develop a reliable method that could be used in the preoperative assessment and postoperative follow-up of children with a tethered cord syndrome (TCS)., Methods: From 1989 to 1997, 15 children underwent spinal cord untethering for TCS. Preoperatively, patients were assessed with MRI and UDS. Postoperative UDS were repeated at 6- to 12-month intervals. Four UDS parameters were identified, graded, and added to obtain a UDS score. A group of 38 children without dysraphic condition was used as control and allowed the calculation of a normal score., Conclusions: There was a statistically significant difference in the preoperative UDS scores between the control group and the study group ( p<0.001). Postoperatively, there was a statistically significant improvement ( p<0.001) in UDS scores. UDS score is a reliable tool for identifying and quantifying neuro-urological disorders in patients with TCS. Postoperatively, this score was useful in the early diagnosis of spinal cord retethering.

In patients suffering from sickle cell disease (SCD), bone is a preferred site of infection. We report the case of a five-year-and-eight-month-old black African boy with homozygous-SS disease who developed a cranial epidural abscess. This intracranial infectious complication originated from a Salmonella enteritidis osteitis of the frontal bone. Antibiotic treatment alone did not control the disease, so surgery was necessary to remove the necrotic bone and to evacuate the epidural pus. The numerous factors interfering with normal healing of a septic focus in sickle cell anemia, particularly in this previously undescribed intracranial complication, emphasize the need for a primary and early surgical treatment in similar situations.

Owing to the frequent occurrence of minor head injuries in their daily practice, pediatricians and primary care physicians have a constant concern about their management. Evaluation of a neurotraumatised children must take account of the epidemiological, anatomical, pathophysiological and clinical specificity of each age class. Recent publications of the American Academy of Pediatric, with evidence based medicine review of the literature and experts consensus have permitted to define precise guidelines for the management of children over and below 2 years of age. Taking account in the initial evaluation of the indirect signs of severity and pediatric mechanisms criteria allows to better define the principles of triage.

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., (Copyright 2003 S. Karger AG, Basel)

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.

Objective: To assess the value of three-dimensional CT (3D CT) in the diagnosis and management of suspected paediatric craniofacial malformations., Materials and Methods: Twenty-eight children (12 girls, 16 boys) with a mean age of 4 years, suffering from craniofacial or cervical malformations, underwent craniofacial spiral CT. 3D reformatting was performed using an independent work-station., Results: 3D CT allowed the preoperative evaluation of 16 patients with craniosynostosis and the post-surgical management of 2 patients. 3D CT clearly depicted malformations of the skull base involving the petrous bone in seven patients (four cases of Goldenhar-Gorlin syndrome, one case of Treacher-Collins syndrome and two cases of Crouzon's disease). Four patients with craniofacial clefts were also evaluated. Radiological findings were confirmed by the clinical and intraoperative findings in all patients that underwent surgical treatment. Movement artefacts and "Lego effect" related to abrupt change of cranial vault border were encountered and are discussed., Conclusions: 3D CT of the skull can safely and reliably identify paediatric craniofacial malformations involving bone, and it should be used as morphological mapping to help the surgeon in planning surgical treatment.

The management and prognosis of gliomas are significantly different in children and in adults. Fortunately, the proportion of gliomas that are malignant is smaller in children than in the adult population. The different types of gliomas encountered in the pediatric population are reviewed, taking account of the most recent contributions on this subject. The importance of considering both localization and histological classification for better definition of the prognostic factors of each subgroup is emphasized. A brief review of the possible causes of gliomas is presented. Unfortunately, the information obtained by molecular and genetic study of these tumors has still not resulted in anything that can help the children suffering from gliomas in a concrete way. Surgery, with all its recent refinements, remains the best treatment for the majority of benign gliomas providing they can be removed without unacceptable sequelae. The role of chemotherapy has emerged recently for the treatment of nonresectable low-grade gliomas, such as hypothalamic-chiasmatic tumors, especially for infants, in whom the adverse effects of radiotherapy can be severe and irreversible. On the eve of the new millennium, there is renewed hope that the problem of malignant gliomas will be solved in the not-too-distant future.

A case of colonization of a CSF shunt device by racemous cysts during a long lasting course of neurocysticercosis is reported.

A 4-year-old girl underwent craniofacial reconstruction for giant cystlike encephalocele deriving from the temporo-maxillary region and giving impression of the duplicated head. The case of temporal encephalocele in this report is especially unusual in the extent of encephalocele, the degree to which it had expanded the zygomatic arch, mandible, cranial vault, and the radiologically undetectable bony defect. The use of craniofacial principles in the resection and reconstruction of the temporal encephalocecle are described.

Acute haemorrhagic presentation of a meningioma is an extremely rare event. In a review of the literature, 44 such cases were found and only eight of them presented with sudden onset of coma. The outcome in these eight cases was, with few exceptions, poor. We report the case of a 72-year-old woman who presented with sudden onset of coma due to a massive haemorrhage into a frontal meningioma. The diagnosis was suspected on the basis of a non-enhanced computed tomographic (CT) scan and considering the clinical status of the patient, emergency evacuation of the haematoma and complete macroscopic resection of the tumour was performed without further diagnostic procedures. The patient fully recovered. Clinical examination and contrast enhanced CT scan at 3 months follow-up were considered normal. The prognosis for haemorrhagic presenting meningiomas is poor especially for those presenting with acute onset of coma. The outcome seems to be improved with rapid decompression and tumour removal. We would like to stress the need to keep the diagnostic procedures to a minimum once a haemorrhagic decompensating meningioma is suspected despite the risks associated with an incomplete radiological work up before surgery. Complete or at least gross tumour removal at the time of emergency surgery seems to be associated with a better outcome and should be attempted in all patients, even those presenting with acute signs of herniation.

Two new cases of leptomeningeal cysts subsequent to vacuum extraction are reported. Both children presented with a huge, nonpulsating, transilluminating subgaleal collection over the anterior fontanel that appeared soon after instrument delivery. Plain X-rays, computed tomography, and magnetic resonance imaging confirmed that the subgaleal collection was cerebrospinal fluid and showed the presence of a diastatic coronal suture in both cases. Treatment consisted of duraplasty with periosteal flaps and application of fibrin glue. In one case, an associated porencephalic cyst was treated with a cystoperitoneal shunt. Surgical treatment of leptomeningeal cyst due to vacuum extraction is simple and should not be postponed, despite the tendency for the extracranial cyst to regress, because of the potential risk of continuous growth of an underlying porencephalic cyst and risk of neurological damage.

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.

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.

This paper reports the case of an English speaking, fully left-handed patient (DS) with a left unilateral writing deficit occurring after a subarachnoid hemorrhage due to the rupture of an anterior communicating aneurysm. DS's performance in spelling and in right-handed writing was entirely preserved while his left-handed writing was characterized by the production of errors which could generally be spontaneously self-corrected. Errors produced with lower-case letters differed from the ones produced with upper-case letters: The former usually corresponded to letter substitutions which were characterized by a high degree of physical similarity between the target letter and the one produced. The latter tended to result in the production of aborted letters. This impairment is discussed in the context of cognitive models of writing. It is suggested that graphic motor patterns for lower-case letters and for upper-case letters are different in nature and consequently that production processes may also differ.

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.

To reproduce diastematomyelia, a sagittal incision was carried out at the level of the rhomboidal sinus of 36- to 40-h-old chick embryos. A small piece of membrane shell, a small agar screen, or a piece of quail isochronous isotopic notochord was inserted into the gap. The embryos were killed and fixed after 9 days' incubation. Diastematomyelia was obtained in several embryos treated with interposition of a membrane screen or a piece of quail notochord. Microscopic examination revealed two hemicords, each containing its own central canal; in some cases one of the cords showed hydromyelia. Absence of the rump was seen in association with experimental diastematomyelia. The interposition of a resorbable agar screen did not succeed in reproducing diastematomyelia. The results of these surgical manipulations suggest that diastematomyelia cannot be explained by a primary disorder of neurulation. It supports the theory of noninvolution of a firm midline structure (probably the neurenteric canal, rapidly surrounded by mesodermal cells originating from the notochord), which prevents the fusion of the separated parts.

Three unusual patients who developed subacute facial numbness as the heralding symptom of an expanding tumor that involved the trigeminal nerve fibers are reported. The first patient had clinical and electrophysiological evidence of an isolated mental neuropathy as a result of metastatic lesions with bone destruction from a renal cell carcinoma. The second patient had a sensorimotor trigeminal neuropathy caused by a direct compression of the semilunar ganglion by a cavernous hemangioma of Meckel's cave. The last patient experienced facial numbness as the unusual presenting manifestation of a primary brainstem lymphoma. Patients 1 and 3 died a few weeks after the admission, whereas patient 2 poorly recovered. Despite the availability of new techniques for early diagnosis, this report demonstrates how difficult it can initially be to differentiate a 'benign' trigeminal neuropathy from serious conditions and underscores the poor prognosis of fifth nerve fibers involvement by an expanding mass. Early referral with clinical and electrophysiological evaluation appears to be of crucial importance.

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 sciatic pain syndrome is reported. Computed tomography showed evidence of gas in the spinal canal, and at operation a gas-filled pseudocyst was found in the spinal canal, without herniated disc. The cause of this unique case is discussed in relation to the spinal vacuum phenomenon.

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.

In a surgical series of 755 pituitary adenomas, 51 cases (6.75%) showed intratumoral calcifications on microscopic examination and 13 of these were visible on roentgenographic examination (1.72%). Thirty-eight of these 51 cases were prolactinomas, the highest incidence being in male patients. There was no correlation between age, sex, levels of hormone, size of the tumor, and the presence of calcifications. Four patterns of radiological calcifications were encountered. Histologically, the calcifications were most frequently found within the tumor masses. Their rare occurrence within fibrous or degenerative areas speaks against their dystrophic nature. Previously reported calcified bodies in fetal and newborn pituitaries and the recently described physiological hyperprolactinemia in early infancy suggest a possible hormonal influence in the genesis of calcifications in prolactinomas.

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.

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.

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%).

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.