Ultrastructure of the wall of the Dandy-Walker cyst

Summary The ultrastructure of the wall of the Dandy-Walker cyst has been described rarely. A boy aged 2 years was confirmed clinically, neuroradiologically, and operatively as having a Dandy-Walker cyst in the posterior fossa. The cyst wall obtained during surgery consisted of an outer arachnoid cell layer, intermediate interwoven neuroglial strands, and an inner layer of cells which lacked the characteristic appearance of ependyma. An unusual finding was a small, buried island of ependymal cells in the intermediate layer of the neuroglial tissue. Ultrastructural study of the cyst wall provides a better understanding of the pathogenesis of the Dandy-Walker syndrome.     

Dermoid cyst in the posterior fossa accompanied by Klippel-Feil syndrome

Objects: Dermoid cysts accompanied by Klippel-Feil syndrome are uncommon, and the coincidence of these two rare diseases suggests an interesting idea about the pathogenesis of a dermoid cyst, which the authors consider with reference to an actual case. A posterior fossa dermoid cyst with dermal sinus and Klippel-Feil syndrome are reported in the same patient. A 23-year-old woman was admitted to hospital with progressive headaches. Methods: Cervical radiography demonstrated C4–5 vertebral fusion, and magnetic resonance imaging revealed a large cystic mass in the midline of the posterior fossa. The cystic lesion was totally removed along with the accompanying dermal sinus. It was histopathologically diagnosed as a dermoid cyst. Conclusion: The rare coincidence of a dermoid cyst and Klippel-Feil syndrome suggests the possibility that a disturbance in the mesoderm before the fourth week of gestation might play an important part in the causation of dermoid cyst. Received: 11 February 2000     

Rapidly enlarging dermoid cyst over the anterior fontanel: a case report and review of the literature

A case report of a rapidly enlarging dermoid cyst over the anterior fontanel is presented. Our presentation demonstrates the course of rapid enlargement of the tumor with radiological images, which were examined at birth and during the process of the tumor enlargement. The literature is reviewed with respect to the nature of this tumor, especially to the relationship of tumor enlargement. Received: 1 November 1999     

Cranial aneurysmal bone cyst: a diagnostic problem With a review of the literature

Aneurysmal bone cysts (ABC) are benign osteolytic lesions that are more common in young people than in adults and involve the skull only exceptionally. The origin of ABC is still debated; indeed, some authors consider ABC to be an anomalous bony reaction that is secondary to traumas or tumours. Conversely, others consider ABC to be a distinct entity. A case of a healthy young female affected by a left frontal ABC is reported here. The clinical onset was characterised by the sudden appearance of a tender and mildly painful frontal mass. Neuroradiological assessment showed a well-circumscribed lytic lesion of the frontal bone with predominantly outward extension. En bloc surgical removal of the lesion was successfully achieved; a reconstructive cranioplasty was also performed to repair the cranial defect. The rarity of the condition described, together with the absence of clear guidelines, prompted us to review the more recent literature with the twin goals of identifying radiological features and becoming able to address the diagnosis and rules for treatment of such a rare entity. Received: 28 July 2000 Revised: 25 September 2000     

Spontaneous regression of a symptomatic pineal cyst after endoscopic third-ventriculostomy

With the advent of modern diagnostic tools for neuroimaging, the incidental detection of pineal cysts in asymptomatic subjects has increased. Only rarely do pineal cysts present with the clinical signs and symptoms of increased intracranial pressure or with neurological deficits in relation to compression and distortion of the adjacent nervous structures and cerebrospinal fluid pathways. While asymptomatic cysts are considered to be normal variants for which no further investigations are usually required, surgical treatment is suggested for symptomatic cysts, with the goal of eliminating the block in the cerebrospinal fluid circulation and/or the mass effect exerted by the lesion. In this report we describe a pediatric case of symptomatic pineal cyst, revealed by repeated episodes of headache caused by secondary obstructive hydrocephalus. Following an endoscopic third-ventriculostomy, serial magnetic resonance imaging studies demonstrated that not only had the ventriculomegaly resolved but also that the pineal cyst had regressed over time. A to-and-fro movement of fluid through the cyst wall, the direction of which depends on the equilibrium existing between the inner pressure of the cyst and the outer cerebrospinal fluid pressure, is suggested as a possible mechanism accounting for this unexpected result – to our knowledge, the first reported in literature. Received: 16 February 2000     

Incidence of post-lumbar puncture syndrome reduced by reinserting the stylet: a randomized prospective study of 600 patients

The post-lumbar puncture syndrome (PLPS) can best be explained by prolonged spinal fluid leakage owing to delayed closure of a dural defect. Its incidence after spinal anaesthesia is much lower than after diagnostic lumbar puncture (LP). This difference could be caused by a strand of arachnoid, which might enter the needle with the outflowing cerebrospinal fluid (CSF) during diagnostic LP and upon removal of the needle be threaded back through the dura to produce prolonged CSF leakage. To find a technique that further reduces the incidence of PLPS, this hypothesis was tested by evaluating the effect that reinserting the stylet before removing the needle had on the incidence of PLPS. By reinserting the stylet to the tip of the needle, the hypothesized strand would be pushed out, thereby reducing the frequency of PLPS. Sprotte’s “atraumatic needle” (21 gauge) was used for LP. A total of 600 patients participated in the prospective study. They were randomized into two groups and questioned about their complaints every day for up to 7 days after the LP. All LPs were performed by two experienced neurologists (T.B., M.S.). In 300 patients, the stylet was reinserted to the tip of the needle; in the other 300 it was not reinserted. Whereas 49 of the 300 patients without reinsertion developed PLPS, only 15 of the 300 patients with reinsertion did. This significant difference (16.3 vs 5.0%, P < 0.005, chi square test) supports our hypothesis. On the basis of our results, we recommend reinserting the stylet before removing the needle in order to reduce the incidence of PLPS. Received: 30 September 1997 Received in revised form: 9 March 1998 Accepted: 20 March 1998     

Medulloepithelioma of the optic nerve with intradural extension – report of two cases and a review of the literature

Medulloepithelioma is an uncommon tumour. Among the various sites of origin the optic nerve is rare. We report here two cases of medulloepithelioma of the optic nerve that had extended intradurally into the cranial cavity. A review of the published cases is also given. Received: 30 April 1999 Revised: 17 June 1999     

Refined mapping and characterization of the recessive familial amyotrophic lateral sclerosis locus (ALS2) on chromosome 2q33

Amyotrophic lateral sclerosis (ALS) is a progressive degenerative neuromuscular disease that shows familial, autosomal dominant inheritance in 10%–15% of cases. Previous genetic analysis of one large family linked a recessive form of familial ALS (FALS-AR type 3) to the chromosome 2q33–35 region. Using additional polymorphic markers, we have narrowed the size of the linked region to approximately 1.7 cM by linkage and haplotype analysis. We have also established a yeast artificial chromosome contig across the locus that covers an approximate physical distance of 3 million bases. Based on this contig, genes and expressed sequences that map near the 2q33 region have been examined to determine whether they are located within this ALS2 candidate locus. Five identified genes and 34 expressed sequence tags map within the region defined by crossover analysis and merit further consideration as candidate genes for this disease. Accepted: July 15, 1998 / Published online: October 28, 1998     

Linkage of a commoner form of recessive amyotrophic lateral sclerosis to chromosome 15q15-q22 markers

Autosomal recessive familial amyotrophic lateral sclerosis (RFALS) is a rare form of ALS that usually presents at an early age with slow progression of symptoms. RFALS is clinically and genetically heterogeneous and the locus of RFALS type 3 was mapped to 2q33 (ALS2) in a single family. We now report linkage of a more-common form of RFALS to chromosome 15q15-q22 markers (ALS5) and show further genetic locus heterogeneity in RFALS. ALS5 is the locus for most families with RFALS and appears to be present in both North African and European populations. Received: July 22, 1998 / Accepted: September 18, 1998 / Published online: December 18, 1998     

Reliability and validity of a child-specific cross-cultural instrument for assessing posttraumatic stress disorder

 The Posttraumatic Stress Symptoms in Children (PTSS-C) was developed as a cross-cultural semi-structured interview to diagnose posttraumatic stress disorder (PTSD) and to identify PTSD-non-related posttraumatic stress symptoms in children after various traumatic experiences. The psychometric properties were studied in two different child populations in Iraqi Kurdistan (the survivors of the military operation “Anfal”, and the orphans), in a sample of Kurdistanian refugee children in Sweden, and in a comparison sample of Swedish children. The instrument yielded satisfactory internal consistency, high interrater agreement, and excellent validity on cross-validation with the Child Posttraumatic Stress Disorder Reaction Index (CPTSD-RI) and the Diagnostic Interview for Children and Adolescents (DICA) according to the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV). Accepted: 14 March 2000     

Multicentric juvenile pilocytic astrocytoma occurring primarily in the trigone of the lateral ventricle

The authors report a juvenile pilocytic astrocytoma (JPA) in the trigone of an 11-year-old girl, apparently with disseminated lesions that disappeared spontaneously after the removal of primary lesion. Gadolinium-enhanced magnetic resonance images (MRIs) showed features suggestive of the development of multicentric spread of the tumor at the initial diagnosis. Histologically the primary tumor showed the typical features of a JPA. Serial postoperative MRIs demonstrated that everything except a remnant of the primary tumor seemed to regress spontaneously without any adjuvant therapy. The site of origin of this tumor is thought to be the subependymal glia of the occipital lobe. Received: 22 September 1997 Revised: 6 January 1998     

Comparing the German versions of the Strengths and Difficulties Questionnaire (SDQ-Deu) and the Child Behavior Checklist

 The Strengths and Difficulties Questionnaire (SDQ) is a brief behavioural screening questionnaire that can be completed in about 5 minutes by the parents and teachers of 4–16 year olds. The scores of the English version correlate well with those of the considerably longer Child Behavior Checklist (CBCL). The present study compares the German versions of the questionnaires. Both SDQ and CBCL were completed by the parents of 273 children drawn from psychiatric clinics (N = 163) and from a community sample (N = 110). The children from the community sample also filled in the SDQ self-report and the Youth Self Report (YSR). The children from the clinic sample received an ICD-10 diagnosis if applicable. Scores from the parent and self-rated SDQ and CBCL/YSR were highly correlated and equally able to distinguish between the community and clinic samples, with the SDQ showing significantly better results regarding the total scores. They were also equally able to distinguish between disorders within the clinic sample, the only significant difference being that the SDQ was better able to differentiate between children with and without hyperactivity-inattention. The study shows that like the English originals, the SDQ-Deu and the German CBCL are equally valid for most clinical and research purposes. Accepted: 22 February 2000     

Lack of association between apolipoprotein E genotype and sporadic amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a neuro-degenerative disorder with both sporadic and familial forms. Approximately 20% of autosomal dominant ALS is caused by mutations in the Cu/Zn superoxide dismutase (SOD1) gene. The causes of the remaining forms of ALS are unknown. The apolipoprotein E (APOE) gene is a known genetic risk factor for Alzheimer disease (AD), another neuro-degenerative disease. The APOE-4 allele increases risk and decreases age at onset in AD. Studies examining ALS and APOE have failed to show a significant effect of APOE on overall risk in ALS. Studies examining the effect of APOE-4 on site of onset in ALS (bulbar or limb) have been contradictory, with some studies showing an APOE association with bulbar onset and others showing no effect. Sample size was limited in these previous reports, particularly with respect to the number of bulbar onset cases (n = 33, 34 and 53). The present study examines a large collaborative data set of ALS patients (n = 363; 95 with bulbar onset) and age-matched neurologically normal controls. The results for these data showed no significant differences in the percentage of subjects with the APOE-4/4 and APOE-4/X genotypes (X = APOE-2 or APOE-3) when comparing cases and controls in both the overall data set or in the data set stratified by site of onset. Similarly, logistic regression analysis in the overall and stratified data set while controlling for sex showed no increase or decrease in risk of ALS associated with the APOE-4 allele. In addition, there were no significant differences in age at onset between patients with APOE-X/X, and APOE-4/4 or APOE-4/X genotypes, overall or stratified by site of onset. We conclude based on these data that the APOE gene is not a major genetic risk factor for site of onset in ALS. Received: June 30, 1997 / Accepted: August 10, 1997     

Gunshot wound of the atlas in a youth

The case of a bullet retained, without causing neurological symptoms, in the anterior arch of a youth’s atlas after a gun had been fired a short distance from his mouth is reported. The patient was managed with external stabilization. Received: 10 February 1999     

Meningiomas of the central nervous system occurring below the age of 17: report of 24 cases not associated with neurofibromatosis and review of literature

The objective of this work was to gain more insight into the controversial characteristics of meningiomas occurring during childhood and adolescence. Management of meningiomas is an important field in pediatric neurosurgery. Every pediatric neurosurgeon has tried to resolve the problems relating to the clinical characteristics, biological behavior and outcome of this interesting and almost benign pathology, which rarely occurs in the first two decades of life. The records on central nervous system (CNS) tumors held by the two major neurosurgery centers of Tehran Medical University and Arad General Hospital were prospectively collected during last 15 years. Complete medical records are available for all 24 cases, and long-term follow-up was achieved 19 patients. All the cases were diagnosed and treated after the introduction of computed tomographic (CT) scanning. Angiography and magnetic resonance imaging (MRI) were performed as complementary studies in some cases. The sample consisted of 13 girls and 11 boys. The age range at the time of diagnosis varied between 2 and 17 years, with a mean of 9.47 and standard deviation of 3.43. Fifteen patients were below 10 years of age (62.5%), and 9 of them were between 10 and 17 years old (37.5%). The most common presenting symptoms, in declining order of frequency, were headache, epilepsy and focal neurological deficits. Similar cases associated with neurofibromatosis either at the time of presentation with meningioma or during the follow-up period were excluded (5 cases). The size of the presenting tumor was more than 5 cm in diameter in 17 cases. The locations of the lesions, taken as the site of the presumed widest dural base in each case were: spinal, orbital, ethmoidal and sphenoethmoidal in 1 case each, petroclival in 2, and tentorial or supratentorial in 18 patients. The only predisposing factor in this series of childhood meningiomas was whole-axis irradiation for previous malignancy in the case presenting with cervical intradural meningioma. There have been no surgical deaths, and gross total excision of the lesions was achieved in 21 cases. Tumor recurrence was observed during the follow-up period in 6 cases (25%). The follow-up period varied between 2 and 165 months, with a median interval of 130.2 months. This series of pediatric CNS meningiomas comprises almost 1.08% of all meningiomas operated on by the authors during the last 15 years and it also accounts for about 1.1% of all pediatric CNS tumors encountered. This series of patients has certain characteristics regarding sex distribution, unusual size, peculiar localizations, special histological features and benign clinical behavior distinguishing it from other series reported in the literature. Received: 15 February 1999 Revised: 18 July 1999     

Emotional and behavioural problems in a sample of Finnish three-year-olds

 Behavioural and emotional problems in Finnish three-year-olds was studied using the Child Behavior Checklist for ages 2–3 as a standardised rating scale at well-baby clinics in two cities in Finland. Altogether 374 questionnaires were analysed which is 71% of the target population. When compared with studies from other countries the mean scores and prevalences of preschool children's problem behaviours were rather similar. Boys were reported to be more destructive than girls. Parental education level, living in one-parent family and parental unemployment were associated with a higher level of problem behaviours. No significant differences were found in emotional or behavioural problems related to the child's day care. Co-sleeping with parents was associated with sleep problems. Accepted: 30 August 2000     

Surgical outcome of pediatric hydrocephalus treated by endoscopic III ventriculostomy: prognostic factors and interpretation of postoperative neuroimaging

In order to analyze the surgical outcome according to clinical characteristics and to evaluate the correlation between clinical improvement and neuroimaging changes, we retrospectively reviewed 32 children who had undergone endoscopic III ventriculostomy (ETV) from February 1994 to May 1998. There were 15 boys and 17 girls, with a mean age of 5.2 years (range: 1 month to 13 years). The etiology of the hydrocephalus was primary aqueductal stenosis in 18 patients, secondary aqueductal stenosis caused by tumors in 5, IV ventricle outlet obstruction in 5, and hydrocephalus associated with meningomyelocele in 4. The mean duration of follow-up was 19.4 months (range 1–50 months). Overall, surgical outcome was regarded as good in 21 of 29 patients. Surgical outcome was poor in patients younger than 1 year (P<0.05). Neuroimaging 1 month after ETV showed a decrease in ventricular size in 11 of the 16 patients with good surgical outcomes. Five showed minimal changes only. In patients with good outcomes, ventricular size tended to decrease as time passed. Resolution of periventricular edema, flow void in the III ventricle on T2-weighted axial images, and cine-MR imaging were sensitive indicators of good outcome. We suggest that ETV be considered as a primary treatment option in patients older than 1 year of age with noncommunicating hydrocephalus. In addition, time factors should be taken into consideration when surgical outcome is judged. Changes in ventricular size could not predict surgical outcome completely in themselves. Therefore, a comprehensive postoperative assessment should be made with the help of T2-weighted MRI and cine-MRI. Received: 24 May 1999 Revised: 7 August 1999     

Clinical feature and outcome of pediatric cerebrovascular disease: a neurosurgical series

To investigate the clinical features and surgical outcome of pediatric cerebrovascular disease (CVD), we retrospectively reviewed 280 children (up to 15 years of age) who underwent neurosurgical procedures for CVD between 1979 and 1998. Open surgery (n=448), endovascular procedures (n=22), and stereotactic radiosurgery (n=14) were the main neurosurgical procedures adopted. Clinical features and surgical outcomes were described according to the etiology of the CVD and the number of years of management. The mean duration of follow-up was 34 months. The mean age was 7.1 years, and the male-to-female ratio was 1.2:1. The most frequent CVD was moyamoya disease (62%). The surgical mortality related to CVD was 0.7%. Eighty-seven percent had Karnofsky Performance Scale scores of more than 70. We demonstrate increasing detection rates and improving outcomes in recent years. The clinical course in this study shows that recovery from CVD in children is good after neurosurgical procedures. Received: 24 October 1999 Revised: 20 December 1999     

Distribution of measles virus in the central nervous system of HIV-seropositive children

In an autopsy study the distribution of measles virus (MV) in the central nervous system (CNS) of 18 measles-infected children (13 HIV seropositive, 5 HIV seronegative), in Abidjan, Ivory Coast was examined using immunocytochemistry and in situ hybridization. Of these children 17 died from measles giant cell pneumonia. In 3 of the 13 HIV-seropositive patients MV antigens and genomic RNA was detected in the CNS. One of these positive patients had an MV encephalitis with abundant virus throughout most of the CNS. MV was not detected in the CNS of any of the 5 HIV-seronegative patients. These findings, albeit in a small number of cases, would suggest there may be an increased susceptibility to infection of the CNS with MV in HIV-positive children. In this respect entry and growth of MV in the CNS in HIV-seropositive individuals may be similar to the occurrence of measles inclusion body encephalitis in immunocompromised individuals. Furthermore, comparison of the HIV-MV encephalitis patient with two patients with subacute sclerosing panencephalitis (SSPE) demonstrated a paucity of virus in neuronal processes in the HIV-MV encephalitis. Unlike in SSPE, MV maturation by budding through the plasma membrane may occur, thereby minimizing build up of and intracellular movement of incomplete virus. Received: 6 March 1998 / Revised, accepted: 2 June 1998