Prevalence of central nervous system diseases--a large retrospective cohort study of adolescents.

At age sixteen, most Israeli nationals must undergo medical evaluation for compulsory military duty. All potential conscripts are referred to the Israel Defense Forces (IDF) recruiting office. Therefore, medical screening of a vast number of adolescents is performed, offering a unique opportunity to study the prevalence of neurological diseases in an entire age cohort. Hence, screening is not affected by diagnostic or methodological bias. We performed a retrospective neuroepidemiological large cohort study of adolescents from the database of the Israel Defense Forces recruiting office during the years 1998-2002. The survey included 409 492 adolescents, among them 162 079 (39.5%) females. The most prevalent diagnoses were: headache (754 per 10 000 adolescents), permanent brain damage (197 per 10 000), epilepsy (167 per 10 000) and movement and coordination disorders (36 per 10 000). These were followed by cranial nerve disorders, sleep disorders, cranio-spinal bone defects, and chronic progressive CNS disorders. The relative risk for male adolescents within the specific disease groups was higher for movement-coordination, sleep and cranial nerve disorders. Multivariate analysis revealed gender and severity prevalence and sex-grade, or year-grade interactions in the distinct groups of diseases. This study provides important information on the prevalence of neurological diseases in adolescents and demonstrates some significant epidemiological trends.

A normal second-trimester ultrasound does not exclude intracranial structural pathology.

OBJECTIVE: To report the prenatal diagnosis and management of 34 fetuses with various intracranial structural pathologies diagnosed following a normal second-trimester ultrasound examination. METHODS: We retrospectively reviewed the images of 203 abnormal central nervous system ultrasound examinations performed between 13 and 37 weeks of gestation at our prenatal diagnosis unit. In 34 (16.7%) of them at least one previous second-trimester ultrasound examination had been performed and considered normal. These 34 fetuses represent the study group. RESULTS: The following intracranial pathologies were diagnosed: dysgenesis of the corpus callosum, ventriculomegaly, cerebral cysts or hemorrhage, migrational disorders, vermian dysgenesis, arachnoid cysts, macrocephaly, enlarged subarachnoid space, brain calcifications and microcephaly. CONCLUSION: A normal second-trimester ultrasound scan does not rule out significant intracranial anomalies. Parents and physicians should be informed about the limitations of second-trimester sonography as far as brain diagnosis is concerned. A repeat third-trimester scan may enable more accurate diagnosis and counseling.

White matter abnormalities in Leber's hereditary optic neuropathy due to the 3460 mitochondrial DNA mutation.

Leber's hereditary optic neuropathy is a maternally inherited disorder characterized by acute or subacute loss of central vision leading to severe optic atrophy. It is caused by mutations in the mitochondrial genome. Primary mutations are located at nucleotide positions 3460, 11778 and 14484 in genes encoding subunits of complex I of the respiratory chain. The occurrence of a demyelinating disease such as multiple sclerosis has been reported mainly in females with the 11778 mutation. We report a patient with Leber's hereditary optic neuropathy, kyphosis and white matter lesions in association with the 3460 mtDNA mutation. It is suggested that multiple sclerosis-like illness and deformities of the vertebral column may be associated pathogenically with Leber's hereditary optic neuropathy.

The fetal cerebellar vermis: normal development as shown by transvaginal ultrasound.

OBJECTIVE: To determine the normal appearance and development of the fetal cerebellar vermis using mid-sagittal planes obtained by transvaginal sonography. SUBJECTS AND METHODS: A total of 101 fetuses were evaluated by transvaginal sonography between 21 and 39 weeks of gestation. The mid-sagittal antero-posterior and cranio-caudal vermian diameters, circumference and surface area were measured and calculated. Nomograms were produced. RESULTS: The vermis was observed in 96% of the cases and all the measurements were performed in 92%. The vermis grows in a linear fashion throughout pregnancy. The growth pattern correlates well with gestational age, biparietal diameter, head circumference and transverse cerebellar diameter. CONCLUSION: Transvaginal sonography is a valuable tool in the study of the fetal vermis during the second half of pregnancy. Knowledge of its normal appearance may help identify developmental anomalies and enable accurate prenatal counseling.

Absence seizures aggravated by valproic acid.

PURPOSE: To report on pediatric patients with absence epilepsy who experienced absence seizure aggravation while receiving valproic acid (VPA). METHODS: The charts of all children from four pediatric epilepsy clinics receiving VPA for absence epilepsy were reviewed. Patients were evaluated and followed up between 1994 and 2000. RESULTS: Eight cases (six boys) of absence seizure aggravation were detected. Mean age at seizure onset was 5.8 years (range, 3-12 years). Six patients had simple absence seizures, one had myoclonic absences, and one had absences with automatisms. The electroencephalogram in all cases depicted generalized 3-Hz spike-and-wave activities. All eight patients experienced an increase in the frequency of absence seizures within days of VPA introduction. Dose increments resulted in further seizure aggravation. Serum levels of VPA were within therapeutic range in all patients. No case was attributed to VPA-induced encephalopathy. All patients improved on VPA discontinuation. In five children, VPA was reintroduced, resulting in further seizure aggravation. CONCLUSIONS: VPA can occasionally provoke absence seizure aggravation in patients with absence epilepsy.

Benign intracranial hypertension associated with budesonide treatment in children with Crohn's disease.

Oral budesonide in adult studies is a potent corticosteroid with decreased systemic bioavailability and an improved adverse effect profile in comparison with prednisone. It has recently been introduced for the treatment of inflammatory bowel disease in Europe, Canada, and Israel. Benign intracranial hypertension has rarely been associated with corticosteroid therapy but has not been reported in association with budesonide therapy. Three adolescents with Crohn's disease and poor nutritional status developed benign intracranial hypertension while receiving oral budesonide. All three patients had previously received multiple courses of prednisone during the course of their disease, without developing intracranial hypertension. Benign intracranial hypertension resolved after medication withdrawal and did not recur with subsequent use of prednisone. Evaluation for benign intracranial hypertension should be considered in patients with inflammatory bowel disease who develop headache while receiving oral budesonide. This side effect may be associated with poor nutritional status.

Febrile convulsions, ataxia, developmental delay, and obesity: a new syndrome?

We describe the association of recurrent complicated febrile convulsions, developmental delay, ataxia, and obesity in three unrelated girls. The three girls, aged 3 to 4 years, were all born to healthy, nonconsanguineous parents and have normal siblings. Their birth weight was appropriate for gestational age. They are not dysmorphic and have normal head circumference. Development is delayed; they all walked with an ataxic gait after the age of 2 years and started speaking at 3 years. Their growth charts are remarkably alike: they initially had a normal growth curve and around 24 months of age started to gain weight excessively. They all continue to suffer from complicated febrile seizures, which started before 12 months of age, and are resistant to prophylactic anticonvulsants. Metabolic evaluation is normal. They have normal magnetic resonance images and electroencephalograms. Fragile X and Prader-Willi syndromes were ruled out. We suggest that this is a new mental retardation syndrome that should be considered in children with recurrent febrile convulsions, developmental delay, and obesity. In a recent study, mutations in the beta4 calcium channel were identified in the mutant epileptic mouse that presents with epilepsy, mental retardation, and ataxia. We hypothesize that a calcium channel gene may be involved in this syndrome.

Attitude and knowledge of attention deficit hyperactivity disorder and learning disability among high school teachers.

The aims of the study were to investigate teachers' knowledge and attitudes towards attention deficit hyperactivity disorder (ADHD) and learning disabilities (LD). Forty-six high school teachers were interviewed in this regard. The 46 teachers were divided into two groups: 25 teachers taught at an academic school (School 1); and 21 teachers taught at special education school (School 2) and dealt with ADHD/LD cases regularly. General knowledge about ADHD (71%) and about LD (74%) was relatively low among both groups. Thirteen percent of all teachers considered LD to be the result of parental attitudes, namely 'spoiling' the children. The score for attitude and understanding of ADHD children was relatively low (72.5%) for both groups, whereas Group B teachers scored higher regarding LD cases. Almost 40% considered that ADHD children should be rebuked and/or punished in a manner similar to non-ADHD kids. Regarding long-term outcome, 45.7% of the teachers expected ADHD children to experience multiple difficulties in family life during adulthood. In relation to LD cases, the overall scoring for positive attitude was 75%. However, this score was higher for Group B teachers. Three-quarters of the teachers favored increasing peer awareness and comprehension as to the problems LD kids encounter at school. Ninety-five percent believed LD patients should enjoy a more lenient school education. There was no correlation between teachers, knowledge of ADHD and LD and their attitude. The main sources for this knowledge were: specialized textbooks, continuous education, TV shows, journals and newspapers, and medical personnel.

Common peroneal neuropathy due to surfing.

Common peroneal neuropathy is uncommon in children and adolescents. In this population, it is usually caused by direct nerve injury at the fibular head level. Most commonly, the nerve is damaged during sports-related blunt trauma. Other etiologies such as hereditary neuropathies and bone tumors are much less frequent. In some cases, repetitive microtrauma to the peroneal nerve is felt to cause neuropathy. We describe the case of a teenager who developed common peroneal neuropathy in association with prolonged wave-surfing in the presence of weight loss.

Acute pediatric rhabdomyolysis.

Rhabdomyolysis is a relatively common condition that may occur intermittently in chronic and inflammatory myopathy, muscular dystrophy, and metabolic myopathy. Rhabdomyolysis can also present acutely in otherwise healthy individuals. The list of etiologies for acute muscle cell lysis is enormous, with new causes described yearly. Series on acute pediatric rhabdomyolysis have not yet been published. This article describes a retrospective review of children admitted to the authors' institution during an 8-year period in whom rhabdomyolysis was recognized as a complication during their hospital stay. Patients with intermittent or relapsing rhabdomyolysis were excluded. Nineteen children were identified. Trauma (five cases), nonketotic hyperosmolar coma (two cases), viral myositis (two cases), dystonia (two cases), and malignant hyperthermia-related conditions (two cases) were the most common causes of rhabdomyolysis. Acute renal failure was the most frequent complication, occurring in 42% of cases. The mean age of renal failure patients was 13.9 years, compared to 8 years for non-renal failure children. Careful assessment of the initial urinalysis would have suggested a diagnosis of rhabdomyolysis in 9 of 16 patients tested.

Neurologic presentations of mitochondrial disorders.

This article describes the neurologic presentations of children with mitochondrial disorders. The charts of 42 children with highly suspect mitochondrial disorders were reviewed. Thirty-seven children were diagnosed as having definite mitochondrial disorders based on a suggestive clinical presentation and at least one accepted criteria, while in five patients the diagnosis remained probable. All patients had nervous system involvement, but it was the presenting symptom in 28 of 42. Eighteen children had normal intelligence and 24 had mental retardation or developmental delay at the onset of their disease. Twenty-five patients had either an acute regression or a progressive encephalopathy. The most frequent neurologic manifestations were abnormal tone, seizures, extrapyramidal movements, and autonomic dysfunction. The eyes were involved in 11 children. Nerve deafness was found in seven patients. Myopathy was found in only six patients. In conclusion, a complex neurologic picture, especially with other organ involvement, warrants a full mitochondrial evaluation.

Encephalopathy as the presenting symptom of Hashimoto's thyroiditis.

In recent years, encephalopathy has increasingly been recognized as a complication of Hashimoto's thyroiditis. It can begin abruptly as a stroke-like event, acute seizures, or confusion, or as an insidious decline in cognitive function. Most reported cases have been on adult patients, although this encephalopathy does affect children as well. This form of encephalopathy should be considered in the differential diagnosis of children and adults with unexplained neurologic deterioration. We describe the case of a child in whom acute encephalopathy was the presenting symptom of Hashimoto's thyroiditis.

Cauda equina syndrome due to lumbosacral arachnoid cysts in children.

We describe the clinical, neuroradiological and surgical aspects of two children in whom symptoms attributable to cauda equina compression were caused by spinal arachnoid cysts. The first patient presented with recurrent urinary tract infections due to neurogenic bladder dysfunction, absent deep tendon reflexes and sensory deficit in the lower limbs. The second child presented with unstable gait as a result of weakness and diminished sensation in the lower extremities. Spinal magnetic resonance imaging revealed a lumbosacral arachnoid cyst in both patients. During surgery the cysts were identified and excised. Two years after surgery, the sensory deficits of the first patient have disappeared and patellar and ankle reflexes can be elicited, but there is no improvement in bladder function. Neurological examination of the second patient was normal. We conclude that the diagnosis of cauda equina syndrome should prompt a vigorous search for its aetiology. Lumbosacral arachnoid cysts are a rare cause of cauda equina syndrome in children.

Demyelinative brainstem encephalitis responsive to intravenous immunoglobulin therapy.

We describe 2 patients with acute demyelinating brainstem encephalitis who were treated with intravenous immunoglobulin (IVIG). Both patients improved rapidly, concomitant with the course of therapy. To the best of our knowledge, these are the first reported cases of brainstem demyelinating lesions in children treated with IVIG. Our experience suggests that IVIG should be considered as first-line treatment for similar cases.

New antiepileptic drugs in children: present and future.

The pharmacologic management of epilepsy has progressed greatly during the last decade. New medications are available for the management of refractory patients, and more are being developed. For some patients, these productions offer added efficacy, better tolerability, and some pharmacodynamic advantages. Since preapproval studies include few pediatric trials, the final role of these medications in the treatment of childhood epilepsy will be dictated by additional studies and postmarketing experience.

Fatal multiple organ failure following massive hornet stings.

We describe a fatal outcome in a three-year-old child following massive stings by the oriental hornet (Vespa orientalis). The primary clinical features were coma, respiratory failure, coagulopathy, renal failure and liver dysfunction. On postmortem the main organs involved were brain, lungs, kidney and liver.