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.

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.

Outpatient treatment of serious community-acquired pediatric infections using once daily intramuscular ceftriaxone.

Pediatric patients with serious infections are usually hospitalized for parenteral antibiotic treatment. We studied prospectively 74 pediatric patients with community-acquired serious infections and used once daily intramuscular ceftriaxone. Seventeen patients (23%) were initially hospitalized and 57 (77%) patients were treated entirely as outpatients. An initial intramuscular dose of 75 mg/kg was followed by daily doses of 50 mg/kg (maximum, 1.5 g). Infections treated included periorbital/buccal cellulitis, other cellulitis, urinary tract infections, pneumonia, osteomyelitis, mastoiditis, suppurative arthritis and orbital cellulitis. Organisms were recovered from cultures of 37 (50%) patients and 6 (8%) patients were bacteremic. Bacteria included Gram-positive (mostly Staphylococcus aureus) and Gram-negative (mostly enteric bacilli and Haemophilus influenzae organisms). No serious side effects were observed. Of 74 patients 72 (97%) were cured and improvement was usually observed within 24 hours. Two patients did not improve: one with chronic Pseudomonas mastoiditis; and one with lung abscess. Based on previous experience it is estimated that 376 hospitalization days were saved. All 72 successfully treated patients and their parents resumed normal activity within 72 hours of starting therapy. Our data suggest that ceftriaxone can be used for outpatient treatment of some infectious diseases.

Safety and immunogenicity in young infants of Haemophilus b-tetanus protein conjugate vaccine, mixed in the same syringe with diphtheria-tetanus-pertussis-enhanced inactivated poliovirus vaccine.

Because inactivated poliovirus vaccine (IPV) and Haemophilus influenzae b vaccine are advised in many programs and may be incorporated further in other programs, we undertook a study to determine whether the administration of a tetravalent preparation of diphtheria-tetanus-pertussis-IPV mixed in one syringe with tetanus-conjugate H. influenzae b vaccine (DTP-IPV-PRPT) is associated with increased reactogenicity or interference with immunogenicity of individual vaccine components. In a placebo-controlled, double blind study, a total of 161 infants were enrolled (80 DTP-IPV-PRPT and 81 DTP-IPV-placebo). Vaccine was administered at 2, 4 and 6 months of age. Oral poliovirus vaccine was added at 7 months of age and a booster of oral poliovirus vaccine and DTP-IPV was also administered at 12 months of age, according to the policy in Israel. Local and systemic side effects were similar in both groups except for irritability after the second dose and use of acetaminophen which we observed slightly but significantly more often in the DTP-IPV-PRPT recipients. After the third dose the geometric mean titers of anti-polyribosyl-ribitol phosphate antibodies were 3.7 and 0.05 micrograms/ml in the PRPT and placebo groups, respectively (P < 0.001). Higher tetanus antitoxin titers were observed among recipients of DPT-IPV-placebo (1.1 IU/ml vs. 0.7 IU/ml, P = 0.003). A similar trend was found for pertussis agglutinin titers (93.4 vs. 65.4, P = 0.054). No difference was observed for anti-diphtheria toxoid and poliovirus 1, 2, and 3.(ABSTRACT TRUNCATED AT 250 WORDS)

Safety and immunogenicity of Haemophilus type b-tetanus protein conjugate vaccine, mixed in the same syringe with diphtheria-tetanus-pertussis vaccine in young infants.

As new vaccines are developed there is increasing interest in reducing the number of injections given to children by combining vaccines in one syringe. We studied the safety and immunogenicity of Haemophilus influenzae type b-tetanus protein conjugate vaccine (PRP-T) administered at ages 2, 4 and 6 months mixed in the same syringe with DTP vaccine and its effects on the seroresponse to DTP vaccine. A group of 112 healthy 2-month-old infants received DTP-PRP-T or DTP-placebo mixed immediately before immunization in the same syringe. The addition of PRP-T to DTP did not increase the rate of local or systemic reactions. After the first, second and third dose, the PRP-T recipients showed a geometric anti-PRP antibody mean of 0.13, 2.31 and 6.40 micrograms/ml vs. 0.07, 0.05 and 0.05 micrograms/ml among the DTP-placebo recipients, respectively. Of the PRP-T recipients, 94 and 98% attained antibody concentration of greater than or equal to 0.15 micrograms/ml protein after the second and third dose, respectively, and 65 and 94% attained a concentration of greater than or equal to 1.0 micrograms/ml after the second and third dose, respectively. At the age of 1 year 94 and 52% of the DTP-PRP-T recipients vs. 12% and 0% of the placebo recipients still maintained titers of greater than or equal to 0.15 and greater than or equal to 1.0 micrograms/ml, respectively. The administration of DTP in the same syringe with PRP-T did not affect significantly the antibody response to diphtheria and tetanus toxoid and to pertussis agglutinins. It is concluded that PRP-T vaccine could be administered in the same syringe as DTP.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Phytophotodermatitis due to figs.

Phytophotodermatitis is an acute skin reaction that may be easily confused with other causes of contact dermatitis. It is characterized by sunburn, blisters, and/or hyperpigmentation. The reaction takes place when certain plant substances known as psoralens, after being activated by ultraviolet light from the sun, come in contact with the skin. The condition is usually mild and self-limited but hyperpigmentation may persist for many weeks. Failure to recognize phytophotodermatitis in a child may lead to a mistaken diagnosis of child abuse. It is also important to be aware of perfumes and grocery products as causes of this phenomenon.

[Childhood brucellosis in the Negev].

33 children (22 girls) with brucellosis seen between 1972-1988 were studied retrospectively. All but 1 were Bedouins. The mean age at diagnosis was 9.8 years (range: 17 months-17 years). Duration of illness prior to diagnosis was less than 1 week in 13 (39%), 1-4 weeks in 8 (24%) and 1-3 months in 10 (30%). In 2 cases the symptoms lasted 6 and 8 months, respectively, before diagnosis. Presenting symptoms included fever (85%), articular involvement (65%), hepatomegaly (45%) and splenomegaly (33%). Less common manifestations were anorexia (30%) and weight loss (15%) cases. Meningoencephalitis developed in 2 patients and uveitis and glomerulonephritis in 1 each. Diagnosis was based on positive agglutination titers (greater than 160), which were found in all. Brucella melitensis was isolated in blood cultures in 8 of the 33. 18 were treated with tetracycline and 9 with tetracycline and streptomycin, all of whom responded well. 3 of the 6 treated with trimethoprimsulphamethoxazole were only cured when therapy was changed to tetracycline in 2 and tetracycline plus streptomycin in 1. All patients recovered without sequelae. We conclude that brucellosis due to Brucella melitensis is endemic among the Bedouin of the Negev. An increased incidence of brucellosis among hospitalized children has been noted in the past 2 years, indicating the need for diagnostic awareness.