Antiepileptic drug-induced osteopenia in ambulatory epileptic children receiving a standard vitamin D3 supplement.

Dual energy X-ray absorptiometry (DEXA) is a non-invasive, rapid, accurate and highly reproducible method for the assessment of antiepileptic drug (AED)-induced osteopenia in epileptic children. In this study, we investigated bone mineral density (BMD) using DEXA in 56 epileptic children receiving long-term AED treatment for at least 2 years. All children received AED monotherapy or polytherapy plus a standard vitamin D3 supplement (400 U/day). BMD measurements were made from lumbar spine (L2-L4) regions. Age- and sex-specific BMD SD scores were calculated for each child. Osteopenia was defined as SD scores less than -1.5. There was no significant difference in mean BMD values between epileptic children receiving monotherapy or polytherapy. The results were also compared to the age- and sex-specific BMD SD scores obtained from healthy Turkish children. Only three patients (5%) receiving AED therapy had a BMD SD score less than -1.5. This rate is relatively lower than the rates of previous studies conducted on ambulatory children on long-term AED treatment without vitamin D3 supplementation.

Prognostic correlative values of the late-infancy MRI pattern in term infants with perinatal asphyxia.

The aim of this study was to define the risk ratios of the late-infancy magnetic resonance imaging pattern for long-term outcome in term infants with perinatal asphyxia. We evaluated 65 term infants with perinatal asphyxia and performed magnetic resonance imaging examinations between 4-12 months of age. Magnetic resonance imaging scans were classified as follows: (1) periventricular leukomalacia in 21 (32%) infants, (2) marked cortical atrophy in 17 (26%) infants, (3) multicystic encephalomalacia in 10 (15%) infants, (4) deep gray matter involvement in 8 (12%) infants, (5) focal cortical involvement in 6 (9%) infants, (6) myelination delay in 3 (5%) infants. The overall outcome was favorable in 19 (29%) of 65 infants. Infants with diffuse cortical involvement (multicystic encephalomalacia and marked cortical atrophy) are four times (odds ratio: 4.4 and 4.1 respectively) more likely to attain the unfavorable outcome than the infants with other patterns of magnetic resonance imaging. Infants with focal cortical involvement had relatively favorable outcome in 60% of the cases. In conclusion, it appears that the overall outcome of infants with perinatal asphyxia correlated well with the magnetic resonance imaging patterns obtained between 4 and 12 months of age.

Subacute sclerosing panencephalitis: a case with fulminant course after ACTH.

We report a 24-month-old male who developed rapidly progressive subacute sclerosing panencephalitis 17 months after measles infection. This patient had a history of measles infection at the age of 7 months and manifested acute encephalitis 1 month later. Developmental delay observed after encephalitis began to improve after a few months. His control electroencephalogram was normal at the age of 14 months. He was admitted to the hospital with flexor spasms and sudden head drops at the age of 24 months. His electroencephalogram revealed slow waves in the posterior regions of the brain. Vigabatrin was begun; his seizures increased with vigabatrin, and adrenocorticotropic hormone was added to the therapy. After five doses of adrenocorticotropic hormone, his clinical findings deteriorated rapidly. His second electroencephalogram revealed periodic discharges synchronized with myoclonias. He was diagnosed as having subacute sclerosing panencephalitis on the basis of clinical and laboratory findings. He lapsed into a vegetative state within a week and died at the age of 25 months. We report this rapidly progressive case to emphasize the importance of recognition of subacute sclerosing panencephalitis before applying steroids in children with myoclonic seizures.

Deletion analysis and clinical correlations in patients with Xp21 linked muscular dystrophy.

We carried out molecular deletion analysis on 142 patients with Duchenne/Becker muscular dystrophy which covered 25 exons of the dystrophin gene. We also evaluated the results by comparing with the clinical findings and examples in the literature. A deletion ratio of 63.7% was achieved. Exon 46 was the most frequently affected region. Interestingly we also observed four cases with muscle promoter (Mp) region deletions which have been rarely reported in the literature.

Gastroesophageal reflux in children with cerebral palsy: efficacy of cisapride.

The aim of this study was to investigate the presence of gastroesophageal reflux with 24-hour pH monitoring in children with cerebral palsy. In the second part of the study, we started cisapride with the children with documented gastroesophageal reflux and evaluated the efficacy of cisapride with the second 24-hour pH monitoring. This study was performed before discontinuation of cisapride with US Food and Drug Administration reports in Turkish markets. Twenty-eight children who had been followed up in the Department of Pediatric Neurology between 1999 and 2000 were enrolled in the study. Twenty-four-hour pH monitoring was performed on all patients. Two parameters were evaluated as pathologic: a reflux index (percentage of time the pH value was <4) over 4.5% and reflux longer than 15 minutes even when the reflux index was below 4.5%. Cisapride treatment was assigned to the patients with pathologic monitoring results at a dose of 0.2 mg/kg/day for 3 months. Electrocardiograms (ECGs) were analyzed before and after cisapride treatment. Symptoms suggestive of gastroesophageal dysfunction were dysphagia in 18 cases (64.3%), constipation in 8 cases (28.6%), vomiting in 6 (14.2%) cases, and recurrent pneumonia in 2 cases (8.5%). The reflux index was > or =4.5% in 13 (46.4%) of the 28 cases. Reflux was longer than 15 minutes in 2 (7.1%) cases. Cisapride was started in 15 cases with pathologic monitoring results. Appetite improved in 6 cases and dysphagia disappeared in 8 cases after cisapride therapy (P < .05). pH monitoring was repeated in 12 cases after 3 months and was normal in 8 of them. Improvement in the reflux index and total reflux episodes was statistically significant after therapy (P = .008). No adverse effects occurred. Even though the drug is no longer marketed, we concluded that it improved the symptoms and quality of life in spastic children with gastroesophageal reflux.

The use of botulinum toxin type A treatment in children with spasticity.

The current modalities in managing spastic children have some limitations; thus, alternative therapeutic agents are in need. The purpose of this study is to investigate whether intramuscular botulinum toxin type A administration may be an alternative agent in the treatment of children with cerebral palsy. Eighteen children who were aged between 3 and 17 years and manifested cerebral palsy were administered intramuscular botulinum toxin type A with a total dose of 6 U/kg body weight. Outcome measurements were determined with four methods, including Ashworth Spasticity Scale, standardized videotape assessments, observational gait analysis, and walking velocity. Ashworth Spasticity Scale and videotape assessments were statistically significant before and after treatment in all muscles (P < 0.001). The best improvement in video gait analysis was evident at week 8. The botulinum toxin type A injections yielded an improved walking velocity at all visits. The observational gait analysis and walking velocity demonstrated an improvement after treatment in the gastrocnemius-injected group (P < 0.001). In conclusion, intramuscular botulinum toxin type A administration may be effective in children with cerebral palsy, especially at week 4 and when injected in gastrocnemius.

Outcome of axonal and demyelinating forms of Guillain-Barré syndrome in children.

Previous reports have suggested that outcome is worse in the axonal compared with the demyelinating form of Guillain-Barré syndrome (GBS). We performed a retrospective study of 23 children with electrophysiologically confirmed cases of predominant subtypes of GBS to investigate this issue. The patients were classified based on the electrodiagnostic features: Ten (44%) had acute inflammatory demyelinating polyradiculoneuropathy, eight (35%) had acute motor axonal neuropathy, and five (21%) had acute motor-sensory axonal neuropathy. All patients received a standard intravenous immunoglobulin therapy (0.4 g /kg /day for 5 consecutive days). In the acute phase of the disease, patients with the axonal forms of GBS were more disabled than were those with the demyelinating GBS, as measured by GBS scores. Mechanical ventilation was required in five (38%) patients in the axonal group compared with one (10%) patient in the demyelinating group. There was no significant difference at 6 months in GBS scores between demyelinating and axonal forms of GBS. All 20 survivors recovered completely by 12 months. After standard intravenous immunoglobulin therapy, children with axonal forms of GBS recover more slowly than those with the demyelinating form, but outcome at 12 months appears to be equally favorable in two groups.

Tension-type headache in children: a clinical evaluation.

BACKGROUND: Headache Society (IHS) criteria for episodic tension-type headache were included in the present study. Pain characteristics, associated symptoms, and stress-triggering factors were evaluated. Psychiatric and psychosocial evaluations were performed according to DSM-IV criteria. RESULTS: Pain was bilateral in 93.7% of patients and bitemporal in 50% of children. The intensity of pain increased with motion and stress in more than half of the patients, while pain decreased with rest and massage in 43.7% of patients. Ten of the 16 (62.5%) patients were diagnosed as having a psychiatric disorder. The most common stress-triggering factors were difficulty in adaptation at school and relationship problems with family members. All of the children reported 26 stress factors. Of these stress factors, 20 (76.9%) were reported by children diagnosed with psychiatric disorder. CONCLUSION: These results suggest that in children with tension-type headache a thorough psychiatric evaluation should be performed to rule out underlying psychiatric disorders.

Oxcarbazepine in the treatment of childhood epilepsy.

In this study, oxcarbazepine was began as monotherapy to evaluate the efficacy and safety of the drug. Forty-two patients (19 females, 23 males) with partial or generalized epilepsy more than 4 years of age were included (mean age, 11.9 +/- 3.4 years). The mean age at epilepsy onset 8.9 +/- 4 years. Complete blood count, liver function tests, electrolytes, lipid levels, electrocardiography, electroencephalography, and magnetic resonance imaging were performed in all patients. Oxcarbazepine dose was begun at 10 mg/kg/day twice daily and increased to 30 mg/kg/day at the end of the second week. Patients with inadequate seizure control even with the dose of 45 mg/kg/day or intolerable side effects were excluded. Intolerable headache and leukopenia led to discontinuation of the drug in two patients. At the sixth month, 35 of the patients (87.5%) were seizure free (91.7% of the generalized epilepsy patients and 81.2% of the partial epilepsy patients). The most frequent tolerable side effect was drowsiness in 12 patients. As a result, we found oxcarbazepine safe and effective in children with either generalized or partial epilepsy.

[Landau-Kleffner syndrome (acquired epileptic aphasia)]. / Landau-Kleffner sendromu (edinsel epileptik afazi).

Landau-Kleffner syndrome is characterized by a complex group of symptoms including deterioration in language skills, seizures, and abnormal electroencephalography findings. A six-year-old male patient had aphasia for three years and generalized tonic-clonic epileptic seizures for two years. Pure-tone audiometry and auditory brain-stem response audiometry findings were normal. He had verbal auditory agnosia rather than true aphasia. Cranial computed tomography and magnetic resonance imaging studies did not show any abnormal findings. Single-photon emission computed tomography showed hypoperfusion in the right hemispheric temporal lobe. Despite treatment with corticosteroids and intravenous immunoglobulins, multiple exacerbations were interspersed in a four-year follow-up period.

Sodium valproate prophylaxis in childhood migraine.

UNLABELLED: Migraine is a cause of recurrent headache in childhood. The efficacy of sodium valproate is well known in the prophylactic treatment of adult migraine, but there are few studies involving the drug's effect in pediatric migraine. OBJECTIVE: To determine the efficacy of sodium valproate in the prophylactic treatment of childhood migraine. METHODS: Fifteen children with migraine according to International Headache Society criteria were included in the study. Headache severity was measured and assessed by Algology unit by a using visual analog scale and a numerical rating scale. All of the subjects were asked to keep a headache diary for 8 weeks. Three subjects who had no headache attacks during the baseline period and two cases who were lost to follow up were excluded. Thus, sodium valproate was initiated in 10 subjects (six boys, four girls), 500 mg/night, and the daily dose was increased up to 1000 mg according to blood levels. Their ages ranged from 9 to 17 (mean age 13.6 +/- 3.2 years). Therapy continued for at least 12 weeks. RESULTS: Headache severity as measured via the mean visual analog score was 6.8 +/- 1.8 at baseline and was 0.7 +/- 1.2 at the end of the treatment period (P = 0.000). Mean headache attacks per month were 6 +/- 4.2 at baseline and were 0.8 +/- 1.9 at the end of the treatment period (P = 0.002). The duration of headache was significantly decreased from a mean of 5.5 +/- 3.9 hours to 1.1 +/- 2.5 hours with treatment (P = 0.001). The observed side effects were dizziness, drowsiness, and increase in appetite; none required drug withdrawal. In two cases, headache attacks recurred after the cessation of valproate, and therapy was restarted. Headache control lasted for six months following cessation of the drug in the remainder of the subjects. CONCLUSION: Sodium valproate appears to be effective and safe in selected patients with childhood migraine.

Reflex sympathetic dystrophy in childhood.

Reflex sympathetic dystrophy is characterized by constant burning pain and hyperesthesia in an extremity. Lower extremities are usually affected. Pain is accompanied by swelling, sweating, vasomotor instability and sometimes trophic changes. There may be a history of minor injury or not. Muscle spasms, myoclonus or focal dystonia may occur. Diffuse pain, loss of function and autonomic dysfunction are three main criteria suggested for diagnosis. Symptoms can last a few days to as long as a year. In this report we present a girl with multiple limb involvement of stage I RSD. The sympathetic skin responses were tested during a remission period. She had milder attacks with a recurrence rate of 4 per year in the following three years from onset.

Coagulation abnormalities and acquired von Willebrand's disease type 1 in children receiving valproic acid.

Valproic acid is commonly used in the management of childhood epilepsy. The known hematologic side effects of the drug are hemorrhagic diatheses, thrombocytopenia, and hypofibrinogenemia. We studied coagulation parameters in 29 epileptic children receiving valproic acid for at least 6 months. Their ages ranged between 2 and 18 years (10.2 +/- 4.9 years). The total valproic acid dose was 250 to 1000 mg/day equivalent to 20 to 30 mg/kg/day. Treatment duration ranged from 6 to 57 months. These children had not previously had a hemostatic defect and had no family history of bleeding disorders. Platelet count, prothrombin time, activated partial thromboplastin time, bleeding time, fibrinogen, platelet aggregation assays, and ristocetin cofactor activity levels were determined in all of the patients, but von Willebrand's factor antigen levels could be determined in only 14 patients. The values of von Willebrand's factor antigen ranged from 53 to 218% (104.1 +/- 42.3), and ristocetin cofactor activity levels ranged from 11.5 to 218% (94.5 +/- 43.1). Six of the 29 children (20.7%) had decreased values of ristocetin and cofactor activity and were considered to have acquired von Willebrand's disease. The decreases in coagulation parameters were not dependent on either valproic acid dose or treatment duration. Two patients with low ristocetin cofactor activity values had mild epistaxis, which did not require discontinuation of therapy. In patients receiving valproic acid therapy, this side effect must be considered, especially before surgical intervention and serious traumatic conditions.

Hemiplegic migraine with prolonged symptoms: case report.

Hemiplegic migraine is defined by the occurrence of migraine during attacks of unilateral weakness. Neurologic symptoms last for 15 to 60 minutes in most cases. Attacks usually start in childhood, adolescence, or early adulthood. Diagnosis may be delayed if there is no relevant family history. A 16-year-old girl who was diagnosed with hemiplegic migraine presenting with prolonged left hemiparesis is reported. The importance of this case is that the pediatrician will also consider migraine in the differential diagnosis of a child presenting with hemiparesis even if there is no previous headache and family history.

Landau-Kleffner syndrome beginning with stuttering: case report.

Landau-Kleffner syndrome is marked by an acquired aphasia in children who have had normal language and motor development. A 3.5-year-old girl was referred to our clinic with stuttering. She was diagnosed as having benign myoclonic epilepsy of infancy at 3.5 months of age and treated with valproic acid. Her electroencephalogram (EEG) returned to normal at the end of the first year. The therapy was stopped after a 2-year seizure-free period. She started to stutter prominently 3 months after the discontinuation of antiepilepsy drugs. She had no verbal agnosia. Her EEG revealed multiple spike and wave discharges. She was diagnosed as having Landau-Kleffner syndrome. Her previous epilepsy history had contributed to her having obtained an EEG in the early period. We suggest that if a child with normal language function starts to stutter, Landau-Kleffner syndrome must be considered in the differential diagnosis.