Haydar Ali Tasdemir

Dyke–Davidoff–Masson syndrome

We reported five cases of Dyke-Davidoff-Masson syndrome (DDMS) with different clinical and radiological findings. The evaluated parameters were the location of the lesions, midline structural shift effect, pathological and morphological changes on the ipsilateral calvarium, paranasal sinuses and mesencephalon, presence of compensatory contralateral hypertrophy. With the help of both magnetic resonance (MR) and computerized tomography (CT) images, changing degrees of all the evaluated parameters were observed in all five of our patients. In conclusion, no relationship was found between parenchymal and calvarial changes and between the time after onset of the disease and amount of the morphologic and pathological changes.

The influence of valproic acid treatment on hair and serum zinc levels and serum biotinidase activity.

Some of the side effects that develop during long-term valproic acid (VPA) treatment are similar to symptoms of zinc and partial biotinidase deficiencies. This situation suggests an association between these side effects and biotinidase and/or zinc deficiencies. In 32 pediatric patients (22 females, 10 males) receiving VPA treatment, hair and serum zinc levels and serum biotinidase activity (BA) were measured prior to and in the 3rd and 6th months of treatment. Also, serum VPA levels were measured in the 3rd and 6th months of treatment. The mean serum and hair zinc levels were found to be reduced in the 3rd and 6th months of treatment as compared with the pre-treatment values, while the mean serum BA was lower than the pre-treatment values in the 3rd month of treatment, but returned to initial values in the 6th month of treatment. In the 3rd and 6th months of treatment, patients complaining about hair loss had lower hair and serum zinc levels and serum BA but greater mean serum VPA than those who did not. However, the differences between parameters were not statistically significant. Our findings suggest that hair loss in patients can be attributed to zinc and BA depletion within the first 3 months, and to zinc depletion only by the 6th month.

Cerebral vasculitis in a child with Henoch-Schönlein purpura and familial Mediterranean fever.

In this case report, a 10-year-old girl with Henoch–Schönlein purpura (HSP) with severe central nervous system involvement and also having familial Mediterranean fever (FMF) is presented.

Sturge-Weber syndrome involved frontoparietal region without facial nevus

Classic Sturge-Weber syndrome is characterized by a facial nevus and hamartomatous lesion in the brain. Hamartomatous lesions are usually located in the ipsilateral occipital region of the facial nevus. The other lobes may be involved. A few cases of Sturge-Weber syndrome without facial nevus have been reported. A 9-month-old male was admitted with the complaint of afebrile seizures two times. We observed the third seizure, which was complex partial, in the clinic. There was no facial hemangioma. On computed tomography and magnetic resonance imaging of the brain we observed findings concordant with the angioma in the right frontoparietal region, although the occipital region was intact. This patient appears to be the first with Sturge-Weber syndrome without facial nevus, and with involvement of the frontoparietal region but not the occipital region. We do not know the embryologic basis of this association.

Gallbladder motility in children with Down syndrome.

Objective: The aim of this study was to investigate gallbladder motility in children with Down syndrome by measuring gall-bladder volume and contraction index. Methods: This study, performed between January 2001 and December 2002 at the Ondokuz Mayis University, School of Medicine, Department of Paediatric Neurology, Samsun, Turkey, included 21 patients with Down syndrome (study group) and 22 healthy children (control group). After an 8-hour fast, gallbladder diameters in both groups were measured in length, width, and height by ultrasonography before and 30 minutes after a test meal. The volume of gallbladder before and after a test meal was determined, and the contraction index was calculated. Blood triglyceride and cholesterol levels were measured, and 5-hydroxyindoleacetic acid (5-HIAA) levels in urine were determined. Results: Mean gallbladder volume before test meal in the study group and controls was 8,412.4 ± 5,174 mm3 and 16,516.8 ± 6,796.1 mm3 (P < 0.001), respectively. The mean contraction index of the study group was 41.2% ± 19.4% and of controls, 75.0% ± 12.3% (P < 0.001). The mean triglyceride level of the study group was significantly higher than controls (P < 0.05). The mean urine 5-HIAA level of the study group was lower than controls (P < 0.05). Conclusion: CI was lower in patients with Down syndrome, suggesting gallbladder hypomotility. Hypomotility may be a feature associated with the high prevalence of gallstones in Down syndrome.

Pseudotumor cerebri complicating measles : A case report and literature review

A previously healthy 8-year-old girl patient was referred with the complaints of severe headache associated with nausea and vomiting. Three weeks prior to her admission, she had measles manifested with fever and typical skin eruptions. Fundoscopic examination revealed bilateral swollen optic discs with tortuous blood vessels. Other physical examinations were unremarkable, except for photophobia. Lumbar puncture demonstrated a cerebrospinal fluid opening pressure of 30 cm H(2)O, no cells, normal levels of glucose and protein. Serum measles immunoglobulin M level was elevated (183 AU). Our further investigations revealed that the patient had pseudotumor cerebri (PTC) following measles infection.

Self-Esteem and Psychiatric Features of Turkish Adolescents with Psychogenic Non-Epileptic Seizures: A Comparative Study with Epilepsy and Healthy Control Groups

Objective: Children and adolescents with psychogenic non-epileptic seizures (PNES) and epilepsy are known to have psychosocial problems. The aim of the present study was to compare the psychosocial difficulties, history of stressful life events/abuse, psychiatric diagnosis, and self-esteem of adolescents with PNES to the ones with epilepsy and healthy controls at a tertiary care center in Turkey. Method: Thirty-four adolescents with PNES diagnosed by video-EEG were compared with 23 adolescents that have epilepsy and 35 healthy volunteers. Comorbid psychiatric diagnoses of participants were examined by semi-structured interviews using Schedule for Affective Disorders and Schizophrenia for School Age Children—Present and Lifetime Version (KSADS-PL). Self-esteem of adolescents was evaluated by Rosenberg Self Esteem Scale (RSES). Results: No differences in sociodemographic features were observed between the groups. The PNES group showed significantly higher rates of parental conflicts, difficulties in relationship with siblings/peers, school under-achievement, and history of stressful events/abuse. The rates of comorbid psychiatric disorders were 64.7% in PNES and 47.8% in epilepsy group. The most common disorders in both groups were attention deficit hyperactivity disorder (ADHD) and depressive disorder. The rate of posttraumatic stress disorder (PTSD) was significantly increased in the PNES group. Additionally, adolescents with PNES displayed significantly lower levels of self-esteem than the other groups. Conclusion: It could be concluded that both disorders involved a high risk for developing psychiatric disorders; additionally, adolescents with PNES have higher rates of stressors and lower levels of self-esteem. Findings from this investigation point to the importance of psychiatric interventions in pediatric PNES and also epilepsy.

Intravenous immunoglobulin for Guillain-Barré syndrome: how effective?

Guillain-Barré syndrome is an acute inflammatory demyelinating neuropathy characterized by progressive symmetric polyradiculoneuritis, predominantly manifested by weakness and areflexia. In this article, we report our findings in 25 children treated with intravenous immunoglobulin and compare them with the remaining 30 children who received supportive care only. Only supportive care was given to 30 children who were not able to receive intravenous gammaglobulin because of shortcomings in intravenous gammaglobulin availability owing to a poor import during those years. Twenty-five patients were treated with intravenous gammaglobulin; they received intravenous gammaglobulin 0.4 g/kg/day for 5 consecutive days. Seventeen of the intravenous gammaglobulin group had received intravenous gammaglobulin within 10 days after the first symptoms, and eight of them had received intravenous gammaglobulin after the first 10 days. The average time elapsed for the symptoms to reach the maximum level was 6.9 (range 4—12) days in patients receiving intravenous gammaglobulin in the first 10 days, and it was significantly shorter than the time elapsed for the supportive care group (6.9 versus 8.8 days, respectively) (P < .05). Admission to the hospital after the first symptom, disability grade, time to improve in disability grade, the period of hospitalization, and mortality were not different in the intravenous gammaglobulin and supportive care groups (P > .05). Our suggestion for intravenous gammaglobulin treatment in Guillain-Barré syndrome is that if the patient has risk factors for respiratory insufficiency, then the treatment should be started. We more confidently carry out the follow-up of these patients after the results of this study. In conclusion, although it has been reported that intravenous gammaglobulin facilitates improvement in the disease and the decrease in mortality in children with Guillain-Barré syndrome, it has been mentioned in some studies that the intravenous gammaglobulin treatment was not better than supportive care, as in our study. However, further studies are essential to determine when intravenous gammaglobulin should be given to patients having which clinical and laboratory findings. (J Child Neurol 2006;21:972—974; DOI 10.2310/ 7010.2006.00214).

An epileptic case with mosaic ring chromosome 6 and 6q terminal deletion

Ring chromosomes are rare chromosome disorders that arise usually de novo. Children with ring chromosome 6 have a wide range of intellectual functioning and congenital anomalies. We report an epileptic case of a 10-year-old boy to be mild psychomotor retardation and dysmorphic traits including microcephaly, brachycephaly, flat occiput, large and apparently low set ears, and bilateral syndactyly between his second and third fingers with mosaic ring chromosome 6 and 6q terminal deletion. Peripheral chromosome and fluorescent in situ hybridisation (FISH) analysis of the patient showed mos46,XY,r(6)(p24;q26),del(6)(q27) [30]/46,XY,del(6)(q27) [20] de novo. We presented the patient in the light of literature because the mosaic ring 6 and 6q terminal deletion was different caryotypically from other mosaic ring 6 patients.

Evaluation of Nerve Conduction Studies in Obese Children With Insulin Resistance or Impaired Glucose Tolerance

The aim of the study was to investigate nerve conduction studies in terms of neuropathic characteristics in obese patients who were in prediabetes stage and also to determine the abnormal findings. The study included 69 obese adolescent patients between April 2009 and December 2010. All patients and control group underwent motor (median, ulnar, tibial, and peroneal) and sensory (median, ulnar, sural, and medial plantar) nerve conduction studies and sympathetic skin response test. Sensory response amplitude of the medial plantar nerve was significantly lower in the patients with impaired glucose tolerance and insulin resistance. To our knowledge, the present study is the first study demonstrating the development of sensory and autonomic neuropathy due to metabolic complications of obesity in adolescent children even in the period without development of diabetes mellitus. We recommend that routine electrophysiological examinations be performed, using medial plantar nerve conduction studies and sympathetic skin response test.