Aysel Öztürk

Comparison of the Effectiveness of Topiramate and Sodium Valproate in Pediatric Migraine

Frequent migraine headaches can have a significant impact on disability, prompting the need for early recognition and treatment. The objective of this study is to compare the efficacy of topiramate and sodium valproate for the prevention of pediatric migraine, retrospectively. Mean monthly migraine frequency, intensity, and duration in the 28 patients treated with topiramate decreased from 15.3 ± 10.1 to 4.4 ± 5.5 episode, from 6.8 ± 1 to 3.2 ± 1, and from 10.2 ± 9.4 to 2.4 ± 3.1 hours, respectively. Headache disability improved with a reduction of Pediatric Migraine Disability Assessment score from 36 ± 29.5 to 4.6 ± 6.5 (P < .05). Similarly, mean monthly headache frequency, headache intensity, headache duration, and Pediatric Migraine Disability Assessment score in the 20 patients treated with sodium valproate decreased from 20.1 ± 10.2 to 6.6 ± 8.6, from 7.1 ± 1 to 3.4 ± 2.1, from 7 ± 12 to 1.4 ± 2.5 hours, and from 20.5 ± 16.1 to 5.5 ± 9.2, respectively (P < .05). In conclusion, valproate and topiramate seem to be able to manage successfully childhood migraine without substantial differences in efficacy.

Effect of nutritional support in children with spastic quadriplegia.

Malnutrition is a common problem in patients with cerebral palsy. We evaluated the effect of nutritional support on clinical findings in children with spastic quadriplegia. Feeding history, numbers of lower respiratory tract infections, and gastrointestinal and neurologic findings were evaluated via questionnaire. Weight, height, head circumference, midarm circumference, and triceps skinfold thickness were measured. Height for age, weight for age, weight for height, body mass index, and weight and height z-scores were calculated. Clinical findings and anthropometric parameters were re-evaluated after nutritional support for 6 months. Forty-five patients were enrolled. No difference was evident between the first and the last height z-scores of 31 patients who completed the follow-up. Weight, height, weight z-scores, weight for age, weight for height, body mass index, midarm circumference, and triceps skinfold thickness exhibited improvement. Moreover, a significant decrease in number of infections was evident. Frequency of seizures and Gross Motor Function Classification System status did not change. Constipation decreased significantly. Nutritional therapy revealed improvements in some anthropometric findings and a decrease in number of infections. Although there was no difference regarding motor development or seizure frequency, further studies with a longer follow-up are required.

Humoral immune response against 38- and 16-kDa mycobacterial antigens in childhood tuberculosis.

Several enzyme‐linked immunosorbent assays (ELISAs) based on mycobacterial antigens have been tried for the rapid diagnosis of tuberculosis (TB). In this study, the value of the 16 and 38‐kDa mycobacterial antigens in the diagnosis of TB was investigated in pediatric patients in Izmir, Turkey in whom they were found using clinical and/or bacteriological methods. A commercial ELISA kit was used for measuring IgG against 38 and 16‐kDa recombinant antigens. The humoral immune response was analyzed in a group of 32 TB patients (24 pulmonary, 3 lymphadenitis and 2 pleuritis, 2 meningitis and a disseminated TB) and in control groups consisting of 20 healthy children and 20 pulmonary diseases other than TB cases. The sensitivity, specificity, positive predictive value, and the negative predictive value of the test were found to be 25%, 90%, 66.7%, and 60%, respectively, in the TB cases. The ELISA test shows very good specificity, but low level of sensitivity and negative predict value. It was thought that it might be used in combination with other methods to increase diagnostic accuracy, especially for culture‐negative TB pediatric cases, which are difficult to diagnose. Pediatr Pulmonol. 2009; 44:839–844.

Nephrocalcinosis in glucose-galactose malabsorption: nephrocalcinosis and proximal tubular dysfunction in a young infant with a novel mutation of SGLT1.

We report an association of proximal renal tubular dysfunction in a 50-day-old girl with glucose-galactose malabsorption who was found to have nephrocalcinosis, but no sign of nephrolithiasis. A novel homozygous nonsense mutation at 267Arg →stop (CGA→TGA) in the Na+-dependent glucose transporter (SGLT1) was found in loop 5 connecting transmembrane segments 6 and 7, indicating the complete loss of glucose transport activity. This case indicates that hypercalcaemia, nephrocalcinosis and proximal tubular dysfunction may be seen in association with glucose-galactose malabsorption and that most of these abnormalities improve with a glucose-galactose-free diet.

Sarcolemmal alpha and gamma sarcoglycan protein deficiencies in Turkish siblings with a novel missense mutation in the alpha sarcoglycan gene.

BACKGROUND The sarcoglycan alpha gene, also known as the adhalin gene, is located on chromosome 17q21; mutations in this gene are associated with limb-girdle muscular dystrophy type 2D. We describe two Turkish siblings with findings consistent with limb-girdle muscular dystrophy type 2D. The evaluation excluded a dystrophinopathy, which is the most common form of muscular dystrophy. PATIENTS Both siblings had very high levels of creatinine phosphokinase and negative molecular tests for deletions and duplications of the dystrophin gene. The older boy presented at 8 years of age with an inability to climb steps and an abnormal gait. His younger brother was 5 years old and had similar symptoms. The muscle biopsy evaluation was performed only in the older brother. RESULTS The muscle biopsy showed dystrophic features as well as a deficiency in the expression of two different glycoproteins: the alpha sarcoglycan and the gamma sarcoglycan. Sarcolemmal expressions of dystrophin and other sarcoglycans (beta and delta) were diffusely present. DNA analysis demonstrated the presence of previously unknown homozygous mutations [c.226 C > T (p.L76 F)] in exon 3 in the sarcoglycan alpha genes of both siblings. Similar heterozygous point mutations at the same locus were found in both parents, but the genes of beta, delta, and gamma sarcoglycan were normal in the remaining family members. CONCLUSIONS We describe two siblings with limb-girdle muscular dystrophy type 2D with a novel missense mutation. These patients illustrate that the differential diagnosis of muscular dystrophies is impossible with clinical findings alone. Therefore, a muscle biopsy and DNA analysis remain essential methods for diagnosis of muscle diseases.

SUNCT syndrome in a child: a rare cause of paroxysmal headache.

Ann Saudi Med 28(5) September-October 2008 www.kfshrc.edu.sa/annals 386 SUNCT (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing) syndrome is a rare type of headache, first described in 1978 by Sjaastad.1 The syndrome is characterized by mild-to-severe burning, stabbing or electrical orbital/periorbital headache accompanied by autonomic signs. Increased intraocular pressure and eyelid edema occur on the symptomatic side in the course of headache attacks. Two to 40 pain attacks, each lasting for 2 to 60 minutes, may occur daily. Most SUNCT cases have been observed in men at a mean age of 50 years.2 In this report, we describe a rare case of a 10year-old child who was diagnosed with this syndrome.

Recurrent peripheral facial palsy in a child with familial Mediterranean fever.

BACKGROUND Recurrent peripheral facial palsy is uncommon in children. It mostly occurs as an idiopathic disorder and to a lesser extent in the setting of some infectious, genetic, or systemic disorders. However, its association with familial Mediterranean fever has not been reported before. PATIENT We present a 14-year-old girl who experienced three episodes of right-sided peripheral facial palsy during a 9-month interval. She had a diagnosis of familial Mediterranean fever (homozygous with M694V mutation) and she had been receiving colchicine for 8 years. Recurrent peripheral facial palsy could be a neurological manifestation of vasculitis in familial Mediterranean fever. CONCLUSION Recurrent peripheral facial palsy may be a manifestation of familial Mediterranean fever in children.

Steroid-induced psychosis in a child: treatment with risperidone

Steroid-induced psychosis is one of the most serious adverse effects of steroid therapy but is a little-known complication in children. There is no clear mechanism model for steroid-induced behavioral disturbance, but it may be related with dose or level of free fraction of steroids. Our case is a 12-year-old boy diagnosed with steroid-induced psychosis and treated with risperidone, an atypical antipsychotic, due to distinct psychotic symptoms. Pediatricians should be aware of this rare complication when administering corticosteroids for various medical illnesses.

Lack of association of childhood partial epilepsy with brain derived neurotrophic factor gene.

Brain-derived factor (BDNF) is a member of neurotrophin family and is localized and upregulated in areas implicated in epileptogenesis. Several lines of evidence make the BDNF gene a plausible candidate gene for predisposition to epilepsy. In this study, we tested that BDNF might be involved in the etiology of childhood PE. To assess whether BDNF gene C270T polimorphism could be implicated in vulnerability to PE, we conducted a case-control association analysis (112 partial epileptic and 100 controls) in Turkish children. Epileptic children were divided into two groups: 1—idiopathic (n = 85) and 2—symptomathic epilepsy (n = 27). There was no significant difference in genotypic distribution and allelic frequencies of the BDNF gene C270T polimorphism between the PE and control groups. However, the BDNF gene TT genotype was more frequently seen in the epileptic children (15 versus 11 patients, resp.). Interestingly, in the epilepsy group, both two children with TT genotype have posttraumatic epilepsy. The data indicate a possible association with the 270T genotype of the BDNF gene with a posttraumatic epilepsy. To draw any conclusion, further studies using larger sample sizes should be carried out in various ethnic populations in childhood epilepsies.

Visceral Larva Migrans Presenting with Hypereosinophilia

Toxocariasis is an infection caused by the ingestion of larvae of the dog Toxocara canis or the cat Toxocara cati. A 2.5 year old boy was admitted to our clinics with fever, abdominal pain and loss of appetite. His medical history included geophagia (pica) and amebiasis infection. On admission, the physical examination revealed hepatomegaly and pallor. There was marked eosinophilia with leukocytosis, anemia, hypergammaglobulinemia and elevated serum Ig E titers. Toxocariasis was confirmed by anti-Toxocara IgG and Western blot. After 7 days of albendazole therapy, leukocytosis persisted and a second course of albendazole combined with prednisolone was administered. After 3 weeks, the eosinophil count had decreased and the patient showed resolution of hepatomegaly, but Toxocara serology remained elevated.