Intractable seizure disorders: efficacy of the classic ketogenic diet

The ketogenic diet is a high-fat, low carbohydrate, adequate protein diet, developed in the 1920s for the management of intractable seizure disorders in children. To evaluate efficacy and tolerability of the classic ketogenic diet, we analyzed records of the children started on the diet from 1999 to 2006 at the Mofid children's hospital. The subjects were 87 children, mean age 55 months. Before initiation of the diet, 55% of the patients had seizures, at least 1-4 times per day, 36%-5 or more per day and 9%-2 to 4 times per week. Mean number of Anti Epileptic Drugs [AEDs] tried for them was 8 and 67% were receiving three or more drugs. The ketogenic diet showed drastic improvement, with at least 50% reduction in seizure frequency in 87% of our patients, 39% of whom showed complete seizure control in the third month. After one year, in 80% of the patients who returned, improvement continued, with 26% of them being seizure free; besides, 23% had one AED decreased, 36% had two or three AEDs decreased, and 25% [one child] had all AEDs discontinued. Of the 30 improved cases, 20%, at the end of the first year, had improved behavior as well, and 23% of them had become more alert. The medium diet duration of the improved group was 15 months. The improvement in our patients, low side effects, and the duration of diet by families reveal that the ketognic diet can still be a very useful alternative therapy in certain epileptic children

Primary central nervous system lymphoma

Primary central nervous system lymphoma [PCNSL] is an extremely rare condition in childhood. We report the first case of PCNSL in a child in Iran. A nine-year-old boy was referred to Mofid Hospital with the history of headache of four months and seizure of 2 months duration. Magnetic resonance imaging of the brain revealed a hyper-intense lesion in left fronto-parietal area with secondary satellite lesions. Biopsy of the brain mass was performed. Pathologic findings showed brain lymphoma and immunohistochemistry confirmed this diagnosis. The treatment started with intrathecal and systemic chemotherapy in combination with radiotherapy

Comparison between diazepam and phenobarbital in prevention of febrile seizure: clinical trial

Febrile convulsions [FC] are the most common convulsive events in childhood, occurring in 2-5% of children. About one third of these children will have a recurrence during a subsequent febrile infection. This sudden neurologic problem is extremely frightening and emotionally traumatic for parents so some physicians try to prevent recurrence of FC by prescribing different drugs. This is a randomized clinical trial in 85 healthy children, aged 6 months to 5 years, who were not treated before. These children received randomly either oral diazepam [0.33 mg/kg/TDS for two days during febrile illness] or continuous oral Phenobarbital [3-5mg/kg /24 h]. Ultimately 64 patients completed the study and were followed up for an average of 13 months [12-18 months]. The rate of recurrence of febrile seizure was 18.2% in diazepam group and 32.3% in Phenobarbital group; the difference is not statistically significant [p=0.16]. There was no significant difference between intermittent oral diazepam and continuous oral Phenobarbital for FC prevention

Sodium valproate and phenobarbitol: weight complications of treatment in epileptic children

The aim of this study was to evaluate and compare the effects of Na Valproate and Phenobarbital on changes in the weight of epileptic patients following treatment for their condition using the drugs mentioned. Sixty epileptics were assigned into two groups of 30 patients each, the case and controls. The diagnosis was made on the basis of the International League Against Epilepsy [ILAE] characteristics. BMI was defined. In the case group, the patients received 20mg/kg/day of Na Valproate, while the 30 controls received 5mg/kg/day of Phenobarbital for 6 months. Using the Me Nemar and Chi-2 tests, BMI changes were compared after 6 months between the groups. Fisher's exact test was used to evaluate the role of age, sex, and primary weight on the weight increase due to Na Valproate usage. There were no specific changes in age, sex, primary BMI and fatness between the 2 groups; in the case group, 20 patients[66.7%] and in the controls 4[13.3%] gained weight [P<0.001]. There were higher chances of weight gain in children who were older and fatter at the beginning of the study [P<0.2]. The results indicate that epileptic children, aged over 10 years, and those who are overweight have more chances of gaining weight or becoming fatter, following treatment with Na Valproate. Further studies investigating the issue are warranted

efficacy of propofol and nlidazolam in treatment of refractory status epilepticus in children

In this study, we compared the efficacy and safety of propofol and midazolam in treatment of children's refractory status epilepticus. We recruited 32 patients with refractory status epilepticus. Of those, 16 were treated primarily with midazolam and 16 received propofol. We achieved complete seizure control in 6 [38%] patients treated by midazolam, and in 10 [63%] of 16 patients receiving propofol. After drug withdrawal, seizure recurred in 2 of 6 children who had complete seizure control with midazolam and in 2 of 10 patients who were successfully treated with propofol. Overall treatment with propofol failed in 4 [25%] patients, while in the midazolam group, the failure was 50%. Complications in the midazolam group consisted of bra-dycardia which led to cardiac arrest in one patient who fortunately recovered following cardiopulmonary resuscitation, and rise in serum creatine phosphokinase in another. Untoward reactions seen in the propofol group included elevated serum creatine phosphokinase in 5 patients and dyslipidemia in another 5. Untoward reactions in children who received propofol consisted of rise in serum creatine phosphokinase in 5 and increase in serum triglyceride and cholesterol in 5 patients. No significant change was observed in the frequencies of apnea, hypotension, sepsis, electrolyte imbalance and median duration of stay in intensive care unit between the two treatment groups. Propofol, if used appropriately, can quickly and effectively terminate episodes of refractory status epilepticus in children

Vitamin B6 and treatment of infantile spasms: a comparison with standard steroid therapy

Considering the inadequacies of current therapeutic regimens for infantile spasms [IS], and the frequent and serious side effects of Some regimens, the ongoing search for more enhanced protocols is understandable. We have compared the therapeutic and adverse effects of vitamin B6 given in high doses with those of prednisolone in a randomized controlled clinical trial. Vitamin B6 [40mg/kg/24hr] and prednisolone [1.5mg/kg/day] were given to in 22 and 15 patients respectively, and the patients were followed for at least 6 months. Response to treatment was slightly better in the prednisolone group but the difference was not significant [p=0.4]. On the other hand adverse effects were also seen more frequently with prednisolone. We conclude that high dose vitamin B6 should be considered as an alternative method of treatment; it seems that it can be safely used where there is contraindication to use other antiepileptic drugs or where they have failed; even in newly diagnosed cases of IS

Effects of oral iron supplement on breath-holding spells in children

Breath holding spells are one of the most frequent and important diagnostic challenges in pediatrics. The aim of this study, conducted on pediatric patients referring to the pediatric neurology clinic in Hormozgan province, was to evaluate therapeutic effects of iron on breath holding spells 35 children [19 males and 16 females], aged between 3 to 60 months, with a history of breath-holding spells, were included in the trial. To obtain all relevant data a specifically designed questionnaire requiring information on sex, age, age of onset of spells, type of spells, frequency of attacks before and after treatment with oral iron supplement, and determinants of body iron stores was completed for all the patients, based on the mother's statements. The patients were treated by an oral iron preparation for three months. The age of onset of spells ranged between 6 to 24 months. The cyanotic type of spell was detected in 31 children, the pallid type in 3, and the mixed type in one child. There were 14 children with iron deficiency anemia and 20 children with reduced iron stores. Just one child had a normal iron profile. Complete therapeutic response was documented in 24 children, good response in 9, and poor response in one and in one child no change in frequency of spells was seen. Although no significant therapeutic difference was seen in the different response groups, it seems that iron supplement may play an important role in reducing breath holding spells in children

[Hearing function of children with meningitis by auditory brainstem response]

Sensorineural hearing loss is one of the most important complications' of meningitis, however, it can be neglected during the acute course of the disease. By early detection and medical intervention, the retardation of lingual development can be avoided. The hearing function of 40 children with meningitis was evaluated at 24- 72 hours after diagnosis [acute period] and 24 hours before discharging from hospital [recovery period] with auditory brainstem responses [ABR]. During the acute period, ABR test revealed normal hearing in 35 patients [87.5%] and severe to profound sensorineural hearing loss in 5 patients [12.5%]. The same results were obtained in re-evaluation in the recovery period. There were significant differences between the female gender, positive culture of cerebrospinal fluid, the age below 2 years of old and hearing loss. [P value = 0.03, 0.02, 0.02, respectively]. ABR can be used for early detection of hearing loss during the acute and recovery period of meningitis

[Comparison between diazepam and phenobarbital in prevention of febrile seizure]

Febrile seizures are the most common convulsive events in childhood, occurring in 2-5% of children. About one third of these children will have a recurrence during a subsequent febrile infection. This is a randomized clinical trial in 85 healthy children, aged 6 months to 5 years, who were not treated before. These children received randomly either oral diazepam [0.33 mg/kg/tds for two days during febrile illness] or continuous oral Phenobarbital [3-5mg/kg /24 h]. Ultimately 64 patients completed the study and were followed up for an average of 13 months [12-18 months]. The rate of recurrence of febrile seizure was 18.2% in diazepam group and 32.3% in phenobarbital group; the difference is not statistically significant [p=0.16]. There was no significant difference between intermittent oral diazepam or continuous oral phenobarbital

Lamotrigine as add-on therapy in children with drug-resistant epilepsy [Iranian experience]

Lamotrigine [LTG], a newly developed antiepileptic drug [AED], is efficacious in treating refractory epilepsy. This study was designed to evaluate the efficacy and safety of LTG as add-on therapy in 40 children with refractory epilepsy. The trial was an open-labeled prospective study in children with drug-resistant epilepsy aged <14 years, who had at least 4 seizures per month in spite of receiving at least 3 AED's. Initial LTG dose and titration was adjusted based upon the AED's which were taken simultaneously. Lamotrigine was increased in steps to maximal dose within 4 weeks and maintained for 3 months while pre-existing AED's remained unchanged. Overall efficacy was defined if>50% reduction of seizure frequency was achieved during 3 months follow up. Hematological and biochemical parameters were checked before and after the trial in all patients. The evaluation of drug safety consisted of chart review for treatment-emergent adverse events. Among 40 patients who completed the trial, 21 of them [52.5%] had >50% reduction in seizure frequency. Lamotrigine was effective in all seizure types, particularly typical absence. Lennox-Gastaut syndrome also responded well. Skin rashes occurred in 5 patients [10.6%] and resulted in LTG discontinuation. No significant changes were noted in laboratory results. These results indicated that LTG is well tolerated and is effective in controlling a variety of seizure types, especially absence epilepsy