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

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

Acute disseminated encephalomyelitis in a 5-month old infant

This article reports on an 5-month-old infant who was admitted to our hospital for prolonged fever, focal status epilepticus and encephalopathy. Brain magnetic resonance imaging [MRI] demonstrated a single hyperintense lesion in right putamen in T2-weighted images. A diagnosis of acute disseminated encephalomyelitis was made based on the clinical features, treatment response and clinical course. An executive literature review revealed a few cases under the age of one year .This patient is the youngest are who is reported from Iran

Acute disseminated encephalomyelitis in childhood; epidemiologic, clinical, and laboratory features

Acute disseminated encephalomyelitis [ADEM], which occurs spontaneously or after systemic viral infection and vaccination mostly affects children. This study aims at describing the epidemiologic, clinical, neuroimaging, laboratory features, treatment and outcome in children who diagnosed as having ADEM and admitted in a referral center in Tehran. This descriptive prospective study was conducted on patients with a diagnosis of ADEM over a period of 30 months, between Sep 2003 and Mar 2006, admitted to the neurology ward of Mofid Pediatric Hospital. All these patients were visited in regular follow-up every 6 months for two years. Eighteen patients with the mean age of 6 years [range 5 months - 12 years] were studied. No sex predominance was noted. Sixty-one percent had prodromal illness, and two patients had been vaccinated before the illness. Thirteen [72%] patients presented in fall or winter. Motor deficits and cranial nerve palsy were the most common features, which had occurred in 13 [72%] patients. Abnormal findings in cerebrospinal fluid evaluation were detected in 33% of the patients. Brain computed tomography was normal in all but one patient. Electroencephalograms done in nine patients were normal in 54%. Magnetic resonance imaging showed lesions were most commonly in the subcortical and periventricular areas [76%]. Nine [50%] patients were treated with corticosteroids and intravenous immunoglobulin. The mortality rate was 5.5%, and the relapse occurred only in one case. In two-thirds of the patients, prognosis for complete recovery was excellent. Childhood ADEM is a benign condition, affecting both sexes equally. Recurrent infections have been its common cause in our center

[Prednisolone therapy in refractory childhood epilepsy]

Steroids are effective medicaions for controlling the infantile spasms, but there have been few reports of steroid effect in other childhood epileptic syndromes, especially refractory syndromes. The objective of this study was to determine the efficacy of prednisolone in 35 children with intractable epilepsy. This prospective, uncontrolled study was undertaken at the Mofid paediatric Hospital from September 2004 to September 2005. Thirty five children [12 girls and 23 boys], aged bteween 1 to 12 years old with intractable epilepsy were enrolled. Prednisolone [1 mg/kg/day] was prescribed for 12 weeks [Once a day for 6 weeks follwed by every other day for another 6 weeks] in addition to their regular antiepileptic medications. The parents kept seizure diaries. After 1 year of follow up, 17 patients [49%] became seizure-free on prednisolone, whereas another 8 [23%] experienced a significant decreases more than 50% in seizure frequency. In contrast, ten patients [28%] had no changes in seizure frequency. The best response were seen in idiopathic epilepsy [95%] and atypical absence ones [86%]. The least therapeutic effect was seen in symptomatic myoclonic epilepsy [54%]. Side effects such as behavioral disturbances, weight gain and gastrointestinal bleeding were developed in 8 patients and also 14 patients were found to have cushingoid face. All the side effects were regressed after drug discontinuation Prednisolone therapy is a safe and effective adjunctive treatment for children with intractable epilepsy and it should be considered as an alternative treatment for older children with refractory epilepsy

[Comparing the effect of valproate and phenobarbital on weight gaining of epileptic children]

Respecting the high incidence of epilepsy among children and several reports coresponding the weight gaining of some during the valproate therapy, this study was conducted in "Mofid Children's Hospital" during 1384 to determine and compare the occurrence and severity of weigh gain among children treated with either valproate or phenobarbital. This clinical trails study was performed studying on 60 epileptic patients. We applied "International League against Epilepsy" classification to diagnose the patients. They were randomly equally divided into two groups [random allocation]. First group was treated with valproate 20 mg/kg/day, whereas second one received Phenobarbital 5 mg/kg/day [in divided dose]. Patients were followed for a six-month period. Body mass index [BMI] was measured at first, 2[nd], 4[th] and 6[th] months of therapy and BMI[6] was compared with expected BMI[6] [according to BMI curve] and the difference of latter two BMIs [BMI[6] and expected BMI[6]] were calculated. McNemar's test was used to compare the weight difference in each group and Chi-square test was applied for between-groups comparison. In valproate-treated patients whose their weights increased, some characteristics [including age, sex and primary weight] were evaluated by Fisher's exact test. Two groups were identical with respect to their age, sex, primary BMI and weight. 4 patients of phenobarbital group [13.3%] versus 20 patients of valproate group [66.7%], had weight gain [P=0.6 and P<0.0001, respectively]. Final-stage data revealed that compared to phenobarbital, valproate resulted in further weight gaining of children and adolescent [P<0.001]. Moreover, in valproate treated group, patients with older age and higher BMI, were more at risk of gaining weight. The risk of weight gainning in epileptic children treated with valproate is high. It seems that in patients above 10 years old and fat patients, this risk become more. We recommend further study to evaluate the effect of valproate on weight of epileptic patients older than 10 years and also fat patients

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

case of gastroenteritis ends in convulsion?

Gastroenteritis has a diverse etiology; many pathogens can cause this condition. Of the extraintestinal manifestations, one is convulsions, which may be attributable to fever, type of bacteria, or electrolyte imbalance. To assess the risk of occurrence of convulsions, in this study we investigated the association between the paraclinical and clinical findings of children with gastroenteritis and the risk of occurrence of convulsions. In this prospective study, conducted between March 2004 and February 2005, we studied 50 patients admitted to the Mofid Childrens' Hospital, with gastroenteritis and convulsions. They were matched with the case group in terms of age, sex, and month of admission and number. Data was collected using a specific checklist. Stool samples were obtained for investigations of electrolyte imbalances and type of gastroenteritis. A control group consisting of patients admitted simultaneously with gastroenteritis but no convulsions was selected as well. The stool exam [SE] showed 31 cases [62%] had inflammatory diarrhea and 19 [38%] had the non-inflammatory type. In the control group, 21 cases [42%] had inflammatory and 29 [58%] had non-inflammatory diarrhea. Stool culture [SC] results showed 11 [22%] subjects had Shigella, 27 [54%] revealed no organism, and 12 [24%] did not have SCs in their medical records. In the control group SC results revealed Shigella in 2 cases [4%], 38 patients [76%] showed no organism, and 10[20%] did not have SCs. Six cases [12%] had hyponatremia ranging between 125-130meq/lit. In the control group, 4 [8%] had electrolyte imbalances, 3 had hyponatremia ranging between 125-130meq/lit, and 1 had hypokalemia. No significant relation was found between inflammatory gastroenteritis and the incidence of convulsion [P value=0.0716]. Although a significant relation was found between Shigella and convulsion [P value=0.0113], no significant relation existed between electrolyte imbalance and the incidence of convulsion [P value=0.7389]

[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

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

Headache as a sole manifestation of non-convulsive status epilepticus

Non-convulsive status epilepticus [NCSE] may present with several manifestations, many of which may not be obvious. The most important tool for the diagnosis of NCSE is the electroencephalograph [EEG] pattern. This is a case report of a boy 9 years of age presenting with severe and continuous headache. He had received chemotherapy for histiocytosis, diagnosed at the age of 3.5 years. No evidence of central nervous system [CNS] involvement was found. He was diagnosed as having NCSE and following anticonvulsant therapy the headache and EEG abnormality disappeared completely within 24 hours. Headache and seizure disorder may coexist in different situations; to our knowledge this maybe the first report of NCSE with headache as a sole manifestation