Iranian Journal of Child Neurology (IJCN)
Volume:8 Issue: 1, Winter 2014

As acute liver failure (ALF) and chronic liver disease (cirrhosis) continue to increase in prevalence, we will see more cases of hepatic encephalopathy.Primary care physician are often the first to suspect it, since they are familiar with the patient’s usual physical and mental status. This serious complication typically occurs in patients with severe comorbidities and needs multidisciplinary evaluation and care. Hepatic encephalopathy should be considered in any patient with acute liver failure and cirrhosis who presents with neuropsychiatric manifestations, decrease level of consciousness (coma), change of personality, intellectual and behavioral deterioration, speech and motor dysfunction.Every cirrhotic patient may be at risk; potential precipitating factors should be addressed in regular clinic visits. The encephalopathy of liver disease may be prominent, or can be present in subtle forms, such as decline of school performance, emotional outbursts, or depression.“Subtle form” of hepatic encephalopathy may not be obvious on clinical examination, but can be detected by neurophysiologic and neuropsychiatric testing.

Infantile spasms (IS) are among the most catastrophic epileptic syndromes of infancy. The purpose of this study was to compare efficacy and safety of topiramate (TPM) and nitrazepam (NZP) as first-line drugs in the treatment of IS.In a parallel single-blinded randomized clinical trial, 50 patients with IS referred to Pediatric Neurology Clinic of Shahid Sadoughi University of Medical Sciences, Yazd, Iran, were evaluated from September 2008 to March 2010.Patients were randomly assigned to two groups to be treated with TPM or with NZP for 6 months. The primary endpoint was efficacy in cessation of all spasms or reduction of more than 50% in weekly seizure frequency, which was evaluatedbefore and 6 months after the drug use. Secondary outcome was clinical sideeffects of the drugs.Twenty boys (40%) and 30 girls (60%) with the mean age of 9.4±3.8 months were evaluated. Cessation of all spasms occurred in 12 (48%) infants in TPM group and 4(16%) in NZP group. Eight (32%) children in TPM group and 7 (28%) in NZP group had more than 50% reduction in spasms frequency. So,TPM was more effective. Side effects were seen in 32% of TPM and in 36% of NZP groups.Topiramate is an effective and safe drug, which might be considered as the firstline drug for the treatment of ISs.

Some studies suggest that detection of epileptic discharge is unusual during the first postictal week of febrile seizure and others believe that EEGs carried out on the day of the seizure are abnormal in as many as 88% of the patients. In thisstudy, we intend to compare early and late EEG abnormalities in febrile seizure. EEG was recorded during daytime sleep, 24-48 hours (early EEG) and 2 weeks (late EEG) after the seizure in 36 children with febrile seizure (FS), aged between 3 months and 6 years. EEGs that showed generalized or focal spikes, sharp, spike wave complex, and slowing were considered as abnormal EEG.Abnormalities of the first EEG were compared with those of second EEG.The most common abnormal epileptiform discharges recorded in the early EEG were slow waves (27.6%) and sharp waves in late EEG (36%). Distribution of abnormalities in early and late EEG showed no significant statistical difference.The early and late EEG recording had the same results in patient with febrile seizure.

Although the use of Complementary and Alternative Medicine (CAM) has been evaluated globally, there are few studies in our country on this subject. The purpose of this study was to determine the prevalence, pattern of use, parental sources of information, and benefits of CAM in epileptic children in Tehran.One hundred thirty-three parents or relatives of epileptic children who were referred to outpatient clinics or admitted in neurologic ward of four major hospitals in Tehran, were interviewed by our researcher based on a structured questionnaire; from 2009 to 2010. The information obtained comprised the demographic data of patients and their parents, frequency and morphology of convulsions, the type and sources of CAM and finally, the benefits and adverseeffects of this practice.Forty-four percent of the respondents had used CAM methods either alone or in combination with other methods. The most frequently used CAM was written prayers followed by oral herbs and special diets. CAM was mainly introduced to them by relatives. Only 16.7% of these parents had discussed this matter with their children’s physicians. No efficacy to control seizure was observed for most of these methods.This study showed that use of CAM in our study group is relatively common and may have a potentially hazardous role in the treatment process. So, it is necessary for physicians to have enough information about CAM practice in their patients.

Friedreich’s ataxia is the most common form of hereditary ataxia with autosomal recessive pattern. More than 96% of patients are homozygous for GAA repeat extension on both alleles in the first intron of FXN gene and the remainingpatients have been shown to be heterozygous for a GAA extension in one allele and point mutation in other allele.In this study, exons of 1, 2, 3, and 5 of frataxin gene were searched by single strand conformation polymorphism polymerase chain reaction (PCR-SSCP) in 5 patients with GAA extension in one allele. For detection of exact mutation,samples with band shifts were sent for DNA sequencing. Three novel point mutations were found in patients heterozygous for the GAA repeat expansion, p.S81A, p.Y123D, and p.S192C. Our results showed that these point mutations in one allele with GAA extension in another allele are associated with FRDA signs. Thus, these results emphasize the importance of performing molecular genetic analysis for point mutations inFRDA patients.

The present study was performed to examine the effects of two speech therapy methods on six verbal behaviors of autistic children, including oral speech, listening, organizing, speaking, semantics, and syntax.In this study, thirty 6-8 years old children with autism were assigned to one of two groups: imitative and cognitive groups. Before starting the main procedures of the study, the children of both groups were homogenized concerning their autism level. In the first phase of the study, the speech development level of the two groups was measured in a pre-test, in which both groups showed similar results. Then, both groups of children received 6 months of speech therapy instruction, during which one group was taught using an imitative method, while the other group was being worked with cognitive method.After 6-month treatment period, a post-test was done, and the t-tests based on the data of the two groups revealed a significant difference between the results.The statistics showed that after the teaching period, autistic that worked with cognitive method gained a better development in their speech abilities, comparing to those worked with the imitative method.

In chronically ill children who are hospitalized, many mood changes occur. For example, in children with cancer or renal failure, prolonged hospitalization and chemotherapy can lead to depression. With the improved survival of childhoodmalignancies, the effect of treatment on child’s psychosocial well-being becomes increasingly relevant. In this study, we examined the prevalence of depression in hospitalized children with chronic and acute conditions in Dr Sheikh Pediatrics Hospital in Mashhad.After receiving the approval from the Ethics Committee of Mashhad University of Medical Sciences, we did this cross-sectional descriptive study, from April to June 2012 in Dr Sheikh Pediatric Hospital in Mashhad. Ninety children, aged between 8 to 16 years, were screened for depression. The sampling method was census. Children with a history of depressive or other mental disorders were excluded.Three groups of children (children with chronic renal disease, malignancy, and acute disease) were evaluated for depression using standard Children Depression Inventory Questionnaire (CDI). Two specifically trained nurses with the supervisionof a psychiatrist filled out the questionnaires at patients’ bedside. Depression scores were then analyzed by SPSS software.Of 90 children, 43(47.7%) were male and 47(52.2%) were female. The Children’s mean age was 11±2.3 years, and the mean length of hospitalization was 8±5.3 days.Depression was detected in various degrees in 63% of patients (N=57), and 36.6% of children (N=32) had no symptoms of depression. Severe depression was not seenin any of the patients with acute illness. More than half of patients with cancer and chronic kidney disease had moderate to severe depression.There was a significant statistical relationship between the duration of illness and severity of depression. There was also a significant correlation between severity of depression and frequency of hospitalization. Children who had been hospitalized more than 3 times in the last year, experienced more severe levels of depression. We also found a significant correlation between pubertal age and severity of depressionin patients with cancers and chronic renal failure.Children who are hospitalized due to chronic conditions are at a higher risk for mood disorders in comparison with the ones with acute conditions. It is therefore advisable to consider more practical plans to improve the care for hospitalizedchildren’s mental health.

Children with leukemia and their families face a long period of medical treatment and uncertainty about the future. These families may suffer from short- and long-term emotional problems. The aim of the present study was to assess theeffect of supportive psychotherapy on the anxiety of mothers whose children suffer from leukemia.The current research were performed on mothers who had a child with leukemia hospitalized in Mofid Children’s Hospital, Tehran, Iran. They were randomly selected. The research method was a pseudo-experimental study with pretest/posttest design. The pretest Kettles’ anxiety questionnaire was given to all the mothers and after seven sessions of supportive psychotherapy, the posttest was performed and the grades were compared.Ten mothers finished all seven therapeutic sessions. There was a statistically significant difference between the pretest and posttest mean scores, confirming the mothers’ reduced anxiety level.Finding effective and newer approaches to improve the well-being of parents with a sick child is an important challenge of today’s medical researches. Based on our findings, it is possible to reduce the anxiety in mothers of children withleukemia through supportive psychiatric therapies.

Propionic acidemia is one of the rare congenital neurometabolic disorders with autosomal recessive inheritance. This disorder is caused by a defect in the propionyl-CoA carboxylase enzyme and can be presented with life-threatening ketoacidosis, lethargy, failure to thrive, and developmental delay.The patients diagnosed as having propionic acidemia in Neurology Department of Mofid Children’s Hospital in Tehran, Iran, between 2002 and 2012 were include in our study. This disorder was confirmed by clinical manifestations, neuroimaging findings, and neurometabolic assessment in the reference laboratory in Germany. Our study was conducted to define the sex, age, gender, past medical history, developmental status, clinical findings, and neuroimaging manifestations in 10 patients with propionic acidemia. Seventy percent of patients were offspring of consanguineous marriages. In this study, only one patient had microcephaly at birth, but at detection time, 30% of patients had head circumference and weight below the 3rd percentile. The patients were followed for approximately 5 years and this follow-up showed that the patients with early diagnosis had a more favorable clinical response.Neuroimaging findings included brain atrophy, white matter and globus pallidus involvement.Finally we suggest that early diagnosis and treatment have an important role in the prevention of disease progression.

Systemic Lupus Erythematosus (SLE) is an autoimmune, multisystem disorder with various manifestations. There are limited reports on the neuropsychiatric findings as the first manifestation of SLE in children. Herein, we reported a 14-year-old Iranian boy with a two-year history of cognitive dysfunction and behavioural problems as well as a recent history of epistaxis. The patient workup ended with a diagnosis of Klinefelter’s syndrome associated with juvenile SLE. Patients with Klinefelter’s syndrome may exhibit behavioural problems and psychological disease. These psychiatric disorders could be complicated with lupus in children. In fact, psychiatric symptoms may occur as the first manifestation of juvenile SLE. Specially, if accompanied with Klinefelter’s syndrome. We suggest the diagnosis of SLE must be strongly considered in all children with neuropsychiatric manifestations.

Vincristine (VCR) is a vinca alkaloid that is used for treatment of many malignancies.The vinca alkaloids are neurotoxic, usually causing a peripheral neuropathy, but cranial neuropathies are rare as side effects. Described here is the case of a 2.5-year-old boy, a known case of Wilms’ tumor, treated by vincristine (0/067 mg/kg/day) and dactinomycin (0/045 mg/kg/day) after surgery. Three weeks after treatment, he presented with bilateral ptosis.Neurological examination revealed bilateral ptosis with normal pupillary reflex and eye movement. He received 3.015 mg cumulative dose of vincristine before development of ptosis.Treatment with pyridoxine (150 mg/m2 p.o. BID) and pyridostigmine (3 mg/kg p.o. BID) started as neuroprotective agents, and after 7 days the problem disappeared.The treatment continued for 6 weeks and there were no signs of ptosis or a recurrence in follow up 2 months later.

This case study is about an 11-year-old girl with bilateral facial weakness, abnormal taste sensation, and deep tendon reflexes of both knees and ankles were absent. However, the muscle power of the lower and upper extremities across all muscle groups was normal. After 2 days, she developed paresthesia and numbness in the lower extremities. Other neurologic examinations, such as fundoscopic evaluation of the retina were normal with the muscle power of both upper- and lower-extremities intact. A lumbar puncture revealed albumincytological dissociation. EMG and NCV were in favor of Guillain-Barre syndrome, for which IVIG was prescribed and the abnormal sensations in the lower limbs rapidly improved. Bilateral facial diplegia without weakness and paresthesia is a variant of Guillain-Barre syndrome that mostly presents with acute onset, rapid progression with or without limb weakness, paresthesia, and decreased or absent DTR and albumin-cytological dissociation.