Myasthenia gravis in Iranian children

Considering the marked difference between the clinical course and management of juvenile myasthenia gravis, congenital/genetic myasthenia gravis and transient neonatal MG, the differential diagnosis is very important. This study was undertaken to evaluate the clinical spectrum of myasthenia gravis in children and determine the factors helping clinicians in their diagnosis and management of the disease. In a retrospective study from 1994 to 2002, all pediatric patients with myasthenia gravis [MG] admitted to Department of Pediatric Neurology in Mofid Children Hospital affiliated to Shahid Beheshti University were enrolled. Of 32 children, 7 and 25 suffered from congenital and juvenile types of MG, respectively. The initial symptoms in congenital MG were ptosis [7/7], limitation of eye movement [2/7] and mild generalized weakness [6/7]. Although 85% of cases with congenital MG, tested positive for Tensilon test, no myasthenia crisis or spontaneous remission was observed in any of the patients. The female to male ratio was 1.5/1 which was correlated to adult MG. In children with juvenile MG, the mean age was 5.7 +/- 4.2SD years. The most common symptoms were ptosis in 96% and generalized weakness in 76% of the cases. 32% of patients experienced one myasthenia crisis. EMG was diagnosed in 83% and tensilon test was positive in 84% of the cases. One patient had hyperthyroidism and another had hypothyroidism and both were epileptic. Eight patients underwent thymectomy microscopically. Thymic follicular hyperplasia was observed in five cases [62%], and the remaining three cases were normal. 12.5% of patients recovered completely after thymectomy and there was no need for medication during the follow up. 50% of cases showed relative improvement but it was negligible in 37% of patients. This study revealed that thymectomy lacks remarkable prognostic influence

Risk of seizure recurrence following a first unprovoked seizure in childhood

There is still a question whether first seizure leads to epilepsy. Several risk factors have been reported in this relation. This study was undertaken to determine the risk of recurrence after a first unprovoked seizure in children In a prospective study between December 2003 and December 2005, 156 children who presented with a first unprovoked seizure were enrolled and followed for at least 18 months. Potential predictors of recurrence were compared, using the Cox Proportional Hazard model in a univariable and multivariate analysis. Survival analysis was performed, using the Kaplan-Meire curves. Seventy two children [46.2%] experienced subsequent seizures. The cumulative risk of seizure recurrence was 28.8%, 41.7% and 46.2% at 6, 12, and 24 months following the first seizure, respectively. The median time for repeated seizure was 4 months while 62.5% of the recurrence occurred within 6 months, 88.9% within 1 year and 100% till the end of the second year. On multiple analysis, risk factors for resumption of seizure consisted of abnormal electroencephalography [EEG], seizure during sleep, abnormal brain imaging and history of perinatal problems. On univariable analysis, abnormal EEG, abnormal imaging [remote etiology of seizure], history of neonatal problems, previous febrile seizure, and family history of afebrile seizure increased the risk of recurrence. The study revealed that the risk of seizure recurrence in our patients was relatively high. Those who had abnormal electroencephalography, past history of prenatal problems, remote etiology for seizure, abnormal brain imaging, and seizure during sleep were at greater risk for recurrence of seizure

Myasthenia gravis in iranian children

This study was undertaken to evaluate the clinical spectrum of myasthenia gravis in children and determine factors that help the clinician in his/her diagnosis and management. A retrospective review was performed on all pediatric patients suffering from myasthenia gravis [M.G] admitted in the department of pediatric neurology of the Mofid Hospital of the Shaheed Beheshti University, between 1994 and 2002. Of the thirty-two children with M.G. enrolled in our study, seven were suffering from the congenital type while the remaining [25 cases] had the juvenile M.G. Initial symptoms of congenital M.G were ptosis [7/7], limitation of eye movement [2/7] and mild generalized weakness [6/7]. Although the Tensilon test was positive in 85% of congenital M.G cases, no myasthenia crisis or spontaneous remission was observed in any of them. In children with juvenile M.G, the age of presentation was 1.2 to 12.5 years, mean age 5.7 +/- 4.2 years [15 girls and 10 boys]. The most common presenting symptoms in juvenile group were ptosis in 96% and generalized weakness in 76%. Eight of them [32%] had had at least one myasthenia crisis. EMG was diagnostic in 83% and the tensilon test was positive in 84%. One patient had hyperthyroidism and had already been diagnosed with hypothyroidism; two of them were epileptics. Eight patients underwent thymectomy microscopically; in specimens examined, five [62%] showed thymic follicular hyperplasia while in remaining three results were normal. One patient [12.5%] recovered completely after thymectomy with no need for medication during the follow up. Four patients [50%] showed relative improvement and in three cases [37%] improvement was negligible. The results showed a female to male ratio of 1.5/1 which was correlated to adult M.G. The most common presenting symptoms consisted of ophtalmoplegia, with bilateral ptosis being the most significant. Although this study revealed that thymectomy lacks any remarkable prognostic influence, all patients had thymectomy within two years of disease onset. Some reports have indicated positive results if surgery was performed within two years of onset of disease

Facial palsy as first presentation of acute lymphoblastic leukemia: a case report

Facial paralysis in children is very often idiopathic and isolated facial nerve palsy, resulting from leukemic infiltration is a rare occurrence. Here we present the case of a 14 year-old boy with acute lymphoblastic leukemia, who first presented with isolated right side peripheral facial nerve paralysis and was initially diagnosed with Bell's palsy. The presence of Bell's palsy in young children requires a complete evaluation, keeping in mind the possibility of leptomeningeal disease