objective the lennox-gastaut syndrome (lgs: the triad of intractable seizures of various types, a slow spike-wave pattern in eeg and mental retardation) is one of the most difficult epilepsy syndromes to treat. the aim of this study was to evaluate the efficacy and safety of zonisamide (zns) as add-on therapy in seizures of children with lgs. materials & methods in a quasi- experimental study, ...

A 15-month-old girl presented with developmental delay, intractable right focal motor seizures, hyperpigmented patch on the left side of the face, facial asymmetry, and right hemiparesis (figure 1). A diagnosis of linear nevus sebaceous syndrome (LNSS) was considered. Brain MRI revealed hemimegalencephaly (figure 2). EEG showed spike/wave discharges in left occipital leads. Ophthalmologic and s...

The prevalence of epilepsy in sub-Saharan Africa is higher than in other parts of the world, but it is short of the effective measure on treating intractable epilepsy. Epilepsy surgery is not easy to be performed due to the high cost and demand of operational skills. The authors planned to perform ketogenic diet therapy for the children with intractable epilepsy in Ghana with regard to its low ...

INTRODUCTION Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, fatal neurodegenerative disease of childhood and early adolescence caused by defective measles virus. The initial symptoms of SSPE usually involve regression in cognitive functioning and behavior or recurrent myoclonic jerks. Seizures revealing SSPE and epilepsy during the clinical course can occur. OBJECTIVE The ...

BACKGROUND This study was performed in children aged <15 years, at the Pediatric Neurology Clinic of Imam Reza Hospital affiliated to Mashhad University of Medical Sciences. The objective of this study was to recognize the main predisposing factors that result in uncontrolled seizures in patients so that we can start the treatment accurately. METHODS There were two groups of patients; group I...

Ring chromosome 14 syndrome is a rare genetic disorder. Typically, children with this syndrome have distinct facial features, development delay, microcephaly, seizures, ocular abnormalities, and recurrent respiratory infections. Epilepsy associated with ring chromosome 14 generally shows intractable seizures. We describe a six-month-old girl with ring chromosome 14 syndrome who presented with e...

Isolated Hemimegalencephaly (iHME) is a rare form of congenital malformation of cortical development.It is characterized by enlargement of all or part of one cerebral hemisphere. It typically presents with intractable seizures, mental retardation, developmental delay, contralateral hemiparesis and hemianopia. The patient was a five and half month's old baby girl who presented first with focal s...

PURPOSE Epileptic spasms (ES) beyond infancy are a highly refractory type of seizures that require the development of an effective treatment. We therefore studied the efficacy and safety of topiramate (TPM), which is a drug that is indicated to be effective for intractable childhood epilepsy, for ES. METHODS Out of 58 children with ES, we enrolled 33 patients treated with TPM at ≤ 12 years of...

Menkes disease is an infantile-onset X-linked recessive neurodegenerative disorder caused by diverse mutations in a copper-transport gene, ATP7A. Affected patients are characterized by progressive hypotonia, seizures, failure to thrive and death in early childhood. Here, we report a case of Menkes disease presented by intractable seizures and infantile spasms. A 3-month-old male infant had visi...