Mohammad B. Ghofrani

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Nonconvulsive status epilepticus can present with several manifestations, many of which might not be obvious. The most important for the diagnosis of nonconvulsive status epilepticus is the electroencephalogram (EEG) pattern. This is a case report of a 9-year-old boy with severe and continuous headache. He received chemotherapy for histiocytosis and was(More)
BACKGROUND AND AIMS The ketogenic diet is an effective medical therapy for intractable childhood epilepsy. However, it has drawbacks in that it restricts calories, fluids and protein. The Atkins diet may also induce ketosis without those restrictions. Our objective was to evaluate the efficacy of a modified Atkins diet in children with intractable childhood(More)
Oxidative stress and generation of reactive oxygen species are strongly implicated in a number of neuronal and neuromuscular disorders, including epilepsy. The functions of selenium as an antioxidant trace element are believed to be carried out by selenoproteins that possess antioxidant activities and the ability to promote neuronal cell survival. Because(More)
The aim of this study was to investigate the effect of nano-silver treatment on some physical and mechanical properties of compressed low density wood species. Wood specimens were prepared from spruce (Picea abies), impregnated with water or nano-silver solution by empty cell process and compressed through radial direction in a hot press. The results showed(More)
OBJECTIVE Methylmalonic acidemia is one of the inborn errors of metabolism resulting in the accumulation of acylcarnitine in blood and increased urinary methylmalonic acid excretion. This disorder can have symptoms, such as neurological and gastrointestinal manifestations, lethargy, and anorexia. MATERIALS & METHODS The patients who were diagnosed as(More)
OBJECTIVE GM2-Gangliosidosis disease is a rare autosomal recessive genetic disorder that includes two disorders (Tay-Sachs and Sandhoff disease).These disorders cause a progressive deterioration of nerve cells and inherited deficiency in creating hexosaminidases A, B, and AB. MATERIALS & METHODS Patients who were diagnosed withGM2-Gangliosidosis in the(More)
OBJECTIVE 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. MATERIALS & METHODS The patients diagnosed as(More)
OBJECTIVE Biotinidase deficiency is one of the rare congenital metabolic disorders with autosomal recessive inheritance. If this disorder is diagnosed in newborn period, could be prevented well from mental and physical developmental delay and most of clinical manifestations. MATERIALS & METHODS The patients were diagnosed as biotinidase deficiency in(More)
Niemann-Pick disease type C is a rare neurodegenerative disorder with autosomal recessive inheritance that can be broadly categorized into different forms dependent on age at disease onset: pre-/perinatal, early infantile, late infantile, juvenile, and adolescent/adult. This study was conducted to define the age at onset, clinical manifestations,(More)
OBJECTIVE Canavan's disease is a lethal illness caused by a single gene mutation that is inherited as an autosomal recessive pattern. It has many different clinical features especially in the non-Ashkenazi Jewish population. MATERIAL & METHODS 45 patients were referred to the Pediatric Neurology Department of Mofid Children's Hospital in Tehran-Iran from(More)