HIGH AND LOW DOSE IVIG THERAPY IN GUILLAIN-BARRE SYNDROME CHILDREN: A COMPARISON

Objective:Acute inflammatory demyelinating peripheral neuropathy (Guillain-Barre-Syndrome) is by far the most common cause of immune-medicated peripheral nervous system disease in children; with the near disappearance of poliomyelitis, GBS is responsible for the great majority of cases of acute flaccid paralysis. So far, in several controlled studies, corticosteroids, plasmapheresis and IVIG have been utilized in pediatric patients, afflicted with GBS. Regarding IVIG therapy, two methods have been used; the high dose (1 gr/kg/day for 2 days), and the low dose (400mg/kg/day for 5 days). Review of literature shows that a faster rate of recovery can be accomplished in patients who receive total dose of IVIG in 2 days as compared to the dose being given over 5 days.Materials & Methods:In this study we have compared these two types of treatment in an investigation, conducted in the Mofid Children Hospital on pediatric patients who had sudden onset of acute flaccid  paralysis, and were diagnosed as having GBS. Based on histories, physical examination and electrodiagnosis, subjects were divided in two groups, the high dose IVIG treatment, 1gr/kg/day for 2 days (experimental group), and the low dose IVIG treatment, 400 mg/kg/day for 5 days (control group). Statistical analyses were then carried out using the appropriate software.Results:Result of this study showed a faster rate of recovery for patients in the high dose IVIG group; in this group duration of weakness of limbs was shorter and returning of DTR was faster than in controls. In fact, in this type of treatment, the relationship between high dose IVIG therapy and drug side effects was not significant.Conclusion:Base upon the finding in the present study, we conclude that the high dose IVIG therapy is superior to low dose, in view of faster duration of recovery and shorter hospital stay. Also we may infer that shorter hospital stay could be a factor in reducing of more nasocomial infection. In conclusion, we suggest using high dose IVIG treatment of choice in GBS.Keywords:Gulliain Barre Syndrome-High dose, Low dose IVIGcomparison of two types of treatmen

100 CHILDREN WITH ACUTE ATAXIA; A SURVEY IN MOFID CHILDREN'S HOSPITAL

Objective:The term "Ataxia" refers to disturbances of body posture and movement that are normally controlled by the cerebellum, frontal lobes and the posterior columns of the spinal cord. The primary symptom and the most prominent feature of ataxia is abnormal gait which is characterized by lurching and wide base walking. Ataxia was considered acute, if it had occurred within the two preceding weeks. Knowing how frightening acute-onset Ataxia is for the family is not surprising that the condition prompts an immediate visit to the physician.Material & Methods:In view of the lack of information in our country, on the etiology of sudden-onset Ataxia, the authors enrolled 100 children with the chief complaint of acute loss of equilibrium, who came to the attention of the Pediatric Neurology Department over a two year duration (Sept.2001-Sept 2003); they were admitted to the Mofid Childrens' Hospital and all necessary investigations were carried out.Results & Conclusion:The results revealed that Acute Cerebellar Ataxia was the most common cause of the problem, the second most frequent being drug intoxication, which most commonly occurred in patients, 2- 4years old. The remaining causative factors in order of descending frequency consisted of infectious polyneuropathy, migraine, opsoclonus-myoclonus, brain tumor, acute disseminated encephalomyelitis, multiple sclerosis, and epilepsy.Key words:Acute ataxia, Children, Acute cerebellar ataxi

A novel c.973G>T mutation in the ε-subunit of the acetylcholine receptor causing congenital myasthenic syndrome in an iranian family

Congenital myasthenic syndrome (CMS) constitutes a group of inherited disorders of neuromuscular junctions. The majority of postsynaptic syndromes result from mutations in the CHRNE gene that causes muscle nicotine acetylcholine deficiency. In this study, we report on a 2 and a half-year-old boy with normal developmental milestones and bilateral ptosis. Clinical courses, electrophysiological studies and molecular genetic analysis were assessed. Polymerase chain reaction (PCR) and direct DNA sequencing of the CHRNE gene were performed for the proband and all the family members. A novel homozygous missense mutation of c.973G>T was found in the CHRNE gene. Segregation studies were suggested to be the genetic cause of the disease. Using three in silico tools and the American College of Medical Genetics and Genomics/Association for Molecular Pathology (ACMG/AMP) variant classification guidelines indicated that the novel variant c.973G>T was likely pathogenic. Our results recommended first screening of the CHRNE gene for pathogenic mutations in Iranian origin