Last modified: 2014-04-03
Abstract
Abstract
Introduction:
Neurometabolic diseases are a diverse group of brain disorders. The main objective of our study was to collect clinical information of patients 0-19 years age with a clinical picture suspicious of neurometabolic disease in order to creation of a childhood neurometabolic registry system.
METHODS:
During years 2010-2012,we collected clinical information’s of patients with suspected neurometabolic disease according to two classification system: Dyken classification and the other system, small and large molecules disorders .
RESULTS:
During two years, clinical data of 138 patients were collected. Based on Dyken classification, patients were divided as follow: 72 patients with classic and unclassified leukodystrophies ,28 patients with different types of poliodystrophies,6 patients with diffuse encephalopathies and 3 patients with spinocerebellopathies. According to small and large molecules disorders patients were divided as follow: the first group(20 cases) and second group (34 cases).
DISCUSSION
we could obtain relative incidence of some neurometabolic disorders in Tehran province by isolating those patients that were residents in Tehran province. incidence of classic leukodystrophy and classic and unclassified leukodystrophy with together were 12.7 and 21.7 cases/1 million children. Relative incidence of inherited secondary leukoencephalopathies and total inherited white matter disorders were 5.4 cases/1 million children and 18.1 cases/1 million children, respectively. It seems that due to the high rate of consanguineous marriages in Iran, incidence and prevalence of neurometabolic disorders are very significant. Similar studies are also necessary to obtain a more accurate statics of neurometabolic disease incidence in Iran.
Keywords
References
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