الفهرس 
Hypomyelinating white matter disorders MRI approach
AbstractOnly 14 pages are availabe for public view
 |  | from | 141 |  |  |
| | | from | 141 | | |
Abstract
White matter disorders (WMDs) are major source of handicapping at all ages. They often lead to progressive neurological dysfunction and early death. The underlying pathology ranges from hypomylination, white matter rarefaction to demyelination. Clinically, it is not easy to differentiate between the various hypomyelinating disorders. It is, therefore, usually not easy to reach a specific diagnosis and often an elaborate diagnostic work-up is performed in patients with an MRI indicative of hypomyelination. Understanding these disorders can aid in counseling patients and their families and, perhaps, developing and testing therapies. The aim of the present study was to differentiate types of hypomyelinating white matter disorders on the basis of MRI findings and correlate these findings with the clinical picture of the children. Thirty children were included in the present study. Common neurological findings are developmental delay, nystagmus, cerebellar ataxia and seizures. Close inspection of the results of the present study reveals that the most prevalent neurological presentation was delayed walking (76.6%) followed by delayed speech (60%). The current study raises the expectation that MRI may also be a valuable tool in the study of hypomyelinating disorders. The most important diagnostic item of MRI findings in the present study includes homogeneity of white matter signal on T2_weighted images occurring in almost all patients (100%), followed by early cerebellar atrophy (63.3%). So we concluded that MRI has proven to be pivotal in the diagnostic workup of patients with leukoencephalopathies