Magnetic resonance findings in leucodystrophies and MS.

[alexander disease]

White matter diseases are a frequent diagnosis problem in adult patients . They are divided into leucodystrophy , defined by abnormal white matter from the beginning , and leucoencephalopathy , with an initial normal white matter . In addition , two different natures have to be considered : vascular and non-vascular . Vascular diseases are mainly acquired and related to atherosclerosis . Genetic vascular disorders are mostly secondary to Notch 3 mutations , defined as cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL ). Occurrence of leucoaraiosis and lacunae on T 2 sequences , and microbleeds on Gradient Echo sequences , strongly suggest this diagnosis . Some magnetic resonance (MR ) patterns can help to identify genetic leucodystrophies , such as childhood ataxia with central nervous system hypomyelination /leucoencephalopathy with vanishing white matter disease (progressive rarefaction and cystic degeneration of the affected white matter , replaced by water ); Alexander disease (hypointense signals on T 2 sequences involving grey matter , brainstem and cervical cord , with marked atrophy ); megalencephalic leucoencephalopathy with subcortical cysts (diffuse , symmetrical white matter lesions , with constant frontoparietal and anterotemporal subcortical cysts ); leucoencephalopathy with brainstem and spinal cord involvement and high lactates syndrome (extensive demyelination , involvement of the brainstem , i .e . cerebellar peduncles , intraparenchymal and mesencephalic trigeminal nerves and spinal cord , mainly in the lateral corticospinal tracts and dorsal columns ). Half of the genetic adult leucodystrophies remain without any precise diagnosis . This review describes MR in the adult leucoencephalopathies and in multiple sclerosis (MS ). The first part will focus on MR patterns of vascular and nonvascular adult leucoencephalopathies , the second part on MR findings in MS and MS -related diseases . Specific MR patterns in both diseases will be summarized and compared .

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alexander disease

well-differentiated liposarcoma

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