The neuroimaging of XXXX : case series and review of the literature.

@entity1 @entity6220 @entity3308 @entity75 @entity8433 @entity179 @entity74 @entity194 @entity62 @entity2383
UNASSIGNED: @entity8433 by definition is (1) a @entity179 with variable symptoms, (2) caused by @entity2383 from a @entity74 and (3) accompanied by @entity6220 . A genetic etiology is confirmed in approximately 50% of @entity1 , with more than 60 identified mutations in the nuclear and mitochondrial genomes. Here we review the clinical features and imaging studies of @entity8433 and describe the neuroimaging findings in a cohort of 17 @entity1 with genetically confirmed @entity8433 . MR findings include lesions in the brainstem in 9 @entity1 (53%), basal ganglia in 13 (76%), thalami in 4 (24%) and dentate nuclei in 2 (12%), and global @entity194 in 2 (12%). The brainstem lesions were most frequent in the @entity3308 . With follow-up an increased number of lesions from baseline was observed in 7 of 13 @entity1 , evolution of the @entity75 was seen in 6, and complete regression of the lesions was seen in 3. No cerebral @entity62 were found in any of the 17 @entity1 . In concordance with the literature, we found that @entity8433 follows a similar pattern of bilateral, symmetrical basal ganglia or brainstem changes. Lesions in @entity8433 evolve over time and a lack of visible lesions does not exclude the diagnosis. Reversibility of lesions is seen in some @entity1 , making the continued search for treatment and prevention a priority for clinicians and researchers.
