Skip to main content
AJNR: American Journal of Neuroradiology logoLink to AJNR: American Journal of Neuroradiology
. 1994 Oct;15(9):1755-9.

Cerebellar MR in Fukuyama congenital muscular dystrophy: polymicrogyria with cystic lesions.

N Aida 1, A Yagishita 1, K Takada 1, Y Katsumata 1
PMCID: PMC8333743  PMID: 7847224

Abstract

PURPOSE

To determine the MR appearance of cerebellar abnormalities in Fukuyama congenital muscular dystrophy.

METHODS

We reviewed brain MR images of 25 patients with Fukuyama congenital muscular dystrophy and examined the autopsy specimens of a 23-month-old girl with the disease to determine the pathologic nature of the MR findings.

RESULTS

MR studies revealed two characteristic cerebellar abnormalities: (a) disorganized cerebellar folia (16 cases) that were recognized as unusual distortions of the cortex; and (b) clusters of intraparenchymal cysts (23 cases). The two lesions were located close to each other, and milder lesions tended to affect only the superior semilunar lobule. The autopsy specimen revealed small cerebellar cysts, which consisted of dilated subarachnoid spaces buried beneath the malformed cortex.

CONCLUSION

The disorganized folia represent cerebellar polymicrogyria, and the presence of cerebellar cysts is related to the polymicrogyria. These two MR changes are often present in Fukuyama congenital muscular dystrophy and are distinct enough to suggest the radiologic diagnosis.

Full Text

The Full Text of this article is available as a PDF (2.5 MB).


Articles from AJNR: American Journal of Neuroradiology are provided here courtesy of American Society of Neuroradiology

RESOURCES