Abstract
A newly described disease is characterized by anterior bilateral temporal lobe cysts
associated with multilobar leukoencephalopathy and a non-progressive clinical course.
We report a patient with bilateral anterior temporal lobe cystic changes associated
with a non-progressive neurological disorder, microcephaly, spasticity, mental retardation,
and sensorineural deafness. From the literature, 12 other patients have shown a similar
phenotype. The common neuroradiological findings in these patients have been bilateral
anterior temporal lobe cystic changes and non-progressive leukoencephalopathy. By
contrast, variability in the clinical phenotype has been observed, ranging from severe
neuromotor handicap with mental retardation and microcephaly to spasticity in the
lower limbs associated with normal cognitive function. The pathological basis of the
defect remains to be defined.
Keywords
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Article info
Publication history
Accepted:
April 30,
2004
Received in revised form:
April 26,
2004
Received:
December 3,
2003
Identification
Copyright
© 2004 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
