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Intracranial findings in progressive facial hemiatrophy
J A Fry1, A Alvarellos, C W Fink
1Division of Pediatric Neurology, University of Texas Southwestern Medical Center, Dallas 75235.
The Journal of Rheumatology
|June 1, 1992
Summary
Cerebral lesions may be more common in children with progressive facial hemiatrophy. Magnetic resonance imaging (MRI) can detect white matter abnormalities, even in asymptomatic patients, suggesting its diagnostic value.
Area of Science:
- Neurology
- Pediatrics
- Radiology
Background:
- Progressive facial hemiatrophy is a rare condition.
- Cerebral lesions have been infrequently reported in association with this condition.
Purpose of the Study:
- To evaluate the occurrence of cerebral lesions in children with progressive facial hemiatrophy.
- To compare the diagnostic sensitivity of cranial computed tomography (CT) and magnetic resonance imaging (MRI) in detecting these lesions.
Main Methods:
- Evaluation of six children diagnosed with progressive facial hemiatrophy.
- Cranial CT scans were performed on five patients.
- Cranial MRI scans were performed on five patients, focusing on T2-weighted images.
Main Results:
- Four out of six patients presented with neurological deficits, including seizures, hemiparesis, and learning problems.
- Cranial CT revealed bony and soft tissue defects in five patients, with cerebral calcifications noted in only three.
- Cranial MRI demonstrated ipsilateral white matter abnormalities on T2-weighted images in all five patients with upper facial atrophy, irrespective of neurological symptoms.
Conclusions:
- Ipsilateral cerebral lesions associated with progressive facial hemiatrophy may be more prevalent than previously thought.
- MRI appears to be more sensitive than CT in identifying white matter abnormalities in these patients.
- MRI can detect subclinical white matter changes, highlighting its importance in the diagnostic evaluation of progressive facial hemiatrophy.