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Parry-Romberg syndrome: follow-up imaging during suppressive therapy
H Goldberg-Stern1, T deGrauw, M Passo
1Department of Neurology, Children's Hospital Medical Center, Cincinnati 45229-3039, USA.
Abstract:
Parry-Romberg syndrome is a poorly--understood disorder characterized by progressive hemifacial atrophy involving the skin, soft tissue, and bone. Involvement of the central nervous system with impairment in neurologic function occurs infrequently. We describe a child with this syndrome in whom central nervous system involvement, documented on serial MRI, played a prominent role. We have attempted to correlate the clinical course with the radiologic findings, and to determine the impact of prednisone and methotrexate on the intracranial lesions.
Insights
Parry-Romberg syndrome, a rare disorder of facial atrophy, can affect the central nervous system. This case study details a child with significant neurological involvement, monitored via MRI, and explores treatment impacts.
Area of Science:
- Neurology
- Dermatology
- Radiology
Background:
- Parry-Romberg syndrome is a rare, progressive disorder causing unilateral facial atrophy affecting skin, soft tissues, and bone.
- Central nervous system (CNS) involvement with neurological deficits is an infrequent but serious complication of Parry-Romberg syndrome.
Observation:
- This report details a pediatric case of Parry-Romberg syndrome with prominent CNS involvement.
- Serial magnetic resonance imaging (MRI) documented the progression and extent of intracranial lesions.
Findings:
- The study correlates the clinical presentation and neurological status with radiologic findings in the affected child.
- The impact of immunosuppressive therapy, specifically prednisone and methotrexate, on the intracranial lesions was investigated.
Implications:
- This case highlights the importance of considering CNS involvement in Parry-Romberg syndrome, even when facial atrophy is the primary presentation.
- Understanding the correlation between clinical and radiological findings can guide therapeutic strategies and improve patient outcomes for this rare condition.