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Involuntary movement in pediatric moyamoya disease patients: consideration of pathogenetic mechanism using
Ji Yeoun Lee1, Seung-Ki Kim, Kyu-Chang Wang
1Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, South Korea.
Purpose:
Involuntary movement is a rare symptom of moyamoya disease (MMD). No consensus has been reached regarding its clinical features and pathogenetic mechanism. Therefore, pediatric MMD patients presenting with involuntary movement were retrospectively analyzed, focusing on the image findings.
Methods:
A total of 513 patients who were treated for MMD were reviewed. After exclusion of MMD syndromes and those with accompanying conditions related to involuntary movements, five patients (mean age: 11.6 years, range: 5-13 years) were evaluated.
Results:
All of the patients improved their symptoms rapidly after the indirect bypass operations to the contralateral hemisphere. All remained symptom-free during the long follow-up period. Comprehensive evaluation of the preoperative imaging findings failed to suggest a characteristic feature in common, corresponding to the existing hypotheses or a new hypothesis. Only one patient showed infarction preoperatively, and only one patient showed prominently enhanced collateral vessels in the basal ganglia. Although a decrease in vascular reserve was observed in all patients, the location and laterality were nonspecific.
Conclusion:
There still appears to be confusion regarding the pathogenetic mechanism of involuntary movement in MMD with no repetitive, established imaging features to explain the phenomenon. Nonetheless, with its excellent response to surgical treatment, clinical awareness of this rare symptom of MMD should be emphasized as a differential diagnosis for secondary movement disorder in children.
Insights
Involuntary movements in pediatric moyamoya disease (MMD) lack clear imaging markers but rapidly improve after bypass surgery. This highlights MMD as a crucial differential diagnosis for childhood movement disorders.
Area of Science:
- Neurology
- Pediatric Neurology
- Vascular Neurology
Background:
- Involuntary movement is an uncommon presentation of moyamoya disease (MMD).
- The clinical characteristics and underlying mechanisms of MMD-associated involuntary movements remain unclear.
- Pediatric MMD with involuntary movements requires further investigation.
Purpose of the Study:
- To retrospectively analyze pediatric moyamoya disease patients with involuntary movements.
- To investigate the clinical features and imaging findings associated with involuntary movements in pediatric MMD.
- To explore potential pathogenetic mechanisms and diagnostic clues.
Main Methods:
- Retrospective review of 513 MMD patients.
- Exclusion of MMD syndromes and confounding conditions.
- Detailed evaluation of five pediatric MMD patients with involuntary movements, focusing on preoperative imaging.
Main Results:
- All five patients experienced rapid symptom improvement following indirect bypass surgery.
- No characteristic preoperative imaging findings were consistently identified.
- Vascular reserve decrease was noted in all patients, but imaging features were nonspecific.
Conclusions:
- The pathogenetic mechanism of involuntary movement in MMD remains poorly understood, with no definitive imaging features.
- Surgical intervention, specifically indirect bypass, demonstrates significant efficacy.
- Clinical awareness of MMD as a cause of secondary movement disorder in children is essential for timely diagnosis and treatment.
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