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Hemifacial spasm associated with Marfan's syndrome: a case report
T Nagatani1, S Inao, J Yoshida
1Department of Neurosurgery, Nagoya University School of Medicine, Japan.
Neurosurgical Review
|October 31, 1998
Summary
This study reports a rare case of hemifacial spasm in a patient with Marfan syndrome. Microvascular decompression successfully treated the condition by relieving vascular compression of the facial nerve.
Area of Science:
- Neurology
- Genetics
- Cardiology
Background:
- Marfan syndrome is a genetic disorder affecting connective tissue.
- Cerebrovascular abnormalities are a known, though uncommon, complication of Marfan syndrome.
- Hemifacial spasm is a neuromuscular disorder characterized by involuntary contractions of facial muscles.
Observation:
- A 24-year-old woman with Marfan syndrome presented with a 6-year history of left hemifacial spasm.
- Imaging revealed bilateral, tortuous, and elongated vertebral arteries.
- The patient had undergone prior surgeries for ectopia lentis and kyphoscoliosis, consistent with Marfan syndrome features.
Findings:
- Surgical decompression of the left seventh cranial nerve at its root exit zone was performed.
- The facial nerve was compressed by the anterior inferior cerebellar artery (AICA) and the left vertebral artery.
- Post-operative resolution of hemifacial spasm was complete after Teflon padding was placed.
Implications:
- This case highlights a potential link between vascular anomalies in Marfan syndrome and hemifacial spasm.
- Microvascular decompression is an effective treatment for hemifacial spasm caused by vascular compression.
- Further research into cerebrovascular manifestations in Marfan syndrome is warranted.