Journal of Korean Neurosurgical Society

The Korean Neurosurgical Society (대한신경외과학회)
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Microvascular Decompression for Familial Hemifacial Spasm : Single Institute Experience

  • Park, Jae-Han (Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Jo, Kyung-Il (Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Hyun-Seok (Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Jung-A (Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Park, Kwan (Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • Received : 2012.09.10
  • Accepted : 2013.01.07
  • Published : 2013.01.28
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Abstract

Objective : The purpose of this study was to evaluate the characteristics and surgical outcomes of familial hemifacial spasm (HFS) and to discuss the role of genetic susceptibility. Methods : Between 2001 and 2011, 20 familial HFS patients with ten different pedigrees visited our hospital. The data from comprehensive evaluation of these patients, including clinical, radiological and electrophysiological data and surgical outcomes were reviewed to characterize familial HFS and to compare the characteristics between familial HFS and sporadic HFS. Results : According to the family tree, the inheritance pattern was difficult to define clearly using these data. Radiologic findings suggested that the vertebral artery (VA) was a more frequent offender in familial HFS than in sporadic cases (35.0% vs. 10.0%, p<0.001). Chi-square test showed that there were no correlation between VA tortuosity and underlying morbidity such as diabetes or hypertension (p=0.391). Eighteen out of 19 patients who underwent microvascular decompression showed no residual spasm. Other features of familial HFS overlap with sporadic cases. These findings suggest that certain genetic susceptibilities rather than hypertension or diabetes may influence vascular tortuosity and HFS development. Conclusion : In this study, familial HFS seems not so different from sporadic cases. Authors thought familial HFS could have heterogeneous etiology. Further study of familial HFS including clinical, anatomic, genetic, and molecular information may help identify a gene or trait that can provide insight into the mechanisms of sporadic and familial HFS.

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References

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