구척수근위축증에서 감각신경병의 단면적 및 종적 연구

Abstract

Objectives: Bulbo-spinal muscular atrophy (BSMA) is an inherited motor neuronopathy, but it is known that subclinical sensory neuropathy can be found. The objective of this study is to clarify the features of sensory neuropathy by cross-sectional and longitudinal studies. Method: We analyzed the clinical and electrodiagnostic data of 41 BSMA patients who were genetically confirmed. Follow-up studies were performed in 10 patients among them. Results: Of 41 patients, 11 complained of sensory symptoms (26.8%), such as numbness or paresthesia of distal extremities. However, sensory neuropathy was observed in 23 patients (56.1%) with nerve conduction study (NCS). Reduced amplitude of action potentials was the most remarkable finding of the group with sensory neuropathy. For 10 patients with follow-up NCS, the mean follow-up interval was 8 years. There was no significant temporal change between the first and the follow-up sensory NCS. Conclusion: Subclinical sensory neuropathy was found in 56.1% of BSMA patients. The longitudinal study shows that subclinical sensory neuropathy in BSMA may not progress over time.