Improved clinical symptoms in three patients with spinocerebellar ataxia through a surgical decompression procedure of the posterior cranial fossa with subsequent flap transplantation of the cerebellum

Abstract

Objective:

This study aimed to explore the possibility and outcomes of surgical treatments for managing hereditary spinocerebellar ataxia (SCA).

Methods:

Three patients diagnosed with SCA and strongly willing to get surgical treatments were selected for surgery. Under general anesthesia, posterior cranial fossa decompression and extensive arachnoid resection were conducted. The bilateral occipital muscles flapped with arteries were anatomically separated, transferred, and adhered to the surface of the cerebellum. Then, clinical presentations pre- and post-operation were compared.

Results:

All symptoms of the three patients were significantly improved after surgery. In Case 1, after one day post-operation, bucking symptoms disappeared. Also, while standing and walking abilities gradually improved on the 6th day, self-care, speech, and normal handwriting abilities were recovered one month post-rehabilitation. However, during the 6-month follow-up, the patient was still in further recovery. In Case 2, the patient's handwriting function was restored on the 4th day after the operation. Moreover, the piebald skin on both lower limbs disappeared one week post-operation. One month later, the standing ability of the patient was also recovered. In Case 3, the four symptoms experienced were significantly recovered one day after surgery, including speech, bucking, lower limbs trembling, and unstable walking.

Conclusions:

Surgery is therefore a promising and brilliant option for treating hereditary SCA. After operations, neurological deficits improved fast, as shown evidently by recovery results, at a rate that was better than any other treatment way. Nevertheless, improvement mechanisms should further be explored.