Unravelling the etiology of sporadic late-onset cerebellar ataxia in a cohort of 205 patients: a prospective study.

BACKGROUND: Despite recent progress in the field of genetics, sporadic late-onset (> 40 years) cerebellar ataxia (SLOCA) etiology remains frequently elusive, while the optimal diagnostic workup still needs to be determined. We aimed to comprehensively describe the causes of SLOCA and to discuss the relevance of the investigations. METHODS: We included 205 consecutive patients with SLOCA seen in our referral center. Patients were prospectively investigated using exhaustive clinical assessment, biochemical, genetic, electrophysiological, and imaging explorations. RESULTS: We established a diagnosis in 135 (66%) patients and reported 26 different causes for SLOCA, the most frequent being multiple system atrophy cerebellar type (MSA-C) (41%). Fifty-one patients (25%) had various causes of SLOCA including immune-mediated diseases such as multiple sclerosis or anti-GAD antibody-mediated ataxia; and other causes, such as alcoholic cerebellar degeneration, superficial siderosis, or Creutzfeldt-Jakob disease. We also identified 11 genetic causes in 20 patients, including SPG7 (n = 4), RFC1-associated CANVAS (n = 3), SLC20A2 (n = 3), very-late-onset Friedreich's ataxia (n = 2), FXTAS (n = 2), SCA3 (n = 1), SCA17 (n = 1), DRPLA (n = 1), MYORG (n = 1), MELAS (n = 1), and a mitochondriopathy (n = 1) that were less severe than MSA-C (p < 0.001). Remaining patients (34%) had idiopathic late-onset cerebellar ataxia which was less severe than MSA-C (p < 0.01). CONCLUSION: Our prospective study provides an exhaustive picture of the etiology of SLOCA and clues regarding yield of investigations and diagnostic workup. Based on our observations, we established a diagnostic algorithm for SLOCA.

Robotic-assisted laparoscopic repair of rectovesical fistula after Hartmann's reversal procedure.

The case is of a 59-year-old male with history of severe ischemic colitis following emergent intervention for a ruptured infrarenal aortic aneurysm who subsequently underwent left hemicolectomy, partial proctectomy, and Hartmann colostomy. The patient later underwent reversal of the Hartmann colostomy with diverting ileostomy. The surgery was complicated by a right ureteral and posterior bladder injury that resulted in a large rectovesical fistula involving the right hemitrigone and right ureteral orifice. An attempt to repair the rectovesical fistula at an outside facility was unsuccessful. Then, he underwent a robotic-assisted laparoscopic repair of rectovesical fistula, including simple prostatectomy, excision of rectovesical fistulous tract, rectal closure, peritoneal and omental flap interposition, bladder neck reconstruction, vesicourethral anastomosis and right ureteral reimplantation. There were no intraoperative or postoperative complications, and the patient was discharged at postoperative day 4; cystoscopy at 6-week follow-up demonstrated a successful closure of the fistula, at which time the ureteral stents were removed.