To analyze survival and prognostic survival factors in published case studies of ALSP patients. 

ALSP is a fatal and rare genetic disease characterized by neuronal and glial cell pathology, and rapidly progressive neurological symptoms (executive dysfunction, motor impairment, neuropsychiatric complications). ALSP is underrecognized with an estimated prevalence of 10,000 in the U.S., 15,000 in the EU27+UK and 4,000 in Japan. There are no approved treatments for ALSP.

Published case studies were identified based on prespecified selection criteria through a MEDLINE search (1/1/1980-04/302021). The search identified 76 reports with data extracted from 274 patients. This review represents the largest ALSP case series. 

Kaplan-Meier survival analysis was performed with survival time and disease duration variables related to age of onset of symptoms. Survival data included the number (%) of deceased patients, Kaplan-Meier estimates of time to death (25^th, 50^th and 75^th percentiles) and impact of selected categorical variables through a log-rank test with p-values. Estimates of median time to death were calculated with 95% confidence intervals.

This is the first survival analysis conducted for ALSP. Our data confirm the rapid and progressive nature of ALSP, support early intervention, and serve as a foundation for future clinical trial designs.