Frequency, Symptomology, and Course of HIV-Associated ALS: Case Series and Review

The pathogenesis of Amyotrophic Lateral Sclerosis (ALS) is not entirely understood, but there appears to be a relationship between ALS and viral infections including HIV-1. The impact of HIV infection and antiretroviral therapy on the presentation, diagnosis, and progression of ALS is not well-defined. This is a retrospective chart review in which we analyzed records of patients who presented to the ALS Clinic from September 2006 to June 2018. We sought to identify HIV-positive patients receiving antiretroviral therapy who were subsequently diagnosed with ALS. Our primary goals were to analyze prevalence of HIV positivity among our ALS patients, describe their ALS presentation and progression, and compare our study cases to ALS characteristics in the general population. We hypothesize HIV-associated ALS is clinically different from ALS in the absence of HIV in several respects, specifically in that it exhibits on average younger age of onset, slower progression, milder symptomology, and better prognosis. We analyzed records of 322 patients who presented to the ALS Clinic from September 2006 to June 2018, and from that group identified three HIV-positive ALS patients. All three were males with cervical onset classic form of ALS, had been diagnosed with HIV for at least several years, and were undergoing HAART treatment. Each had slower disease progression and younger age of both onset and diagnosis than that described with ALS on average. All three were negative for family history of neurodegenerative disorders. Although limited in its number of patients, this small case series suggests a distinct profile of patients with HIV subsequently diagnosed with ALS. Confirmation with a large case control study or analysis of a large national database may lead to an emerging focus of ALS research related to the modulation of the immune system and its impact on ALS