This retrospective study was conducted to describe clinical and investigational findings and to determine the effect of
thiamine treatment on mortality in patients admitted with acute non-infectious
encephalopathy to a hospital in Sikkim between October 2019 and March 2021. Amongst 37 included patients the median age was 4 mo (IQR 3-5), 62.2% were males, 75% were exclusively breastfed infants, 67.6% and 89.2% patients had ophthalmologic and respiratory abnormalities respectively. Multisystem involvement was common. Bilateral basal ganglia involvement was noted in 75% of neuroimaging. Biochemical
thiamine deficiency was confirmed in one infant. None of the 11 patients who received
thiamine died whereas 20 among 26 patients who did not receive
thiamine died [case fatality rate (CFR) 76.9%].
Thiamine treatment was significantly associated with reduced odds of mortality (aOR 0.046, 95% CI 0.0024-0.86, p 0.039). In patients with acute non-infectious
encephalopathy and bilateral basal ganglia involvement
thiamine use was associated with decreased mortality.