Thelansis

 Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is an uncommon neurological disorder characterized by demyelination in the brain's white matter, enlarged axons, and pigmented glial cells. ALSP encompasses two clinicopathologically similar conditions formerly identified as hereditary diffuse leukoencephalopathy with spheroids (HDLS) and pigmentary orthochromatic leukodystrophy (POLD). The genetic, structural, and neuro-pathophysiological abnormalities associated with ALSP lead to various neurological symptoms, including cognitive dysfunction, movement disorders, motor impairment, familial dementia, subcortical gliosis of the Neumann type, and neuropsychiatric complications, ultimately resulting in a decline in quality of life and premature death. Currently, no approved targeted therapy for ALSP exists, and only a few off-label treatments have shown short-term efficacy. ALSP falls within the category of leukodystrophy disorders, accounting for 10 to 2...