A hereditary, autosomal dominant, adult-onset, rapidly progressive NEURODEGENERATIVE DISORDER characterized by variable behavioral, cognitive, and motor changes. Patients often die of DEMENTIA within 6 years of onset. NEUROIMAGING shows patchy abnormalities in the cerebral WHITE MATTER, predominantly affecting the FRONTAL LOBE and PARIETAL LOBE. Mutations in the CSF1R gene have been identified. OMIM: 221820
Also Known As:
Adult-Onset Leukodystrophy with Neuroaxonal Spheroids; Autosomal Dominant Leukoencephalopathy with Neuroaxonal Spheroids; Dementia, Familial, Neumann Type; Gliosis, Familial Progressive Subcortical; Hereditary Diffuse Leukoencephalopathy with Axonal Spheroids; Leukoencephalopathy with Neuroaxonal Spheroids, Autosomal Dominant; Leukoencephalopathy, Diffuse Hereditary, With Spheroids; Neuroaxonal Leukodystrophy; Subcortical Gliosis of Neumann