Adult-onset leukoencephalopathy with neuroaxonal spheroids and pigmented glia mimicking systemic lupus erythematosus cerebral vasculitis

- Consider a leukodystrophy in patients with a 'refractory' cerebral vasculitis
- CSF1R mutation analysis and a brain biopsy are often required to exclude mimics
- ALSP may present with early onset dementia and personality changes
- A progressive motor and gait disturbance is often seen, occasionally with seizures