Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is a rare autosomal dominant progressive degenerative white matter disease characterized by axonal loss, axonal spheroids and pigmented microglia, caused by heterozygous mutations in the colony stimulating factor-1 receptor (CSF1R) gene. Patients commonly present with progressive cognitive decline and psychiatric symptoms. Motor symptoms such as gait ataxia, spasticity and dystonia may also occur. The authors report a unique case of ALSP that presented initially as a young stroke with acute onset of left sided hemiparesis, in the absence of preceding history of cognitive decline or behaviour change. Subsequent cognitive decline and behavioural change lead to a consideration of an alternative diagnosis. The authors believe that the initial sites of the associated neurodegeneration in our patient could have primarily involved the bilateral corona radiata, resulting in motor deficit as the first symptom, which led to the patient initially being diagnosed as having a stroke. This is a novel presentation of acute stroke-like symptoms in a patient with ALSP. There has been no previous report of a similar presentation in the current literature. The authors hope that clinicians could learn from our experience to keep an open mind in considering various differentials when encountering a case of possible “stroke”.

ADULT-ONSET LEUKOENCEPHALOPATHY WITH AXONAL SPHEROIDS AND PIGMENTED GLIA PRESENTING WITH ACUTE STROKE-LIKE SYMPTOMS