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Alternating hemiplegia of childhood (AHC) is a rare neurodevelopmental disorder with an incidence of one per million, characterized by paroxysmal alternating hemiplegia, eye movement abnormalities, dystonia, and epilepsy.1 In 2012, mutations in the ATP1A3 gene encoding the Na+-K+-ATPase α3 subunit (originally discovered in rapid-onset dystonia-parkinsonism) were identified as the cause for AHC.1,2 However, the diagnosis of AHC is still often delayed or missed. We report a case of AHC diagnosed in adulthood; to our knowledge, this is the first report in a person of Malay ethnicity. There are very few studies utilizing more advanced neuroimaging in AHC, and none have specifically used diffusion tensor imaging (DTI). DTI allows detection of loss of white matter tract (WMT) integrity even when conventional MRI appears normal.3 Here, we documented novel DTI abnormalities in AHC. A 24-year-old Malay woman with global developmental delay since infancy was referred with a seven-year history of recurrent painful paroxysmal left hemidystonia. The frequency of attacks ranged from 3–4 times daily to 2–3 times a month. Each attack usually lasted a few minutes and occasionally up to one hour. Triggers included excitement, bright lights, and crowded places. There was no association with sudden movements or prolonged exercise. Interictally, she could ambulate and perform activities independently. Family history was negative. Further probing revealed a history of paroxysmal hemiplegia, alternating from side to side, starting at age 13 months. Each hemiplegic episode lasted a few days and occurred 4–5 times a month with gradual resolution by age two years. General examination revealed normal stature with no visual, hearing or bony abnormalities. There was apraxia of horizontal eye movements, severe dysarthria, and right torticollis (Supplementary Video 1 in the online-only Data Supplement). There was dystonic posturing in the fingers, which was worse on the left and more pronounced during writing. Repetitive hand movements were slow. There was no tremor, rigidity or dysmetria. Limb power was full with normal tendon and plantar reflexes. Gait was mildly ataxic. The Montreal Cognitive Assessment score was 4/30. Two episodes of painful hemidystonia lasting a few minutes were witnessed during consultation, with left-sided wrist flexion and ankle inversion. Routine blood tests, brain MRI and electroencephalogram were normal. Analysis of the ATP1A3 gene confirmed the diag-

Alternating Hemiplegia of Childhood in a Person of Malay Ethnicity with Diffusion Tensor Imaging Abnormalities