Teaching NeuroImage: Ventricular Garlands in Late-Onset Alexander Disease

A 41-year-old woman developed an ataxic gait, dysarthria, and urinary incontinence after undergoing a cesarean section over 2 years ago.Before that, she had experienced a 23-year history of frequent falls.Physical examination revealed mildly decreased muscle strength in all 4 limbs (more pronounced distally and on the right side), normal sensory examination, and appendicular and gait ataxia.Brain MRI showed abnormalities in the deep white matter, brainstem, and cerebellum (Figure 1).In addition, ventricular garlands were observed (Figure 2).Genetic testing revealed a heterozygous variation (c.262C>T, p.Arg88Cys) in the glial fibrillary acidic protein gene, leading to a diagnosis of late-onset Alexander disease. 1