Palato-Lingual Tremor in a Child With Juvenile-Onset Alexander Disease

Abstract

Alexander disease is an autosomal dominant leukodystrophy associated with mutations in the gene encoding the glial fibrillary acidic protein (GFAP). The patients present with progressive motor dysfunction in the form of gait disturbance, fine motor difficulties, and bulbar symptoms, particularly palatal tremor. In this article, we have presented an interesting illustrative case of palato-lingual tremor in a 10-year-old girl with juvenile-onset Alexander disease. Typically, the term “palatal myoclonus” is used to describe the abnormal movement of soft palate in patients with Alexander disease.