Tongue atrophy in facioscapulohumeral muscular dystrophy

Involvement of the lingual muscle is considered one of the exclusion criter ia of facioscapulohumeral muscular dystrophy (FSHD) In a series of 151 Japa nese patients with 4q35-FSHD, seven patients (4.6%) had tongue atrophy with abnormal MRI findings and typical myogenic patterns of electromyography. A ll seven patients belong to a group of early-onset FSHD with large gene del etions on chromosome 4q35. Our result suggests that the patients with 4q35- FSHD could have myopathic tongue atrophy.