Jaw drop in Kennedy’s disease

Kennedy’s disease, or spinal and bulbar muscular atrophy (SBMA), is an inherited X-linked degenerative disease of sensory and motor neurons caused by a trinucleotide (CAG) repeat expansion in the first exon of the androgen receptor (AR) gene.1 Several reports2-4⇓⇓ have emphasized the distinguishing clinical features of the disease, which include slowly progressive proximal greater than distal limb weakness, bulbar weakness involving primarily facial and tongue muscles, perioral fasciculations, sensory involvement, elevated creatine kinase (CK), and signs of androgen insensitivity (gynecomastia and testicular atrophy). Despite improved awareness of SBMA, patients are still frequently misdiagnosed with other disorders, such as, ALS, myopathy, or MG. Recently, we encountered two patients with SBMA who had been misdiagnosed after presenting with “jaw drop.” A 60-year-old man with diabetes had difficulty chewing and a generalized sense of fatigue. He manually manipulated his jaw to talk or chew and avoided foods …