Dystonia (tongue protrusion) helped by sensory trick in pantothenate kinase-associated neurodegeneration

This 32-year-old man had onset of dystonic foot inversion and handwriting difficulties due to hand dystonia at 17 years of age. He later developed gait difficulties, falls, dysarthria with unintelligible speech, and difficulties with chewing. He now exhibits oromandibular and lingual dystonia, which interfere with his swallowing, chewing, and speech. Placing a cigarette applicator into his mouth markedly relieves his dystonic tongue protrusion (a sensory trick). An MRI scan of the brain shows the typical "eye-of-the-tiger" hypointensity in the globus pallidus bilaterally on T2-weighted images. The CT shows bilateral globus pallidus hyperintensities. This patient is the brother of the teenage girl and a cousin of the 28-year-old woman who also have generalized dystonia due to PKAN and are shown in other clips. (Contributed by Dr. Joseph Jankovic.)