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

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.)

Associated Disorders

  • Abetalipoproteinemia
  • Antiphospholipid syndrome
  • Ataxia telangiectasia
  • Ataxia with CoQ10 deficiency
  • Ataxia with vitamin E deficiency
  • Athetosis
  • Ballism
  • Benign developmental movement disorders
  • Benign hereditary chorea
  • Benign idiopathic dystonia of infancy
  • Benign neonatal sleep myoclonus
  • Benign paroxysmal torticollis
  • Bobble-head doll syndrome
  • Bradykinesia
  • Cerebral palsy
  • Chorea
  • Cortical myoclonus
  • Dopa-responsive dystonia
  • Drug-induced movement disorders
  • Drug-induced myoclonus
  • Essential tremor
  • Focal dystonia
  • Friedrich ataxia
  • Galactosemia
  • Generalized dystonia
  • Geniospasm
  • Glutaric aciduria
  • Gluten-associated ataxia
  • Hartnup disease
  • Head-nodding
  • Infantile shuddering attacks
  • Jitteriness
  • Juvenile Huntington disease
  • Juvenile neuronal ceroid lipofuscinosis
  • Juvenile parkinsonism
  • Leigh syndrome
  • Lesch-Nyhan disease
  • Methylmalonic acidemia
  • Miller-Fischer syndrome
  • Myoclonus-dystonia
  • Neuroacanthocytosis
  • Neurodegeneration with brain iron accumulation
  • Niemann Pick C
  • Oculogyric crisis
  • Oppenheimer dystonia
  • Opsoclonus myoclonus
  • Paroxysmal exertional dyskinesia
  • Paroxysmal kinesigenic dyskinesia
  • Paroxysmal tonic upgaze of infancy
  • Phenylketonuria
  • Physiologic myoclonus
  • Post-infectious chorea
  • Post-pump chorea
  • Postinfectious cerebellitis
  • Postinfectious myoclonus
  • Provisional tic disorder (Transient tic disorder)
  • Refsum disease
  • Restless legs syndrome
  • Sandifer syndrome
  • Self-stimulatory behavior
  • Spasmus nutans
  • Spinal myoclonus
  • Stereotypies
  • Sydenham chorea
  • Tardive dyskinesia
  • Tic disorders
  • Tourette syndrome
  • Tyrosine hydroxylase deficiency
  • Wernicke encephalopathy
  • Wilson disease
  • Withdrawal emergent syndrome