title: Effect of Intrathecal Baclofen on Delayed-Onset Paroxysmal Dystonia due to Compression Injury Resulting From Congenital and Progressive Spinal Bone Deformities in Chondrodysplasia Punctata.
abstract: BACKGROUND
  Dystonia due to spinal lesions in adult patients is characterized by the provocation and/or amelioration of the spasm by somatosensory stimulation with a sensory trick.
  
  
  METHODS
  An infant with brachytelephalangic chondrodysplasia punctata developed flaccid tetraplegia due to cervical cord compression resulting from congenital atlantoaxial dislocation. Episodic, tonic extension of the extremities, neck, and trunk had appeared daily since age two years and was often provoked by tactile stimulation. Although decompression surgery was performed at age three years, progressive spinal deformity resulted in the aggravation of episodic dystonia thereafter, lasting for hours. Foot dorsiflexion and wearing a truncal brace for scoliosis inhibited these spasms. Intrathecal baclofen bolus injection transiently ameliorated the paroxysmal dystonia and detrusor-sphincter dyssynergia in the lower urinary tract.
  
  
  CONCLUSIONS
  Paroxysmal dystonia is unusual in children with spinal cord lesions; however, it should be recognized for appropriate individualized clinical management.
authors.affiliation: Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Yonago, Japan. Electronic address: t-okazaki@med.tottori-u.ac.jp.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Okazaki
authors.affiliation: Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Saito
authors.affiliation: Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Riyo
authors.initials: R
authors.lastname: Ueda
authors.affiliation: Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Susumu
authors.initials: S
authors.lastname: Sugihara
authors.affiliation: Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Tamasaki
authors.affiliation: Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Nishimura
authors.affiliation: Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Koyo
authors.initials: K
authors.lastname: Ohno
authors.affiliation: Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Yonago, Japan; Department of Pediatrics, Tottori Prefectural Central Hospital, Tottori, Japan.
authors.forename: Masami
authors.initials: M
authors.lastname: Togawa
authors.affiliation: Western Shimane Medical and Welfare Center for the Disabled, Shimane, Japan.
authors.forename: Takako
authors.initials: T
authors.lastname: Ohno
authors.affiliation: Department of Pediatrics, Shimane University, Faculty of Medicine, Shimane, Japan.
authors.forename: Akiyoshi
authors.initials: A
authors.lastname: Horie
authors.affiliation: Division of Urology, Department of Surgery, Tottori University Faculty of Medicine, Yonago, Japan.
authors.forename: Masashi
authors.initials: M
authors.lastname: Honda
authors.affiliation: Division of Urology, Department of Surgery, Tottori University Faculty of Medicine, Yonago, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Takenaka
authors.affiliation: Department of Orthopedic Surgery, Tottori University, Yonago, Tottori, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Nagashima
authors.affiliation: Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Maegaki
mesh_terms: D001176:Arthrogryposis / Q000150:complications* / Q000209:etiology; D001418:Baclofen / Q000627:therapeutic use*; D002675:Child, Preschool; D002806:Chondrodysplasia Punctata / Q000150:complications* / Q000000981:diagnostic imaging; D004421:Dystonia / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology*; D015417:Hereditary Sensory and Motor Neuropathy / Q000150:complications* / Q000209:etiology; D006801:Humans; D007278:Injections, Spinal; D008279:Magnetic Resonance Imaging; D008297:Male; D009125:Muscle Relaxants, Central / Q000627:therapeutic use*
chemical_list: D009125:Muscle Relaxants, Central; D001418:Baclofen
keywords: atlantoaxial dislocation; brachytelephalangy; chondrodysplasia punctata; detrusor-sphincter dyssynergia; intrathecal baclofen; myelopathy; paroxysmal dystonia; spinal cord