Effect of Intrathecal Baclofen on Delayed-Onset Paroxysmal Dystonia due to Compression Injury Resulting From Congenital and Progressive Spinal Bone Deformities in Chondrodysplasia Punctata

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• 作者：Tetsuya Okazaki ; MD^a ; ^{t-okazaki@med.tottori-u.ac.jp" class="auth_mail" title="E-mail the corresponding author} ; Yoshiaki Saito ; MD ; PhD^a ; Riyo Ueda ; MD^a ; Susumu Sugihara ; MD^a ; Akiko Tamasaki ; MD ; PhD^a ; Yoko Nishimura ; MD^a ; Koyo Ohno ; MD^a ; Masami Togawa ; MD^a ; ^b ; Takako Ohno ; MD ; PhD^c ; Akiyoshi Horie ; MD ; PhD^d ; Masashi Honda ; MD ; PhD^e ; Atsushi Takenaka ; MD ; PhD^e ; Hideki Nagashima ; MD ; PhD^f ; Yoshihiro Maegaki ; MD ; PhD^a
• 关键词：brachytelephalangy ; spinal cord ; myelopathy ; atlantoaxial dislocation ; paroxysmal dystonia ; chondrodysplasia punctata ; detrusor-sphincter dyssynergia ; intrathecal baclofen
• 刊名：Pediatric Neurology
• 出版年：2016
• 出版时间：March 2016
• 年：2016
• 卷：56
• 期：Complete
• 页码：80-85.e2
• 全文大小：2875 K

文摘

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.

Patient Description

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.

Conclusion

Paroxysmal dystonia is unusual in children with spinal cord lesions; however, it should be recognized for appropriate individualized clinical management.