NO TO HATTATSU
Online ISSN : 1884-7668
Print ISSN : 0029-0831
ISSN-L : 0029-0831
A Case of Pseudoachondroplasia with Atlanto-axial Dislocation
Kazuyo KuzumeHideo NagaoMitsugi TakahashiTakehiko MorimotoNozomi SanoShinzi HabaraHiroshi Matsuda
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JOURNAL FREE ACCESS

1983 Volume 15 Issue 6 Pages 507-511

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Abstract

Pseudoachondroplasia is a short-limbed dwarfism with marked ligamentous laxity. Dwarfism is not recognized until after the second year of life, and the body proportion resembles achondroplasia. However, the head and face are normal. In some patients cervical myelopathy caused by atlanto-axial dislocation is revealed.
A 14-year-old boy with pseudoachondroplasia associated with chronic cervical cord myelopathy was reported. He was admitted to Ehime University Hospital for evaluation and treatment of short-limbed dwarfism, complete paraplegia and respiratory failure. Roentgenographically, fragmentation and irregular changes of the epiphyses in the extremities, hypoplasia of vertebrae and odontoid process, and atlanto-axial dislocation were seen.
Surgical treatment of atlanto-axial dislocation was performed. However, no improvement of neurologicaland respiratory signs was obtained.
This report indicates the importance of early diagnosis and treatment of atlanto-axial dislocation.

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© Japanese Society of Child Neurology
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