Chiari Malformation and Syringomyelia Associated with Hirayama Disease.
Atlantoaxial instability
Chiari formation
Hirayama disease
Syringomyelia
Journal
World neurosurgery
ISSN: 1878-8769
Titre abrégé: World Neurosurg
Pays: United States
ID NLM: 101528275
Informations de publication
Date de publication:
Mar 2020
Mar 2020
Historique:
received:
03
11
2019
revised:
15
12
2019
accepted:
16
12
2019
pubmed:
28
12
2019
medline:
25
3
2020
entrez:
28
12
2019
Statut:
ppublish
Résumé
To the best of our knowledge, the presence of Chiari formation and Hirayama disease in the same patient has not been previously reported. On the basis of the presented case, we have attempted to identify the common pathogenesis of both of these clinical entities. We have reported a case of a 23-year-old male patient who had presented with complaints of pain in the nape of neck and shoulders of 9 months' duration, weakness and stiffness in all 4 limbs, and wasting and weakness of muscles of both hands of 6 months' duration. The investigations showed Chiari formation and syringomyelia. Additional investigations depicted the characteristic radiological features associated with Hirayama disease. The patient was treated by atlantoaxial and multisegmental subaxial cervical spinal fixation. Spinal instability seems to be the nodal point of the pathogenesis of both Chiari formation and Hirayama disease.
Sections du résumé
BACKGROUND
BACKGROUND
To the best of our knowledge, the presence of Chiari formation and Hirayama disease in the same patient has not been previously reported. On the basis of the presented case, we have attempted to identify the common pathogenesis of both of these clinical entities.
CASE DESCRIPTION
METHODS
We have reported a case of a 23-year-old male patient who had presented with complaints of pain in the nape of neck and shoulders of 9 months' duration, weakness and stiffness in all 4 limbs, and wasting and weakness of muscles of both hands of 6 months' duration. The investigations showed Chiari formation and syringomyelia. Additional investigations depicted the characteristic radiological features associated with Hirayama disease. The patient was treated by atlantoaxial and multisegmental subaxial cervical spinal fixation.
CONCLUSIONS
CONCLUSIONS
Spinal instability seems to be the nodal point of the pathogenesis of both Chiari formation and Hirayama disease.
Identifiants
pubmed: 31881343
pii: S1878-8750(19)33133-X
doi: 10.1016/j.wneu.2019.12.101
pii:
doi:
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
241-244Commentaires et corrections
Type : CommentIn
Type : CommentIn
Informations de copyright
Copyright © 2019 Elsevier Inc. All rights reserved.