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A case of Arnold-Chiari syndrome with flaccid paralysis and huge syringomyelia.
Spinal Cord. 2004 Sep; 42(9):541-4.SC

Abstract

STUDY DESIGN

A case report.

SETTING

Department of Orthopaedic Surgery, Shiga University of Medical Science, Japan.

PATIENT

A 13-year-old woman presented progressive weakness in the lower extremities, with predominance on the right. Magnetic resonance (MR) imaging revealed a huge syrinx. The patient also showed scoliosis, cleft palate, hearing impairment, excessive sweating, hairiness, dural ectasia, and malformation of the skull. METHOD AND OBJECTIVES: We treated a very rare case of Arnold-Chiari syndrome, which presented with flaccid paralysis. Methods of differential diagnosis and suitable treatment are discussed.

RESULTS AND CONCLUSION

Both the syrinx and muscle strength were quickly improved following placement of a syringo-peritoneal (S-P) shunt, after which the patient recovered the ability to walk. However, transient hypesthesia in the right hand occurred after the operation. The syrinx around the conus was thought to play a crucial role in the etiology of the patient case, which showed unique symptoms.

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Publisher Full Text

Authors+Show Affiliations

Mimura T
Department of Orthopaedic Surgery, Shiga University of Medical Science, Tsukinowa-cho, Seta, Otsu, Shiga, Japan.
Asajima S
No affiliation info available
Saruhashi Y
No affiliation info available
Matsusue Y
No affiliation info available

MeSH

AdolescentArnold-Chiari MalformationCerebrospinal Fluid ShuntsFemaleGait Disorders, NeurologicHumansMuscle HypotoniaMuscle WeaknessParalysisSeverity of Illness IndexSyringomyeliaTreatment Outcome

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

15111990

Citation

Mimura, T, et al. "A Case of Arnold-Chiari Syndrome With Flaccid Paralysis and Huge Syringomyelia." Spinal Cord, vol. 42, no. 9, 2004, pp. 541-4.
Mimura T, Asajima S, Saruhashi Y, et al. A case of Arnold-Chiari syndrome with flaccid paralysis and huge syringomyelia. Spinal Cord. 2004;42(9):541-4.
Mimura, T., Asajima, S., Saruhashi, Y., & Matsusue, Y. (2004). A case of Arnold-Chiari syndrome with flaccid paralysis and huge syringomyelia. Spinal Cord, 42(9), 541-4.
Mimura T, et al. A Case of Arnold-Chiari Syndrome With Flaccid Paralysis and Huge Syringomyelia. Spinal Cord. 2004;42(9):541-4. PubMed PMID: 15111990.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A case of Arnold-Chiari syndrome with flaccid paralysis and huge syringomyelia. AU - Mimura,T, AU - Asajima,S, AU - Saruhashi,Y, AU - Matsusue,Y, PY - 2004/4/28/pubmed PY - 2004/10/20/medline PY - 2004/4/28/entrez SP - 541 EP - 4 JF - Spinal cord JO - Spinal Cord VL - 42 IS - 9 N2 - STUDY DESIGN: A case report. SETTING: Department of Orthopaedic Surgery, Shiga University of Medical Science, Japan. PATIENT: A 13-year-old woman presented progressive weakness in the lower extremities, with predominance on the right. Magnetic resonance (MR) imaging revealed a huge syrinx. The patient also showed scoliosis, cleft palate, hearing impairment, excessive sweating, hairiness, dural ectasia, and malformation of the skull. METHOD AND OBJECTIVES: We treated a very rare case of Arnold-Chiari syndrome, which presented with flaccid paralysis. Methods of differential diagnosis and suitable treatment are discussed. RESULTS AND CONCLUSION: Both the syrinx and muscle strength were quickly improved following placement of a syringo-peritoneal (S-P) shunt, after which the patient recovered the ability to walk. However, transient hypesthesia in the right hand occurred after the operation. The syrinx around the conus was thought to play a crucial role in the etiology of the patient case, which showed unique symptoms. SN - 1362-4393 UR - https://www.unboundmedicine.com/medline/citation/15111990/A_case_of_Arnold_Chiari_syndrome_with_flaccid_paralysis_and_huge_syringomyelia_ L2 - https://doi.org/10.1038/sj.sc.3101607 DB - PRIME DP - Unbound Medicine ER -