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Treatment of ZC4H2 Variant-Associated Spastic Paraplegia with Selective Dorsal Rhizotomy and Intensive Postoperative Rehabilitation: A Case Report.

Authors :
Toshiki Inotani
Akira Horaguchi
Yuko Morishita
Ayuko Yoshida
Misaki Otomo
Makoto Suzuki
Takehiko Inui
Yukimune Okubo
Shigemasa Komatsu
Chika Mizuno
Yuko Takahashi
Tatsuhiro Ochiai
Takeshi Kinjo
Takashi Asato
Jun Takayama
Gen Tamiya
Naoya Saijo
Atsuo Kikuchi
Kazuhiro Haginoya
Source :
Tohoku Journal of Experimental Medicine; Apr2024, Vol. 262 Issue 4, p239-244, 6p
Publication Year :
2024

Abstract

Selective dorsal rhizotomy (SDR) has been used to treat children with spastic cerebral palsy (CP), and its beneficial effect on quality of life and ambulation has been confirmed in long-term follow-up studies. However, the role of SDR in the treatment of spasticity in patients with hereditary spastic paraplegia (HSP) and related disorders is not well-established. Here, we report the first patient with the ZC4H2 variant who underwent SDR to treat spastic paraplegia. Abnormal gait was discovered during a regular checkup at the age of 3 years and 9 months, and she was diagnosed with spastic paraplegia. She was heterozygous for the ZC4H2 variant and underwent SDR at the age of 5 years and 11 months, which alleviated the spasticity. The patient underwent inpatient postoperative rehabilitation for 4 months and continued outpatient physiotherapy after discharge. The Gross Motor Function Measure-88 score and maximum walking speed decreased transiently 1 month postoperatively, but gradually recovered, and continuously improved 6 months postoperatively. SDR and postoperative intensive rehabilitation were effective in improving motor and walking functions up to 6 months after surgery, although long-term follow-up is needed to draw conclusions. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
00408727
Volume :
262
Issue :
4
Database :
Supplemental Index
Journal :
Tohoku Journal of Experimental Medicine
Publication Type :
Academic Journal
Accession number :
177303380
Full Text :
https://doi.org/10.1620/tjem.2024.J004