Zhang ZY, Ouyang ZY, Zhao GH, Fang JJ. Mills’ syndrome is a unique entity of upper motor neuron disease with N-shaped progression: Three case reports. World J Clin Cases 2022; 10(19): 6664-6671 [PMID: 35979278 DOI: 10.12998/wjcc.v10.i19.6664]
Corresponding Author of This Article
Jia-Jia Fang, PhD, Chief Doctor, Department of Neurology, The Fourth Affiliated Hospital, Zhejiang University School of Medicine, No. N1 Shangcheng Road, Yiwu 322000, Zhejiang Province, China. fangjjiaj@zju.edu.cn
Research Domain of This Article
Clinical Neurology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Zhi-Yun Zhang, Guo-Hua Zhao, Jia-Jia Fang, Department of Neurology, The Fourth Affiliated Hospital, Zhejiang University School of Medicine, Yiwu 322000, Zhejiang Province, China
Zhi-Yuan Ouyang, Department of Neurology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310000, Zhejiang Province, China
Author contributions: Zhang ZY and Ouyang ZY both wrote and revised the text; Zhao GH and Fang JJ revised the manuscript for intellectual content; All authors read and approved the final manuscript.
Supported bythe Public Welfare Technology Research Program of Zhejiang Province, No. LGF20H090011; the Key Projects of Major Health Science and Technology Plan of Zhejiang Province, No. WKJ-ZJ-2129; and the Educational Reform Project of Medical College of Zhejiang University, No. jgyb20202033.
Informed consent statement: Three patients exhibiting progressive spastic hemiparesis similar to Mills’ original description were identified from the Inpatient Department of Neurology, Affiliated Hospital of Zhejiang University. All the procedures were approved by the ethics committees of The Fourth Affiliated Hospital, Zhejiang University School of Medicine. Informed consents were obtained from all the patients.
Conflict-of-interest statement: The authors did not provide a conflict of interest statement.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Jia-Jia Fang, PhD, Chief Doctor, Department of Neurology, The Fourth Affiliated Hospital, Zhejiang University School of Medicine, No. N1 Shangcheng Road, Yiwu 322000, Zhejiang Province, China. fangjjiaj@zju.edu.cn
Received: January 10, 2022 Peer-review started: January 10, 2022 First decision: March 15, 2022 Revised: April 10, 2022 Accepted: May 16, 2022 Article in press: May 16, 2022 Published online: July 6, 2022 Processing time: 164 Days and 18.6 Hours
Abstract
BACKGROUND
Mills’ syndrome is an extremely rare degenerative motor neuron disorder first described by Mills in 1900, but its nosological status is still not clear. We aimed to analyze the clinical features of Mills’ syndrome.
CASE SUMMARY
Herein, we present 3 cases with similar features as those described in Mills’ original paper and review the related literature. Our patients showed middle- and older-age onset, with only upper motor neuron symptoms evident throughout the course of the disease. Spastic hemiplegia began in the lower extremity with a unique progressive pattern.
CONCLUSION
We consider that Mills’ syndrome is a unique entity of motor neuron disorder with an N-shaped progression. Clinicians should maintain a high index of suspicion for the diagnosis of Mills’ syndrome when the onset involves lower extremity paralysis without evidence of lower motor neuron or sensory involvement.
Core Tip: Mills’ syndrome is an extremely rare degenerative motor neuron disorder, whose nosological status is currently uncertain. We report 3 cases with similar features as those described in Mills’ original paper. All patients had initial symptoms in one lower extremity that spread up to the homolateral upper limb, followed by the contralateral lower limb, and finally the contralateral upper limb. It is necessary to clarify the clinical features to receive more attention from clinicians.
