Published online Jul 6, 2022. doi: 10.12998/wjcc.v10.i19.6664
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
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.
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.
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.