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Mills' syndrome revisited.

Stephan R Jaiser1,2,3, Dipayan Mitra1,4, Timothy L Williams2

  • 1Institute of Neuroscience, Medical School, Newcastle University, Framlington Place, Newcastle upon Tyne, NE2 4HH, UK.

Journal of Neurology
|January 12, 2019
PubMed
Summary

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Mills' syndrome is a rare motor neuron disorder causing progressive leg weakness. This study proposes diagnostic criteria, distinguishing it from other motor neuron diseases like ALS.

Area of Science:

  • Neurology
  • Neuroscience
  • Motor Neuron Diseases

Background:

  • Mills' syndrome is an idiopathic, progressive spastic hemiparesis.
  • Understanding its distinct clinical and electrophysiological profile is crucial for accurate diagnosis.

Observation:

  • Three long-term cases of Mills' syndrome are presented, with symptom onset in the leg.
  • MRI revealed cervical cord T2 hyper-intensity, with abnormal motor evoked potentials and beta-band intermuscular coherence.

Findings:

  • Mills' syndrome presents with progressive spastic hemiparesis, often starting in the leg.
  • Electrophysiological studies show prolonged central motor conduction times and altered intermuscular coherence.
  • Amyotrophy can develop, suggesting overlap with motor neuron disease spectrum.
Keywords:
MRIMills’ syndromeNeurophysiologySpastic hemiparesis

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Implications:

  • Mills' syndrome is a distinct neurological entity within the motor neuron disorder spectrum.
  • Proposed diagnostic criteria aid in differentiating it from ALS and PLS.
  • Estimated prevalence is at least 1.2:1,000,000, highlighting its rarity.