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Published on: March 23, 2022
[Mills' syndrome: a rare clinical entity]
S Lachaud1, M H Soriani, E Delmont
1Service d'EFSN, Hôpital Pasteur, Nice, France. Lachaud.s@chu-nice.fr
Introduction:
Mills' syndrome is a rare motor neuron disease, initially described by Mills in 1900 as a progressive ascending or descending hemiplegia without significant sensory involvement. This syndrome is of uncertain nosological status, and is supposedly due to unilateral primary degeneration of corticospinal pathway. Some authors have suggested that it could represent a variant of primary lateral sclerosis.
Methods:
We retrospectively studied the clinical and paraclinical data from eight patients with suspected Mills' syndrome hospitalized for diagnosis.
Results:
For all patients, the clinical course was slowly progressive, with motor deficiency, unilateral pyramidal signs (or bilateral with asymmetry), without bulbar signs, fasciculations or sensory deficit. Final diagnosis was Mills' syndrome (n=3), primary lateral sclerosis (n=1), myelitis of unknown origin (n=2), progressive primary multiple sclerosis (n=1), and antiphospholipid syndrome (n=1). The main arguments for final diagnosis were brought by electrophysiology and brain and spinal MRI.
Conclusion:
Mills' syndrome is a rare clinical diagnosis, requiring exhaustive investigations.
Insights
Mills' syndrome, a rare motor neuron disease, presents with progressive hemiplegia. Diagnosis requires extensive investigation, as other neurological conditions can mimic its symptoms.
Area of Science:
- Neurology
- Neuroscience
- Motor Neuron Diseases
Context:
- Mills' syndrome is a rare, poorly understood motor neuron disease characterized by progressive hemiplegia.
- Its exact nosological status remains uncertain, with potential links to primary lateral sclerosis.
- Understanding Mills' syndrome is crucial for accurate diagnosis and patient management.
Purpose:
- To retrospectively analyze clinical and paraclinical data of eight patients with suspected Mills' syndrome.
- To differentiate Mills' syndrome from other neurological disorders presenting with similar symptoms.
- To highlight the diagnostic challenges and the importance of comprehensive investigations.
Summary:
- Eight patients with suspected Mills' syndrome were evaluated.
- Clinical features included slowly progressive motor deficiency with unilateral or asymmetric pyramidal signs.
- Final diagnoses varied, with only three confirmed as Mills' syndrome, underscoring diagnostic complexity.
- Electrophysiology and MRI were key in differentiating diagnoses.
Impact:
- This study emphasizes the rarity and diagnostic complexity of Mills' syndrome.
- It underscores the necessity of thorough investigations, including advanced imaging and electrophysiology.
- Accurate diagnosis is vital for appropriate patient care and management of this rare motor neuron disease.
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