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Facial onset sensory and motor neuronopathy (FOSMN syndrome): Cases series and systematic review

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Abstract

Objective

To provide new and comprehensive evidence for diagnosis and management of FOSMN syndrome.

Methods

We reviewed our database to identify patients with FOSMN syndrome. Online database including PubMed, EMBASE, and OVID were also searched for relevant cases.

Results

We identified a total of 71 cases, including 4 cases from our database and 67 ones from online searching. A predominance of male was observed [44 (62.0%)] with median onset age of 53 (range: 7–75) years old. The median (range) disease duration was 60 (3–552) months at the time of the visit. The initial symptoms could be sensory deficits in face (80.3%) or oral cavity (4.2%), bulbar paralysis (7.0%), dysosmia (1.4%), dysgeusia (4.2%), weakness or numbness of upper limbs (5.6%), or lower limbs (1.4%). Abnormal blink reflex was presented in 64 (90.1%) patients. CSF tests showed elevated protein level in 5 (7.0%) patients. Six (8.5%) patients had MND-related gene mutation. Five (7.0%) patients showed transient responsiveness to immunosuppressive therapy, then deteriorated relentlessly. Fourteen (19.7%) patients died, with an average survival time of around 4 years. Among them, five patients died of respiratory insufficiency.

Conclusion

The age of onset, progress of disease course, and prognosis of FOSMN syndrome could be varied significantly. The prerequisites of diagnosis were progressive and asymmetric lower motor neuron dysfunction, with sensory dysfunction which usually showed in face at the onset. Immunosuppressive therapy could be tried in some patients with suspected inflammatory clues. In general, FOSMN syndrome tended to be motor neuron disease with sensory involvement.

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Acknowledgements

Authors would like to thank all the authors of the original articles.

Funding

National High Level Hospital Clinical Research Funding (2022-PUMCH-B-017), CAMS Innovation Fund for Medical Sciences (CIFMS 2021-I2M-1–003), Beijing Natural Science Foundation (7202158).

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Authors and Affiliations

  1. Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, 100730, Beijing, China

    Nan Hu, Lei Zhang, Xunzhe Yang, Hanhui Fu, Liying Cui & Mingsheng Liu

Authors
  1. Nan Hu
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  2. Lei Zhang
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  3. Xunzhe Yang
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  4. Hanhui Fu
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  5. Liying Cui
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  6. Mingsheng Liu
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Contributions

Nan Hu: data analysis and writing of the main manuscript; Lei Zhang: data collection; Xunzhe Yang: data collection; Hanhui Fu: data collection; Liying Cui: design of the project; Mingsheng Liu: design of the project and revision of the main manuscript. All authors have read and approved the manuscript.

Corresponding author

Correspondence to Mingsheng Liu.

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The study was approved by the Ethics Committee of the PUMCH (Peking Union Medical College Hospital).

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All included patients were provided written, informed consent.

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Hu, N., Zhang, L., Yang, X. et al. Facial onset sensory and motor neuronopathy (FOSMN syndrome): Cases series and systematic review. Neurol Sci 44, 1969–1978 (2023). https://doi.org/10.1007/s10072-023-06703-1

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  • DOI: https://doi.org/10.1007/s10072-023-06703-1

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