Meige’s syndrome



Meige’s syndrome is a segmental form of dystonia. It is also known as Brueghel's syndrome and oral facial dystonia.

Named after Henri Meige, the French neurologist who first described the symptoms in detail in 1910 1)

The incidence is about one case in 20,000 people.


Meige's syndrome and hemifacial spasm (HFS) are two different forms of dystonic movement disorder, but their difference in terms of resting state functional connectivity (rsFC) remains unclear. Chen et al. applied resting state fMRI on the patients and quantified their functional connectivity with graph theoretical measures, including the degree centrality and the betweenness centrality. Fifteen Meige's syndrome patients and 19 HFS patients matched in age and gender were recruited and their MRI data were collected. To analyze the rsFC, they adopted the Anatomical Automatic Labeling (AAL) template, a brain atlas system including 90 region of interest (ROIs) covering all the brain regions of the cerebral cortex. For each participant, the time course of each ROI was extracted, and the corresponding degree centrality and betweenness centrality of each ROI were computed. These measures were then compared between the Meige's syndrome patients and the HFS patients. Meige's syndrome patients showed higher betweenness centrality and degree centrality of the bilateral superior medial frontal cortex, the left cerebellum cortex, etc. than the HFS patients. Our results suggest that the rsFC pattern in Meige's syndrome patients might become more centralized toward the prefrontal and vestibular cerebellar systems, indicating less flexibility in their functional connections. These results preliminarily revealed the characteristic abnormality in the functional connectivity of Meige's patients and may help to explore better treatment 2).

Primary Meige syndrome is characterized by blepharospasm and orofacial-cervical dystonia: hemifacial spasm with oral movements.

The symptoms usually begin between the ages of 30 and 70 years old and appear to be more common in women than in men (2:1 ratio). The combination of upper and lower dystonia is sometimes called cranial-cervical dystonia.


2)
Chen H, Deng K, Zhang Y, Jiang X, Wang Y. Higher betweenness and degree centrality in the frontal and cerebellum cortex of Meige's syndrome patients than hemifacial spasm patients. Neuroreport. 2023 Feb 1;34(2):102-107. doi: 10.1097/WNR.0000000000001865. Epub 2022 Dec 17. PMID: 36608166.
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