Regional metabolic and network changes in Meige syndrome.

To contribute to the understanding of the aetiology and pathogenesis of Meige syndrome, the metabolic networks of patients with Meige syndrome were investigated using 18F-fluoro-D-glucose positron emission tomography (18F-FDG-PET) imaging of cerebral glucose metabolism. Fifty right-handed and unmedicated primary Meige syndrome patients enrolled between September 2017 and September 2020 at the Department of Neurosurgery, Peking University People's Hospital, and 50 age- and sex-matched healthy control subjects participated in the study. Metabolic connectivity and graph theory analysis were used to investigate metabolic network differences based on 18F-FDG-PET images. Glucose hypometabolism was detected in the left internal globus pallidus and parietal lobe, right frontal lobe and postcentral gyrus, and bilateral thalamus and cerebellum of patients with Meige syndrome. Clustering coefficients (Cps) (density threshold: 16-28%; P < 0.05) and shortest path lengths (Lps) (density threshold: 10-15%; P < 0.05) were higher in Meige syndrome patients than in healthy controls. Small-worldness was lower in Meige syndrome patients than in healthy controls, and centrality was significantly lower in the right superior occipital gyrus and pallidum and higher in the right thalamus. Hypometabolism in the globus pallidus and thalamus may indicate basal ganglia-thalamocortical motor circuit abnormalities as a pathogenic mechanism of Meige syndrome, providing a possible explanation for the efficacy of deep brain stimulation (DBS) in improving symptoms. Meige syndrome patients had abnormal small-world properties. Centrality changes in the right pallidus and thalamus verified the important roles of these regions in the pathogenesis of Meige syndrome.

Positron emission tomography in patients with benign essential blepharospasm.

PURPOSE: To identify possible abnormalities in regional cerebral glucose metabolism in patients with benign essential blepharospasm or Meige syndrome using positron emission tomography. METHODS: Ten patients with benign essential blepharospasm and one patient with Meige syndrome were examined using positron emission tomography with deoxyglucose labeled with fluorine 18. The severity of muscle spasm at the time of positron emission tomography was recorded in each patient. In five patients, positron tomography was performed on two occasions (once before and again 1 to 2 weeks after botulinum injection) to look for reproducibility of the findings on positron emission tomography also to see if there was any correlation between the severity of symptoms and positron emission tomography findings. RESULTS: The mean reference ratio for fluorodeoxyglucose F18 metabolism was significantly elevated in the striatum compared with the frontal, temporal, or parietal regions. Glucose metabolism was also elevated in the thalami. There was no correlation between the severity of blepharospasm and the degree of hyperactivity in the striatum. In the patients who underwent positron emission tomography scanning on two occasions, there was no significant difference between the two studies in any of the regions analyzed. CONCLUSIONS: The authors' findings support the hypothesis that benign essential blepharospasm and Meige syndrome may be associated with overactivity of the striatum and the thalamus. Positron emission tomography may prove to be a useful research tool and a possible adjunct diagnostic technique for benign essential blepharospasm and Meige syndrome.

Meige syndrome in the spectrum of Lewy body disease.

We report a patient with Meige syndrome (segmental cranial dystonia) who had neuropathologic changes of Parkinson's disease on postmortem examination. Neuropathologic examination showed typical and atypical Lewy bodies in the pigmented nuclei of the brainstem (substantia nigra, locus ceruleus), the nucleus basalis of Meynert, and the nucleus ambiguus. Neurochemical analysis of postmortem brain tissue showed evidence for decreased dopamine turnover in the substantia nigra, striatum, and nucleus accumbens. We propose that some cases of Meige syndrome may be included in the spectrum of Lewy body disease.

[A comparative study of the catecholamine content of the blood and urine in patients with facial paraspasm (Meige's syndrome)]. / Sravnitel'noe issledovanie soderzhaniia katekholaminov v krovi i moche u bol'nykh s litsevym paraspazom (sindrom Mezha).

Catecholamines and DOPA excretion and televant levels in blood were measured in 17 patients with facial paraspasm and in 24 healthy subjects. The findings gave evidence for sympathetic-adrenal system adrenal activation and more intensive catecholamine metabolism in patients, especially marked in those with generalised facial hyperkinesia.

Relationship between Meige syndrome and alpha-methyldopa-induced parkinsonism.

We studied two patients with Meige syndrome who developed alpha-methyldopa-induced parkinsonism. Opposite responses of parkinsonian and dystonic symptoms to antiparkinson drugs in some cases suggest a functionally reciprocal relationship between these disorders.