BACKGROUND: Benign essential blepharospasm (BEB) is a neurologic disorder characterized by an adult-onset focal dystonia that causes involuntary blinking and eyelid spasms. The pathophysiology of BEB patients remains unclear. This study investigated intrinsic low-frequency fluctuation in BEB patients during resting state functional magnetic resonance imaging (fMRI). METHODS: The study included 9 patients with BEB (mean age, 61.7 years; range, 52-66 years), in whom the average duration of symptoms was 2.7 ± 1.8 years, and another 9 subjects from an age- and sex-matched control group. Resting state fMRI was performed in both the patients with BEB and the normal controls. Voxel-based analysis was used to characterize the alteration of amplitude of low-frequency fluctuation (ALFF) in both patients with BEB and the normal controls. RESULTS: The whole brain analysis indicated that in comparison with the normal control group, there was a significantly increased ALFF in the left putamen, pallidum, insular lobe, and medial prefrontal cortex and a significantly decreased ALFF in the bilateral somatosensory regions, thalami, cerebellum, and medial and posterior cingulate cortex. CONCLUSION: The present study suggests that both an abnormal default mode network and corticostriatopallidothalamic loop may play a role in the pathophysiology of BEB.
BACKGROUND:Benign essential blepharospasm (BEB) is a neurologic disorder characterized by an adult-onset focal dystonia that causes involuntary blinking and eyelid spasms. The pathophysiology of BEB patients remains unclear. This study investigated intrinsic low-frequency fluctuation in BEB patients during resting state functional magnetic resonance imaging (fMRI). METHODS: The study included 9 patients with BEB (mean age, 61.7 years; range, 52-66 years), in whom the average duration of symptoms was 2.7 ± 1.8 years, and another 9 subjects from an age- and sex-matched control group. Resting state fMRI was performed in both the patients with BEB and the normal controls. Voxel-based analysis was used to characterize the alteration of amplitude of low-frequency fluctuation (ALFF) in both patients with BEB and the normal controls. RESULTS: The whole brain analysis indicated that in comparison with the normal control group, there was a significantly increased ALFF in the left putamen, pallidum, insular lobe, and medial prefrontal cortex and a significantly decreased ALFF in the bilateral somatosensory regions, thalami, cerebellum, and medial and posterior cingulate cortex. CONCLUSION: The present study suggests that both an abnormal default mode network and corticostriatopallidothalamic loop may play a role in the pathophysiology of BEB.
Authors: Vikram G Shakkottai; Amit Batla; Kailash Bhatia; William T Dauer; Christian Dresel; Martin Niethammer; David Eidelberg; Robert S Raike; Yoland Smith; H A Jinnah; Ellen J Hess; Sabine Meunier; Mark Hallett; Rachel Fremont; Kamran Khodakhah; Mark S LeDoux; Traian Popa; Cécile Gallea; Stéphane Lehericy; Andreea C Bostan; Peter L Strick Journal: Cerebellum Date: 2017-04 Impact factor: 3.847
Authors: Jesse C DeSimone; Marcelo Febo; Priyank Shukla; Edward Ofori; Luis M Colon-Perez; Yuqing Li; David E Vaillancourt Journal: Neurobiol Dis Date: 2016-07-09 Impact factor: 5.996
Authors: Maggie Fagan; Laura Scorr; Doug Bernhardt; Ellen J Hess; Joel S Perlmutter; Carlos A Pardo; H A Jinnah Journal: Exp Neurol Date: 2021-08-28 Impact factor: 5.330