Altered Functional Connectivity and Sensory Processing in Blepharospasm and Hemifacial Spasm: Coexistence and Difference

Abstract

Background: Blepharospasm (BSP) and hemifacial spasm (HFS) are both facial hyperkinesia however BSP is thought to be caused by maladaptation in multiple brain regions in contrast to the peripherally induced cause in HFS. Plausible coexisting pathophysiologies between these two distinct diseases have been proposed.Objectives: In this study, we compared brain resting state functional connectivity (rsFC) and quantitative thermal test (QTT) results between patients with BSP, HFS and heathy controls (HCs).Methods: This study enrolled 12 patients with BSP, 11 patients with HFS, and 15 HCs. All subjects received serial neuropsychiatric evaluations, questionnaires determining disease severity and functional impairment, QTT, and resting state functional MRI. Image data were acquired using seed-based analyses using the CONN toolbox.Results: A higher cold detection threshold was found in the BSP and HFS patients compared to the HCs. The BSP and HFS patients had higher rsFC between the anterior cerebellum network and left occipital regions compared to the HCs. In all subjects, impaired cold detection threshold in the QTT of lower extremities had a correlation with higher rsFC between the anterior cerebellar network and left lingual gyrus. Compared to the HCs, increased rsFC in right postcentral gyrus in the BSP patients and decreased rsFC in the right amygdala and frontal orbital cortex in the HFS subjects were revealed when the anterior cerebellar network was used as seed.Conclusions: Dysfunction of sensory processing detected by the QTT is found in the BSP and HSP patients. Altered functional connectivity between the anterior cerebellar network and left occipital region, especially the Brodmann area 19, may indicate the possibility of shared pathophysiology among BSP, HFS, and impaired cold detection threshold. Further large-scale longitudinal study is needed for testing this theory in the future.

Spreading of Trigeminal Neuralgia and Hemifacial Spasm to Blepharospasm: What the Role for Neurovascular Compression of the Vth Cranial Nerve?

“…All figures are the original one made by the authors for this article and have not been published previously. and sensory processing [9]. Relevance of this case report stands on absence of vascular compression of the facial nerve (VII th cranial nerve) and the presence of compression of the trigeminal nerve.…”

“…Although this finding might be only accidental and not pathogenic, one hypothesis may be launched based on the scientific background already known. Trigeminal and facial connections within the encephalic trunk manage the blink reflex and involve both afferent and efferent pathways [9]. Impairment of the inhibitory trigeminofacial responses in the perioral muscles suggest a central control of these reflexes [10].…”

Altered brain connectivity in hyperkinetic movement disorders: A review of resting-state fMRI

“…For dystonia, 46 articles were included, ( Zhang et al, 2020 , Pan et al, 2019 , Jochim et al, 2018 , Putzel et al, 2018 , Mantel et al, 2018 , Ni et al, 2017 , Huang et al, 2017 , Li et al, 2017 , Long et al, 2017 , Haslinger et al, 2017 , Kiyuna et al, 2017 , Battistella et al, 2016 , Sarasso et al, 2020 , Liu et al, 2016 , Ren et al, 2015 , Bharath et al, 2015 , Dresel et al, 2014 , Delnooz et al, 2015 , Hinkley et al, 2013 , Delnooz et al, 2013 , Delnooz et al, 2012 , Mohammadi et al, 2012 , Wei et al, 2021 , Jiang et al, 2019 , Pan et al, 2021 , Feng e al., 2021 , de Faria et al, 2020 , Norris et al, 2020 , Glickman et al, 2020 , Mantel et al, 2020 , Ma et al, 2021 , Corp et al, 2019 , Fang et al, 2021 , Giannì et al, 2022 , Qin et al, 2019 , Zito et al, 2022 , Kim et al, 2022 , Hou et al, 2022 , Ekmen et al, 2022 , Nieuwhof et al, 2022 , Filip et al, 2022 , Battistella and Simonyan, 2019 , Bianchi et al, 2019 , Kita et al, 2018 , Fuertinger and Simonyan, 2018 , Qin et al, 2018 ) concerning cervical dystonia (CD; n = 14), writer’s cramp (n = 10), laryngeal dystonia (n = 8), musician's dystonia (n = 5), paroxysmal kinesigenic dyskinesia (n = 6), blepharospasm (n = 6), oromandibular dystonia (n = 2), dyskinetic cerebral palsy (n = 2), spastic cerebral palsy (n = 1), dystonic tremor (n = 1), and generalized dystonia (n = 1). Most studies matched patients and healthy participants for age and gender.…”

Cerebellar gray matter alterations predict deep brain stimulation outcomes in Meige syndrome

“…The cerebellum, as an indispensable portion of the sensorimotor circuit, has been proven with compelling evidence to be engaged in the physiopathological substrate of dystonia ( Morigaki et al, 2021 ), especially of MS ( Pandey and Sharma, 2017 ). Firstly, cerebellar abnormalities in MS have been identified in gray matter (GM) changes ( Obermann et al, 2007 , Piccinin et al, 2014a , Ramdhani et al, 2014 , Tomic et al, 2021 ), white matter (WM) ( Ramdhani et al, 2014 , Yang et al, 2014 ), structural connectivity ( Chirumamilla et al, 2019 ), neural activity ( Nguyen et al, 2020 , Zhou et al, 2013 ), functional connectivity (FC) ( Fang et al, 2021 , Jochim et al, 2018 ), and metabolic activity ( Hutchinson et al, 2000 , Liu et al, 2021 ). Secondly, the lesions in the cerebellum accounted for a large part of secondary blepharospasm patients ( Khooshnoodi et al, 2013 ).…”