Animal Models for Investigating Benign Essential Blepharospasm

Evinger, Craig

doi:10.2174/157015913804999441

Cited by 10 publications

(10 citation statements)

References 118 publications

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“…MS was also found to be associated with decreased inhibition in the cerebral cortex caused by environmental factors and genetic susceptibility ( 9 , 14 , 40 ). Animal studies have demonstrated that abnormal interactions among trigeminal blink circuits, basal ganglia, and the cerebellum contribute to the disease ( 41 ). Although there is no definitive evidence for the existence of MS susceptibility genes, some studies indicate that it is a low penetrance autosomal dominant disorder ( 33 , 42 – 45 ).…”

Section: Pathophysiologymentioning

confidence: 99%

Blepharospasm, Oromandibular Dystonia, and Meige Syndrome: Clinical and Genetic Update

Liang-jun

et al. 2021

Front. Neurol.

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Meige syndrome (MS) is cranial dystonia characterized by the combination of upper and lower cranial involvement and including binocular eyelid spasms (blepharospasm; BSP) and involuntary movements of the jaw muscles (oromandibular dystonia; OMD). The etiology and pathogenesis of this disorder of the extrapyramidal system are not well-understood. Neurologic and ophthalmic examinations often reveal no abnormalities, making diagnosis difficult and often resulting in misdiagnosis. A small proportion of patients have a family history of the disease, but to date no causative genes have been identified to date and no cure is available, although botulinum toxin A therapy effectively mitigates the symptoms and deep brain stimulation is gaining increasing attention as a viable alternative treatment option. Here we review the history and progress of research on MS, BSP, and OMD, as well as the etiology, pathology, diagnosis, and treatment.

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Section: Pathophysiologymentioning

confidence: 99%

Blepharospasm, Oromandibular Dystonia, and Meige Syndrome: Clinical and Genetic Update

Liang-jun

et al. 2021

Front. Neurol.

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“…An animal model of BSP has been reported in which a 6-hidroxi-dopamine lesion of the striatum together with a peripheral nerve lesion may lead to enhanced reflex gain ( 54 , 55 ). This may apply to humans with some predisposition to develop dystonia, where unilateral peripheral facial nerve lesions lead to enhancement of blink reflex excitability recovery in the side contralateral to paresis.…”

Section: Review Of Known Bsp Phenomenologymentioning

confidence: 99%

Blepharospasm: Update on Epidemiology, Clinical Aspects, and Pathophysiology

Valls‐Solé

Defazio

2016

Front. Neurol.

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Blepharospasm (BSP) is a rather distressing form of focal dystonia. Although many aspects of its pathophysiological mechanisms are already known, we lack fundamental evidence on etiology, prevention, and treatment. To advance in our knowledge, we need to review what is already known in various aspects of the disorder and use these bases to find future lines of interest. Some of the signs observed in BSP are cause, while others are consequence of the disorder. Non-motor symptoms and signs may be a cue for understanding better the disease. Various cerebral sites have been shown to be functionally abnormal in BSP, including the basal ganglia, the cortex, and the cerebellum. However, we still do not know if the dysfunction or structural change affecting these brain regions is cause or consequence of BSP. Further advances in neurophysiology and neuroimaging may eventually clarify the pathophysiological mechanisms implicated. In this manuscript, we aim to update what is known regarding epidemiology, clinical aspects, and pathophysiology of the disorder and speculate on the directions of research worth pursuing in the near future.

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“…This may be interpreted as changes in neurotransmitter (NT) production, NT transmission, and NT receptor functions as age increases. In previous animal model, having both depleting the dopamine NT and eye irritation condition produce the BSP, and cerebellum blocked blink amplitude and duration (Evinger, 2013 ; Hall et al., 2006 ). It can be interpreted that NT depletion according to age can contribute to the etiology of BSP.…”

Section: Discussionmentioning

confidence: 98%

Blink index as a response predictor of blepharospasm to botulinum neurotoxin‐A treatment

Jang

Lew

2021

Brain and Behavior

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Purpose:We investigated the blink profiles and blink index using ocular surface interferometer in the patients with blepharospasm (BSP) and identified points to consider predictive factor after BSP treatment. Methods:In total, 117 eyelids of 59 elderly patients and 20 eyelids of 10 age-matched control group were studied. All BSP patients applied botulinum toxin-A (BoNT-A) injection for treatment of BSP. An ocular surface interferometer (LipiView; TearScience, Morrisville, NC, USA) was used to measure blink profile and blink index; total and incomplete blinks/20 s, and the partial blink ratio (PBR). Eyelid blink time (including lid closing time, closure time, lid opening time), interblink times (IBT), closing speeds (OS), and opening speeds (OS) were analyzed using 600 blinks recorded over 20 s.Results: Total blink rate was significantly higher in BSP patients compared to the age-matched control group (p = .029) but other time-related and speed-related index including interpalpebral fissure, PBR, blink time, closure time (CT), interblink time, CS, and OS were not significantly different. In the responder of BSP patients, the average age was higher, CT was shorter, CS was faster than nonresponder (age; p = .016, CT; p < .001, CS; p = .042). Conclusion:The blink index by analyzing the blink profile using ocular surface interferometer, and this blink index may be used as a predictive factor for evaluating the clinical response after BoNT-A injection in blepharospasm patients. K E Y W O R D Sblepharospasm, blink index, blink profile, botulinum neurotoxin-A INTRODUCTIONBlepharospasm (BSP) is the focal dystonia involving periocular muscles and result in involuntary and abnormal eyelid movement. BSP patients have increased blink rate at rest and during conversation compared to healthy people (Bentivoglio et al., 2006). In addition, increased blink rate may precede BSP that the results of more than 5 years of follow-up on the patients with increased blinkingThis is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

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Animal Models for Investigating Benign Essential Blepharospasm

Cited by 10 publications

References 118 publications

Blepharospasm, Oromandibular Dystonia, and Meige Syndrome: Clinical and Genetic Update

Blepharospasm, Oromandibular Dystonia, and Meige Syndrome: Clinical and Genetic Update

Blepharospasm: Update on Epidemiology, Clinical Aspects, and Pathophysiology

Blink index as a response predictor of blepharospasm to botulinum neurotoxin‐A treatment

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