Prevalence of blepharospasm in Parkinsonism, Essential tremor and Cervical Dystonia

Original Paper Eur Neurol 2012;68:318–321 DOI: 10.1159/000341621 Received: May 3, 2012 Accepted: July 1, 2012 Published online: October 11, 2012 Prevalence of Blepharospasm and Apraxia of Eyelid Opening in Patients with Parkinsonism, Cervical Dystonia and Essential Tremor Abdul-Qayyum Rana a Ashish Kabir b Okan Dogu c Ami Patel d Sumaiya Khondker b a Parkinson’s Clinic of Eastern Toronto and Movement Disorders Centre, Toronto, Ont., Canada; All Saints University School of Medicine, Roseau, Dominica; c Department of Neurology, Faculty of Medicine, Mersin University, Mersin, Turkey; d University of Toronto, Toronto, Ont., Canada b Abstract The objective of this study was to determine the prevalence of blepharospasm (BSP), with and without apraxia of eyelid opening (AEO), in patients with parkinsonism, cervical dystonia (CD), and essential tremor (ET). BSP, with or without AEO, is associated with parkinsonism. There have been several reports of BSP in other dystonic conditions, but few looked at the incidence of BSP in ET patients. This study included 659 patients of which 357 had parkinsonism (276 idiopathic Parkinson’s disease (IPD) and 81 atypical parkinsonism (57 progressive supranuclear palsy; 11 multiple system atrophy 13 corticobasal degeneration)), 274 had ET, 22 had CD, and 6 had spinocerebellar ataxia. Our results indicate that BSP (with or without AEO) was more prevalent in atypical parkinsonism (6 out of 81, 7.41%) than IPD (9 out of 276, 3.26%). The study also followed 10 (of the 28) patients with BSP to screen for the development of other movement disorders – of these, 2 developed Parkinson’s disease. We conclude then that BSP is common in parkinsonism and that BSP is more prevalent in atypical parkinsonism. We also conclude that BSP is not a common feature in ET patients (0 out of 274 patients reported BSP symptoms). Copyright © 2012 S. Karger AG, Basel © 2012 S. Karger AG, Basel 0014–3022/12/0685–0318$38.00/0 Fax +41 61 306 12 34 E-Mail karger@karger.ch www.karger.com Accessible online at: www.karger.com/ene Introduction Blepharospasm (BSP) is a focal dystonia characterized by intermittent, involuntary eyelid closure, increased blinking rate, ocular discomfort and dry eyes [1, 2]. BSP is often associated with apraxia of eyelid opening (AEO) [2]. AEO has on occasion been reported in association with atypical parkinsonism, especially progressive supranuclear palsy (PSP) [2]. Furthermore, Yoon et al. [2] report that BSP and AEO are coexisting dystonia in atypical parkinsonism (commonly seen in PSP patients) and indicate that further research is necessary to confirm and clarify this correlation. Parkinsonism can be subclassified into primary, i.e. idiopathic Parkinson’s disease (IPD), secondary and atypical parkinsonism – which includes PSP, multiple system atrophy (MSA), and cortical basal degeneration (CBD) [3]. Patients with atypical parkinsonism usually have pseudobulbar symptoms without tremor. In addition, they demonstrate impairment of ocular movements of the supranuclear type, dementia and a lack of therapeutic response to levodopa [4]. This study examines the records of a significant population of PD patients for BSP symptoms. Spinocerebellar ataxia (SCA) is a genetic disorder characterized by a slowly progressive loss of coordinated gait and is often associated with poor coordinated Dr. Abdul-Qayyum Rana Parkinson’s Clinic of Eastern Toronto and Movement Disorders Centre 404-2863 Ellesmere Road Toronto, ON M1E 5E9 (Canada) Tel. +1 416 724 9850, E-Mail ranaaq @ yahoo.com Downloaded by: Medical College Wisconsin 198.143.32.33 - 10/25/2015 4:23:55 AM Key Words Blepharospasm ⴢ Essential tremor ⴢ Parkinsonism ⴢ Cervical dystonia ⴢ Spinocerebellar ataxia Patients BSP, n Idiopathic Parkinson’s disease Atypical Parkinson’s: PSP Atypical Parkinson’s: MSA Atypical Parkinson’s: CBD Essential tremor Cervical dystonia SCA type I SCA type II SCA type III hand, speech and eye movements. SCA subtypes are numerous – each with its own characteristic features [5]. This study looks at BSP in patients with SCA type I, II or III. Cervical dystonia (CD) (also known as ‘spasmodic torticollis’) and essential tremor (ET) – a kinetic tremor of the arms possibly in addition to other motor and nonmotor features, are types of movement disorders [6, 7]. Reports indicate that BSP is associated with CD [8]. However, BSP has not been commonly reported in patients with other movement disorders such as ET. There have been many reports that indicate that BSP is much more prevalent in IPD, atypical parkinsonism [2] and other movement disorders including CD [1, 8]. Different studies have examined this relationship but the results have not unequivocally indicated an association between BSP and parkinsonism or CD. Also, few studies have attempted to determine a correlation between BSP and ET. This study is therefore novel as it investigates the prevalence of BSP within a large sample size of ET patients and a smaller sample of patients with SCA. Methods This study was a retrospective chart review of 659 patients in total, 357 of which had parkinsonism. Among these, 276 patients had IPD and 81 had atypical parkinsonism. The 81 patients with atypical parkinsonism included 57 with PSP, 11 with MSA, and 13 with CBD. The study also retrospectively reviewed the charts of 274 patients with ET, 22 with CD, and 6 with SCA type I, II or III to identify the prevalence of BSP. The study also prospectively followed 10 out of the 28 patients with BSP over time to screen for the development of clinical features of parkinsonism or other movement disorders. These patients were seen in our communitybased Parkinson’s Disease and Movement Disorder Centre between 2005 and 2011. The study protocol was reviewed by the lo- Prevalence of BSP in Patients with Parkinsonism, CD and ET 9/276 (3.26%) 6/57 (10.52%) 0/11 0/13 0/274 2/22 (9.09%) 0/2 0/2 2/2 Gender male female 6 (66.67%) 5 (83.33%) – – – 2 – – 0 3 (33.33%) 1 (16.67%) – – – – – – 2 cal ethics board and regulatory approval was exempted. Patients with the various movement disorders were regularly followed two to three times a year at this center. A chart review of the medical records of each patient including a detailed analysis of the assessment from every follow-up visit was performed. Diagnosis of IPD was made using United Kingdom Brain Bank criteria and diagnosis of PSP [9], MSA [10] and CBD as well as BSP were made using established diagnostic criteria [11]. Every patient was assessed by a neurologist with specialized training in movement disorders. Diagnostic criteria proposed by Lepore and Duvoisin [12] were used to diagnose AEO. In CD patients, disease was assessed using the Tsui scale [13]. ET was diagnosed using established criteria based on medical history [11] which included: a visible and persistent bilateral postural tremor of the upper extremities that worsened with action in the absence of drugs that enhance physiological tremor, cerebellar signs, a diagnosis of Parkinson’s disease, dystonia, hyperthyroidism, anxiety, peripheral neuropathy other medical conditions or alcoholism [7]. Hoehn & Yahr staging was used during the assessment of every PD patient. Results We identified 28 patients (15 men, 13 women) in this study who had BSP. Of 276 IPD patients, 9 (3.26%) had BSP. Of 57 PSP patients, 6 (10.52%) had BSP among which 2 (3.50%) had BSP with AEO. Of 13 CBD patients and 11 MSA patients, none had BSP. BSP was more frequent in atypical PD than in IPD. The study found that BSP was more prevalent in men (66.67%) than women (33.33%) in IPD and in PSP. Of 274 ET patients, none had BSP. To discern the prevalence of BSP in SCA, the study looked at a total of 6 SCA patients. Of this group, 2 patients, both of whom had SCA type III, had BSP while none of the other 4 SCA patients did. Of the CD patients, 2 of 22 (9.09%) had BSP. Moreover, none of the 274 patients had BSP. Eur Neurol 2012;68:318–321 319 Downloaded by: Medical College Wisconsin 198.143.32.33 - 10/25/2015 4:23:55 AM Table 1. Features of each form of parkinsonism and movement disorders Discussion The study examined the prevalence of BSP in patients with parkinsonism (idiopathic and atypical), CD, ET and SCA. Previous studies have indicated that BSP is the most common form of focal dystonia in parkinsonism, especially in patients with IPD and atypical parkinsonism [14]. In our study, the frequency of BSP was increased in patients with atypical parkinsonism, for example in PSP (10.52%) versus in IPD (3.26%). In contrast to the findings of Yoon et al. [2], our findings did not indicate the presence of BSP among MSA patients, possibly due to the small number of MSA and also CBD patients in our study. We also prospectively followed 10 patients with BSP and of those, only 2 developed IPD. While this may indicate that some patients with BSP may eventually develop parkinsonism, further research where a larger cohort with BSP is followed in a prospective study is required to establish this correlation. BSP in isolation seems to be more prevalent in females, however results on this topic seem to vary – one previous study found that gender was not associated with the presence of BSP in IPD [15], while our study found BSP to be more prevalent in men with IPD and PSP. Studies conducted previously report that AEO either combined with or without BSP may occur in parkinsonism, particularly in PSP [2, 16, 17]. Our results correlate with those from previous studies and in addition examined the prevalence of BSP in ET patients – which, to the best of our knowledge, has not been done before. This study indicated that BSP with AEO was more prevalent in atypical parkinsonism, especially PSP. This study found that among 6 patients with various SCA types, 2 (33.33%) patients, both with SCA type III, had BSP. SCA type III is an autosomal dominant multisystem neurodegenerative disorder that presents with ataxia and pyramidal and extrapyramidal signs. Further investigation (with larger sample sizes) into the association between SCA type III and BSP is warranted to determine whether or not BSP is a clinical characteristic of SCA type III. Our study confirmed the findings of previous studies that BSP is more prevalent among CD patients. If oromandibular dystonia is present in these patients, a diagnosis of Meige’s syndrome is certainly also a consideration. We did, initially, expect to find an association between BSP and ET, but our study indicated that none of the patients with ET had BSP and therefore we conclude that BSP is not prevalent in ET patients. Limitations of this study include the lack of a control group of healthy individuals. This study was also mainly cross-sectional; however, 10 patients with isolated BSP were followed prospectively and a detailed retrospective analysis of all parkinsonism cases was performed. The SCA and CD patient sample sizes were relatively small and so those results merit further investigation. Conclusion The results of this study confirm that BSP with or without AEO is more frequently observed in atypical parkinsonism than in IPD. BSP and AEO may therefore be a unique feature of atypical parkinsonism and CD. This study does indicate that no association exists between BSP and ET. 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