|Year : 2020 | Volume
| Issue : 3 | Page : 156-162
Nonmotor symptoms and sleep disturbances in patients with blepharospasm
Somdattaa Ray, Pramod K Pal, Ravi Yadav
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India
|Date of Submission||19-Jan-2020|
|Date of Decision||13-Feb-2020|
|Date of Acceptance||06-Jul-2020|
|Date of Web Publication||07-Nov-2020|
Dr. Ravi Yadav
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka.
Source of Support: None, Conflict of Interest: None
Blepharospasm is a disabling focal dystonia, which is characterized by involuntary bilateral synchronous, excessive blinking and closure of the eyes. This leads to functional impairment in vision and consequences such as inability to carry out daily activities such as driving a vehicle and walking. In addition, these patients can develop many nonmotor symptoms. There is a significant prevalence of anxiety, depression, and obsessive compulsion disorders in patients with blepharospasm. Sleep problems such as poor quality of sleep, delayed sleep latency, and reduced sleep duration are also seen in blepharospasm. This review aims to summarize the literature on the impact of nonmotor symptoms in patients with blepharospasm.
Keywords: Anxiety, blepharospasm, depression, nonmotor, sleep
|How to cite this article:|
Ray S, Pal PK, Yadav R. Nonmotor symptoms and sleep disturbances in patients with blepharospasm. Ann Mov Disord 2020;3:156-62
|How to cite this URL:|
Ray S, Pal PK, Yadav R. Nonmotor symptoms and sleep disturbances in patients with blepharospasm. Ann Mov Disord [serial online] 2020 [cited 2020 Dec 5];3:156-62. Available from: https://www.aomd.in/text.asp?2020/3/3/156/300281
| Introduction|| |
Blepharospasm is a form of adult-onset focal dystonia, which is characterized by involuntary contraction of orbicularis oculi muscles that is synchronous and bilateral., Increased rate of blinking may precede the development of blepharospasm.,
The incidence of blepharospasm ranges from 12 to 133 per million. Blepharospasm is more common in women compared to men in the ratio of 2.6:1 to 3:1.,, In various studies, the age of onset of symptoms ranged commonly from 29 to 84 years of age., However, onset before 24 years of age is also known. In a study of 111 patients from Taiwan, 35% of the patients had onset of symptoms between 51 and 60 years, and 44% of the patients had age of onset after 60 years. Of the patients with blepharospasm, 62% reported unilateral-onset symptoms, and 22% of the patients had bilateral symptoms at onset. The median time of onset to peak of symptoms is approximately 3 years. Among 240 patients with blepharospasm studied by Peckham et al., 25% of the patients reported photophobia and 22% of the patients reported preexisting eye disease. Of the patients with blepharospasm, 32% had family history of focal dystonia. Head trauma, exposure to antipsychotic medication, and antiemetics predisposed to the development of the blepharospasm., Head trauma may result in insult to basal ganglia that could predispose to focal dystonia. Of 111 patients with blepharospasm, in a study from South Taiwan, 81% reported improvement of symptoms following sleep. Of the patients, 77% reported functional blindness.
Blepharospasm tends to spread to adjacent sites, cervical and oromandibular region being the commonest sites., A study reported that 30% of the patients with blepharospasm had concurrent dystonia of the orofacial and the cervical region. The spread of dystonia is more likely to occur at an older age of onset of blepharospasm. The probable pathophysiology could be the age-induced maladaptive changes in the circuits that lead to reduced intracortical inhibition.,,
Geste antagoniste or sensory tricks have been reported in 70% of the patients with blepharospasm. The probable mechanism by which sensory tricks improve dystonia is by normalization of the deranged cortical inhibition that is a characteristic of dystonia. This is modulated by attenuating the output from the supplementary motor cortex.,
Primary essential blepharospasm is a disorder wherein the patient has dystonia without any underlying degenerative or hereditary cause. It is characterized by normal imaging. Secondary blepharospasm is associated with radiological evidence of lesions in cerebellum, basal ganglia, pons, and diencephalon.,,, An increase in the size of bilateral putamen with a decrease in the volume of inferior parietal lobule has been observed in blepharospasm. On the basis of the functional imaging, other regions implicated in the pathogenesis of blepharospasm are anterior cingulate and visual cortex, primary motor cortex, superior cerebellum, and thalamus. Fluorodeoxyglucose–positron emission tomography studies have shown hypermetabolism of cerebellum and pons.
| Methodology|| |
PubMed (MEDLINE) search was carried out using key words such as “blepharospasm” and “sleep,” “non motor symptoms,” “anxiety,” “depression,” “quality of life,” “sexual dysfunction,” “cognition,” “memory,” and “autonomic.” Studies that described nonmotor symptoms after administration of botulinum toxin were excluded.
A total of 214 studies were found in the category of blepharospasm, and among them, 27 studies were found relevant for the review. The flow diagram of methodology of selection is in [Figure 1]. In this article, we reviewed the currently available literature on the nonmotor symptoms of blepharospasm.
|Figure 1: Flow of information and methodology chosen in shortlisted articles for review as per PRISMA guidelines|
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| Pathophysiology of Nonmotor Symptoms in Blepharospasm|| |
With recognition of subcortical circuits concerned with saccadic eye movements, executive function, motivation, and behavioral aspects, the role of basal ganglia was found to be pivotal in the pathogenesis of nonmotor symptoms of dystonia. Cortico-striato-thalamo-cortical loop has been hypothesized to be involved in cognition in dystonia. Among patients with blepharospasm, altered circuits connecting basal ganglia with cerebellum, supplementary motor cortex, cuneus, precuneus, premotor area, parietal, and visual area have been postulated. This widespread abnormal connectivity explains the coexistence of motor and multiple nonmotor symptoms in blepharospasm.
| Psychiatric Symptoms in Blepharospasm|| |
A variety of psychiatric symptoms have been observed as comorbidities in blepharospasm. These include anxiety, depression, mood disorders, and obsessive compulsive disorders. Fabbrini et al. noted that the onset of psychiatric symptoms preceded the development of dystonia by an average of 18 years (range being 1–52 years) in 68% of the patients with focal dystonia (cervical dystonia, blepharospasm, hand dystonia, and laryngeal dystonia). Stressors preceding the onset of blepharospasm were reported in 73% of the patients in another study.
Psychiatric symptoms have been reported in 67.9% of patients with blepharospasm by Fabbrini et al. Depression was observed in 13.3%–37% of the patients,,, and anxiety was reported in 20%–41.3% in various studies.,, Mood disorders were reported in 39.3% of the patients. Depression was seen more commonly among female patients with blepharospasm. A correlation was found between gender and Beck Depression Inventory (BDI) score, and females with blepharospasm reportedly had higher incidence of depression compared to their male counterparts. An epidemiological study of 1325 patients with blepharospasm revealed that anxiety was present in 21.4% of the patients, and depression was present in 9.9% of the patients. In this study, obsessive compulsive disorders were relatively rare, and they were seen in 0.8% of the population.
In a study, patients with blepharospasm had obsessive compulsive behavior concerning cleanliness, control, and order, although they did not satisfy the criteria of obsessive compulsive disorder. Other studies have also reported a higher prevalence of obsessive compulsive disorders in patients with blepharospasm [Table 1].
In a study comparing nonmotor symptoms among various types of focal dystonia, patients with blepharospasm appeared to have a significantly higher depression and anxiety scores compared to those with cervical dystonia and limb dystonia. Psychiatry disorders present at a similar frequency in both blepharospasm and hemifacial spasm, a similar movement disorder involving the face.
No significant correlation was found between depression and anxiety scores with the severity of motor symptoms. The severity of the psychiatric disturbance also lacked correlation with the frequency of botulinum injections. Depression was associated with an increased probability of diagnosis with blepharospasm. The presence of coexisting anxiety and depression was found to shorten the duration of action of botulinum toxin. In certain patients, depression could be a reaction to the disability as a result of dystonia.
| Sleep in Blepharospasm|| |
Sleep impairment significantly adds to the comorbidity with 43.2%–72% of patients with blepharospasm reporting poor sleep.,,, Patients with blepharospasm appear to have a significantly higher Pittsburg Sleep Quality Index (PSQI) scores compared to those with cervical dystonia and limb dystonia, hence inferring that the sleep impairment was worst among patients with blepharospasm compared to other focal dystonia [Table 1].
When analyzing the scores that contributed to impaired sleep, patients with blepharospasm had poor sleep latency and duration. The impairment in sleep was not associated with excessive daytime sleepiness, which might reaffirm the postulate that sleep dysfunction might be a comorbidity of dystonia. Components such as sleep latency, efficiency, duration, and medication use were contributory to the high PSQI scores in comparison to the high PSQI scores in cervical dystonia in which only sleep efficiency played an important role. Depression showed positive correlation with poor sleep as evidenced by BDI., But these patients did not show any significant correlation between impairment of sleep and severity of blepharospasm or disease duration. Moreover, impaired sleep was more common in women with blepharospasm.
| Excessive Daytime Sleepiness in Blepharospasm|| |
Of patients with blepharospasm, 6% reported excessive daytime sleepiness. Excessive daytime sleepiness was found in 25% of patients with blepharospasm in another study [Table 1].
| Cognitive Disturbances|| |
Subclinical cognitive impairment was noted in patients with primary dystonia. Impaired mental rotation of body parts also was noted in patients with DYT1 mutation. Certain studies in patients with primary dystonia revealed normal language skills with impaired attention and executive functioning. These results were conflicting, as few other studies in primary dystonia showed that patients had poor verbal fluency with normal attention and executive skills. Studies on cognition in patients with blepharospasm also revealed impaired tactile object recognition, poor motor dexterity, executive dysfunction, and flawed planning of complex motor movements. Attention and visuospatial working memory were also affected. Reasoning using visuospatial memory worsened with increasing age and severity of the disease. In one study, 32.3% of patients with blepharospasm had cognitive deficits, with deficits being predominantly in visuospatial (30.9%), language (30.9%), and attention domain (26%). Verbal fluency was the least affected in these patients, with 22% showing deficits in verbal fluency. Similar finding was noted in another study with impairment in visuospatial domain, memory, and attention. In yet another study comparing executive function in blepharospasm and hemifacial spasm, the authors concluded that executive function was unaffected in blepharospasm. Yang et al. reported that 35% of the patients with blepharospasm had cognitive deficits that correlated with age and severity of the illness.
| Other Nonmotor Symptoms in Blepharospasm|| |
Autonomic dysfunction was noted in secondary unilateral blepharospasm secondary to cluster headache. Attacks of bilateral blepharospasm with autonomic symptoms and photophobia have also been reported. Association of primary blepharospasm with autonomic dysfunction is not described.
Sensory symptoms have been reported among patients with blepharospasm either before or at the onset of the illness. These include photophobia, foreign body sensation, and dryness of eyes that were reported in 40%–60% of the patients.,, In another study, photophobia was reported in 25% of patients with blepharospasm. Ocular illnesses such as iritis, blepharitis, and conjunctivitis have been reported in only 22% of patients with blepharospasm. Pain accounted for impaired quality of life among patients with blepharospasm.,
Sexual dysfunction has been studied in blepharospasm. Patients reported a higher level of dissatisfaction, infrequency, premature ejaculation, and vaginismus compared to controls. In another study, 39% of patients with blepharospasm reported sexual dysfunction.
| Blepharospasm and Quality of Life|| |
The pathogenetic circuits in blepharospasm could be a primary factor affecting the quality of life. In a study comparing the quality of life between blepharospasm and hemifacial spasm, two disorders with similar symptoms but with different pathogenesis, patients with blepharospasm were found to have higher depression and anxiety scores compared to patients with hemifacial spasm. Vision-related scores were also reported to be higher among patients with blepharospasm. Novaretti et al. reported that quality of life was worse among patients with blepharospasm in comparison to cervical dystonia and limb dystonia.
In a study by Müller et al., quality of life was better in patients with blepharospasm with a longer duration of the illness. Among patients with blepharospasm, women had a higher degree of impaired quality of life. Anxiety, depression, pain, and unemployment were associated with impaired quality of life in patients with blepharospasm. Although anxiety and depression relieved with botulinum toxin, there was no improvement in the quality of life following botulinum toxin administration in patients with blepharospasm. Other factors determining the quality of life was driving, ophthalmological symptoms, and dependency. The summary of the interactions of nonmotor symptoms in blepharospasm is shown in [Figure 2].
|Figure 2: A schematic diagram showing the main groups of nonmotor symptoms in patients with blepharospasm|
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| Management of Nonmotor Symptoms in Blepharospasm|| |
Photochromatic modulation has been suggested as a potential treatment for photophobia in blepharospasm. Sleep impairment and excessive daytime sleepiness in blepharospasm can be traced to the effect of medication and psychiatric comorbidities. Addressing these concerns will be beneficial in alleviating sleep impairment. Interestingly, botulinum toxin has been stated to improve anxiety and depression in patients with blepharospasm in addition to motor symptoms.
| Conclusion|| |
Although blepharospasm is a focal dystonia, nonmotor symptoms and sleep abnormalities add significant morbidity to the illness. Thus, patients with blepharospasm may need adequate management strategies for the nonmotor symptoms also, while managing the dystonia.
We express gratitude to National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India, for providing the facilities to carry out this study.
Financial support and sponsorship
This study was funded by Golden Jubilee Fund of Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]