Abstract
BackgroundsStudies have reported that non-motor symptoms are an important component of primary dystonia. However, evidence supporting cognitive impairment in primary dystonia is limited and contradictory.MethodsWe applied the Chinese version of the Addenbrooke’s Cognitive Examination-Revised and the Mini-Mental State Examination (MMSE) to screen for cognitive impairment in patients with primary blepharospasm. In addition, we investigated the relationship between performance on the Addenbrooke’s Cognitive Examination-Revised and quality of life as measured by the Medical Outcomes Study 36-item Short-Form (SF36).ResultsThe study included 68 primary blepharospasm patients and 68 controls matched by age, sex and education. The prevalence of cognitive deficits was 22.0% and 32.3% in primary blepharospasm patients group, as measured by the MMSE and the Addenbrooke’s Cognitive Examination-Revised, respectively. Primary blepharospasm patents had a broad range of cognitive deficits, with the most frequently affected domains being visuospatial function (30.9%) and language (30.9%), followed by memory (27.9%), orientation/attention (26.4%) and verbal fluency (22.0%). Patients with cognitive deficits had lower total SF36 scores, especially in the subdomains of physical functioning, role-physical and social functioning, compared to those without cognitive deficits. Scores on the Addenbrooke’s Cognitive Examination-Revised were significantly correlated with both the SF36 scores and the scores on the subdomains of physical functioning and social functioning.ConclusionsSome patients with primary blepharospasm have cognitive deficits. Poor performance on the Addenbrooke’s Cognitive Examination-Revised is related to poorer quality of life.
Highlights
Dystonia is a common heterogeneous movement disorder that is characterized by sustained or intermittent muscle contractions, which results in twisting movements and/or abnormal postures[1]
Primary blepharospasm patents had a broad range of cognitive deficits, with the most frequently affected domains being visuospatial function (30.9%) and language (30.9%), followed by memory (27.9%), orientation/attention (26.4%) and verbal fluency (22.0%)
The etiology and pathophysiology of primary dystonia remain incompletely understood, dystonia is associated with basal ganglia dysfunction, and there is increasing evidence that the basal ganglia plays a role in both cognitive functions and motor control [4]
Summary
Dystonia is a common heterogeneous movement disorder that is characterized by sustained or intermittent muscle contractions, which results in twisting movements and/or abnormal postures[1]. Dystonia can be classified according to its etiology and clinical characteristics, including age of onset, body distribution, temporal pattern and associated features[1]. Despite the prominent motor symptoms, clinical studies have revealed that patients with primary dystonia suffer from a wide range of non-motor symptoms, including the more commonly reported sensory and neuropsychiatric abnormalities and the less frequent cognitive abnormalities[2]. Both motor and non-motor symptoms have been shown to decrease quality of life (QoL) in patients with dystonia[3]. Non-motor features, such as abnormalities in sensory processing, neuropsychiatric problems and cognitive impairments, are not surprising. [2, 6]
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