Abstract:The pathophysiology of cervical dystonia is not completely understood. Current concepts of the pathophysiology propose that it is a network disorder involving the basal ganglia, cerebellum and sensorimotor cortex. These structures are primarily concerned with sensorimotor control but are also involved in non-motor functioning such as the processing of information related to the chemical senses. This overlap lets us hypothesize a link between cervical dystonia and altered sense of smell and taste. To prove this… Show more
“…Clinical parameters, such as the degree of cervical dystonia, did not predict olfactory decline. Non-motor manifestations of dystonia, such as cognitive and psychiatric alterations, which potentially impact the performance of olfactory tests [ 161 , 164 , 168 , 169 , 170 , 171 , 172 , 173 , 174 , 175 , 176 , 177 , 178 , 179 ], were not assessed as co-factors in this study.…”
Section: Olfactory Deficits In Dystonia: the Current Statementioning
confidence: 99%
“…To comprehensively assess the chemical sense in dystonia, a more recent study appraised the sense of smell, as well as the sense of taste in subjects with cervical dystonia, which was compared to carefully matched healthy controls ( Table 1 ) [ 161 ]. Moreover, cognitive and psychiatric alterations as non-motor manifestations of dystonia were assessed by the use of an extensive test battery.…”
Section: Olfactory Deficits In Dystonia: the Current Statementioning
confidence: 99%
“…Moreover, cognitive and psychiatric alterations as non-motor manifestations of dystonia were assessed by the use of an extensive test battery. For the best possible comparison of the two groups and to exclude other causes that could impact the chemical senses as suggested by a consensus panel [ 47 ], the researchers applied an extensive list of exclusion criteria [ 161 ]. Dystonia subjects on treatment with botulinum toxin were assessed 3 months after their last injections, when the effects of botulinum toxin treatment had wasted.…”
Section: Olfactory Deficits In Dystonia: the Current Statementioning
confidence: 99%
“…It is possibly too early to define olfaction as a trait marker for dystonia. However, two independent studies revealed diminished odor threshold and diminished odor identification as the pattern of olfactory decline in subjects with cervical dystonia [ 160 , 161 ]. Findings are possibly related to the involvement of the basal ganglia, cerebellum, and sensorimotor cortex in both the pathophysiology of dystonia and the processing of olfactory information.…”
Section: Olfaction As a Potential Marker For Dystonia: Further Dirmentioning
Dystonia is a heterogeneous group of hyperkinetic movement disorders. The unifying descriptor of dystonia is the motor manifestation, characterized by continuous or intermittent contractions of muscles that cause abnormal movements and postures. Additionally, there are psychiatric, cognitive, and sensory alterations that are possible or putative non-motor manifestations of dystonia. The pathophysiology of dystonia is incompletely understood. A better understanding of dystonia pathophysiology is highly relevant in the amelioration of significant disability associated with motor and non-motor manifestations of dystonia. Recently, diminished olfaction was found to be a potential non-motor manifestation that may worsen the situation of subjects with dystonia. Yet, this finding may also shed light into dystonia pathophysiology and yield novel treatment options. This article aims to provide background information on dystonia and the current understanding of its pathophysiology, including the key structures involved, namely, the basal ganglia, cerebellum, and sensorimotor cortex. Additionally, involvement of these structures in the chemical senses are reviewed to provide an overview on how olfactory (and gustatory) deficits may occur in dystonia. Finally, we describe the present findings on altered chemical senses in dystonia and discuss directions of research on olfactory dysfunction as a marker in dystonia.
“…Clinical parameters, such as the degree of cervical dystonia, did not predict olfactory decline. Non-motor manifestations of dystonia, such as cognitive and psychiatric alterations, which potentially impact the performance of olfactory tests [ 161 , 164 , 168 , 169 , 170 , 171 , 172 , 173 , 174 , 175 , 176 , 177 , 178 , 179 ], were not assessed as co-factors in this study.…”
Section: Olfactory Deficits In Dystonia: the Current Statementioning
confidence: 99%
“…To comprehensively assess the chemical sense in dystonia, a more recent study appraised the sense of smell, as well as the sense of taste in subjects with cervical dystonia, which was compared to carefully matched healthy controls ( Table 1 ) [ 161 ]. Moreover, cognitive and psychiatric alterations as non-motor manifestations of dystonia were assessed by the use of an extensive test battery.…”
Section: Olfactory Deficits In Dystonia: the Current Statementioning
confidence: 99%
“…Moreover, cognitive and psychiatric alterations as non-motor manifestations of dystonia were assessed by the use of an extensive test battery. For the best possible comparison of the two groups and to exclude other causes that could impact the chemical senses as suggested by a consensus panel [ 47 ], the researchers applied an extensive list of exclusion criteria [ 161 ]. Dystonia subjects on treatment with botulinum toxin were assessed 3 months after their last injections, when the effects of botulinum toxin treatment had wasted.…”
Section: Olfactory Deficits In Dystonia: the Current Statementioning
confidence: 99%
“…It is possibly too early to define olfaction as a trait marker for dystonia. However, two independent studies revealed diminished odor threshold and diminished odor identification as the pattern of olfactory decline in subjects with cervical dystonia [ 160 , 161 ]. Findings are possibly related to the involvement of the basal ganglia, cerebellum, and sensorimotor cortex in both the pathophysiology of dystonia and the processing of olfactory information.…”
Section: Olfaction As a Potential Marker For Dystonia: Further Dirmentioning
Dystonia is a heterogeneous group of hyperkinetic movement disorders. The unifying descriptor of dystonia is the motor manifestation, characterized by continuous or intermittent contractions of muscles that cause abnormal movements and postures. Additionally, there are psychiatric, cognitive, and sensory alterations that are possible or putative non-motor manifestations of dystonia. The pathophysiology of dystonia is incompletely understood. A better understanding of dystonia pathophysiology is highly relevant in the amelioration of significant disability associated with motor and non-motor manifestations of dystonia. Recently, diminished olfaction was found to be a potential non-motor manifestation that may worsen the situation of subjects with dystonia. Yet, this finding may also shed light into dystonia pathophysiology and yield novel treatment options. This article aims to provide background information on dystonia and the current understanding of its pathophysiology, including the key structures involved, namely, the basal ganglia, cerebellum, and sensorimotor cortex. Additionally, involvement of these structures in the chemical senses are reviewed to provide an overview on how olfactory (and gustatory) deficits may occur in dystonia. Finally, we describe the present findings on altered chemical senses in dystonia and discuss directions of research on olfactory dysfunction as a marker in dystonia.
“…Because of the anatomical overlap of structures involved in the pathophysiology of idiopathic BSP and the chemical senses, an impairment of the sense of smell and taste may be found in subjects with idiopathic BSP. This idea is supported by diminished chemical senses in subjects with cervical dystonia (CD) (Marek et al 2018 ; Herr et al 2020 ), where alterations in the sensorimotor control network are also found (Jinnah et al 2013 , 2017 ). Although largely overlooked, the chemical senses fulfill fundamental aspects important for daily life (Croy et al 2014 ; Doty 2019 ).…”
The pathophysiology of blepharospasm is incompletely understood. Current concepts suggest that blepharospasm is a network disorder, involving basal ganglia, thalamus, cortex, and, possibly, the cerebellum. Tracing, imaging, and clinical studies revealed that these structures are also concerned with olfaction and taste. Because of this anatomical overlap, dysfunction of the chemical senses in blepharospasm is expected. Injections of botulinum toxin into the eyelid muscles are the first-line treatment of blepharospasm. Yet, the effects of botulinum toxin on the chemical senses have not been systematically assessed. To contribute to a better understanding of blepharospasm, olfactory and gustatory abilities were assessed in 17 subjects with blepharospasm and 17 age-/sex-matched healthy controls. Sniffin Sticks were used to assess odor threshold, odor discrimination, and odor identification. Results of these three Sniffin Sticks subtests were added to the composite olfactory score. The Taste Strips were applied to assess taste. In an adjacent study, we assessed the sense of smell and taste in eight subjects with blepharospasm before and 4 weeks after botulinum toxin treatment. Subjects with blepharospasm had significantly lower (= worse) scores for odor threshold and for the composite olfactory score than healthy controls, while odor discrimination, odor identification, and the composite taste score were not different between groups. The adjacent study revealed that botulinum toxin did not impact the chemical senses. In this study, subjects with blepharospasm had a lower (= worse) odor threshold than healthy controls. As olfaction is important in daily life, findings justify further research of olfaction in blepharospasm.
BackgroundCognitive dysfunction has been reported in idiopathic adult‐onset dystonia (IAOD), but whether this is a primary or secondary component of the disorder remains uncertain.ObjectiveHere, we aimed to analyze the key domains of abnormal cognitive performance in IAOD and whether this is associated with motor or mood changes.MethodsArticle selection for our critical review was guided by PRISMA guidelines (mesh terms “dystonia” and “cognitive,” publication period: 2000–2022). Only peer‐reviewed, English‐language original case–control studies involving patients with IAOD who were not exposed to dopamine‐ or acetylcholine‐modulating agents and validated cognitive assessments were included.ResultsAbstract screening ultimately yielded 22 articles for full‐text review and data extraction. A greater proportion of studies (17 of 22, 82%) reported abnormal cognitive performance in IAOD. Most of these studies focused on blepharospasm (BSP) and cervical dystonia (10 and 14, respectively). Most studies reporting cognitive impairment (11 of 17) identified multidomain impairment in cognition. Executive functions were the domain most frequently explored (14 of 22 studies), 79% of which detected worse performance in people with dystonia. Results related to other domains were inconclusive. Cognitive abnormalities were independent of motor symptoms in most studies (7 of 12) that explored this relationship and independent of mood status in all 8 that investigated this.ConclusionsWithin IAOD, cognitive dysfunction (in particular, executive dysfunction) has been documented mainly in BSP and cervical dystonia. More comprehensive testing is warranted to assess abnormalities in other domains and in other forms of IAOD, as well as to evaluate longitudinal progression of cognitive disturbances in this condition.
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