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The Phenomenology of Functional (Psychogenic) Dystonia.

ABSTRACT: From the very first descriptions of dystonia, there has been a lack of agreement on the differentiation of organic from functional (psychogenic) dystonia. This lack of agreement has had a significant effect on patients over the years, most particularly in the lack of access to appropriate management, whether for those with organic dystonia diagnosed as having a functional cause or vice versa. However, clinico-genetic advances have led to greater certainty about the phenomenology of organic dystonia and therefore recognition of atypical forms. The diagnosis of functional dystonia rests on recognition of its phenomenology and should not be, as far as possible, a diagnosis of exclusion. Here, we present an overview of the phenomenology of functional dystonia, concentrating on the three main phenotypic presentations: functional cranial dystonia; functional fixed dystonia; and functional paroxysmal dystonia. We hope that this review of phenomenology will aid in the positive diagnosis of functional dystonia and, through this, will lead to more rapid access to appropriate management.

SUBMITTER: Ganos C

PROVIDER: S-EPMC6183180 | biostudies-literature | 2014 Apr

REPOSITORIES: biostudies-literature

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The Phenomenology of Functional (Psychogenic) Dystonia.

Ganos Christos C Edwards Mark J MJ Bhatia Kailash P KP

Movement disorders clinical practice 20140410 1

From the very first descriptions of dystonia, there has been a lack of agreement on the differentiation of organic from functional (psychogenic) dystonia. This lack of agreement has had a significant effect on patients over the years, most particularly in the lack of access to appropriate management, whether for those with organic dystonia diagnosed as having a functional cause or vice versa. However, clinico-genetic advances have led to greater certainty about the phenomenology of organic dysto ...[more]

PMID: 30363921

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Project description:The neurobiological basis of psychogenic movement disorders remains poorly understood and the management of these conditions difficult. Functional neuroimaging studies have provided some insight into the pathophysiology of disorders implicating particularly the prefrontal cortex, but there are no studies on psychogenic dystonia, and comparisons with findings in organic counterparts are rare. To understand the pathophysiology of these disorders better, we compared the similarities and differences in functional neuroimaging of patients with psychogenic dystonia and genetically determined dystonia, and tested hypotheses on the role of the prefrontal cortex in functional neurological disorders. Patients with psychogenic (n = 6) or organic (n = 5, DYT1 gene mutation positive) dystonia of the right leg, and matched healthy control subjects (n = 6) underwent positron emission tomography of regional cerebral blood flow. Participants were studied during rest, during fixed posturing of the right leg and during paced ankle movements. Continuous surface electromyography and footplate manometry monitored task performance. Averaging regional cerebral blood flow across all tasks, the organic dystonia group showed abnormal increases in the primary motor cortex and thalamus compared with controls, with decreases in the cerebellum. In contrast, the psychogenic dystonia group showed the opposite pattern, with abnormally increased blood flow in the cerebellum and basal ganglia, with decreases in the primary motor cortex. Comparing organic dystonia with psychogenic dystonia revealed significantly greater regional blood flow in the primary motor cortex, whereas psychogenic dystonia was associated with significantly greater blood flow in the cerebellum and basal ganglia (all P < 0.05, family-wise whole-brain corrected). Group × task interactions were also examined. During movement, compared with rest, there was abnormal activation in the right dorsolateral prefrontal cortex that was common to both organic and psychogenic dystonia groups (compared with control subjects, P < 0.05, family-wise small-volume correction). These data show a cortical-subcortical differentiation between organic and psychogenic dystonia in terms of regional blood flow, both at rest and during active motor tasks. The pathological prefrontal cortical activation was confirmed in, but was not specific to, psychogenic dystonia. This suggests that psychogenic and organic dystonia have different cortical and subcortical pathophysiology, while a derangement in mechanisms of motor attention may be a feature of both conditions.

Project description:Dystonia is a common movement disorder, involving sustained muscle contractions, often resulting in twisting and repetitive movements and abnormal postures. Dystonia may be primary, as the sole feature (isolated) or in combination with other movement disorders (combined dystonia), or as one feature of another neurological process (secondary dystonia). The current hypothesis is that dystonia is a disorder of distributed brain networks, including the basal ganglia, cerebellum, thalamus and the cortex resulting in abnormal neural motor programs. In comparison, functional dystonia (FD) may resemble other forms of dystonia (OD) but has a different pathophysiology, as a subtype of functional movement disorders (FMD). FD is the second most common FMD and amongst the most diagnostically challenging FMD subtypes. Therefore, distinguishing between FD and OD is important, as the management of these disorders is distinct. There are also different pathophysiological underpinnings in FD, with for example evidence of involvement of the right temporoparietal junction in functional movement disorders that is believed to serve as a general comparator of internal predictions/motor intentions with actual motor events resulting in disturbances in self-agency. In this article, we present a comprehensive review across the spectrum of FD, including oromandibular and vocal forms and discuss the history, clinical clues, evidence for adjunctive "laboratory-based" testing, pathophysiological research and prognosis data. We also provide the approach used at the Massachusetts General Hospital Dystonia Center toward the diagnosis, management and treatment of FD. A multidisciplinary approach, including neurology, psychiatry, physical, occupational therapy and speech therapy, and cognitive behavioral psychotherapy approaches are frequently required; pharmacological approaches, including possible targeted use of botulinum toxin injections and inpatient programs are considerations in some patients. Early diagnosis and treatment may help prevent unnecessary investigations and procedures, while facilitating the appropriate management of these highly complex patients, which may help to mitigate frequently poor clinical outcomes.

Dataset Information

The Phenomenology of Functional (Psychogenic) Dystonia.

Publications

The Phenomenology of Functional (Psychogenic) Dystonia.

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