A History of Dystonia: Ancient to Modern

Before 1911, when Hermann Oppenheim introduced the term dystonia, this movement disorder lacked a unifying descriptor. While words like epilepsy, apoplexy, and palsy have had their meanings since antiquity, references to dystonia are much harder to identify in historical documents. Torticollis is an exception, although there is difficulty distinguishing dystonic torticollis from congenital muscular torticollis. There are, nevertheless, possible representations of dystonia in literature and visual art from the pre-modern world. Eighteenth century systematic nosologists such as Linnaeus, de Sauvages, and Cullen had attempted to classify some spasmodic conditions, including torticollis. But only after Charcot's contributions to clinical neuroscience were the various forms of generalized and focal dystonia clearly delineated. They were categorized as névroses: Charcot's term for conditions without an identifiable neuroanatomical cause. For a time thereafter, psychoanalytic models of dystonia based on Freud's ideas about unconscious conflicts transduced into physical symptoms were ascendant, although there was always a dissenting “organic” school. With the rise of subspecialization in movement disorders during the 1970s, the pendulum swung strongly back toward organic causation. David Marsden's clinical and electrophysiological research on the adult-onset focal dystonias was particularly important in establishing a physical basis for these disorders. We are still in a period of “living history” of dystonia, with much yet to be understood about pathophysiology. Rigidly dualistic models have crumbled in the face of evidence of electrophysiological and psychopathological overlap between organic and functional dystonia. More flexible biopsychosocial frameworks may address the demand for new diagnostic and therapeutic rationales

Cognitive Behavioral Therapy in Movement Disorders. A Review

In addition to motor symptoms, patients with movement disorders often complain of psychiatric disturbances, including mood, anxiety, and impulse-control disorders and psychosis. These abnormalities are often misdiagnosed and left untreated, thus resulting in a worse prognosis and lower quality of life. Besides the use of standard pharmacological treatments, psychiatric abnormalities can be treated by means of nonpharmacological approaches. These approaches include various types of psychological therapies, the most widely used being cognitive behavioral therapy (CBT). We reviewed all articles, conducted until 2014, that contained primary data derived from clinical trials and case reports on the effect of CBT in the most common movement disorders. One randomized, controlled study and several uncontrolled studies on the efficacy of CBT in Parkinson's disease (PD) have shown a short-term benefit of depression and anxiety. In Tourette's syndrome (TS), CBT has been assessed in a number of large controlled clinical trials that have demonstrated an improvement in psychiatric disturbances and tics. There are no controlled studies on the efficacy of CBT in other types of movement disorders, such as dystonia, Huntington's disease, and essential tremor. Only a limited number of studies have evaluated the efficacy of CBT in the management of psychiatric disorders in movement disorders. The evidence available suggests that CBT is useful in TS and probably useful in PD. We recommend the planning of randomized, controlled clinical trials to investigate the effects of CBT and group CBT in the treatment of psychiatric disturbances in movement disorders

Neuroimaging in Functional Movement Disorders.

PURPOSE OF REVIEW: Functional movement disorders are common and disabling causes of abnormal movement control. Here, we review the current state of the evidence on the use of neuroimaging in Functional movement disorders, particularly its role in helping to unravel the pathophysiology of this enigmatic condition. RECENT FINDINGS: In recent years, there has been a shift in thinking about functional movement disorder, away from a focus on high-level psychological precipitants as in Freudian conversion theories, or even an implicit belief they are 'put-on' for secondary gain. New research has emphasised novel neurobiological models incorporating emotional processing, self-representation and agency. Neuroimaging has provided new insights into functional movement disorders, supporting emerging neurobiological theories implicating dysfunctional emotional processing, self-image and sense of agency. Recent studies have also found subtle structural brain changes in patients with functional disorders, arguing against a strict functional/structural dichotomy

Diplopia and eye movement disorders.

Published versio

Dystonia and paroxysmal dyskinesias: under-recognized movement disorders in domestic animals? A comparison with human dystonia/paroxysmal dyskinesias.

Dystonia is defined as a neurological syndrome characterized by involuntary sustained or intermittent muscle contractions causing twisting, often repetitive movements, and postures. Paroxysmal dyskinesias are episodic movement disorders encompassing dystonia, chorea, athetosis, and ballism in conscious individuals. Several decades of research have enhanced the understanding of the etiology of human dystonia and dyskinesias that are associated with dystonia, but the pathophysiology remains largely unknown. The spontaneous occurrence of hereditary dystonia and paroxysmal dyskinesia is well documented in rodents used as animal models in basic dystonia research. Several hyperkinetic movement disorders, described in dogs, horses and cattle, show similarities to these human movement disorders. Although dystonia is regarded as the third most common movement disorder in humans, it is often misdiagnosed because of the heterogeneity of etiology and clinical presentation. Since these conditions are poorly known in veterinary practice, their prevalence may be underestimated in veterinary medicine. In order to attract attention to these movement disorders, i.e., dystonia and paroxysmal dyskinesias associated with dystonia, and to enhance interest in translational research, this review gives a brief overview of the current literature regarding dystonia/paroxysmal dyskinesia in humans and summarizes similar hereditary movement disorders reported in domestic animals

Movement Disorders Presenting in Childhood.

PURPOSE OF REVIEW: This article provides an overview of movement disorders that present in childhood. Key clinical features are discussed, and a brief guide to management strategies is provided. Recent advances in the field of pediatric movement disorders are also a focus of the article. RECENT FINDINGS: Advances in genetic technologies and cell biology have contributed greatly to the elucidation of underlying disease mechanisms in childhood movement disorders. This article discusses the expanding spectrum of both genetic and acquired movement disorders that present in childhood, including benign, acquired, genetic, and psychogenic movement disorders. SUMMARY: Movement disorders in childhood comprise a wide spectrum of both genetic and acquired diseases, ranging from benign self-limiting conditions to more progressive phenotypes associated with significant morbidity and mortality. Elucidation of the underlying cause is achieved through accurate history, detailed clinical examination, review of video footage (including home videos), and, where appropriate, neuroimaging and laboratory investigations. Early accurate diagnosis will facilitate the instigation of appropriate management strategies

Flunarizine and cinnarizine-induced parkinsonism: 25 years of de Melo-Souza's syndrome

Universidade Federal do Paraná Hospital de Clínicas Internal Medicine DepartmentUniversidade Federal de São Paulo (UNIFESP) Neurology and Neurosurgery Department Movement Disorders UnitUNIFESP, Neurology and Neurosurgery Department Movement Disorders UnitSciEL

Síndrome de pessoa-rígida e ataxia cerebelar com teste anti-GAD positivo: duas condições clínicas tratáveis as quais o médico deve permanecer atento

Universidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina (EPM) Neurology, Movement Disorders UnitUNIFESP, EPM, Neurology, Movement Disorders UnitSciEL

Informed consent decision-making in deep brain stimulation

Deep brain stimulation (DBS) has proved useful for several movement disorders (Parkinson’s disease, essential tremor, dystonia), in which first and/or second line pharmacological treatments were inefficacious. Initial evidence of DBS efficacy exists for refractory obsessive-compulsive disorder, treatment-resistant major depressive disorder, and impulse control disorders. Ethical concerns have been raised about the use of an invasive surgical approach involving the central nervous system in patients with possible impairment in cognitive functioning and decision-making capacity. Most of the disorders in which DBS has been used might present with alterations in memory, attention, and executive functioning, which may have an impact on the mental capacity to give informed consent to neurosurgery. Depression, anxiety, and compulsivity are also common in DBS candidate disorders, and could also be associated with an impaired capacity to consent to treatment or clinical research. Despite these issues, there is limited empirical knowledge on the decision-making levels of these patients. The possible informed consent issues of DBS will be discussed by focusing on the specific treatable diseases