Dystonic Tremor: Time to Change.

BACKGROUND: The term dystonic tremor is being increasingly used in neurological publications despite uncertainties about its meaning. We provide here a historical reconstruction from its original introduction in 1984 to help distinguish dystonia from essential tremor. METHODS: A comprehensive Pubmed search of MeSH terms "dystonia", "tremor", and "essential tremor" provided the information base for reconstructing historical usage of the term "dystonic tremor". RESULTS: Over the years, this expression was enriched of additional meanings and sided by companion descriptors, such as tremor associated with dystonia. Dystonic tremor has been considered characteristically coarse, jerky, irregular, directional and asymmetrical. These characteristics, however, are not included in the most recent definitions of tremor. The relationship between tremor and dystonia is not easy to untangle, as the two phenomena are often recognized in association. Tremor and dystonia experts have developed different visions of dystonic tremor that have been variably implemented. There are currently two independent consensus definitions, which are not coincident and imply different pathophysiological interpretations. CONCLUSIONS: This historical reappraisal highlights that usage of the expression dystonic tremor has evolved over time to lose its original meaning. Notwithstanding inconsistencies of current definitions, its usage has steadily increased and it is time now to agree on an updated terminology.

Cervico-facial dystonia as depicted in sculpture before its scientific description.

The authors describe a sculpture from Daumier, called "Le Hargneux" (The peevish one), whose physiognomic study evokes hitherto unrecognized cranial-cervical dystonia. It is probably the first representation of dystonia in sculpture, before its scientific identification by Horatio Wood, in 1887.

[The mucisian's cramp. About the illness of Robert Schumann]. / La crampe du musicien. A propos de la maladie de Robert Schumann.

The musicians are seen in daily neurological practice facing various problems sometimes simple such as skeletal or tendon pain or even compression of a nerve trunk and sometimes more complicated such as focal dystonia. Dystonia often has a dramatic impact on the career of a musician given the complexity of the clinical and therapeutic approach and the results are often disappointing. The history of the German Romantic composer Robert Schumann illustrates this reality; through his story a discussion of both the different pathophysiological hypotheses responsible for focal dystonia, a disorder of brain plasticity, and of the multimodal therapeutic approaches, revisited in the light of neurophysiological findings will be described.

Albert Schweitzer: a patient with writer's cramp.

Albert Schweitzer (1875-1965) the world-famous philosopher, theologian, concert organist, musicologist, philanthropist and winner of the 1952 Nobel Peace Prize suffered throughout most of his life from severe and painful muscle cramps in his right upper extremity which were triggered exclusively by handwriting. They led to tonic finger flexion and wrist extension and produced slow and clumsy handwriting of a reduced character size. Other motor functions including Schweitzer's highly skilful and famous organ playing were not affected. Inheritance from his mother is likely. Schweitzer applied several coping strategies including a specific holding pattern for pens, usage of special pens, avoidance of handwriting and slowing of handwriting. With all these features Schweitzer presents as a classical case of action-specific dystonia in the form of a simple tonic writer's cramp. Interestingly, Schweitzer never received a medical diagnosis, although writer's cramp had already been identified and described as a medical condition. Impairment of his handwriting but not his organ playing may give insight into the multifactorial aetiology of writer's cramp.

Psychogenic movement disorders: past developments, current status, and future directions.

As the field of movement disorders has developed and matured over the past 25 years, psychogenic movement disorders have become increasingly recognized in subspecialty clinics. The diagnosis can be challenging and should be based on positive features rather than a purely exclusionary approach. The clinical phenotype can be quite varied, although certain categories of abnormal movement are more common than others. Electrophysiological studies may be particularly useful in establishing the diagnosis, especially with respect to tremor and myoclonus, and an argument can be made for adding a "laboratory-supported definite" category to earlier classification schemes. The diagnosis of psychogenic dystonia remains a major challenge, although there are some recent promising developments with respect to the evaluation of cortical plasticity that require further study. The pathogenesis of psychogenic movement disorders is poorly understood; insights may be provided from the study of other neurological conversion disorders such as psychogenic hemiparesis. Psychogenic movement disorders typically result in considerable disability and negatively impact quality of life to the same or greater extent than do many organic movement disorders. Treatment is extremely challenging, and many patients experience chronic disability despite various therapeutic interventions. Given the personal and societal impact of these problems, further advances in our understanding of their pathogenesis and the subsequent development of effective therapies are sorely needed.

Early-onset primary dystonia.

"Dystonia" is the term used to describe abnormal movements consisting of sustained muscle contractions frequently causing twisting and repetitive movements or abnormal postures. Dystonia is classified partly by age at onset because this helps guide the diagnostic work-up and treatment decisions. This chapter focuses on early-onset (<26 years old) primary dystonia. The history, clinical features, genetics, pathophysiology, diagnosis, and treatment of early-onset primary dystonia are discussed. Special emphasis is placed on DYT1 dystonia, the most common, autosomal-dominant, early-onset, primary dystonia. A diagnostic algorithm is proposed for gene-negative early-onset dystonia, and treatment recommendations for generalized, early-onset dystonia are made.

[Botulinum toxin treatment of patients with oromandibular dystonia]. / Botulinumtoksin-behandling af patienter med oromandibulaer dystoni.

INTRODUCTION: Oromandibular dystonia (OMD) is a frequently disabling focal dystonia, which may be treated with injections of botulinum toxin in the affected muscles. The aim of the present study was to evaluate the population, effect and side-effects of patients treated in Denmark during a nine year period. METHODS: We evaluated all 45 consecutive patients treated with quantitative EMG guided injections of botulinum toxin for OMD. RESULTS: The OMD symptoms varied but were most often mixed symptoms (n = 13), jaw closing (n = 11) and jaw opening (n = 7). Thirty-two patients (71%) had other focal or generalised dystonia, and in 24 the additional dystonia were also treated with botulinum toxin. The 45 patients had a total of 277 treatments (mean 6.2 treatments pr. patient), each including one to six muscles. Marked effect was observed or experienced after 193 (70%) treatments, and 33 patients (73%) experienced at least one effective treatment. Side-effects occurred after 35 treatments (13%) experienced by a total of 16 patients (35.6%), most frequently as transient mild dysphagia. DISCUSSION: The study shows that botulinum toxin treatment of OMD, guided by quantitative EMG, is safe and effective.

Robert Schumann.

Robert Schumann, one of the giants of early romantic music, was born in Saxony in 1810 and died in an asylum shortly after his 46th birthday. Early in life, he demonstrated extraordinary skills in both music and journalism; he remained active in both areas until his final illness. His marriage to the remarkable pianist, Clara Wieck, provided him with both much-needed emotional support and a highly effective champion of his music throughout her lengthy career. Schumann's plans to be a concert pianist were thwarted at least partially by an injury to his right hand, the nature of which has been the subject of much speculation. After considering what few facts are available, the author concludes that this may have represented focal dystonia. His compositional output waxed and waned dramatically over his professional life, reflecting to some degree his emotional state. It is considered most likely that he suffered from a major affective disorder, bipolar type. This ultimately led to a suicide attempt in February 1854, and to his eventual death in July 1856. Despite wide-spread and reasonable suspicion that he may have died from neurosyphilis, severe malnutrition from self-starvation seems more likely.

Musicians' dystonia: the case of Gary Graffman.

A professional pianist developed career-ending focal dystonia. There is a possible relationship between the pianist's syndrome and his past playing history. Although the patient has derived very little benefit from various treatment modalities, his candor regarding his impairment indirectly led to the establishment of performing arts medicine as a recognized subspecialty of occupational medicine.