Introdução: A cãibra do escrivão (CE) é uma distonia focal com contração sustentada e involuntária das mãos, dedos e/ou braços durante a escrita. Poucos estudos avaliaram desempenho motor e cognição na CE a partir de testes neuropsicológicos. Os achados do estudo podem colaborar na reabilitação da doença. Objetivo: Avaliar os desempenhos motor e cognitivo dos pacientes com CE. Métodos: Foram avaliados 21 pacientes e 21 controles pareados por sexo e idade. Dados clínicos foram colhidos e combinados com avaliação da presença de sintomas de depressão, ansiedade e apatia por meio, respectivamente, dos Inventários de Depressão e Ansiedade de Beck e da Escala de Apatia. A gravidade da distonia foi quantificada pelas escalas Writer\'s Cramp Rating Scale e The Arm Dystonia Disabiliity Scale. O desempenho motor foi avaliado pelo Purdue Pegboard Test (PPT). Demais testes neuropsicológicos aplicados foram: Mini Exame do Estado Mental (MEEM), Frontal Assessment Battery (FAB), Symbol Digit Modalities Test (SDMT), Stroop Test (ST) e Trail Making Test (TMT). Resultados: Pacientes com CE apresentaram desempenho inferior aos controles nos testes: MEEM, FAB e ST parte \"W\". Após correção para anos de estudo, variável demográfica com diferença estatística significativa entre os grupos, manteve-se diferença significativa apenas na FAB. Pacientes apresentaram desempenho inferior aos controles nas tarefas com a mão dominante, com ambas as mãos e no teste de montagem do PPT. Após correção para variável anos de estudo, não houve diferença significativa, apenas tendência estatística à performance inferior de pacientes na tarefa montagem do PPT. O desempenho da tarefa montagem do PPT em pacientes com CE teve forte correlação positiva com testes neuropsicológicos, e não com gravidade motora da distonia. Conclusões: O estudo sugere que pacientes com CE apresentem disfunção executiva. As aterações motoras encontradas na tarefa montagem do PPT dos pacientes com CE estão possivelmente relacionadas ao déficit no funcionamento executivo destes indivíduos. / Introduction: The writer\'s cramp (WC) is a focal dystonia with sustained and involuntary contraction of the hands, fingers and/or arms during writing. Few studies have evaluated motor performance and cognition of these patients using neuropsychological tests. The findings may contribute to rehabilitation of this disease. Objective: evaluate motor and cognitive performance of patients with WC. Methods: Twenty-one patients and 21 matched controls by sex and age were evaluated. Clinical data were collected, with assessment of symptoms of depression, anxiety, and apathy using the respective clinical scales: Beck Depression Inventory, Beck Anxiety Inventory and Apathy Scale. Severity of dystonia was quantified by Writer\'s Cramp Rating Scale and The Arm Dystonia Disability Scale. Motor performance was assessed by Purdue Pegboard Test (PPT). Other neuropsychological tests applied were: Mini Mental State Examination (MMSE), Frontal Assessment Battery (FAB), Symbol Digit Modalities Test (SDMT), Stroop Test (ST) and Trail Making Test (TMT). Results: Patients with WC had inferior performance to the controls in MMSE, FAB and ST part \"W\". After correction for years of study, a demographic variable with significant statistical difference between the groups, a significant difference was maintained only in FAB. Patients had inferior performance to the controls in these tasks of PPT: task with the dominant hand, with both hands and in PPT assembly. After correction for the variable \'years of study\', there was no significant difference, only statistical tendency to inferior performance of patients in PPT assembly. The performance of PPT assembly task in patients with WC had a strong positive correlation with neuropsychological tests, and not with motor severity of dystonia. Conclusions: The study suggests that patients with WC have executive dysfunction. The motor alterations in patients with WC is possibly related to deficit in executive functioning

Introdução: A cãibra do escrivão (CE) é uma distonia focal com contração sustentada e involuntária das mãos, dedos e/ou braços durante a escrita. Poucos estudos avaliaram desempenho motor e cognição na CE a partir de testes neuropsicológicos. Os achados do estudo podem colaborar na reabilitação da doença. Objetivo: Avaliar os desempenhos motor e cognitivo dos pacientes com CE. Métodos: Foram avaliados 21 pacientes e 21 controles pareados por sexo e idade. Dados clínicos foram colhidos e combinados com avaliação da presença de sintomas de depressão, ansiedade e apatia por meio, respectivamente, dos Inventários de Depressão e Ansiedade de Beck e da Escala de Apatia. A gravidade da distonia foi quantificada pelas escalas Writer\'s Cramp Rating Scale e The Arm Dystonia Disabiliity Scale. O desempenho motor foi avaliado pelo Purdue Pegboard Test (PPT). Demais testes neuropsicológicos aplicados foram: Mini Exame do Estado Mental (MEEM), Frontal Assessment Battery (FAB), Symbol Digit Modalities Test (SDMT), Stroop Test (ST) e Trail Making Test (TMT). Resultados: Pacientes com CE apresentaram desempenho inferior aos controles nos testes: MEEM, FAB e ST parte \"W\". Após correção para anos de estudo, variável demográfica com diferença estatística significativa entre os grupos, manteve-se diferença significativa apenas na FAB. Pacientes apresentaram desempenho inferior aos controles nas tarefas com a mão dominante, com ambas as mãos e no teste de montagem do PPT. Após correção para variável anos de estudo, não houve diferença significativa, apenas tendência estatística à performance inferior de pacientes na tarefa montagem do PPT. O desempenho da tarefa montagem do PPT em pacientes com CE teve forte correlação positiva com testes neuropsicológicos, e não com gravidade motora da distonia. Conclusões: O estudo sugere que pacientes com CE apresentem disfunção executiva. As aterações motoras encontradas na tarefa montagem do PPT dos pacientes com CE estão possivelmente relacionadas ao déficit no funcionamento executivo destes indivíduos. / Introduction: The writer\'s cramp (WC) is a focal dystonia with sustained and involuntary contraction of the hands, fingers and/or arms during writing. Few studies have evaluated motor performance and cognition of these patients using neuropsychological tests. The findings may contribute to rehabilitation of this disease. Objective: evaluate motor and cognitive performance of patients with WC. Methods: Twenty-one patients and 21 matched controls by sex and age were evaluated. Clinical data were collected, with assessment of symptoms of depression, anxiety, and apathy using the respective clinical scales: Beck Depression Inventory, Beck Anxiety Inventory and Apathy Scale. Severity of dystonia was quantified by Writer\'s Cramp Rating Scale and The Arm Dystonia Disability Scale. Motor performance was assessed by Purdue Pegboard Test (PPT). Other neuropsychological tests applied were: Mini Mental State Examination (MMSE), Frontal Assessment Battery (FAB), Symbol Digit Modalities Test (SDMT), Stroop Test (ST) and Trail Making Test (TMT). Results: Patients with WC had inferior performance to the controls in MMSE, FAB and ST part \"W\". After correction for years of study, a demographic variable with significant statistical difference between the groups, a significant difference was maintained only in FAB. Patients had inferior performance to the controls in these tasks of PPT: task with the dominant hand, with both hands and in PPT assembly. After correction for the variable \'years of study\', there was no significant difference, only statistical tendency to inferior performance of patients in PPT assembly. The performance of PPT assembly task in patients with WC had a strong positive correlation with neuropsychological tests, and not with motor severity of dystonia. Conclusions: The study suggests that patients with WC have executive dysfunction. The motor alterations in patients with WC is possibly related to deficit in executive functioning

La crampe de l'écrivain est la forme la plus fréquente de dystonies de fonction. Elle se caractérise par l’apparition de contractions musculaires involontaires et soutenues aboutissant à une perturbation sévère de l’écriture. Cette dernière est une tâche motrice séquentielle complexe nécessitant un contrôle continu des informations proprioceptives afin d’adapter la position du membre supérieur et la pression des doigts sur le stylo au cours de l’activité motrice. Dans ce travail de thèse nous avons postulé que l’élément fondamental de la physiopathologie de la crampe de l’écrivain est un trouble de l’intégration sensori-motrice. Pour tester cette hypothèse, nous avons utilisé deux tâches comportementales n’induisant pas de symptôme dystonique. Le sujet devait exécuter une séquence motrice, soit de façon spontanée (tâche SGT), soit sur la base d’une séquence de stimulation tactile précédemment apprise (ST). Les modifications d’activité cérébrale au cours de ces deux tâches ont été étudiées par couplage des techniques d’imagerie par résonance magnétique fonctionnelle (IRMf) et d’électroencéphalographie quantitative (EEGq). Nos résultats suggèrent qu’en l’absence de symptôme dystonique, les patients atteints de crampe de l’écrivain présentent une altération de leur performance motrice dans les deux tâches. Durant l’analyse des informations proprioceptives, l’IRMf montre qu’il existe une diminution du signal Bold au niveau du cortex somesthésique primaire, de l’aire motrice supplémentaire (AMS) et du cortex pariétal. Les données d’EEGq révèlent une diminution de la désynchronisation β (DLEβ) au niveau de l’AMS et celles de la cohérence intra-corticale des perturbations du fonctionnement des réseaux pariéto-prémoteurs. Durant la phase motrice, l’IRMf ne révèle aucune différence inter-groupe, mais l’EEGq montre une augmentation de la DLEβ dans les régions pariétales postérieures pour la situation SGT, associée à une augmentation de cohérence intra-corticale. Après l’exécution du mouvement, des anomalies de synchronisation β (SLEβ) sont également retrouvées dans les deux tâches. Ces données montrent qu’il existe chez les patients présentant une crampe de l’écrivain, des perturbations de l’analyse séquentielle des informations proprioceptives, ainsi que des anomalies de l’intégration sensori-motrice. Elles sont en rapport avec une altération des relations fonctionnelles entre les cortex pariétaux et prémoteurs. Ces éléments expliquent pourquoi ces patients au cours de l’écriture ont des difficultés à élaborer une réponse motrice adaptée sur la base d’information proprioceptive séquentielle. / Writer's cramp is the most frequent form of task-specific dystonia. It is characterized by excessive and inappropriate muscle activation resulting in severe disturbance of writing. Writing is a complex sequential motor task requiring the on-line continuous monitoring of proprioceptive information to adjust the position of the upper limb and finger pressure on the pen. In this experimental work, we postulated that the main feature in the pathophysiology of writer's cramp is a sensorimotor integration disorder. To test this hypothesis, we used two behavioral tasks that does not induce dystonic symptoms. The subject performed a motor sequence, either spontaneously (SGT task), or on the basis of a sequence of proprioceptive stimuli previously presented (ST). Changes in brain activity during both tasks were studied by coupling functional magnetic resonance imaging (fMRI) with quantitative electroencephalography (qEEG). Our results show that writer's cramp patients had impaired motor performances in both tasks whereas they did not exhibit any dystonic symptoms. During the analysis of proprioceptive information, fMRI shows a Bold signal decrease in the primary somatosensory cortex, the supplementary motor area (SMA) and parietal cortex. EEGq data revealed a decrease of β desynchronization (βERD) in SMA and intra-cortical coherence a disruption of the functional connectivity of parieto-premotor networks. During the motor phase, fMRI reveals no between-group difference, but EEGq showed an increase of βERD in the posterior parietal regions for the SGT task associated with an increased intra-cortical coherence. During the post-movement period, abnormal β synchronization (βERS) is also found in both tasks. This data shows that writer's cramp patient exhibited a disruption in the sequential analysis of proprioceptive information, as well as abnormalities in sensorymotor integration during motor planning. These features are related to an alteration of the functional relationships between the parietal and premotor cortices. These phenomenons explain why writer's cramp patient fail to develop an appropriate motor response on the basis of sequential proprioceptive information, as in writing.

Hintergrund: Der genaue Pathomechanismus zur Entstehung des benignen Faszikulations- Crampus-Syndrom ist nicht bekannt, und es wurde noch nie eine größere Anzahl dieser Patienten mittles des threshold trackings untersucht. Material und Methoden: 18 Patienten mit einem BFCS und 15 gesunde Probanden wurden mithilfe des threshold trackings, welches eine Methode zur Messung von Exzitabilitätsparametern von peripheren Nerven in vivo ist, untersucht und die Ergebnisse verglichen. Ergebnisse: Die stärksten Unterschiede zeigten sich im threshold electrotonus, der current/threshold relationship und der stimulus-response-curve. Im threshold electrotonus war insbesondere die Schwellenwertzunahme auf hyperpolarisierende Reize signifikant geringer bei einer Reizstärke von -40%, -70% und -100% des Kontrollimpulses (p < 0,01). Die current/threshold relationship war in hyperpolasierender Richtung bei den BFCS Patienten steiler als bei der gesunden Kontrollgruppe (p < 0,05). Außerdem benötigten sie eine deutlich geringere Reizintensität, um 50% des maximalen Summenaktionspotentials des Muskels auszulösen (p < 0,05). Schlussfolgerung: Die Ergebnisse weisen auf eine veränderte Expression oder Kinetik der HCN-Kanäle an peripheren Nerven der Patienten mit einem BFCS hin und geben folglich neue Hinweise auf die Entstehung dieser Erkrankung. Da zum ersten Mal ein Zusammenhang zwischen der Veränderung der HCN-Kanäle und dem BFCS gezeigt werden konnte, sollten sich zukünftige Studien darauf konzentrieren, genauere Informationen über diese Pathologie zu erlangen.

L’identification des muscles responsables des dystonies focales est un prérequis à l’instauration des traitements par toxine botulique et exercices correcteurs. A partir de deux dystonies apparemment dissemblables: la crampe de l’écrivain et la dystonie cervicale, nous avons montré que la réponse aux traitements est tributaire de la distribution des muscles impliqués. L’enregistrement des mouvements du segment tête-cou dans la dystonie cervicale , au moyen de capteurs inertiels 3-D, a montré qu’au mouvement volontaire de la tête, dans un plan, s’associent des mouvements non physiologiques dans les deux autres plans. Pour déterminer les actions musculaires en cause, nous avons réalisé une modélisation numérique du segment tête-cou permettant d’associer le muscle responsable aux déformations. Par ailleurs, sachant l’importance des phénomènes sensitifs dans le contrôle du mouvement, nous avons, au cours de tâches d’ajustement de la force musculaire, montré que ce contrôle de la force est perturbé dans chacune des deux dystonies focales étudiées.Nos résultats devraient avoir une implication dans les traitements symptomatiques de ces dystonies. / The identification of the muscles responsible for focal dystonia is a prerequisite to the introduction of botulinum toxin treatment and tailored exercises. From two apparently dissimilar dystonia: writer's cramp and cervical dystonia, we showed that the response to the treatments depends on the distribution of the muscles involved. Recording the movement of the head-neck segment in cervical dystonia, using 3-D inertial sensors, showed that voluntary head movement in a plane is associated with non-physiological movement in the two other planes. To determine the muscular actions involved, we performed a digital modeling of the head-neck segment which allows us to link the responsible muscle with abnormal postures.Therefore, knowing the importance of sensory phenomena in the control of movement, we have, during tasks of muscular force adjustment, demonstrated that force control is altered in both studied dystonia.Our results should have implications in the symptomatic treatment of these dystonias.

Dystonia is a neurological syndrome characterized by the involuntary contraction of opposing muscles, causing twisting movements or abnormal postures (modified by Fahn, 1987). Writer's cramp is the most common form of task-specific focal dystonia. In the first study, patients with writer's cramp were evaluated for differences in cortical activation during movements likely to induce cramps (complex movements) and movements which rarely lead to dystonia (simple movements). Although complex patient movements during fMRI were never associated with dystonic cramps, they exhibited abnormally decreased cortical activity. This was not observed in simple movements and was unrelated to the character of handwriting or the presence/absence of visual feedback. Our results support the theory of dualistic sensorimotor system behavior in writer's cramp. As the somatosensory system is believed to be affected in focal dystonia, we focused on modulation of the primary somatosensory cortex (SI) induced by repetitive transcranial magnetic stimulation (rTMS) in the second study, in order to improve writer's cramp. In conclusion, 1 Hz rTMS of the SI cortex can improve manifestations of writer's cramp while increasing cortical activity in both hemispheres. Handwriting as well as subjective assessment improved in most...

Dystonia is a neurological syndrome characterized by the involuntary contraction of opposing muscles, causing twisting movements or abnormal postures (modified by Fahn, 1987). Writer's cramp is the most common form of task-specific focal dystonia. In the first study, patients with writer's cramp were evaluated for differences in cortical activation during movements likely to induce cramps (complex movements) and movements which rarely lead to dystonia (simple movements). Although complex patient movements during fMRI were never associated with dystonic cramps, they exhibited abnormally decreased cortical activity. This was not observed in simple movements and was unrelated to the character of handwriting or the presence/absence of visual feedback. Our results support the theory of dualistic sensorimotor system behavior in writer's cramp. As the somatosensory system is believed to be affected in focal dystonia, we focused on modulation of the primary somatosensory cortex (SI) induced by repetitive transcranial magnetic stimulation (rTMS) in the second study, in order to improve writer's cramp. In conclusion, 1 Hz rTMS of the SI cortex can improve manifestations of writer's cramp while increasing cortical activity in both hemispheres. Handwriting as well as subjective assessment improved in most...

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