Fluctuating Dystonia and Allied Syndromes

Gordon, Neil

doi:10.1055/s-2008-1059614

Cited by 18 publications

(8 citation statements)

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“…Tremor analysis revealed the presence of a significant postural tremor in both brothers with an abnormally low peak frequency in the younger boy. This supports other clinical descriptions of tremors in similar cases (3,5,7,8,13,30,39,40,45).…”

Section: Discussionsupporting

confidence: 91%

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Motor Control in Childhood Onset Dopa-Responsive Dystonia (Segawa Syndrome)*

Müller

Hömberg²,

Lenard³

1989

Neuropediatrics

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To elucidate the pathophysiological features of childhood onset dopa-responsive dystonia (DRD) we used a variety of quantitative analysis techniques to evaluate aspects of reflex- and voluntary motor control in two brothers with this disorder. The observed patterns were compared with those obtained in patients with adult onset Parkinson's disease (PD) and Huntington's disease (HD). In both brothers onset of the disease was in the first decade. Both responded either to treatment with L-Dopa or a combination of L-Dopa with trihexiphenidyl. Neurophysiological studies revealed slowing of different upper extremity voluntary motor activities and a low frequency postural tremor similar to results in other basal ganglia disorders including PD. In contrast to adult onset PD, fastest isometric voluntary index finger contractions did not show the typical kinetic tremor oscillations superimposed on the force trajectories. Also, different to adult PD, no impairment of stance regulating reflexes or saccadic and smooth pursuit eye movements was found in DRD. Magnetoelectrical stimulation of motor cortex showed normal efferent cortical spinal activity. Data indicate that like in other basal ganglia diseases slowing of voluntary motor activity is also a constant feature in DRD. DRD patients show, however, a clear difference to the pattern of motor abnormalities obtainable in PD. Both the pattern of motor control abnormalities is different, and the longlasting effect. In contrast to PD the pathophysiological mechanism in DRD possibly involves a lack of maturation of dopaminergic substantia nigra neurons rather than a degenerative process.

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Section: Discussionsupporting

confidence: 91%

“…In cases of DRD the occurrence of extensor plantar responses has been noted (5,13,17,22,26,29). It remains unclear if this is indicative of involvement of corticospinal tracts or rather reflects a "striatal foot" (12) Le.…”

Section: Discussionmentioning

confidence: 99%

Motor Control in Childhood Onset Dopa-Responsive Dystonia (Segawa Syndrome)*

Müller

Hömberg²,

Lenard³

1989

Neuropediatrics

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“…Judging by their clinical features, these syndromes are easily recognisable, their course is predictable and the results of treatment frustrating. Not exactly knowing the underlying pathophysiological process, the approach to treatment is still on a trial and error basis (Gordon 1982). Recently, interesting papers have been published in relation to the daily course of the dystonic syndromes (Gordon 1982, Oll\'rier 1978 and Se-!…”

Section: Introductionmentioning

confidence: 99%

Treatment of Non-Fluctuating Progressive Dystonia: A Neuropharmacological Approach

Willemse¹,

Nieuwenhuizen²,

Gooskens³

et al. 1984

Neuropediatrics

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HVA and 5-HIAA in CSF were determined in five patients with non-fluctuating progressive dystonia before and after treatment with L-Dopa. The response to L-Dopa was unequivocally favourable in one patient. The HVA-level in her CSF was significantly reduced before treatment, whereas in the other four non-responding patients the pre-treatment HVA was normal. It is postulated, that the biochemical delineation of clinical responsiveness to a neurotransmitter may provide useful clues towards the classification of extrapyramidal disease in childhood.

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“…This type of SUMMARY relapsing course has been documented only rarely (Zeman et af. 1960, Gordon 1982.…”

Section: Discussionmentioning

confidence: 99%

“…A subgroup of progressive idiopathic childhood dystonia, first identified in Japan by Segawa and colleagues, is characterized by marked diurnal fluctuation and a reversal of the symptoms with low doses of L-dopa (Segawa et al 1976, Ouvrier 1978 which also are responsive to L-dopa, unlike most other types of idiopathic childhood dystonia (Gordon 1982). We report a dopa-sensitive progressive idiopathic dystonia with childhood onset and a peculiar course, documented over many years and characterized by lateoccurring fluctuations, regression and exacerbation, and with writer's cramp as one of the main features.…”

Section: A Ferreiramentioning

confidence: 99%

Idiopathic Fluctuating Dystonia: a Case of Foot Dystonia and Writer's Cramp Responsive to L‐dopa

Deonna

Ferreira²

1985

Develop Med Child Neuro

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SUMMARY A forme fruste of progressive idiopathic dystonia is reported in a girl, starting at eight years and followed to 24 years of age. The first symptom was isolated dystonia of the left foot and later writer's cramp developed in the hands. Fluctuations of the dystonia occurred later in the course of the disorder, which were not related to the sleep‐waking cycle. Complete remission of symptoms was obtained with low dosage of L‐dopa. This case broadens the spectrum of fluctuating dopa‐sensitive progressive childhood dystonias and provides evidence for writer's cramp being a form of focal dystonia. RÉSUMÉ Dystonie idiopathique fluctuanle: un cas de dystonie du pied el de erampe de l'écrivain répondant à la L‐dopa L'article rapporte une forme fruste de dystonie idiopathique progressive chez une fille ayant débutéà l'âge de huit ans et suivie jusqu'a l'age de 24 ans. Les symptomes débutèrent par une dystonie isolée du pied gauche et plus tard par une erampe de l'écrivain de la main droite. Les fluctuations de la dystonie, non reliées aux cycles de veille‐sommeil apparurent plus tard au cours du développement et une rémission complète des symptomes fut obtenue par de faibles doses de L‐dopa. Ce cas élargit le spectre des dystonies progressives fluctuantes de l'enfance sensibles à la Dopa et rend évident que la erampe de l'écrivain est une forme de dystonie focale. ZUSAMMENFASSUNG Idiopathische fluktuierende Dystonie: ein Fall mil Fußdystonie und Schreibkrampf, der auf L‐Dopa ansprach Es wird über eine ‘forme fruste’ einer progressiven idiopathischen Dystonie bei einem Mädchen berichtet, die im Alter von acht Jahren begann und bis zum 24. Lebensjahr kontrolliert wurde. Die Symptome begannen mit einer isolierten Dystonie des linken Fußes und später kam ein Schreibkrampf der rechten Hand hinzu. Im weiteren Verlauf traten Fluktuationen der Dystonie auf, die nicht dem Schlaf‐Wach‐Rhythmus unterworfen waren und durch eine niedrige Dosis von L‐Dopa wurde eine komplette Remission erreicht. Dieser Fall erweitert das Spektrum der fluktuierenden Dopa‐sensiblen progressiven Dystonien im Kindesalter und liefert einen Anhalt dafür, daß der Schreibkrampf eine Form der fokalen Dystonie ist. RESUMEN Dislonia fluctuante idiopática. Un caso de distonia de pie y calambre del escribiente sensibles a L‐dopa Se aporta una forma frustre de distonía idiopática progresiva en una niña que se inició a los ocho años de edad y fue seguida hasta los 24 años. Los síntomas se iniciaron con una distonía aislada del pie izquierdo y más tarde con un calambre del escribiente de la mano derecha. Más adelante aparecieron fluctuaciones en la distonia sin relación con el ciclo sueño/vigilia y una remisión completa de los sintomas se obtuvo con dosis bajas de L‐dopa. Este caso amplia el campo de las distonias infantiles fluctuantes Dopa‐sensibles y proporciona evidencia de que el calambre del escribiente es una forma local de distonia.

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Fluctuating Dystonia and Allied Syndromes

Cited by 18 publications

References 0 publications

Motor Control in Childhood Onset Dopa-Responsive Dystonia (Segawa Syndrome)*

Motor Control in Childhood Onset Dopa-Responsive Dystonia (Segawa Syndrome)*

Treatment of Non-Fluctuating Progressive Dystonia: A Neuropharmacological Approach

Idiopathic Fluctuating Dystonia: a Case of Foot Dystonia and Writer's Cramp Responsive to L‐dopa

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