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Vogt-Vogt syndrome (Cécile and Oskar Vogt)

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This syndrome is the result of a lesion of the corpus striatum, and is usually associated with birth injury, especially one causing the athetoid form of cerebral palsy. It is characterized by an abnormal aggregation of myelinated nerve fibres in the nervous system, typically in the corpus striatum, which gives it a marble like appearance. The thalamus, corpus pallidum, and cerebral cortex may also be involved.

Clinically there are marked choreiform and athetoid movements which begin in the first year of life, successively replaced by rigidity, and causing walking problems and speech disorders. Spasmodic laughing or crying, and mental deficiency may be associated

According to some authors this is a no longer commonly used term for an extrapyramidal disturbance with double sided athetosis occurring in early childhood. One of the earliest accounts of this condition was made in 1892 by James Wright Putnam (1860-1938).

A disturbance with onset months after birth when complex motor activity develops, such as sitting, standing, and walking.

It is characterised by slow, writhing, purposeless movements mainly affecting the hands and face (forced laughter and crying). Disturbance of posture is mainly contractures in position with flexion of the knees. There is also disturbance of tonus with over stretchable joint. Besides athetosis spastic and cerebral signs also occur. Lesions of the midbrain, thalamic nuclei, pallidostratium, and internal capsule of the cerebral cortex are the cause of this disorder. Most patients are good-natured and have normal intelligence. Premature infants frequently affected. Both dominant and recessive autosomal types.

Bibliography

  • J. W. Putnam:
    A case of complete athetosis with post-mortem. Journal of Nervous and Mental Disease, Chicago, 1892, n.s. 17: 124-126.
  • C. Vogt:
    Quelques considérations générales à propos du syndrôme du corps strié.
    Journal für Psychologie und Neurologie, Leipzig, 1911, 18: 479-488.
  • C. Vogt, O. Vogt:
    Zur Lehre der Erkrankungen des striären Systems.
    Journal für Psychologie und Neurologie, Leipzig, 1920, volume 25 (supplement 3): 627-846.

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