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Idiopathic (primary) cranial dystonias 

Idiopathic (primary) cranial dystonias
Idiopathic (primary) cranial dystonias

Ivan Donaldson

, C. David Marsden

, Susanne A. Schneider

, and Kailash P. Bhatia

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date: 05 July 2022

Pictorial representation of cranial dystonias may have preceded their description in the medical literature. Close inspection of some 16th century Flemish paintings reveals examples of bizarre facial contortions. The most convincing is that of Pieter Brueghel, The Elder, who has depicted the screwed up eyes and wide open mouth which are so typical of this disorder. The first written description, however, was not until 1910 when Meige, a Parisian neurologist, published a paper entitled ‘Les Convulsion de la Face, une Forme Clinique de Convulsion Faciale Bilateral et Mediane’. He clearly and concisely outlined the important clinical features and distinguished them from other involuntary movement disorders affecting the face, such as tic, hemispasm, and post-paralytic spasm. Although described as ‘a facial convulsion’, he did not imply the disorder was epileptic.

He emphasized that the spasms were bilateral, tended to affect the upper face more than the lower, but could also involve the jaw and floor of the mouth, including the tongue. They were precipitated by a variety of stimuli, could be temporarily restrained by voluntary effort or certain manoeuvres, and disappeared during sleep. He noted improvement following surgical division of the supraorbital nerves. In spite of this excellent description, the disorder was largely ignored and reappeared in the literature over half a century later (Liversedge 1969, Altrocchi 1972, Marsden 1976[a]). Since then there have been a large number of publications on the disorder.

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