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Characteristic "Forcible" Geste Antagoniste in Oromandibular Dystonia Resulting From Pantothenate Kinase-Associated Neurodegeneration.


ABSTRACT: Geste antagonistes are usually considered typical of primary dystonia, although rarely they have been described in secondary/heredodegenerative dystonias. We have recently come across a particular geste antagoniste in 5 of 10 patients with pantothenate kinase-associated neurodegeneration (PKAN) who had prominent oromandibular involvement with severe jaw-opening dystonia. It consists of touching the chin with both hands characteristically clenched into a fist with flexion at the elbows. Because of the resemblance of this geste antagoniste with the praying-like posture of Mantis religiosa, we coined the term "mantis sign." Reviewing videos of PKAN cases in literature, including what is considered the first cinematic depiction of a case of this disorder, 3 additional cases with akin maneuvers were identified. In contrast, examining 205 videos of non-PKAN dystonic patients from our database for the presence of a similar maneuver was unrevealing. Thus, we consider the mantis sign to be quite typical of PKAN and propose it to be added as a clinical hint toward diagnosis.

SUBMITTER: Petrovic IN 

PROVIDER: S-EPMC6183307 | biostudies-literature | 2014 Jun

REPOSITORIES: biostudies-literature

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Characteristic "Forcible" Geste Antagoniste in Oromandibular Dystonia Resulting From Pantothenate Kinase-Associated Neurodegeneration.

Petrović Igor N IN   Kresojević Nikola N   Ganos Christos C   Svetel Marina M   Dragašević Nataša N   Bhatia Kailash P KP   Kostić Vladimir S VS  

Movement disorders clinical practice 20140516 2


Geste antagonistes are usually considered typical of primary dystonia, although rarely they have been described in secondary/heredodegenerative dystonias. We have recently come across a particular geste antagoniste in 5 of 10 patients with pantothenate kinase-associated neurodegeneration (PKAN) who had prominent oromandibular involvement with severe jaw-opening dystonia. It consists of touching the chin with both hands characteristically clenched into a fist with flexion at the elbows. Because o  ...[more]

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