Characteristic “Forcible” Geste Antagoniste in Oromandibular Dystonia Resulting From Pantothenate Kinase‐Associated Neurodegeneration

• Published in: Movement disorders clinical practice (Hoboken, N.J.) N.J.), 2014-06, Vol.1 (2), p.112-114
• Main Authors: Petrović, Igor N., Kresojević, Nikola, Ganos, Christos, Svetel, Marina, Dragašević, Nataša, Bhatia, Kailash P., Kostić, Vladimir S.
• Format: Article
• Language: English
• Subjects: Case Series; dystonia; geste antagoniste; Neurodegeneration; PKAN
• ISSN: 2330-1619; 2330-1619
• DOI: 10.1002/mdc3.12035

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.