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Sensory Tricks in Pantothenate Kinase-Associated Neurodegeneration: Video-Analysis of 43 Patients

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Autores de CIPF

Participantes ajenos a CIPF

  • Martins, J
  • Darling, A
  • Garrido, C
  • Marti, MJ
  • Duenas, BP
  • Temudo, T

Grupos de Investigación

Abstract

Background Sensory tricks are a classic hallmark of primary dystonia and result in specific maneuvers that temporarily improve dystonic posture or movement. Pantothenate kinase-associated neurodegeneration (PKAN) is a progressive neurological disorder that courses with prominent dystonia. Although previously described, sensory tricks are considered to be rare in PKAN. Cases We reviewed videotaped motor examinations of 43 genetically confirmed patients with PKAN in order to identify and classify sensory tricks. All patients presented some feature of dystonia. Eighteen (42%) had one or more well-structured sensory tricks. Twelve different sensory tricks were identified, eight typical and four atypical (forcible motor): four in cervical dystonia, four in limb dystonia, three in oromandibular dystonia, and one in blepharospasm. A characteristic forcible motor maneuver for oromandibular dystonia (previously described as the "mantis sign") was present in 8 patients. Conclusions Sensory tricks are common in PKAN, particularly for oromandibular dystonia. The mantis sign may be a useful clue for the diagnosis.

Datos de la publicación

ISSN/ISSNe:
2330-1619, 2330-1619

Movement Disorders Clinical Practice  John Wiley and Sons Ltd

Tipo:
Article
Páginas:
704-707
PubMed:
31745482

Citas Recibidas en Web of Science: 2

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Keywords

  • sensory tricks; alleviating maneuvers; PKAN; secondary dystonia

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