Objective: To analyze and classify sensory tricks developed by patients with dystonia in the setting of pantothenate kinase-associated neurodegeneration (PKAN).

Background: PKAN is a progressive neurological disorder that courses with prominent dystonia, often with oromandibular involvement. Sensory tricks are voluntary movements or alterations in the sensory input that temporarily improve dystonia.

Methods: We analyzed the videotapes of 45 genetically confirmed patients with PKAN and identified sensory tricks. We classified them according to the segment of dystonia, the different tasks, the phenomenology, the complexity and the utilization of an object.

Results: All patients presented some feature of dystonia; 18/45 (40%) had well-structured sensory tricks. Seven had two or more different tricks. Eighteen different sensory tricks were identified. We classified eight different sensory tricks for oromandibular dystonia; two for blepharospasm; four for cervical dystonia; three for writer’s cramp and one for limb dystonia during gait. Five patients had complex sensory tricks and all presented more severe dystonia. Two patients utilized an object for oromandibular dystonia.

Conclusions: Sensory tricks are well-recognized phenomena in primary dystonia. We have demonstrated that sensory tricks are also frequent in the setting of neurodegenerative secondary dystonia, being the underlying mechanism unclear. In this cohort of PKAN patients, we realized that sensory tricks are common, the phenomenology is extensive and the complexity is related to the severity of dystonia.

References: Acknowledgements: Vincenzo Lupo, Cristina Tello, María José Martí, Sergio Aguilera, Miguel Tomás, Luis González Gutiérrez Solana, Itziar Gastón, Marcos Madruga, Julio Ramos Lizana, Montserrat Pujol, Tania Gavilán Iglesias, Kylee Tustin, Jean Pierre Lin, Giovanna Zorzi, Nardo Nardocci, Loreto Martorell, Gustavo Lorenzo Sanz, Fuencisla Gutiérrez, Pedro J. García, Lidia Vela, Carlos Hernández Lahoz, Juan Darío Ortigoza Escobar, Laura Martí Sánchez, Fradique Moreira, Miguel Coelho, Leonor Correia Guedes, Ana Castro Caldas, Joaquim Ferreira, Paula Pires, Cristina Costa, Paulo Rego, Marina Magalhães, María Stamelou, Daniel Cuadras Pallejá, Carmen Rodríguez-Blazquez, Pablo Martínez-Martín, Leonidas Stefanis and Roser Pons.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/analysis-of-sensory-tricks-in-45-patients-with-pantothenate-kinase-associated-neurodegeneration/