Martins Joana, Darling Alejandra, Garrido Cristina, Espinós Carmen, Martí María José, Dueñas Belen Pérez, Temudo Teresa
Pediatric Neurology Department, Centro Materno Infantil do Norte Centro Hospitalar Universitário do Porto Porto Portugal.
Pediatric Movement Disorders Unit Hospital Sant Joan de Deu Barcelona Spain.
Mov Disord Clin Pract. 2019 Oct 25;6(8):704-707. doi: 10.1002/mdc3.12842. eCollection 2019 Nov.
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.
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.
Sensory tricks are common in PKAN, particularly for oromandibular dystonia. The mantis sign may be a useful clue for the diagnosis.
感觉技巧是原发性肌张力障碍的经典特征，会产生特定动作，可暂时改善肌张力障碍姿势或运动。泛酸激酶相关神经变性（PKAN）是一种进行性神经疾病，伴有明显的肌张力障碍。虽然此前已有描述，但感觉技巧在PKAN中被认为较为罕见。
我们回顾了43例基因确诊的PKAN患者的录像运动检查，以识别和分类感觉技巧。所有患者均有肌张力障碍的某些特征。18例（42%）有一个或多个结构良好的感觉技巧。识别出12种不同的感觉技巧，8种典型和4种非典型（强力运动型）：4种见于颈部肌张力障碍，4种见于肢体肌张力障碍，3种见于口下颌肌张力障碍，1种见于眼睑痉挛。8例患者存在口下颌肌张力障碍的一种特征性强力运动动作（此前称为“螳螂征”）。
感觉技巧在PKAN中很常见，尤其是口下颌肌张力障碍。螳螂征可能是诊断的有用线索。
