Sipos Andrea, Árvai Milán, Varga Dávid, Ruszin-Perecz Brigitta, Janszky József, Hajdú Nándor, Pál Endre
Department of Neurology, Medical School, University of Pécs, 7623 Pécs, Hungary.
Institute of Psychology, Eötvös Loránd University, 1053 Budapest, Hungary.
Neurol Int. 2025 Jul 31;17(8):118. doi: 10.3390/neurolint17080118.
Myotonia is a rare neuromuscular condition characterized by impaired muscle relaxation. In this study, we provide normative values for clinical tests related to myotonia in the Hungarian population and compare them to patients with myotonic dystrophy type 1 (DM1).
Relaxation tests (10 eye openings, tongue extension, and palm openings), handgrip strength, and the nine-hole peg test were conducted on 139 healthy individuals and 31 patients with DM1.
We observed non-significant declines in handgrip strength and relaxation tests with age ( < 0.05). Significant differences were found between controls (n:139) and patients with DM1 (n = 31) in all tests ( < 0.05). Sex differences were noted in the healthy population: men (n:68/139) had stronger handgrip (mean of men 42.45 ± 1.15 vs. women 24.3 ± 0.9) and slower relaxation tests (mean of eye openings in men 3.6 ± 0.2 vs. in women 4.2 ± 0.2, tongue extensions in men 3.7 ± 0.2 vs. in women 4.2 ± 0.2, palm openings in men 4 ± 0.2 vs. in women 4.9 ± 0.2 However, these differences were not present among patients with DM1.
Normal values for relaxation tests across different age groups were established. These results might be useful for further clinical investigations. Our study supports the usage of averages of healthy population instead of age groups of relaxation tests and their clinical relevance in the evaluation of patients with myotonia.
肌强直是一种罕见的神经肌肉疾病，其特征为肌肉放松受损。在本研究中，我们提供了匈牙利人群中与肌强直相关的临床测试的标准值，并将其与1型强直性肌营养不良（DM1）患者进行比较。
对139名健康个体和31名DM1患者进行了放松测试（10次睁眼、伸舌和张掌）、握力测试和九孔插板测试。
我们观察到握力和放松测试随年龄增长无显著下降（<0.05）。在所有测试中，对照组（n = 139）和DM1患者（n = 31）之间存在显著差异（<0.05）。在健康人群中注意到了性别差异：男性（n = 68/139）握力更强（男性平均值42.45±1.15，女性为24.3±0.9），放松测试更慢（男性睁眼平均值3.6±0.2，女性为4.2±0.2；男性伸舌平均值3.7±0.2，女性为4.2±0.2；男性张掌平均值4±0.2，女性为4.9±0.2）。然而，这些差异在DM1患者中不存在。
建立了不同年龄组放松测试的正常值。这些结果可能对进一步的临床研究有用。我们的研究支持在评估肌强直患者时使用健康人群的平均值而非年龄组的放松测试及其临床相关性。
