Vela-Desojo Lydia, Urso Daniele, Osuna-López Mireia, Hoenicka Janet
Hospital Fundación Alcorcón, 28922 Madrid, Spain.
HM Centro Integral en Neurociencias (CINAC), University Hospital HM Puerta del Sur, 28938 Madrid, Spain.
J Clin Med. 2022 Mar 13;11(6):1590. doi: 10.3390/jcm11061590.
-dystonia-parkinsonism (PLAN-DP) is characterized by levodopa responsive parkinsonism and dystonia. While neuropsychiatric symptoms and early cognitive decline are also common in this entity there is little information regarding other non-motor symptoms (NMS). Here, we describe a 26-year-old patient with PLAN-DP whose motor symptoms were preceded by mild cognitive impairment and anxiety, and who developed many other NMS as the disease evolved. Furthermore, we reviewed the NMS described in all the PLAN-DP patients published to date. A total of 50 patients with PLAN-DP were identified, 42 of whom developed NMS and in 23 of these cases, NMS preceded the motor symptoms of the disease. Neuropsychiatric symptoms dominated the premotor phase of this condition and cognitive impairment/dementia was the most prevalent NMS. Other NMS were reported infrequently like sleep disorders, autonomic symptoms, pain and hyposmia, and mostly as the disease evolved. NMS are very frequent in PLAN-DP and they may appear before diagnosis or during the course of the disease. Neuropsychiatric symptoms and cognitive decline are the most frequent NMS. The appearance of neuropsychiatric symptoms like depression, anxiety or personality changes prior to a diagnosis of parkinsonism in younger individuals might suggest the presence of gene mutations.
多巴反应性肌张力障碍-帕金森综合征(PLAN-DP)的特征为左旋多巴反应性帕金森综合征和肌张力障碍。虽然神经精神症状和早期认知衰退在该病症中也很常见,但关于其他非运动症状(NMS)的信息却很少。在此,我们描述一名26岁的PLAN-DP患者,其运动症状之前有轻度认知障碍和焦虑,并且随着疾病进展出现了许多其他NMS。此外,我们回顾了迄今为止已发表的所有PLAN-DP患者中描述的NMS。共确定了50例PLAN-DP患者,其中42例出现了NMS,在其中23例中,NMS先于疾病的运动症状出现。神经精神症状在该病症的运动前阶段占主导地位,认知障碍/痴呆是最常见的NMS。其他NMS如睡眠障碍、自主神经症状、疼痛和嗅觉减退报告较少,且大多在疾病进展过程中出现。NMS在PLAN-DP中非常常见,它们可能在诊断前或疾病过程中出现。神经精神症状和认知衰退是最常见的NMS。在年轻个体中,帕金森综合征诊断前出现抑郁、焦虑或人格改变等神经精神症状可能提示存在基因突变。