Fondazione Santa Lucia, IRCCS, Roma, Italy.
UOC Neurologia, Azienda Ospedaliera "Belcolle", Viterbo, Italy.
Brain Behav. 2019 Dec;9(12):e01448. doi: 10.1002/brb3.1448. Epub 2019 Nov 19.
Phenotypic variants of progressive supranuclear palsy (PSP) are all characterized by the combination of motor symptoms of parkinsonism with a number of neuropsychiatric and cognitive disorders. Despite the strong effort in characterizing these features in PSP, alexithymia and anhedonia have not been investigated at present. Here, we aimed at investigating the qualitative and quantitative differences of alexithymia and anhedonia in the two more frequent variants of PSP, Richardson's syndrome (PSP-RS) and PSP with predominant parkinsonism (PSP-P) compared to Parkinson's disease (PD) patients recruited within 24 months after the onset of motor symptoms.
One hundred fifty-five PD, 11 PSP-P, and 14 PSP-RS patients underwent clinical, neuropsychiatric, and neuropsychological evaluations. Alexithymia was assessed using the Toronto Alexithymia Scale-20 item (TAS-20), and hedonic tone was measured by the Snaith-Hamilton Pleasure Scale (SHAPS).
In PSP-P and PSP-RS patients, the frequency of alexithymia diagnosis was higher than in PD. On the TAS-20 scores, PSP-RS performed worse in the total score and in F2 sub-scale when compared to PD. Among patients with diagnosis of depression, PSP-RS showed higher scores in TAS-20 total and TAS-20 F2 than PD. No significant differences in TAS-20 scores were found in nondepressed patients. Finally, we did not find significant differences among PD, PSP-P, and PSP-RS groups in the SHAPS scores.
Alexithymia is identifiable very early in PSP-P and PSP-RS patients. Alexithymic symptoms differentiate PSP-RS group from PD group but not between the two subtypes of PSP. Further, alexithymia in PSP seems to be predicted by the presence of depression. Altered emotional capability could be related to specific neurophysiological dysfunction occurring precociously in PSP; therefore, its identification could orient the diagnosis toward PSP cases.
进行性核上性麻痹(PSP)的各种表型变异均以帕金森病运动症状与多种神经精神和认知障碍的组合为特征。尽管目前已经在 PSP 中对这些特征进行了强烈的特征描述,但尚未对其进行述情障碍和快感缺失的研究。在这里,我们旨在研究与帕金森病(PD)患者相比,在两个更常见的 PSP 变体,即 Richardson 综合征(PSP-RS)和以帕金森病为主的 PSP(PSP-P)中,述情障碍和快感缺失的定性和定量差异,这些患者均在运动症状发作后 24 个月内招募。
155 名 PD、11 名 PSP-P 和 14 名 PSP-RS 患者接受了临床、神经精神和神经心理学评估。使用多伦多述情障碍量表 20 项(TAS-20)评估述情障碍,使用 Snaith-Hamilton 快感量表(SHAPS)测量快感缺失。
在 PSP-P 和 PSP-RS 患者中,述情障碍的诊断频率高于 PD。在 TAS-20 评分中,PSP-RS 在总分和 F2 子量表上的得分均低于 PD。在诊断为抑郁症的患者中,PSP-RS 在 TAS-20 总分和 TAS-20 F2 上的得分均高于 PD。在非抑郁患者中,TAS-20 评分无显著差异。最后,我们在 PD、PSP-P 和 PSP-RS 组之间的 SHAPS 评分中未发现显著差异。
述情障碍在 PSP-P 和 PSP-RS 患者中很早就可以识别出来。述情障碍症状将 PSP-RS 组与 PD 组区分开来,但不能区分两种 PSP 亚型。此外,PSP 中的述情障碍似乎是由抑郁的存在预测的。情感能力的改变可能与 PSP 中早期发生的特定神经生理功能障碍有关;因此,其识别可以将诊断指向 PSP 病例。