Department of Neurology, University of Cologne, Cologne, Germany.
CNS Neurosci Ther. 2012 Feb;18(2):149-59. doi: 10.1111/j.1755-5949.2011.00247.x.
Patients with neurodegenerative disease frequently experience hallucinations and illusionary perceptions. As early symptoms, hallucinations may even have diagnostic relevance (i.e., for the diagnosis of Lewy Body Dementia). In the later course of the disease, hallucinations may appear as characteristic symptoms and often constitute a particular challenge for therapeutic endeavors. Here, the distinction of disease-inherent hallucinations from medication-associated perceptual disturbances is particularly relevant. Synucleinopathies and tauopathies have different risk profiles for hallucinations. In synucleinopathies hallucinations are much more frequent and phenomenology is characterized by visual, short-lived hallucinations, with insight preserved for a long time. A “double hit” theory proposes that dysfunctionality of both associative visual areas and changes of limbic areas or the ventral striatum are required. In contrast, in tauopathies the hallucinations are more rare and mostly embedded in confusional states with agitation and with poorly defined or rapidly changing paranoia. The occurrence of hallucinations has even been proposed as an exclusion criterion for tauopathies with Parkinsonian features such as progressive supranuclear palsy. To date, treatment remains largely empirical, except the use of clozapine and cholinesterase inhibitors in synucleinopathies, which is evidence-based. The risk of increased neuroleptic sensitivity further restricts the treatment options in patients with Lewy Body Dementia. Coping Strategies and improvement of visual acuity and sleep quality may be useful therapeutic complements.
神经退行性疾病患者常出现幻觉和错觉。幻觉作为早期症状,甚至可能具有诊断意义(例如,对路易体痴呆的诊断)。在疾病后期,幻觉可能出现为特征性症状,且常常给治疗带来特别的挑战。此时,区分疾病本身引起的幻觉与药物相关的知觉障碍尤为重要。α-突触核蛋白病和 tau 病的幻觉风险特征不同。在 α-突触核蛋白病中,幻觉更为常见,其表现为短暂的视觉幻觉,长时间保持洞察力。“双重打击”理论提出,需要同时存在关联视觉区域的功能障碍和边缘区域或腹侧纹状体的改变。相比之下,在 tau 病中,幻觉更为罕见,且大多嵌入激越和意识混乱状态,妄想表现为定义不明确或迅速变化。幻觉的发生甚至被提议作为具有帕金森特征的 tau 病(如进行性核上性麻痹)的排除标准。迄今为止,除了在 α-突触核蛋白病中使用氯氮平和胆碱酯酶抑制剂具有循证医学证据外,治疗仍主要为经验性治疗。增加神经安定敏感性的风险进一步限制了路易体痴呆患者的治疗选择。应对策略以及提高视力和睡眠质量可能是有用的治疗补充。