Pakiam A S, Bergeron C, Lang A E
Division of Neurology, Toronto Hospital, Toronto, Canada.
Can J Neurol Sci. 1999 May;26(2):127-31.
The majority of patients with diffuse Lewy body disease have cognitive or psychiatric manifestations as part of their initial presentation. A sizable minority present with parkinsonian features alone. Autonomic features may also occur, typically after the development of cognitive changes. We aim to demonstrate that diffuse Lewy body disease may rarely also present with parkinsonism accompanied by marked autonomic dysfunction in the absence of significant cognitive or psychiatric abnormalities.
Case report based on a retrospective chart review and neuropathological examination.
We report on a patient in whom a clinical diagnosis of multiple system atrophy was made based on a presentation of parkinsonism with prominent and early autonomic involvement. The former included postural tremor, rigidity and bradykinesia, while the latter consisted of repeated falls due to orthostasis and the subsequent development of urinary incontinence midway through the course of her illness. She was poorly tolerant of dopaminergic therapy due to accentuated orthostasis. Benefit from levodopa was limited and only evident when attempted withdrawal resulted in increased rigidity. There was no history of spontaneous or drug-induced hallucinations, delusions or fluctuating cognition, and in contrast to the prominence and progression of her parkinsonian and autonomic features over the first several years, cognitive impairment did not occur until the final stages of her illness, seven years after the onset of initial symptoms. Neuropathological examination revealed numerous Lewy bodies in both neocortical as well as subcortical structures consistent with a diagnosis of diffuse Lewy body disease. There was marked neuronal loss in the substantia nigra as well as the autonomic nuclei of the brainstem and spinal cord.
In addition to cognitive, psychiatric, and parkinsonian presentations, diffuse Lewy body disease may present with parkinsonism and prominent autonomic dysfunction, fulfilling proposed criteria for the striatonigral form of MSA.
大多数弥漫性路易体病患者在疾病初期表现为认知或精神症状。少数患者仅表现为帕金森综合征特征。自主神经功能障碍也可能出现，通常在认知改变之后。我们旨在证明，弥漫性路易体病在罕见情况下也可能表现为帕金森综合征并伴有明显的自主神经功能障碍，而无显著的认知或精神异常。
基于回顾性病历审查和神经病理学检查的病例报告。
我们报告了一名患者，该患者因帕金森综合征伴突出且早期的自主神经受累而被临床诊断为多系统萎缩。前者包括姿势性震颤、强直和运动迟缓，后者包括因体位性低血压导致的反复跌倒，以及在病程中期出现的尿失禁。由于体位性低血压加重，她对多巴胺能治疗耐受性差。左旋多巴的益处有限，仅在尝试停药导致强直加重时才明显。无自发或药物诱发的幻觉、妄想或认知波动病史，与最初几年帕金森综合征和自主神经特征的突出及进展情况相反，认知障碍直到疾病末期才出现，即初始症状出现七年后。神经病理学检查显示新皮质和皮质下结构中有大量路易小体，符合弥漫性路易体病的诊断。黑质以及脑干和脊髓的自主神经核有明显的神经元丢失。
除了认知、精神和帕金森综合征表现外，弥漫性路易体病还可能表现为帕金森综合征和突出的自主神经功能障碍，符合多系统萎缩纹状体黑质型的诊断标准。
