Szirmai Imre, Kovács Tibor
Semmelweis Egyetem, Altalános Orvosi Kar, Neurológiai Klinika, 1083 Budapest, Balassa u. 6.
Ideggyogy Sz. 2002 Jul 20;55(7-8):220-5.
The cognitive (executive) ability of patients with Parkinson's-disease (PD) deteriorates gradually during the progression of the disease. Fluency of speech, word finding, working memory, ability to plan the future and flexibility decline. Cognitive disturbance was found to be proportional with the speech, posture, gait and balance problems and can not be influenced by L-dopa substitution. Apart the dorsal and ventral mesolimbic dopaminergic systems the coerulo-cortical noradrenergic, serotoninergic and cholinergic systems are also impaired in PD. Subcortical dementia in PD can also be explained by the functional disability of dorsolateral and anterior cingular circuits. Attention deficit can be explained by the dopamine depletion of cingular cortex. Cortical Lewy bodies, neurofibrillary tangles, neurit plaques and additional vascular pathology should also play a role in cognitive impairment of PD. In several systemic degenerative diseases associating with Parkinson's syndrome (PS) i.e. progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), multiple system atrophy (MSA) dementia can be detected with various severity, therefore the question arises concerning the correlation between cognitive disability and PS. Parkinson syndrome can also develop in frontotemporal dementias (FTD), Alzheimer's disease and cortical Lewy body disease (CLBD) but no correlation exists between motor disability and severity of dementia. In CLBD dementia can be the initial symptom in 18% of cases but PS can also preceeds the dementia. In PSP profound depletion of other monoaminergic neurotransmitter system was also reported. In FTDs associated with PS degeneration of substantia nigra, locus coeruleus and basal nucleus of Meynert has been reported with increased number of neurofibrillary tangles. In patients with vascular PS (VP) there is generally no tremor and rigidity, but pseudobulbar palsy, dementia, gate disturbance, incontinency appears; L-dopa treatment is generally ineffective. In VP no cellular loss can be found within the substantia nigra, but leukoaraiosis, lacunae in the white matter and basal ganglia are commonly demonstrated.
帕金森病（PD）患者的认知（执行）能力在疾病进展过程中会逐渐恶化。言语流畅性、找词能力、工作记忆、规划未来的能力以及灵活性都会下降。研究发现认知障碍与言语、姿势、步态和平衡问题成正比，且不受左旋多巴替代治疗的影响。除了背侧和腹侧中脑边缘多巴胺能系统外，蓝斑-皮质去甲肾上腺素能、5-羟色胺能和胆碱能系统在PD中也受损。PD中的皮质下痴呆也可由背外侧和前扣带回回路的功能障碍来解释。注意力缺陷可由扣带回皮质的多巴胺耗竭来解释。皮质路易体、神经原纤维缠结、神经炎性斑块以及其他血管病变也应在PD的认知障碍中起作用。在几种与帕金森综合征（PS）相关的全身性退行性疾病中，即进行性核上性麻痹（PSP）、皮质基底节变性（CBD）、多系统萎缩（MSA），可检测到不同严重程度的痴呆，因此出现了关于认知功能障碍与PS之间相关性的问题。帕金森综合征也可发生在额颞叶痴呆（FTD）、阿尔茨海默病和皮质路易体病（CLBD）中，但运动功能障碍与痴呆严重程度之间不存在相关性。在CLBD中，18%的病例痴呆可为首发症状，但PS也可先于痴呆出现。在PSP中，也有其他单胺能神经递质系统严重耗竭的报道。在与PS相关的FTD中，已报道黑质、蓝斑和Meynert基底核发生变性，神经原纤维缠结数量增加。在血管性PS（VP）患者中，通常无震颤和强直，但会出现假性延髓麻痹、痴呆、步态障碍、尿失禁；左旋多巴治疗通常无效。在VP中，黑质内未发现细胞丢失，但常见白质疏松、白质和基底节的腔隙。
