Further evidence of dementia in SPG4-linked autosomal dominant hereditary spastic paraplegia
Further evidence of dementia in SPG4-linked autosomal dominant hereditary spastic paraplegia
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DOI：
10.1212/01.wnl.0000108629.04434.05
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发表时间：
2004-02-10
期刊：
影响因子：
9.9
通讯作者：
Hutchinson, M
中科院分区：
文献类型：
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作者：
McMonagle, P;Byrne, P;Hutchinson, M
目的：探讨SPG4连锁常染色体显性遗传性痉挛截瘫(SPG4-ADHSP)患者认知功能障碍的进展及其行为特征。方法：前瞻性评估来自5个SPG4-ADHSP家系的16例患者，年龄在45岁以上。其中11人来自三个家庭，其中10人接受了两次认知测试，使用剑桥认知评估(CAMCOG)，相隔2.9年。第二次评估时由11例患者的近亲属填写老年人认知功能减退情况调查表(IQCODE)、神经精神病学问卷(NPI)和老年患者护士观察量表(NOSGER)。11名配对对照受试者相隔3.1年进行CAMCOG检查。结果：10例过敏性紫斑狼疮患者首次评估时CAMCOG平均得分(73.5/10 7)低于10例对照组(91.7/10 7，P=0.005)。2.9年后，过敏性紫斑狼疮患者的平均CAMCOG下降了9.1点，降至64.4%(p=0.008)。对照组的CAMCOG平均得分(90.8/107)在3.1年后仅下降了0.9分(p=0.36)。两名HSP患者的CAMCOG评分从轻度(60分至80分)进入中度痴呆类别(35分至59分)，另外三名患者的CAMCOG评分从接近正常的分数(81分至85分)进入轻度痴呆。9/11过敏性紫杉醇患者和1/11对照组IQCODE评分异常(p=0.001)。11名HSP患者中有7名被认为患有痴呆症。结论：本研究表明老年SPG4-ADHSP患者认知功能减退和痴呆的进展活跃。
Objective: To investigate the progression of cognitive impairment and its behavioral aspects in patients with SPG4-linked autosomal dominant hereditary spastic paraplegia (SPG4-ADHSP). Methods: Sixteen patients, 45 years or older, from five families with SPG4-ADHSP were prospectively assessed. Eleven of these, from three families, were followed and 10 had two cognitive examinations using the Cambridge Cognitive Assessment (CAMCOG) 2.9 years apart. The Informant Questionnaire on Cognitive Decline in the Elderly (IQCODE), the Neuropsychiatric Inventory (NPI), and the Nurses' Observation Scale for Geriatric Patients (NOSGER) were completed by close relatives of the 11 patients at the second assessment. Eleven matched control subjects had CAMCOG examinations 3.1 years apart. Results: The mean CAMCOG score at the initial assessment was lower for the 10 HSP patients (73.5/107) than for 10 control subjects (91.7/107, p=0.005). After 2.9 years, the HSP patients experienced a fall in the mean CAMCOG by 9.1 points to 64.4/107 (p=0.008). The mean CAMCOG score for the control subjects (90.8/107) fell by only 0.9 points after 3.1 years (p=0.36). The CAMCOG scores of two HSP patients moved from the mild (60 to 80) into the moderate dementia category (35 to 59) and in three other patients into the mild dementia range from borderline normal scores (81 to 85). IQCODE scores were abnormal in 9/11 HSP patients and 1/11 controls (p=0.001). Seven of the 11 HSP patients were considered as having dementia. Conclusion: This study indicates an active progression of cognitive deterioration and dementia in older patients with SPG4-ADHSP.