小脑认知情感综合征1例报告并文献复习

姚红艳; 唐莲; 王倩; 张玉梅

doi:10.16766/j.cnki.issn.1674-4152.004395

小脑认知情感综合征1例报告并文献复习

doi: 10.16766/j.cnki.issn.1674-4152.004395

姚红艳¹,
唐莲¹,
王倩²,
张玉梅^2, ,

1.
首都医科大学附属北京天坛医院神经病学中心, 北京 100070
2.
首都医科大学附属北京天坛医院康复医学科

基金项目:

国家自然科学基金面上项目 82372555

详细信息

通讯作者:
张玉梅, E-mail: zhangyumei95@aliyun.com

中图分类号: R742.82
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出版历程
- 收稿日期: 2025-06-21
- 网络出版日期: 2026-04-11

A case report and literature review of cerebellar cognitive affective syndrome

1.
Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China

摘要

摘要: 临床上小脑损伤患者会出现认知功能障碍及情感调节障碍，临床表现轻于大脑受损所致的认知障碍，在实际工作中易被忽略。本文报道1例血管母细胞瘤患者因出现小脑或其环路受损导致出现轻度认知功能障碍及情感调节障碍的病例，患者男性，43岁，因“行走不稳3个月、反应减慢、焦虑、视物异常1.5个月”入院，入院后完善认知心理学测评等相关检查，最终诊断为“很可能的小脑认知情感综合征”；经过个体化的功能康复，患者症状均较前改善。本文对该病例进行报道，以期为临床上小脑认知情感综合征的诊断及治疗提供参考。
- 小脑认知情感综合征 /
- 血管母细胞瘤 /
- 普遍小脑转换理论 /
- 思维辨距理论
Abstract: Clinically, patients suffering from cerebellar damage may present with cognitive impairment and affective dysregulation. These clinical manifestations are typically less severe than the cognitive deficits caused by cerebral damage and are often overlooked in clinical practice. This case report describes a patient with hemangioblastoma who developed mild cognitive impairment and affective dysregulation due to cerebellar or cerebellar circuit damage. The patient, a 43-year-old male, was admitted with a three-month history of gait instability, accompanied by slowed responses, anxiety and visual disturbances over the preceding 1.5 months. Following comprehensive neuropsychological assessments, a diagnosis of probable cerebellar cognitive affective syndrome (CCAS) was established. Following the administration of personalized functional rehabilitation, the patient exhibited a substantial improvement in symptoms. The objective of this report is to furnish clinical insights into the diagnosis and management of CCAS.
- Cerebellar cognitive affective syndrome /
- Hemangioblastoma /
- Universal cerebellar transform /
- Dysmetria of thought

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图 1 小脑认知情感综合征患者视物歪斜测验

注：双眼视野分离，左眼正常，右眼会出现物象倾斜。

Figure 1. Test for diplopia in patients with cerebellar cognitive-affective syndrome

下载: 全尺寸图片幻灯片

图 2 小脑认知情感综合征患者术前头颅MRI检查结果

注：四脑室内可见一结节状低T₁WI(A)T₂WI(B)低信号，内可见点条状流空信号，相应于FLAIR(C)上呈稍高信号，大小18 mm×16 mm，边界清楚，四脑室周围及中脑顶盖可见片状水肿(箭头所示)。增强扫描(D)呈明显均匀强化(箭头所示)。

Figure 2. Preoperative cranial MRI examination results of patients with cerebellar cognitive and affective syndrome

下载: 全尺寸图片幻灯片

图 3 小脑认知情感综合征患者术后头颅MRI检查结果

注：术区小脑蚓部呈T₁(A)T₂(B)不均匀信号(箭头所示)，增强未见强化(C)，SWI(D)多发低信号(箭头所示)。

Figure 3. Preoperative cranial MRI examination results of patients with cerebellar cognitive and affective syndrome

下载: 全尺寸图片幻灯片

参考文献(22)

[1]	ARGYROPOULOS G P D, VAN DUN K, ADAMASZEK M, et al. The cerebellar cognitive affective/schmahmann syndrome: a task force paper[J]. Cerebellum (London, England), 2020, 19(1): 102-125. doi: 10.1007/s12311-019-01068-8
[2]	JACOBI H, FABER J, TIMMANN D, et al. Update cerebellum and cognition[J]. J Neurol, 2021, 268(10): 3921-3925. doi: 10.1007/s00415-021-10486-w
[3]	CIAPPONI C, LI Y H, OSORIO B D, et al. Variations on the theme: focus on cerebellum and emotional processing[J]. Front Syst Neurosci, 2023, 17: 1185752. DOI: 10.3389/fnsys.2023.1185752.
[4]	PIERCE J E, THOMASSON M, VORUZ P, et al. Explicit and implicit emotion processing in the cerebellum: a meta-analysis and systematic review[J]. Cerebellum, 2023, 22(5): 852-864.
[5]	REUMERS S, BONGAERTS F, DE LEEUW F E, et al. Cognition in cerebellar disorders: what's in the profile? A systematic review and meta-analysis[J]. J Neurol, 2025, 272(3): 250. DOI: 10.1007/s00415-025-12967-8.
[6]	WALSH K S, PIZER B, SAMARGIA-GRIVETTE S, et al. Proceedings of the first global meeting of the Posterior Fossa Society: state of the art in cerebellar mutism syndrome[J]. Childs Nerv Syst, 2024, 40(7): 2177-2191. doi: 10.1007/s00381-024-06411-x
[7]	SKYE J, BRUSS J, TOESCU S, et al. Neuroanatomy of cerebellar mutism syndrome: the role of lesion location[J]. Brain Commun, 2024, 6(4): fcae197. DOI: 10.1093/braincomms/fcae197.
[8]	INOUE M, OYA S, YAMAGA T, et al. Pearls & Oy-sters: cognitive and affective dysfunction caused by a small cerebellar hemangioblastoma[J]. Neurology, 2023, 101(12): e1272-e1275.
[9]	BLITHIKIOTI C, NUNO L, GUELL X, et al. The cerebellum and psychological trauma: a systematic review of neuroimaging studies[J]. Neurobiol Stress, 2022, 17: 100429. DOI: 10.1016/j.ynstr.2022.100429.
[10]	BAUMANN O, MATTINGLEY J B. Cerebellum and emotion processing[J]. Adv Exp Med Biol, 2022, 1378: 25-39.
[11]	SCHMAHMANN J D. Ferdinando Rossi Lecture: the cerebellar cognitive affective syndrome-implications and future directions[J]. Cerebellum, 2023, 22(5): 947-953.
[12]	LIU Q, ZHANG Y M. A comparative study on cognitive assessment in cerebellar and supratentorial stroke[J]. Brain Sci, 2024, 14(7): 676. DOI: 10.3390/brainsci14070676.
[13]	SERRANO-MUNUERA C, MARTÍNEZ-REGUEIRO R, MARTÍNEZ F E, et al. Validation of the Spanish version of the cerebellar cognitive-affective syndrome scale[J]. Clin Neuropsychol, 2025: 1-17. DOI: 10.1080/13854046.2025.2488453.
[14]	DE OLIVEIRA SCOTT S S, PEDROSO J L, ELIAS V V, et al. Translation, cross-cultural adaptation, and validation to brazilian portuguese of the cerebellar cognitive affective/schmahmann syndrome scale[J]. Cerebellum, 2023, 22(2): 282-294.
[15]	SAADON-GROSMAN N, DU J N, KOSAKOWSKI H L, et al. Within-individual organization of the human cognitive cerebellum: evidence for closely juxtaposed, functionally specialized regions[J]. Sci Adv, 2024, 10(45): eadq4037. DOI: 10.1126/sciadv.adq4037.
[16]	ZHANG X Y, WU W X, SHEN L P, et al. A role for the cerebellum in motor-triggered alleviation of anxiety[J]. Neuron, 2024, 112(7): 1165-1181. doi: 10.1016/j.neuron.2024.01.007
[17]	牛琪惠, 牛明敏, 张力方, 等. 强迫性障碍患者的脑功能活动特点及丘脑与全脑功能连接分析[J]. 中华全科医学, 2020, 18(8): 1272-1275, 1282. doi: 10.16766/j.cnki.issn.1674-4152.001483 NIU Q H, NIU M M, ZHANG L F, et al. Study on characteristics of brain activity and the functional connection between thalamus and whole brain in obsessive-compulsive disorder patients[J]. Chinese Journal of General Practice, 2020, 18(8): 1272-1275, 1282. doi: 10.16766/j.cnki.issn.1674-4152.001483
[18]	郝巧梅, 张海三, 马小静, 等. 3D-ASL技术评估脑梗死患者脑白质血流灌注异常情况及与认知功能的相关性分析[J]. 中华全科医学, 2024, 22(5): 795-799. doi: 10.16766/j.cnki.issn.1674-4152.003505 HAO Q M, ZHANG H S, MA X J, et al. Evaluate the abnormal white matter blood perfusion by 3D-ASL technique and its correlation with cognitive function in patients with cerebral infarction[J]. Chinese Journal of General Practice, 2024, 22(5): 795-799. doi: 10.16766/j.cnki.issn.1674-4152.003505
[19]	ARGYROPOULOS G, VAN DUN K, ADAMASZEK M, et al. The cerebellar cognitive affective/schmahmann syndrome: a task force paper[J]. Cerebellum, 2020, 19(1): 102-125. doi: 10.1007/s12311-019-01068-8
[20]	XUE A, KONG R, YANG Q, et al. The detailed organization of the human cerebellum estimated by intrinsic functional connectivity within the individual[J]. J Neurophysiol, 2021, 125(2): 358-384. doi: 10.1152/jn.00561.2020
[21]	IKENOUCHI A, IDE S, HAMADA S, et al. Cerebellar cognitive affective syndrome caused by cerebellar atrophy associated with Wallerian degeneration after pontine haemorrhage: a case report[J]. Psychogeriatrics, 2024, 24(5): 1168-1172. doi: 10.1111/psyg.13174
[22]	BECKER-BENSE S, KAISER L, BECKER R, et al. Acetyl-DL-leucine in cerebellar ataxia ([¹⁸F]-FDG-PET study): how does a cerebellar disorder influence cortical sensorimotor networks?[J]. J Neurol, 2023, 270(1): 44-56. doi: 10.1007/s00415-022-11252-2