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一位 42 岁女性出现进行性多灶性神经系统疾病。

A progressive multifocal neurological syndrome in a 42-year-old woman.

机构信息

Department of Neuropathology, Beaumont Hospital, Dublin, Ireland.

出版信息

Brain Pathol. 2011 Sep;21(5):611-4. doi: 10.1111/j.1750-3639.2011.00522.x.

Abstract

This is the case of a 42-year-old female who presented with transient dizziness. Her symptoms and signs progressed to include dysarthria, ataxia and cognitive decline over 2 years, such that she was unable to care for herself. She died 4 years after first presentation without a diagnosis. Investigations revealed a normochromic normocytic anaemia. Cerebrospinal fluid was normal. Serial computed tomography brain showed a wedge-shaped frontal infarct but no progressive changes. Examination at autopsy showed discoloration of the gray and white matter of the brain and spinal cord.Microscopy of leptomeningeal and parenchymal vessels showed they were filled with atypical B lymphocytes confined to the intravascular space with multiple infarcts in the brain, cerebellum and spinal cord. A diagnosis of intravascular B cell lymphoma was made and is discussed.

摘要

这是一位 42 岁女性的病例,她表现为一过性头晕。她的症状和体征逐渐加重,包括构音障碍、共济失调和认知能力下降,以至于无法照顾自己。她在首次就诊 4 年后死亡,仍未确诊。检查发现正细胞正色素性贫血。脑脊液正常。连续脑 CT 显示楔形额部梗死,但无进展性改变。尸检检查显示脑和脊髓的灰质和白质变色。软脑膜和实质血管的显微镜检查显示,血管内充满了局限于血管腔内的非典型 B 淋巴细胞,脑、小脑和脊髓多处梗死。诊断为血管内 B 细胞淋巴瘤,并对此进行了讨论。

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