An autopsy case of progressive supranuclear palsy with severe corticospinal tract degeneration.

IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY
Neuropathology Pub Date : 2024-08-24 DOI:10.1111/neup.13001
Satoshi Kuru, Motoko Sakai, Hideyuki Moriyoshi, Hiroaki Miyahara, Yasushi Iwasaki
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引用次数: 0

Abstract

We report an autopsy case of a 70-year-old man who was clinically diagnosed with atypical progressive supranuclear palsy (PSP). He initially presented with gait ataxia and then showed vertical gaze palsy, rigidity, akinesia, dysphagia, and mild cognitive impairment, followed by prominent upper motor signs later in the course of the disease. Cranial magnetic resonance imaging revealed tegmental atrophy of the midbrain. Autopsy revealed severe neuronal loss and gliosis in the motor cortex and corticospinal degeneration and mild to moderate neuronal loss and gliosis in the basal ganglia, substantia nigra, midbrain, and pons. Tufted astrocytes were primarily found in the motor cortex and basal ganglia. Globose-type neurofibrillary tangles were observed in the locus coeruleus and nucleus olivaris inferior. In the cerebellar cortex, mild Purkinje cell loss and scattered axonal torpedoes were observed with tau-positive Purkinje cells. The dentate nucleus displayed severe neuronal loss and gliosis. The present case showed characteristics of both PSP with prominent cerebellar ataxia (PSP-C) and PSP-primary lateral sclerosis (PSP-PLS).

一例伴有严重皮质脊髓束变性的进行性核上性麻痹尸检病例。
我们报告了一例 70 岁男性的尸检病例,他被临床诊断为非典型进行性核上性麻痹(PSP)。他最初表现为步态共济失调,随后出现垂直凝视麻痹、僵直、运动障碍、吞咽困难和轻度认知障碍,病程后期出现明显的上肢运动症状。头颅磁共振成像显示中脑被盖区萎缩。尸检显示,运动皮质和皮质脊髓变性存在严重的神经元缺失和胶质增生,基底节、黑质、中脑和脑桥存在轻度至中度神经元缺失和胶质增生。簇状星形胶质细胞主要出现在运动皮层和基底节。在脑室和橄榄核下部观察到球状神经纤维缠结。在小脑皮层,观察到轻度的浦肯野细胞缺失和散在的轴突鱼雷,浦肯野细胞tau阳性。齿状核显示出严重的神经元缺失和胶质增生。本病例同时表现出小脑共济失调综合征(PSP-C)和小脑原发性侧索硬化症(PSP-PLS)的特征。
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来源期刊
Neuropathology
Neuropathology 医学-病理学
CiteScore
4.10
自引率
4.30%
发文量
105
审稿时长
6-12 weeks
期刊介绍: Neuropathology is an international journal sponsored by the Japanese Society of Neuropathology and publishes peer-reviewed original papers dealing with all aspects of human and experimental neuropathology and related fields of research. The Journal aims to promote the international exchange of results and encourages authors from all countries to submit papers in the following categories: Original Articles, Case Reports, Short Communications, Occasional Reviews, Editorials and Letters to the Editor. All articles are peer-reviewed by at least two researchers expert in the field of the submitted paper.
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