Watanabe Ryohei, Kawakami Ito, Onaya Mitsumoto, Higashi Shinji, Arai Nobutaka, Akiyama Haruhiko, Hasegawa Masato, Arai Tetsuaki
Dementia Reserch Project, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan.
Department of Psychiatry, University of Tsukuba, Ibaraki, Japan.
Neuropathology. 2018 Jun;38(3):281-287. doi: 10.1111/neup.12442. Epub 2017 Nov 7.
Catatonia is a clinical syndrome characterized by symptoms such as immobility, mutism, stupor, stereotypy, echophenomena, catalepsy, automatic obedience, posturing, negativism, gegenhalten and ambitendency. This syndrome occurs mostly in mood disorder and schizophrenic patients, and is related to neuronal dysfunction involving the frontal lobe. Some cases of frontotemporal dementia (FTD) with catatonia have been reported, but these cases were not examined by autopsy. Here, we report on a FTD case which showed catatonia after the first episode of brief psychotic disorder. At the age of 58, the patient had a sudden onset of disorganized behavior and meaningless speech. Psychotropic drugs were effective for catatonic symptoms. However, after remission apathy, hyperorality, socially inappropriate behavior, hoarding, and an instinctive grasp reaction appeared and persisted. Brain MRI showed significant atrophy of the bilateral fronto-temporal lobes. A neuropathological examination revealed extensive trans-activation response DNA-binding protein 43 (TDP-43) positive neurocytoplasmic inclusions and dystrophic neurites in the brain, including the cerebral cortex, basal ganglia, and brainstem. Pathological diagnosis was frontotemporal lobar degeneration (FTLD) with TDP-43 (FTLD-TDP) type C, which was also confirmed by the band pattern of C-terminal fragments of TDP-43 on western blotting of sarkosyl-insoluble fractions extracted from the frozen brain. Dysfunction of the thalamus, globus pallidus, supplementary motor area, amygdala and cingulate cortex have been said to be related to the catatonic syndrome. In this case, these areas were affected, showing abnormal TDP-43-positive structures. Further studies are expected to confirm further clinical - pathological correlations to FTLD.
紧张症是一种临床综合征，其特征为不动、缄默、木僵、刻板动作、模仿现象、僵住症、自动服从、姿势异常、违拗症、抗拒及矛盾意向等症状。该综合征多见于心境障碍和精神分裂症患者，与涉及额叶的神经元功能障碍有关。已有一些伴有紧张症的额颞叶痴呆（FTD）病例报道，但这些病例未进行尸检。在此，我们报告一例FTD病例，该病例在首次短暂精神病性障碍发作后出现紧张症。患者58岁，突然出现行为紊乱和言语无意义。精神药物对紧张症症状有效。然而，缓解后出现了冷漠、口欲亢进、社交不当行为、囤积行为及本能抓握反应，并持续存在。脑部MRI显示双侧额颞叶显著萎缩。神经病理学检查发现大脑包括大脑皮质、基底神经节和脑干中有广泛的反式激活反应DNA结合蛋白43（TDP-43）阳性神经细胞质包涵体和营养不良性神经突。病理诊断为C型TDP-43相关额颞叶变性（FTLD-TDP），从冷冻脑提取的 Sarkosyl不溶性组分的蛋白质免疫印迹上TDP-43 C末端片段的条带模式也证实了这一点。据说丘脑、苍白球、辅助运动区、杏仁核和扣带回皮质功能障碍与紧张症综合征有关。在该病例中，这些区域受到影响，显示出异常的TDP-43阳性结构。期望进一步的研究能证实与FTLD更多的临床-病理相关性。
