Londos Elisabet, Hansson Oskar, Rosén Ingmar, Englund Elisabet
Department of Clinical Sciences Malmö, Lund University, Malmoe, Sweden.
Department of Clinical Sciences Lund, Lund University, Lund, Sweden.
BMJ Case Rep. 2019 Mar 9;12(3):e228177. doi: 10.1136/bcr-2018-228177.
Excessive sleep during the night and for >2 hours during the day is part of the fluctuating wakefulness criterion of dementia with Lewy bodies (DLB). The phenomenon 'sleep days' is not uncommon in nursing homes. Here, we describe a woman who, for months, slept for 3 days and nights in a row and thereafter was awake for 3 days and nights. Electroencephalogram (EEG) showed slow background activity and increased delta activity. No epileptiform activity was detected. Polysomnography showed a severely disturbed, markedly fragmented sleep pattern. On her death, neuropathology revealed degeneration and loss of neurons along with α-synuclein-containing Lewy body inclusions and neurites in the substantia nigra, locus coeruleus, hypothalamus, and neocortex, thus fulfilling the criteria of DLB, cortical type. We propose that the hypothalamic degeneration contributed significantly to the clinical profile in this case. We suggest that patients with sleep days should be investigated for other DLB signs.
夜间睡眠过多且白天睡眠时间超过2小时是路易体痴呆(DLB)波动性觉醒标准的一部分。“睡眠日”现象在养老院并不罕见。在此,我们描述一名女性,她连续数月出现连续3天3夜睡眠,之后又连续3天3夜清醒。脑电图(EEG)显示背景活动缓慢且δ活动增加。未检测到癫痫样活动。多导睡眠图显示睡眠模式严重紊乱、明显碎片化。她去世后,神经病理学检查发现黑质、蓝斑、下丘脑和新皮质出现神经元变性和丢失,伴有含α-突触核蛋白的路易小体包涵体和神经突,从而符合皮质型DLB的标准。我们认为下丘脑变性在该病例的临床特征中起了重要作用。我们建议对有睡眠日的患者进行其他DLB体征的检查。