Progressive supranuclear palsy can mimic idiopathic normal pressure hydrocephalus: A case series.

Progressive supranuclear palsy can mimic idiopathic normal pressure hydrocephalus: A case series.
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DOI:
10.1093/jnen/nlad090
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发表时间:
2023-11-20
影响因子:
3.2
通讯作者:
--
中科院分区:
医学4区
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--
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致编辑:特发性正常压力脑积水(iNPH)的特征是步态障碍、认知障碍和尿失禁三联征。在神经影像学上,它通常伴有脑室扩大和不成比例的蛛网膜下腔扩大(1)。iNPH的临床特征可以模拟神经退行性疾病,如进行性核上性麻痹(PSP)、路易体病或阿尔茨海默病(2-5)。虽然缺乏对这些疾病的根治性治疗,但脑室腹膜分流术(VPS)已被确定为iNPH的治疗方法(6)。因此,区分iNPH与神经退行性疾病非常重要,因为VPS可能使iNPH患者受益,但对其他神经退行性疾病患者无效。我们发现了2例iNPH患者,尸检时发现PSP的神经病理学结果。第一位患者是一位70岁的男性,有6年帕金森病史,伴有进行性步态困难。他还有平衡能力差、短期记忆力下降、尿急、构音障碍、发音减退和抑郁症。他的既往病史是高脂血症,他的家族史是其母亲的帕金森症。死亡前1年神经系统检查,语言、记忆和知识储备正常,但认知反应迟钝。他的颅神经检查无异常,包括眼外运动检查,但他有掩盖的面孔。他的运动功能和肌腱深腱反射正常。他有一个缓慢的,拖着脚走路,步幅减少,没有手臂摆动。脑部MRI显示轻度全身性脑萎缩伴脑室扩大,与脑沟扩张不成比例。高容量腰椎穿刺显示他的症状出现了戏剧性的暂时性改善,从而诊断为iNPH。他在去世前2年接受了VPS置入术;然而,他的症状改善仅持续了几周。
To the Editor: Idiopathic normal pressure hydrocephalus (iNPH) is characterized by a triad of gait disturbance, cognitive impairment, and urinary incontinence. It is typically accompanied by enlarged ventricles and disproportionately enlarged subarachnoid space on neuroimaging (1). The clinical features of iNPH can mimic neurodegenerative disorders, such as progressive supranuclear palsy (PSP), Lewy body disease, or Alzheimer disease (2–5). Although curative treatments for these diseases are lacking, ventriculoperitoneal shunt (VPS) surgery has been established as a treatment for iNPH (6). Therefore, it is important to differentiate iNPH from neurodegenerative disorders because VPS may benefit patients with iNPH, but not patients with other neurodegenerative disorders. We identified 2 patients with iNPH who at autopsy had neuropathologic findings of PSP. The first patient was a 70-year-old man with a 6-year history of Parkinsonism with progressive gait difficulties. He also had poor balance, short-term memory decline, urinary urgency, dysarthria, hypophonia, and depression. His past medical history was notable for hyperlipidemia, and his family history was significant for Parkinsonism in his mother. On neurological examination 1 year before he died, he had normal language, memory, and fund of knowledge, but his cognitive responses were slow. His cranial nerve exam was unremarkable, including exams of extraocular motility, but he had masked facies. His motor function and tendon deep tendon reflexes were normal. He had a slow, shuffling gait, decreased stride length, and no arm swing. An MRI of the brain showed mild generalized cerebral atrophy with ventriculomegaly that was disproportionate to sulcal dilatation. A high-volume lumbar puncture showed dramatic, temporary improvement in his symptoms, leading to the diagnosis of iNPH. He underwent VPS placement 2 years before he died; however, improvement in his symptoms lasted only for several weeks.
经过病理验证的皮质性皮质变性,模仿了理查森综合征与临床和放射学上的反应性正常压力脑化脑化的综合征。
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