Kaur Sukhman, Kwon Kihyun, Ramachandran Sudha, Pisinski Leszek, Krauthamer Alan
Harlem Hospital Center/Columbia University Irving Medical Center, 506 Lenox Ave, New York, NY 10037, USA.
Radiol Case Rep. 2022 May 4;17(7):2289-2294. doi: 10.1016/j.radcr.2022.03.075. eCollection 2022 Jul.
Spontaneous intracranial hypotension is a rare disease that results from low cerebrospinal fluid (CSF) volume caused by leakage of CSF from the spine in the absence of lumbar puncture, spine surgery, or intervention. The most common presentation is the headache that is usually but not invariably orthostatic. The underlying pathology is a CSF leak resulting from dural weakness involving the nerve root sleeves, ventral dural tears associated with calcified disc herniations, or CSF venous fistula. In severe cases, neuropsychiatric symptoms and changes in mental status may develop. Some case reports also mention gait disturbances, slurred speech, and urinary incontinence. The constellation of neuropsychiatric symptoms similar to behavior variant frontotemporal dementia in the presence of "brain sag" on MRI is known as frontotemporal brain sagging syndrome, first described by Wicklund et al. (4). The disease presents a diagnostic challenge to the primary care physicians, who are the first to see these patients. Brain and spine imaging is key to diagnoses but requires a high index of suspicion, as very rarely are all classic findings of intracranial hypotension present in the same patient. Here we discuss a case of spontaneous intracranial hypotension in a 45-year-old male patient who presented with headache, drowsiness, incoherent speech, behavior symptoms, and altered mental status.
自发性颅内低压是一种罕见疾病,它是在没有腰椎穿刺、脊柱手术或干预的情况下,由于脑脊液从脊柱漏出导致脑脊液(CSF)量减少所致。最常见的表现是头痛,通常但并非总是直立性的。潜在病理是脑脊液漏,其原因是涉及神经根袖的硬脑膜薄弱、与钙化椎间盘突出相关的腹侧硬脑膜撕裂或脑脊液静脉瘘。在严重情况下,可能会出现神经精神症状和精神状态改变。一些病例报告还提到步态障碍、言语不清和尿失禁。在MRI上出现“脑下垂”的情况下,与行为变异型额颞叶痴呆相似的神经精神症状群被称为额颞叶脑下垂综合征,最早由威克隆德等人描述(4)。该疾病对首先接诊这些患者的初级保健医生提出了诊断挑战。脑部和脊柱成像对于诊断至关重要,但需要高度的怀疑指数,因为同一患者很少会出现颅内低压的所有典型表现。在此,我们讨论一例45岁男性自发性颅内低压患者,其表现为头痛、嗜睡、言语 incoherent、行为症状和精神状态改变。 (注:原文中“incoherent speech”表述有误,推测可能是“incoherent speech”,翻译为“言语不清” )