Capizzano A A, Lai L, Kim J, Rizzo M, Gray L, Smoot M K, Moritani T
From the Department of Radiology (A.A.C., T.M.), University of Iowa Carver College of Medicine, Iowa City, Iowa
Department of Radiology (L.L.), Stanford University School of Medicine, Stanford, California.
AJNR Am J Neuroradiol. 2016 Jul;37(7):1256-61. doi: 10.3174/ajnr.A4706. Epub 2016 Mar 3.
Atypical clinical presentations of spontaneous intracranial hypotension include obtundation, memory deficits, dementia with frontotemporal features, parkinsonism, and ataxia. The purpose of this study was to compare clinical and imaging features of spontaneous intracranial hypotension with typical-versus-atypical presentations.
Clinical records and neuroimaging of patients with spontaneous intracranial hypotension from September 2005 to August 2014 were retrospectively evaluated. Patients with classic spontaneous intracranial hypotension (n = 33; mean age, 41.7 ± 14.3 years) were compared with those with intracranial hypotension with atypical clinical presentation (n = 8; mean age, 55.9 ± 14.1 years) and 36 controls (mean age, 41.4 ± 11.2 years).
Patients with atypical spontaneous intracranial hypotension were older than those with classic spontaneous intracranial hypotension (55.9 ± 14.1 years versus 41.7 ± 14.3 years; P = .018). Symptom duration was shorter in classic compared with atypical spontaneous intracranial hypotension (3.78 ± 7.18 months versus 21.93 ± 18.43 months; P = .015). There was no significant difference in dural enhancement, subdural hematomas, or cerebellar tonsil herniation. Patients with atypical spontaneous intracranial hypotension had significantly more elongated anteroposterior midbrain diameter compared with those with classic spontaneous intracranial hypotension (33.6 ± 2.9 mm versus 27.3 ± 2.9 mm; P < .001) and shortened pontomammillary distance (2.8 ± 1 mm versus 5.15 ± 1.5 mm; P < .001). Patients with atypical spontaneous intracranial hypotension were less likely to become symptom-free, regardless of treatment, compared with those with classic spontaneous intracranial hypotension (χ(2) = 13.99, P < .001).
In this sample of 8 patients, atypical spontaneous intracranial hypotension was a more chronic syndrome compared with classic spontaneous intracranial hypotension, with more severe brain sagging, lower rates of clinical response, and frequent relapses. Awareness of atypical presentations of spontaneous intracranial hypotension is paramount.
自发性颅内低压的非典型临床表现包括意识模糊、记忆缺陷、具有额颞叶特征的痴呆、帕金森综合征及共济失调。本研究旨在比较具有典型与非典型表现的自发性颅内低压的临床和影像学特征。
回顾性评估2005年9月至2014年8月期间自发性颅内低压患者的临床记录和神经影像学资料。将典型自发性颅内低压患者（n = 33；平均年龄，41.7 ± 14.3岁）与具有非典型临床表现的颅内低压患者（n = 8；平均年龄，55.9 ± 14.1岁）及36名对照者（平均年龄，41.4 ± 11.2岁）进行比较。
非典型自发性颅内低压患者比典型自发性颅内低压患者年龄更大（55.9 ± 14.1岁对41.7 ± 14.3岁；P = 0.018）。与非典型自发性颅内低压相比，典型自发性颅内低压的症状持续时间更短（3.78 ± 7.18个月对2 / 1.93 ± 18.43个月；P = 0.015）。硬膜强化、硬膜下血肿或小脑扁桃体疝方面无显著差异。与典型自发性颅内低压患者相比，非典型自发性颅内低压患者的中脑前后径明显更长（33.6 ± 2.9 mm对27.3 ± 2.9 mm；P < 0.001），脑桥乳头体距离缩短（2.8 ± 1 mm对5.15 ± 1.5 mm；P < 0.001）。与典型自发性颅内低压患者相比，无论治疗与否，非典型自发性颅内低压患者症状缓解的可能性更小（χ(2) = 13.99，P < 0.001）。
在这个8例患者的样本中，与典型自发性颅内低压相比，非典型自发性颅内低压是一种更慢性的综合征，脑下垂更严重，临床缓解率更低且频繁复发。认识自发性颅内低压的非典型表现至关重要。
