Department of Neuroradiology, Medical Center, Faculty of Medicine, University Medical Center Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany.
Department of Neurosurgery, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Neuroradiology. 2024 Oct;66(10):1671-1679. doi: 10.1007/s00234-024-03425-9. Epub 2024 Jul 10.
BACKGROUND & PURPOSE: Around 5% of dementia patients have a treatable cause. To estimate the prevalence of two rare diseases, in which the treatable cause is at the spinal level.
A radiology information system was searched using the terms CT myelography and the operation and classification system (OPS) code 3-241. The clinical charts of these patients were reviewed to identify patients with a significant cognitive decline.
Among 205 patients with spontaneous intracranial hypotension (SIH) and proven CSF leaks we identified five patients with a so-called frontotemporal brain sagging syndrome: Four of those had CSF venous fistulas and significantly improved by occluding them either by surgery or transvenous embolization. Another 11 patients had infratentorial hemosiderosis and hearing problems and ataxia as guiding symptoms. Some cognitive decline was present in at least two of them. Ten patients had ventral dural tears in the thoracic spine and one patient a lateral dural tear at C2/3 respectively. Eight patients showed some improvement after surgery.
It is mandatory to study the (thoracic) spine in cognitively impaired patients with brain sagging and/ or infratentorial hemosiderosis on MRI. We propose the term spinal dementia to draw attention to this region, which in turn is evaluated with dynamic digital subtraction and CT myelography.
约 5%的痴呆患者存在可治疗的病因。为了评估两种罕见疾病的患病率,这些疾病的可治疗病因位于脊柱水平。
使用 CT 脊髓造影和操作和分类系统(OPS)代码 3-241 搜索放射学信息系统。回顾这些患者的临床图表,以识别有明显认知能力下降的患者。
在 205 例自发性颅内低血压(SIH)和已证实的 CSF 漏患者中,我们发现了 5 例所谓的额颞脑下垂综合征患者:其中 4 例存在 CSF 静脉瘘,通过手术或经静脉栓塞闭塞这些瘘管后明显改善。另外 11 例患者出现小脑幕下含铁血黄素沉着症和听力问题以及共济失调等症状。至少有两人存在认知能力下降。10 例患者的胸椎有腹侧硬脑膜撕裂,1 例患者 C2/3 有外侧硬脑膜撕裂。8 例患者手术后有一定程度的改善。
对于 MRI 显示有脑下垂和/或小脑幕下含铁血黄素沉着症的认知障碍患者,必须研究(胸椎)脊柱。我们提出“脊柱性痴呆”一词,以引起对该区域的关注,该区域通过动态数字减影和 CT 脊髓造影进行评估。