Department of Neurology, Shaoxing Second Hospital, Shaoxing, Zhejiang, China.
Department of Emergency Internal Medicine, Shaoxing Second Hospital, Shaoxing, Zhejiang, China.
Medicine (Baltimore). 2024 Jul 26;103(30):e39090. doi: 10.1097/MD.0000000000039090.
Spontaneous intracranial hypotension (SIH) is a well-established condition typically presenting with disabling orthostatic headache. Corpus callosum agenesis (CCA) is one of the most common human brain malformations with a wide spectrum of associated malformations, chromosomal abnormalities, and clinical syndromes.
A 53-year-old woman presented with recurrent orthostatic headache for about 1 month. The head computed tomography examination of the patient showed CCA and the initial pressure of subsequent lumbar puncture was only 5 centimeters cerebrospinal fluid. Magnetic resonance imaging examination confirmed CCA with gray matter heterotopia.
The final diagnose was SIH related headache with CCA.
The patient's symptom improved after oral hydration, intravenous fluids, and bed rest.
Favorable outcome was observed.
Although this co-occurrence of SIH and CCA is probably coincidental, this finding suggests that the developmental malformations of the brain may lead to structural changes in brain tissue or disturbances in cerebrospinal fluid production and reflux, resulting in pathological changes over time.
自发性颅内低血压（SIH）是一种已确立的病症，通常表现为使人致残的直立性头痛。胼胝体发育不全（CCA）是最常见的人类大脑畸形之一，伴有广泛的相关畸形、染色体异常和临床综合征。
一名 53 岁女性因反复直立性头痛约 1 个月就诊。患者的头部计算机断层扫描（CT）显示 CCA，随后的腰椎穿刺初始压力仅为 5 厘米脑脊液。磁共振成像（MRI）检查证实了 CCA 合并灰质异位。
最终诊断为与 CCA 相关的 SIH 性头痛。
患者接受口服补液、静脉补液和卧床休息后症状改善。
观察到良好的结局。
尽管 SIH 和 CCA 的这种同时发生可能是偶然的，但这一发现表明，大脑的发育畸形可能导致脑组织的结构变化或脑脊液产生和回流的紊乱，随着时间的推移导致病理性变化。
