Togha Mansoureh, Mokary Yousef, Jafari Elham
Neurology Department, Sina Hospital, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
Headache Department, Iranian Center of Neurological Research, Neuroscience Institute, Tehran University of Medical Sciences, Tehran, Iran.
J Med Case Rep. 2025 Jan 4;19(1):2. doi: 10.1186/s13256-024-04997-z.
Idiopathic intracranial hypertension (IIH) is a condition where the pressure of the cerebrospinal fluid in the brain increases without a known cause. It typically affects adults but can also occur in adolescents and children, although it is less common. Numerous elements, including coagulopathy, have been documented in previous cases as potential etiological factors of IIH. Nonetheless, our objective was to present the insufficiency of a coagulation factor as an additional contributing factor to IIH, a notion that has not been previously reported.
In this case, a 34-year-old West Asian female patient presented with a subacute generalized headache, bilateral blurred vision, and papilledema. The patient's brain magnetic resonance imaging showed flattening of the posterior globe and empty sella, but no other abnormalities were detected. The results of magnetic resonance venography and cerebrospinal fluid analysis were also normal, except for an opening cerebrospinal fluid pressure of 600 mm HO during the lumbar puncture. Rheumatologic and endocrine disorders were ruled out on the basis of clinical assessment and laboratory tests. The patient was started on acetazolamide (1 g/day, increased to 2 g/day) and furosemide (20 mg/twice a day) and was encouraged to lose weight. These treatments led to some improvement for about 1 year, but her symptoms then worsened without an obvious cause. Given the prolonged duration of the disease and the lack of expected response to treatment, the patient was reevaluated for endocrinopathy and collagen vascular disease, which were negative. An additional workup revealed an antithrombin III (AT III) deficiency, for which the patient was prescribed acetylsalicylic acid (80 mg/day) in addition to the previous medications. As a result, the patient's papilledema, macular thickness, and nerve fiber layer edema decreased, as observed by fundoscopy and optical coherence tomography. Clinical examination and imaging also showed improvement in the patient's symptoms.
This case highlights the importance of considering coagulopathy in cases of IIH and suggests that antiplatelet therapy with acetylsalicylic acid may be beneficial for such patients.
特发性颅内高压（IIH）是一种脑部脑脊液压力升高但原因不明的病症。它通常影响成年人，但也可发生于青少年和儿童，不过较为少见。在先前病例中，包括凝血病在内的众多因素已被记录为IIH的潜在病因。尽管如此，我们的目的是提出凝血因子不足作为IIH的一个额外促成因素，这一观点此前尚未见报道。
在本病例中，一名34岁的西亚女性患者出现亚急性全身性头痛、双侧视力模糊和视乳头水肿。患者的脑部磁共振成像显示眼球后部扁平及空蝶鞍，但未检测到其他异常。磁共振静脉造影和脑脊液分析结果也正常，只是腰椎穿刺时脑脊液初压为600mmH₂O。根据临床评估和实验室检查排除了风湿性和内分泌疾病。患者开始服用乙酰唑胺（1g/天，增至2g/天）和呋塞米（20mg/每日两次），并鼓励其减重。这些治疗在约1年内带来了一些改善，但随后她的症状无明显原因地恶化。鉴于病程较长且对治疗缺乏预期反应，对患者重新评估内分泌病和胶原血管病，结果均为阴性。进一步检查发现抗凝血酶III（AT III）缺乏，除先前药物外，为该患者加用了阿司匹林（80mg/天）。结果，通过眼底镜检查和光学相干断层扫描观察到，患者的视乳头水肿、黄斑厚度和神经纤维层水肿减轻。临床检查和影像学检查也显示患者症状有所改善。
本病例强调了在IIH病例中考虑凝血病的重要性，并表明阿司匹林抗血小板治疗可能对此类患者有益。
