Department of Neurology, Delta General Hospital, Roeselare, Belgium.
Department of Neurosurgery, Delta General Hospital, Roeselare, Belgium.
BMC Neurol. 2024 Sep 4;24(1):315. doi: 10.1186/s12883-024-03803-z.
Acute Disseminated Encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system, characterize by multiple white matter hyperintensities on T2 MRI. Patients usually present with subacute progressive encephalopathy and polyfocal neurological deficits. Possible treatments are corticosteroids, immunoglobulins and plasma exchange. Full clinical recovery is seen in more than half of the cases.
We describe a case of a 62-year-old patient presenting with thunderclap headache as the first symptom, two weeks after an upper respiratory tract infection. The clinical course was complicated by progressive coma and intracranial hypertension mandating external ventricular drainage and sedation. Initial treatment with methylprednisolone was unsuccessful but clinical resolution and radiological regression was achieved after plasma exchanges and cyclophosphamide.
To our knowledge, this is the first reported case of ADEM presenting with thunderclap headache. Intracranial hypertension with the need for invasive neuromonitoring and pressure management is also a very rare complication of ADEM. In this report, we describe the findings of the literature review concerning ADEM, thunderclap headache and intracranial hypertension.
急性播散性脑脊髓炎（ADEM）是一种中枢神经系统的急性脱髓鞘疾病，其特征是 T2 MRI 上有多个白质高信号。患者通常表现为亚急性进行性脑病和多灶性神经功能缺损。可能的治疗方法包括皮质类固醇、免疫球蛋白和血浆置换。超过一半的病例可完全临床康复。
我们描述了一例 62 岁患者，在上呼吸道感染后两周出现雷击样头痛作为首发症状。临床病程复杂，进展为昏迷和颅内压增高，需要行脑室外引流和镇静治疗。最初使用甲泼尼龙治疗无效，但在接受血浆置换和环磷酰胺治疗后，临床症状缓解，影像学改善。
据我们所知，这是首例以雷击样头痛为首发症状的 ADEM 病例。伴有颅内压增高，需要进行有创性神经监测和压力管理，这也是 ADEM 非常罕见的并发症。在本报告中，我们描述了与 ADEM、雷击样头痛和颅内压增高相关的文献综述结果。
