Department of Neurology, Sam Houston State University College of Osteopathic Medicine, Huntsville, TX, USA.
Research Fellow, Houston Medical Clerkship, Sugar Land, TX, USA.
Am J Case Rep. 2022 Jan 10;23:e935019. doi: 10.12659/AJCR.935019.
BACKGROUND Acute brainstem syndrome (ABS), as the initial manifestation of progressive multifocal leukoencephalopathy (PML), is rarely reported. Appropriate history and neurodiagnostic testing are essential to encompass the extended spectrum of clinical and radiological differentials of ABS. CASE REPORT A 47-year-old woman presented to the emergency department with slurred speech, dizziness, right-sided facial droop, and numbness. Brain magnetic resonance imaging (MRI) revealed non-enhancing hyperintensities in the right lateral pons and brachium pontis, eventually extending to the bilateral middle cerebellar peduncle, pons, left>right cerebellar hemisphere, right thalamocapsular region, and midbrain region. Lumbar puncture revealed 3 cerebrospinal fluid-specific oligoclonal bands. Initial diagnosis of multiple sclerosis led to high-dose intravenous steroid treatment. The patient continued to deteriorate, leading to multiple emergency department visits and hospital admissions. Additional history revealing previously diagnosed, treatment-naive HIV prompted a repeat lumbar puncture. Cerebrospinal fluid polymerase chain reaction (PCR) for JC polyomavirus (JCV) was positive, leading to the diagnosis of clinically isolated brainstem PML. Unfortunately, the patient developed pneumonia and hypoxic respiratory failure, which ultimately led to her death. CONCLUSIONS This case highlights the need for considering isolated brainstem PML, as a diagnostic possibility, in patients presenting with acute-subacute brainstem symptoms and compatible neuroimaging findings. Clinicians need to be aware of varying PML presentations with brainstem or diencephalic variants, as well as monofocal lesions. The prognosis for PML has improved somewhat, secondary to immune reconstitution by highly active antiretroviral therapy, risk stratification in drug-induced PML, and other emerging treatments, such as pembrolizumab.
急性脑干综合征（ABS）是进行性多灶性白质脑病（PML）的初始表现，很少有报道。适当的病史和神经诊断测试对于涵盖 ABS 的广泛临床和影像学差异至关重要。
一名 47 岁女性因言语不清、头晕、右侧面瘫和麻木到急诊科就诊。脑部磁共振成像（MRI）显示右侧桥脑和脑桥臂有非增强性高信号，最终延伸至双侧小脑中脑脚、桥脑、左侧>右侧小脑半球、右侧丘脑壳核区和中脑区。腰椎穿刺显示 3 种脑脊液特异性寡克隆带。最初诊断为多发性硬化症，导致高剂量静脉类固醇治疗。患者病情持续恶化，导致多次急诊就诊和住院。进一步询问病史发现患者此前被诊断为未经治疗的 HIV，促使再次进行腰椎穿刺。脑脊液聚合酶链反应（PCR）检测 JC 多瘤病毒（JCV）阳性，诊断为临床孤立性脑干 PML。不幸的是，患者发生肺炎和低氧性呼吸衰竭，最终导致死亡。
本病例强调了在出现急性-亚急性脑干症状和影像学相符的患者中，考虑孤立性脑干 PML 作为一种诊断可能性的必要性。临床医生需要意识到 PML 具有多种表现形式，包括脑干或间脑变异型，以及单灶性病变。由于高效抗逆转录病毒疗法的免疫重建、药物诱导性 PML 的风险分层以及其他新兴治疗方法（如 pembrolizumab），PML 的预后有所改善。
