PDF(Pubmed)
Abstract：
UNASSIGNED: Posterior Reversible Encephalopathy Syndrome, typically characterized by parieto-occipital vasogenic edema, can present atypically, as a bilateral symmetrical vasogenic edema in the basal ganglia, featuring the called \"lentiform fork sign.\" Prompt recognition of such variations is crucial for accurate diagnosis and tailored management, highlighting the complexity of this syndrome\'s manifestations.
UNASSIGNED: Posterior Reversible Encephalopathy Syndrome (PRES) manifests as transient neurological symptoms and cerebral edema, commonly associated with immunosuppressive drugs (ISDs) in transplant recipients. ISDs can lead to endothelial dysfunction and compromise the blood-brain barrier. Typically, PRES exhibits identifiable MRI patterns, often demonstrating vasogenic edema in the bilateral parieto-occipital white matter. Identifying unique presentations, such as the recently observed \"lentiform fork sign,\" commonly seen in uremic encephalopathy, emphasizes this syndrome\'s broad spectrum manifestations. A 19-year-old male, who underwent bilateral lung and liver transplantation, experienced a bilateral tonic-clonic seizure of unknown onset 47 days post-surgery. MRI findings revealed an unconventional PRES pattern, featuring the \"lentiform fork sign\" as bilateral symmetrical vasogenic edema in the basal ganglia, surrounded by a hyperintense rim outlining the lentiform nucleus bilaterally. Subsequent management, including ISD modification and magnesium supplementation, resulted in clinical and neuroimaging resolution. An almost complete clinical and radiological resolution was achieved after 14 days. The occurrence of PRES in transplant recipients highlights the intricate interplay among ISDs, physiological factors, and cerebrovascular dynamics, potentially involving direct neurovascular endothelial toxicity and disruption of the blood-brain barrier. Neuroimaging plays a pivotal role in diagnosis. The distinctive \"lentiform fork sign\" was observed in this patient despite the absence of typical metabolic disturbances. Management strategies usually involve reducing hypertension, discontinuing ISDs, correcting electrolyte imbalances, and initiating antiseizure drugs if necessary. Identifying the presence of the \"lentiform fork sign\" alongside typical PRES edema in a patient lacking renal failure emphasizes that this manifestation is not solely indicative of uremic encephalopathy. Instead, it might represent the final common pathway resulting from alterations in the blood-brain barrier integrity within the deep white matter. Understanding such atypical imaging manifestations could significantly aid earlier and more precise diagnosis, influencing appropriate management decisions.
UNASSIGNED: Posterior Reversible Encephalopathy Syndrome (PRES) manifests as transient neurological symptoms and cerebral edema, commonly associated with immunosuppressive drugs (ISDs) in transplant recipients. ISDs can lead to endothelial dysfunction and compromise the blood-brain barrier. Typically, PRES exhibits identifiable MRI patterns, often demonstrating vasogenic edema in the bilateral parieto-occipital white matter. Identifying unique presentations, such as the recently observed \"lentiform fork sign,\" commonly seen in uremic encephalopathy, emphasizes this syndrome\'s broad spectrum manifestations. A 19-year-old male, who underwent bilateral lung and liver transplantation, experienced a bilateral tonic-clonic seizure of unknown onset 47 days post-surgery. MRI findings revealed an unconventional PRES pattern, featuring the \"lentiform fork sign\" as bilateral symmetrical vasogenic edema in the basal ganglia, surrounded by a hyperintense rim outlining the lentiform nucleus bilaterally. Subsequent management, including ISD modification and magnesium supplementation, resulted in clinical and neuroimaging resolution. An almost complete clinical and radiological resolution was achieved after 14 days. The occurrence of PRES in transplant recipients highlights the intricate interplay among ISDs, physiological factors, and cerebrovascular dynamics, potentially involving direct neurovascular endothelial toxicity and disruption of the blood-brain barrier. Neuroimaging plays a pivotal role in diagnosis. The distinctive \"lentiform fork sign\" was observed in this patient despite the absence of typical metabolic disturbances. Management strategies usually involve reducing hypertension, discontinuing ISDs, correcting electrolyte imbalances, and initiating antiseizure drugs if necessary. Identifying the presence of the \"lentiform fork sign\" alongside typical PRES edema in a patient lacking renal failure emphasizes that this manifestation is not solely indicative of uremic encephalopathy. Instead, it might represent the final common pathway resulting from alterations in the blood-brain barrier integrity within the deep white matter. Understanding such atypical imaging manifestations could significantly aid earlier and more precise diagnosis, influencing appropriate management decisions.
摘要：
■后部可逆性脑病综合征，典型特征为顶枕血管源性水肿，可以非典型地呈现，作为基底神经节的双侧对称血管源性水肿，带有所谓的“象形叉”标志。“迅速识别这种变化对于准确诊断和量身定制的管理至关重要，突出了这种综合征表现的复杂性。
■后部可逆性脑病综合征（PRES）表现为短暂的神经症状和脑水肿，移植受者通常与免疫抑制药物（ISD）相关。ISD可导致内皮功能障碍并破坏血脑屏障。通常,PRES显示可识别的MRI模式，常表现为双侧顶枕白质血管源性水肿。识别独特的演示文稿，例如最近观察到的“象形叉”标志，“常见于尿毒症性脑病，强调这种综合征的广谱表现。一个19岁的男性,进行了双侧肺和肝移植，在手术后47天经历了未知发作的双侧强直阵挛性癫痫发作。MRI发现揭示了非常规的PRES模式，表现为基底神经节的双侧对称血管源性水肿，被两侧概述小豆状核的高强度边缘包围。后续管理，包括ISD修饰和镁补充，导致临床和神经影像学分辨率。14天后达到了几乎完整的临床和放射学分辨率。移植受者中PRES的发生凸显了ISD之间复杂的相互作用，生理因素,和脑血管动力学，可能涉及直接的神经血管内皮毒性和血脑屏障的破坏。神经影像学在诊断中起着关键作用。尽管没有典型的代谢紊乱，但在该患者中观察到了独特的“象牙叉征”。管理策略通常涉及降低高血压，停止ISD，纠正电解质不平衡，并在必要时开始服用抗癫痫药物。在缺乏肾功能衰竭的患者中，确定“象形叉征”和典型的PRES水肿的存在强调，这种表现不仅表明尿毒症脑病。相反，它可能是由深部白质内血脑屏障完整性改变导致的最终共同通路.了解这种非典型的影像学表现可以显着帮助早期和更精确的诊断。影响适当的管理决策。
■后部可逆性脑病综合征（PRES）表现为短暂的神经症状和脑水肿，移植受者通常与免疫抑制药物（ISD）相关。ISD可导致内皮功能障碍并破坏血脑屏障。通常,PRES显示可识别的MRI模式，常表现为双侧顶枕白质血管源性水肿。识别独特的演示文稿，例如最近观察到的“象形叉”标志，“常见于尿毒症性脑病，强调这种综合征的广谱表现。一个19岁的男性,进行了双侧肺和肝移植，在手术后47天经历了未知发作的双侧强直阵挛性癫痫发作。MRI发现揭示了非常规的PRES模式，表现为基底神经节的双侧对称血管源性水肿，被两侧概述小豆状核的高强度边缘包围。后续管理，包括ISD修饰和镁补充，导致临床和神经影像学分辨率。14天后达到了几乎完整的临床和放射学分辨率。移植受者中PRES的发生凸显了ISD之间复杂的相互作用，生理因素,和脑血管动力学，可能涉及直接的神经血管内皮毒性和血脑屏障的破坏。神经影像学在诊断中起着关键作用。尽管没有典型的代谢紊乱，但在该患者中观察到了独特的“象牙叉征”。管理策略通常涉及降低高血压，停止ISD，纠正电解质不平衡，并在必要时开始服用抗癫痫药物。在缺乏肾功能衰竭的患者中，确定“象形叉征”和典型的PRES水肿的存在强调，这种表现不仅表明尿毒症脑病。相反，它可能是由深部白质内血脑屏障完整性改变导致的最终共同通路.了解这种非典型的影像学表现可以显着帮助早期和更精确的诊断。影响适当的管理决策。
