PDF(Pubmed)
Abstract：
UNASSIGNED: Acquired hepatocerebral degeneration (AHD) is a neurological condition associated with cerebral manganese (Mn) accumulation caused by portosystemic shunts (PSS), usually because of advanced liver disease. AHD is diagnosed by the identification of T1-weighted brain magnetic resonance imaging (MRI) hyperintensities coupled with the presence of PSS and neurological symptoms. Clinical presentations primarily involve motor dysfunction and cognitive impairment. As a result of the frequently concurrent hepatic encephalopathy, the psychiatric symptoms of AHD alone remain unclear. This report is the first documentation of unique psychiatric symptoms of AHD due to a congenital PSS (CPSS) and suggests the efficacy of shunt embolization in achieving sustained remission of psychiatric symptoms in such cases.
UNASSIGNED: A 57-year-old Japanese woman presented with recurrent severe depression, pain, and somatosensory hallucinations, along with fluctuating motor dysfunction, including parkinsonism, and cognitive impairments. Psychiatric interventions, including antidepressants, antipsychotics or electroconvulsive therapy, had limited efficacy or did not prevent relapse.
UNASSIGNED: T1-weighted MRI showed bilateral hyperintensity in the globus pallidus. No history of Mn exposure or metabolic abnormalities, including copper, was identified. Furthermore, no evidence of liver dysfunction or hyperammonemia was found. Eventually, a gastrorenal shunt was observed on contrast-enhanced abdominal computed tomography. The diagnosis of AHD due to CPSS was made based on the clinical manifestations and abnormal imaging findings. Shunt embolization was performed, which prevented the relapse of psychiatric symptoms and substantially reduced the T1-weighted MRI hyperintensities.
UNASSIGNED: This case highlights the potential involvement of AHD in adult-onset psychiatric symptoms, even in the absence of liver disease. Furthermore, this case underscores the efficacy of shunt embolization in treating the psychiatric symptoms of AHD due to CPSS.
UNASSIGNED: A 57-year-old Japanese woman presented with recurrent severe depression, pain, and somatosensory hallucinations, along with fluctuating motor dysfunction, including parkinsonism, and cognitive impairments. Psychiatric interventions, including antidepressants, antipsychotics or electroconvulsive therapy, had limited efficacy or did not prevent relapse.
UNASSIGNED: T1-weighted MRI showed bilateral hyperintensity in the globus pallidus. No history of Mn exposure or metabolic abnormalities, including copper, was identified. Furthermore, no evidence of liver dysfunction or hyperammonemia was found. Eventually, a gastrorenal shunt was observed on contrast-enhanced abdominal computed tomography. The diagnosis of AHD due to CPSS was made based on the clinical manifestations and abnormal imaging findings. Shunt embolization was performed, which prevented the relapse of psychiatric symptoms and substantially reduced the T1-weighted MRI hyperintensities.
UNASSIGNED: This case highlights the potential involvement of AHD in adult-onset psychiatric symptoms, even in the absence of liver disease. Furthermore, this case underscores the efficacy of shunt embolization in treating the psychiatric symptoms of AHD due to CPSS.
摘要：
■获得性肝脑变性（AHD）是与门体分流（PSS）引起的脑锰（Mn）积累有关的神经系统疾病，通常是因为晚期肝病。通过识别T1加权脑磁共振成像（MRI）高强度以及PSS和神经系统症状的存在来诊断AHD。临床表现主要涉及运动功能障碍和认知障碍。由于经常并发肝性脑病，仅AHD的精神症状尚不清楚.该报告是有关先天性PSS（CPSS）引起的AHD独特精神症状的第一份文献，并表明在这种情况下，分流栓塞在实现精神症状持续缓解方面的功效。
■一名57岁的日本女性出现反复发作的重度抑郁症，疼痛,和体感幻觉，伴随着波动性的运动功能障碍，包括帕金森病,和认知障碍。精神病学干预，包括抗抑郁药,抗精神病药或电惊厥疗法，疗效有限或不能预防复发。
■T1加权MRI显示苍白球双侧高强度。无锰暴露史或代谢异常，包括铜，已确定。此外，未发现肝功能障碍或高氨血症的证据.最终,在对比增强的腹部计算机断层扫描中观察到胃肾分流。根据临床表现和异常影像学表现诊断为CPSS所致的AHD。进行分流栓塞,这防止了精神症状的复发，并大大降低了T1加权MRI高强度。
该病例突出了AHD在成人发病的精神症状中的潜在参与，即使没有肝病。此外，该病例强调了分流栓塞治疗CPSS引起的AHD精神症状的疗效。
■一名57岁的日本女性出现反复发作的重度抑郁症，疼痛,和体感幻觉，伴随着波动性的运动功能障碍，包括帕金森病,和认知障碍。精神病学干预，包括抗抑郁药,抗精神病药或电惊厥疗法，疗效有限或不能预防复发。
■T1加权MRI显示苍白球双侧高强度。无锰暴露史或代谢异常，包括铜，已确定。此外，未发现肝功能障碍或高氨血症的证据.最终,在对比增强的腹部计算机断层扫描中观察到胃肾分流。根据临床表现和异常影像学表现诊断为CPSS所致的AHD。进行分流栓塞,这防止了精神症状的复发，并大大降低了T1加权MRI高强度。
该病例突出了AHD在成人发病的精神症状中的潜在参与，即使没有肝病。此外，该病例强调了分流栓塞治疗CPSS引起的AHD精神症状的疗效。
