PDF(Pubmed)
Abstract：
BACKGROUND: John Cunningham virus related granule cell neuronopathy (JCV-GCN) is a rare manifestation of the reactivation of infection of the cerebellar granule cells by the JCV, mostly in immunocompromised individuals. The \"hot cross bun\" (HCB) sign is a cruciform hyperintensity seen in the midpons on T2-weighted and fluid attenuated inversion recovery (FLAIR) sequences on magnetic resonance imaging (MRI) of the brain. An index sub-Saharan Africa report of a case of JCV-GCN with HCB sign follows.
METHODS: A 27-year-old HIV positive female with JCV-GCN was re-evaluated for chronic ataxia complicated by subacute progressive horizontal diplopia. Cerebrospinal fluid (CSF) had trace Mycobacterium tuberculosis (MTB) detected by GeneXpert Mycobacterium Tuberculosis/Rifampicin resistance (MTB/RIF) assay test. Brain MRI revealed diffuse severe cerebellar atrophy with a hot cross bun sign and patchy enhancement contiguous to the cerebellar dentate nuclei bilaterally. She continued Highly Active Antiretroviral Therapy (HAART) pending CSF HIV viral load counts and started standard brain TB local treatment regimen protocols with progressive improvement in limb ataxia.
CONCLUSIONS: In conclusion, finding of the HCB sign may be indicative of and aid diagnosis of JCV-GCN in the right clinical context. This could be an important neuroimaging marker in this context, that may radiologically be more evident in later stages of the condition.
METHODS: A 27-year-old HIV positive female with JCV-GCN was re-evaluated for chronic ataxia complicated by subacute progressive horizontal diplopia. Cerebrospinal fluid (CSF) had trace Mycobacterium tuberculosis (MTB) detected by GeneXpert Mycobacterium Tuberculosis/Rifampicin resistance (MTB/RIF) assay test. Brain MRI revealed diffuse severe cerebellar atrophy with a hot cross bun sign and patchy enhancement contiguous to the cerebellar dentate nuclei bilaterally. She continued Highly Active Antiretroviral Therapy (HAART) pending CSF HIV viral load counts and started standard brain TB local treatment regimen protocols with progressive improvement in limb ataxia.
CONCLUSIONS: In conclusion, finding of the HCB sign may be indicative of and aid diagnosis of JCV-GCN in the right clinical context. This could be an important neuroimaging marker in this context, that may radiologically be more evident in later stages of the condition.
摘要：
背景：约翰·坎宁安病毒相关的颗粒细胞神经病（JCV-GCN）是JCV感染小脑颗粒细胞的再激活的一种罕见表现，主要是免疫功能低下的个体。“热十字头”（HCB）标志是在脑磁共振成像（MRI）的T2加权和液体衰减倒置恢复（FLAIR）序列上的中桥中看到的十字形高强度。以下是撒哈拉以南非洲关于带有HCB标志的JCV-GCN病例的索引报告。
方法：重新评估了一名27岁的HIV阳性女性JCV-GCN患者的慢性共济失调并发亚急性进行性水平复视。脑脊液（CSF）通过GeneXpert结核分枝杆菌/利福平耐药（MTB/RIF）试验检测到痕量结核分枝杆菌（MTB）。脑MRI显示弥漫性严重的小脑萎缩，伴有热交叉bun征，并与小脑齿状核两侧相邻。她继续进行高活性抗逆转录病毒治疗（HAART），等待CSFHIV病毒载量计数，并开始标准的脑结核局部治疗方案，逐渐改善肢体共济失调。
结论：结论：在正确的临床背景下，发现HCB体征可能是JCV-GCN的指示和辅助诊断。在这种情况下，这可能是一个重要的神经成像标记，这在放射学上可能在病情的后期阶段更加明显。
方法：重新评估了一名27岁的HIV阳性女性JCV-GCN患者的慢性共济失调并发亚急性进行性水平复视。脑脊液（CSF）通过GeneXpert结核分枝杆菌/利福平耐药（MTB/RIF）试验检测到痕量结核分枝杆菌（MTB）。脑MRI显示弥漫性严重的小脑萎缩，伴有热交叉bun征，并与小脑齿状核两侧相邻。她继续进行高活性抗逆转录病毒治疗（HAART），等待CSFHIV病毒载量计数，并开始标准的脑结核局部治疗方案，逐渐改善肢体共济失调。
结论：结论：在正确的临床背景下，发现HCB体征可能是JCV-GCN的指示和辅助诊断。在这种情况下，这可能是一个重要的神经成像标记，这在放射学上可能在病情的后期阶段更加明显。
