未经评估:磁共振成像(MRI)上的“热交叉发囊标志”(HCBs)最初被认为是具有小脑特征的多系统萎缩的特异性。然而,此后描述了许多其他情况,这可能与这个成像标志有关。我们在此描述了抗Ri和副肿瘤小脑共济失调的患者,并回顾了HCBs在影像学上与各种神经系统自身免疫性疾病的关联。
未经证实:我们报告了一名40岁女性,患有抗Ri相关副肿瘤神经综合征和乳腺癌,脑部MRI显示HCBs在病程后期。我们还回顾了文献中报道的类似病例。
未经证实:患者出现小脑共济失调,多发性神经病,和锥体的迹象。尽管脑部MRI最初并不明显,在随后的随访中观察到双侧小脑中段的HCBs和T2加权高强度.在血清和脑脊液中检测到抗Ri。通过腋窝淋巴结活检证实了乳腺癌。第一次皮质类固醇脉冲后,她的症状部分缓解。然而,随后的免疫疗法和肿瘤治疗无效.文献中发现了4例HCBs的自身免疫性小脑共济失调(2例副肿瘤和2例非副肿瘤)。
未经批准:HCBs可能与副肿瘤和非副肿瘤小脑共济失调有关,这可能反映了继发于自身免疫损伤的神经变性。因此,HCBs不应被视为自身免疫性小脑综合征的禁忌症.
UNASSIGNED: The \"hot cross bun sign\" (HCBs) on magnetic resonance imaging (MRI) has been initially considered specific for multiple system atrophy with cerebellar features. However, a number of other conditions have since been described, which may be associated with this imaging sign. We herein describe a patient with anti-Ri and paraneoplastic cerebellar ataxia, and review the association of the HCBs on imaging with various neurological autoimmune conditions.
UNASSIGNED: We report a 40-year-old woman with anti-Ri-associated paraneoplastic neurological syndrome and breast carcinoma, in whom brain MRI revealed the HCBs late in the disease course. We also reviewed similar cases reported in the literature.
UNASSIGNED: The patient presented with cerebellar ataxia, polyneuropathy, and pyramidal signs. Although brain MRI was initially unremarkable, the HCBs and T2-weighted hyperintensity of the bilateral middle cerebellar peduncles were observed at later follow-up. Anti-Ri was detected in the serum and cerebrospinal fluid. Breast adenocarcinoma was confirmed via an axillary lymph node biopsy. Her symptoms partially resolved after the first corticosteroid pulse. However, subsequent immunotherapy and tumor treatments were ineffective. Four autoimmune cerebellar ataxia cases with the HCBs (two paraneoplastic and two non-paraneoplastic) were identified in the literature.
UNASSIGNED: The HCBs can be associated with paraneoplastic and non-paraneoplastic cerebellar ataxia, which may reflect neurodegeneration secondary to autoimmune injury. Thus, the HCBs should not be considered a contraindication for autoimmune cerebellar syndrome.