Department of Neurology, BLK Super Speciality Hospital, New Delhi, India.
Neurol Sci. 2021 Jun;42(6):2523-2525. doi: 10.1007/s10072-021-05046-z. Epub 2021 Feb 8.
Paraneoplastic Cerebellar degeneration (PCD) is one of the classical paraneoplastic syndromes (PNS) which is characterised by subacute onset, progressive cerebellar ataxia and is usually associated with small cell lung carcinoma, adeno carcinoma of breast and ovary followed by Hodgkin's lymphoma.
We herein report a case of subacute onset, progressive cerebellar ataxia in a 37-year-old female, who on evaluation was found to have non-Hodgkin's lymphoma and experienced good clinical response to treatment.
As compared to solid tumours, chances of association of PNS with Lymphomas is quite low and there are only few case reports in the literature showing association of PCD with non-Hodgkin's lymphoma. As PCD is one of the classical PNS, it is very important to identify subtle cerebellar manifestations in an otherwise apparently normal individual, as early diagnosis and aggressive treatment can immensely improve the mortality and morbidity associated with this syndrome.
This case signifies the importance of suspecting PNS as an important differential diagnosis in a young patient presenting with subacute onset progressive cerebellar ataxia and evaluating her extensively for malignancy in spite of no paraneoplastic antibody been detected as early diagnosis and treatment can lead to gratifying response. We do agree that 2 weeks follow up is a short time interval to determine whether the response was sustained or not, for which a long term follow up is required.
副肿瘤性小脑变性(PCD)是经典的副肿瘤综合征(PNS)之一,其特征为亚急性起病、进行性小脑共济失调,通常与小细胞肺癌、乳腺和卵巢腺癌以及霍奇金淋巴瘤相关。
我们在此报告一例 37 岁女性亚急性起病、进行性小脑共济失调,评估后发现为非霍奇金淋巴瘤,治疗后临床反应良好。
与实体瘤相比,PNS 与淋巴瘤的关联机会较低,文献中仅有少数病例报告显示 PCD 与非霍奇金淋巴瘤相关。由于 PCD 是经典的 PNS 之一,因此非常重要的是在其他方面表现正常的个体中识别出微妙的小脑表现,因为早期诊断和积极治疗可以极大地改善与该综合征相关的死亡率和发病率。
本例表明,在年轻患者出现亚急性起病、进行性小脑共济失调时,应将 PNS 作为重要的鉴别诊断进行怀疑,并对其进行广泛的恶性肿瘤评估,尽管未检测到副肿瘤抗体,因为早期诊断和治疗可以带来满意的反应。我们同意,2 周的随访时间间隔较短,无法确定反应是否持续,因此需要进行长期随访。