Objective: To describe a case of paraneoplastic cerebellar degeneration in a patient with renal cell carcinoma.

Background: Paraneoplastic cerebellar degeneration belongs to a group of neurologic syndromes in which symptoms are indirectly caused by an underlying malignancy. Cerebellar signs and symptoms may be unrelated to a metastatic disorder; usually, the mechanism of injury is autoimmune. The neurologic symptoms may precede the diagnosis of cancer by several years.

Method: Case report of a 75-year-old female who eveloped progressive cerebellar ataxia a week after a viral respiratory infection. She reported dysarthria and gait unsteadiness. Her symptoms worsened for about 2 weeks and then remained stable. She was admitted to our service one year after symptoms onset, and she had been previously diagnosed with parainfectious cerebellar ataxia. She reported a 6-month history of daily nausea and vomiting, weight loss of 66 pounds, and distal four limbs paresthesias.

Results: Brain MRI (Figure 1A) showed cerebellar atrophy. Abdominal MRI disclosed an exophytic heterogeneous renal mass. Radiology determined that it was category III in the Bosniak Classification for complex renal cysts [4], suggesting a high risk of malignancy. Histopathology revealed a clear cell renal carcinoma. There was complete resolution of gastrointestinal symptoms after surgery, and partial dysarthria improvement. Ataxia, however, remained stable.

Conclusion: This case highlights the need to consider paraneoplastic cerebellar degeneration in patients presenting with gradually progressive cerebellar ataxia, not explained by other causes. The failure to reach the correct diagnosis may allow patients to reach more advanced cancer stage.

References: [1] Lancaster E. Paraneoplastic Disorders. Continuum 2017; 23 (6, Neuro-Oncology), 1653 1679. [2] Hens MJ, Camacho B, Maestre A, González V, Villegas I. Carcinoma renal que debuta como cuadro neurológico paraneoplásico. Actas Urológicas Españolas 2008; 32 (6), 645-648. [3] Giri P, Shukla R. Paraneoplastic Cerebellar Degeneration: A Rare but Important Consideration. Journal of Clinical Case Reports. 2016; 6 (1), 10-12. [4] Muglia V, Westphalen A. Bosniak classification for complex renal cysts: history and critical analysis. Radiol Bras. 2014 Nov/Dec; 47(6), 368–373. [5] Muglia V, Prando A. Renal cell carcinoma: histological classification and correlation with imaging findings. Radiol Bras. 2015 May/June; 48 (3), 155-174.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/paraneoplastic-cerebellar-degeneration-as-initial-symptom-of-renal-cell-carcinoma/