Paraneoplastic Cerebellar Degeneration: A Rare but Important Consideration

Prithvi Giri,Rakesh Shukla

doi:10.4172/2165-7920.1000700

Abstract

Paraneoplastic cerebellar degeneration is an uncommon autoimmune disorder characterized clinically by progressive, ultimately incapacitating ataxia and pathologically by destruction of cerebellar Purkinje cells, with variable loss of other cell populations. The paraneoplastic cerebellar degeneration can antedate the recognition of malignancy and hence can be a warning sign for occult tumor. We are reporting two cases of paraneoplastic cerebellar degeneration that presented before any evidence of tumor and later developed advanced stage of malignancy not amenable for surgery.

Highlights

Paraneoplastic Syndromes (PNS) are a rare heterogeneous group of disorders that are indicators of underlying occult malignancy
Patient was planned for contrast enhanced computer tomography (CECT) chest and Fluorodeoxyglucose-Positron Emission Tomography (FDG-PET) scan to search out for primary occult malignancy, but she was lost to follow-up
The diagnostic criteria for PNS recommended by Graus et al divides patients of suspected PNS into definite and possible based on presence of 1) classical or non classical syndrome, 2) associated cancer within five years and 3) presence of well-characterized or partially-characterized paraneoplastic antibodie [7]

Summary

Introduction

Paraneoplastic Syndromes (PNS) are a rare heterogeneous group of disorders that are indicators of underlying occult malignancy. Any part of the brain could be involved in PNS and isolated neurologic manifestations appear months or years before the detection of primary malignancy. It occurs before the presenting complaints of neoplasm in up to 65-80% of cases and so it can help in the early diagnosis of tumor [1,2,3]. It is characterized by rapid to subacute development of severe pancerebellar dysfunction over days to weeks due to extensive loss of Purkinje neurons with many patients stabilizing over months with significant disability in up to 90% [6] It presents with appendicular and truncal ataxia, dysarthria, and nystagmus. We are discussing two interesting cases of sub acute progressive, symmetrical, cerebellar ataxia, which after intensive investigations proved to be cases of paraneoplastic cerebellar degeneration

Case Presentation

Discussion

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Learning points