A Case of Atypical Acute Encephalopathy Unmasking IgA Multiple Myeloma

TL;DR

A rare case shows how multiple myeloma can present as sudden brain dysfunction, highlighting the need for early diagnosis and treatment.

Contribution

Presents a rare neurological manifestation of IgA multiple myeloma as acute encephalopathy.

Findings

The patient's encephalopathy resolved only after bortezomib-based chemotherapy.

MRI showed diffuse microhemorrhages in the brain.

Elevated IgA lambda paraproteins confirmed the diagnosis of multiple myeloma.

Abstract

Multiple myeloma (MM) can have an insidious onset, particularly in its early stages, which may contribute to delays in diagnosis. Acute encephalopathy is a rare and atypical manifestation of MM that can further complicate timely recognition and management. We present the case of a 57-year-old woman who initially reported fatigue, back pain, recurrent falls, and significant unintentional weight loss and was found to have hypercalcemia, anemia, acute renal dysfunction, and bone lesions. Her clinical course rapidly evolved into profound encephalopathy, characterized by a change in mentation from her baseline, agitation, and eventual unresponsiveness requiring intubation.

Extensive investigation, including a bone biopsy, revealed elevated IgA lambda paraproteins consistent with MM. She also had borderline serum hyperviscosity, mildly elevated ammonia levels, and diffuse microhemorrhages on brain MRI. Despite interventions, including plasmapheresis and high-dose steroids, her encephalopathy persisted until initiation of bortezomib-based chemotherapy, resulting in neurological improvement. This case emphasizes the importance of recognizing MM as a potential cause of unexplained encephalopathy and highlights the complexity of its neurological manifestations, underscoring the need for prompt hematologic evaluation and interdisciplinary management.

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