Critical Care: Student/Resident Case Report Poster - Critical Care II |

Not a Stroke: An Unusual Presentation of Hyperammonemia in Multiple Myeloma FREE TO VIEW

Boram Kim, DO; Purav Shah, MD; Eric Yudelevich, MD; Raymonde Jean, MD
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Mt. Sinai St. Luke's West Hospital, New York, NY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):396A. doi:10.1016/j.chest.2016.08.409
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SESSION TITLE: Student/Resident Case Report Poster - Critical Care II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Altered mental status (AMS) associated with multiple myeloma (MM) can be secondary to hypercalcemia, hyperuricemia and hyperviscosity. Only rarely has it been linked to hyperammonemia [1]. We present a unique case of hyperammonemia causing altered sensorium with focal neurologic findings.

CASE PRESENTATION: A 79-year-old female with MM presented to the emergency department with acute AMS 24 hours after being discharged from the hospital. She had been successfully treated for sepsis secondary to multifocal pneumonia. At discharge, she was conversant, able to walk and follow commands appropriately. However, upon presentation 24 hours later, she was stuporous and had right gaze deviation with left sided facial droop. Vital signs were stable. She required intubation for airway protection and was admitted to the intensive care unit (ICU). While in the ICU, she exhibited intermittent gaze deviation from right to left. Computed Tomography and Magnetic Resonance Imaging of the brain and CXR were unremarkable. Laboratory data including CBC, CMP, urinalysis and cerebrospinal fluid analysis showed no evidence of infection, liver failure, hyperuricemia, hypercalcemia or hyperviscosity. A continuous 24-hour EEG was negative for seizures. Her ammonia (NH3) level was noted to be 177 mmol/L and she underwent emergent dialysis for 2 consecutive days. Post dialysis, NH3 levels decreased to 101-110 mmol/L. She became alert, followed simple commands and her neurologic deficits disappeared. However, once NH3 increased back to prior level within six hours of dialysis, she again became stuporous. Chemotherapy was not pursued in accordance with patient’s prior wishes. She expired ten days later.

DISCUSSION: Although there are reports of hyperammonemia as a cause of AMS in multiple myeloma [2], to date there are no reports of associated focal neurologic changes. Our patient presented with AMS as well as rightward gaze with left sided facial droop. In the absence of other justifiable causes such as stroke, seizure and infection, elevated ammonia was identified as the culprit. Her transient improvement in mental status with resolution of focal deficits after dialysis, which has been used in hyperammonemia [3], further corroborates that her AMS correlated with NH3 levels. An interesting observation of unknown significance was how rapidly the hyperammonemia developed after treatment of sepsis.

CONCLUSIONS: In patients with MM presenting with focal neurologic deficits, one should have a high index of suspicion for hyperammonemia. The mechanism for neurologic deficits and the temporal relationship between treatment of sepsis and acute hyperammonemia may be potential areas of further study.

Reference #1: Jones, FM, et al., Hyperammonemic encephalopathy in multiple myeloma. Ann Hematol, 2014

Reference #2: Lora-Tamayo, J, et al., Multiple Myeloma and Hyperammonemic Encephalopathy: Review of 27 Cases Clin Lymphoma Myeloma, 2008

Reference #3: Kwan, L, et al., Hyperammonemic Encephalopathy in Multiple Myeloma. NEJM, 2002

DISCLOSURE: The following authors have nothing to disclose: Boram Kim, Purav Shah, Eric Yudelevich, Raymonde Jean

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