A 91-Year-Old Female with Recurring Coma Due to Atypical Hyperammonemia

Abstract

Background and clinical significance: Acute reduction in vigilance is a frequent reason for emergency department admissions, especially among the elderly. While intracranial causes or infections with fluid depletion are often responsible, there remain cases where imaging, laboratory tests, and clinical examination fail to provide a clear diagnosis. Case presentation: A 91-year-old woman was presented to the emergency department with recurrent episodes of somnolence to deep coma. On admission, her vital signs were stable, and cerebral CT imaging revealed no intracranial pathology. Laboratory analyses, including blood gas measurements, were unremarkable. Empirical treatment for possible intoxications with benzodiazepines or opioids using flumazenil and naloxone had no effect. An Addison's crisis was considered but excluded following methylprednisolone administration without improvement in consciousness. Eventually, an isolated elevation of serum ammonia was identified as the cause of the reduced vigilance. Further investigation linked the hyperammonemia to abnormal intestinal bacterial colonization, likely due to a prior ureteroenterostomy. There was no evidence of liver dysfunction, thus classifying the condition as non-hepatic hyperammonemia. Therapy was initiated with rifaximin, supported by aggressive laxative regimens. Ammonia levels and vital parameters were closely monitored. The patient's condition improved gradually, with serum ammonia levels returning to normal and cognitive function fully restored. Conclusions: This case highlights an uncommon cause of coma due to non-hepatic hyperammonemia in the absence of liver disease, emphasizing the diagnostic challenge when standard evaluations are inconclusive. It underscores the need for broad differential thinking in emergency settings and the importance of considering rare metabolic disturbances as potential causes of altered mental status.

Keywords: coma; hyperammonemia; metabolic encephalopathy.

Figure 1

The No scientific information is lost, since all necessary waveform morphology and intervals can be assessed visually using the standard scaling (1 mm = 40 ms). For readers outside of cardiology, these values are of limited interpretative relevance. Therefore, I believe that the figure remains scientifically complete and appropriate as presented. ECG at admission showed no severe dysrhythmias nor signs of typical intoxications. I–III represent the standard limb leads; aVR, aVL, and aVF are augmented limb leads; V1–V6 are the precordial (chest) leads.

Figure 2

Chest X-ray showed no pneumonia or sign of heart failure. Pneumothorax or pleural effusions were not visible either. The upper mediastinum was slightly broadened due to a known goiter. The letter “L” denotes the patient’s left side and serves as a standard orientation marker in posteroanterior chest radiography.

Figure 3

In the sonographic liver examination, no cirrhosis was detectable. The blue line indicates the orientation marker on the ultrasound probe, placed transversely in the right upper abdominal quadrant.