Dignity Health St. Joseph's Hospital and Medical Center is a hospital in Phoenix, Arizona, United States, operated by Dignity Health. St. Joseph's is a 607-bed, not-for-profit hospital that provides a wide range of health, social and support services, with special advocacy for the poor and underserved. It is home to the Barrow Neurological Institute, the world's largest dedicated neurosurgical center and a renowned leader in neurosurgical training, research, and patient care. St. St.
Spontaneous intracerebral hemorrhage (ICH) accounts for about 10
Abstract Introduction Alpha-1 antitrypsin deficiency (A1ATD) is a rare genetic disorder that can cause both panacinar emphysema and progressive hepatic dysfunction. Development of hepatic encephalopathy (HE) in this setting signals decompensation and carries poor prognosis. Recognition of its characteristic neuroimaging findings can help distinguish metabolic encephalopathy from other causes of altered mentation in patients with end-stage liver and lung disease. Case Description A 58-year-old man with A1ATD, severe panacinar emphysema on 4 L/min home oxygen, and decompensated cirrhosis (MELD-Na 21) on weekly paracenteses and Prolastin infusions presented with progressive dyspnea, cough, and pleuritic chest discomfort. Imaging showed recurrent right-sided pleural effusions and spontaneous pneumothorax requiring thoracenteses and chest tube placement; pleural fluid was transudative, consistent with hepatic hydrothorax (Figure 1A). He was transferred for combined liver and lung transplant evaluation. During hospitalization, he developed confusion with elevated serum ammonia. Brain MRI revealed symmetric T1 hyperintensity in the globus pallidus and subtle diffusion restriction in the insular cortex (Figure 1B). These findings, characteristic of hyperammonemic encephalopathy, reflect ammonia-induced astrocytic swelling and neuronal dysfunction. After multidisciplinary evaluation, the patient was deemed ineligible for dual-organ transplantation due to psychosocial limitations. Discussion This case illustrates the full spectrum of A1ATD-related multiorgan failure, emphasizing that hepatic and pulmonary disease may progress simultaneously. The diagnosis of HE remains primarily clinical, but characteristic MRI findings-symmetric cortical hyperintensity in the insular and cingulate regions and T1 pallidal hyperintensity-support the diagnosis and correlate with the severity and chronicity of hyperammonemia. Early recognition of these radiologic signatures can guide appropriate therapy and prognosis discussions. Although HE changes may reverse with ammonia reduction, recurrent episodes portend advanced liver dysfunction. This case underscores the importance of coordinated transplant evaluation and recognition of imaging patterns that signal metabolic encephalopathy in patients with combined hepatic and pulmonary compromise. This abstract is funded by: None
