(609) Autoimmune GFAP Astrocytopathy in the Medical Intensive Care Unit

Introduction: Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an inflammatory autoimmune disease of the central and peripheral nervous systems that can affect any neurologic region. There is a wide spectrum of presenting symptoms, so it can be difficult to diagnose, particularly in patients in the intensive care unit.

Description: A 43-year-old male with new-onset type 2 diabetes mellitus presented with 1 week of malaise and intractable hiccups. He was admitted for suspected diabetic ketoacidosis. On admission, he had a normal neurological exam, but he became altered and tachypneic requiring endotracheal intubation. Initial Head CT was unremarkable. A lumbar puncture showed an elevated opening pressure and lymphocytic pleocytosis. Viral PCRs and serologies were negative. Cefepime, vancomycin, and acyclovir were initiated. The patient initially improved and was extubated, but, over the next 4 days, he developed profound generalized weakness requiring re-intubation for acute respiratory failure. He also developed right upper extremity myoclonus, right lower extremity hyporeflexia, and bilateral papilledema. A Head MRI revealed 2 punctate lesions to the right hippocampus and midbrain but no signs of encephalitis. Due to the patient's continued decline, corticosteroids, high-dose acyclovir, and IVIG were initiated. Despite this treatment, he remained ventilator dependent with limited mentation. An additional head and spine MRI was performed with nerve conduction studies on hospital day 18 revealing encephalitis not seen previously with evidence of radiculitis. At this time, initial CSF studies elicited the final etiology of his condition: autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. He was started on aggressive physical therapy and extubated on Hospital Day 20.

Discussion: The patient's presentation of intractable hiccups is an uncommon presentation of autoimmune GFAP astrocytopathy. There have been a limited number of documented cases of autoimmune GFAP astrocytopathy associated with intractable hiccups. The pathology of this could be due to autoimmune involvement of the medulla oblongata resulting in Area Postrema Syndrome. Lack of diagnostic criteria and a multitude of presenting features can pose challenges in diagnosis of GFAP astrocytopathy.