A 23-year-old Caucasian male with past medical history of asthma was in normal health after a tonsillectomy in May 2013, when he developed nausea, vomiting, and right upper quadrant pain in June. He presented to an outside hospital, was diagnosed with cholecystitis, and underwent a laparoscopic cholecystectomy in early July 2013. Although he was reportedly eating normally before and after the cholecystectomy, he had unintentionally lost 40 pounds in the last few months and approximately 70 pounds over the past year (possible causes were unknown to the patient and family). He as well as his family denied alcohol consumption or smoking. Family history was noncontributory for cancer or any diseases. The patient denied recent vaccinations or infections. The patient was admitted to the outside hospital in late July 2013 with a diagnosis of gastritis after his cholecystectomy and was discharged on Reglan for nausea and vomiting. At time of discharge, his mother reported that he was unsteady on his feet and was apparently grabbing at objects that were not present. He was readmitted to the outside hospital the following day and had two tonic-clonic seizures (broken by Ativan). Head CT and chest X-ray at the outside hospital were unremarkable. Lumbar puncture there reportedly showed no WBCs/RBCs, glucose 104, and protein 125. Additionally, his laboratory values were significant for transaminitis (AST 41, ALT 207; remainder HFP normal) and leukocytosis (WBC 15.0). Alcohol level was not significant, but HIV-ELISA was also reportedly positive at the outside hospital. He had an MRI at the outside hospital, which was read as a few nonspecific deep white matter changes.
Due to need for acute care monitoring, the patient was transferred to UPMC Mercy Hospital. On admission to the intensive care unit, the patient was protecting his airways and was intubated; pupils were intact and eyes blinked to visual threat, but the patient did not respond to any commands nor was verbalizing. He moved all extremities spontaneously but very little to stimuli. There was no clonus or Babinski. A stat EEG failed to show epileptiform discharges but did show generalized slowing. Keppra 1 g IV BID was started for prevention of the seizures as well as empiric vancomycin/Zosyn and acyclovir for possible infectious meningitis given his leukocytosis. Repeat HIV-ELISA at our hospital was negative, as was UDS. General surgery was consulted regarding possible intraabdominal pathology or other cholecystectomy-related cause of infection. An abdominopelvic CT scan was entirely unremarkable; however, the surgical service deemed no surgical intervention.
On admission day 2, the neurological team ordered a follow-up brain MRI, which showed signal abnormalities involving the hypothalamus, periaqueductal gray matter, and bilateral thalami (Figures 1(a), 1(c), 2(a), 2(c), and 3(a)). In consideration of the radiologic findings, the prioritized differential diagnosis included Wernicke's encephalopathy, possibly acute disseminated encephalomyelitis, and less likely CNS vasculitis or less common infections.
Because this list contained Wernicke's encephalopathy, empiric IV thiamine 500 mg TID was immediately started. At this point, after joint discussion with the neurology, infectious diseases, and critical care services, it was agreed that the patient should be started on a trial of 1 g IV methylprednisolone. It was jointly concluded that therapy neither alone nor in combination would foreseeably harm the patient neurologically or immunologically. An NG tube was also placed for general nutrition since the patient had been having weight loss and intractable vomiting.
To entertain the possibility of rare infections, the infectious diseases service conducted further workup. This workup was negative for California, St. Louis, Equine, and West Nile encephalitis. In addition, workup was negative for measles, mumps, LCMV, CMV, Cryptococcus, JC virus, Mycoplasma, RMSF, typhus, and hepatitis viruses. Subsequently, HIV Western blot was negative (CD4 count was normal as well), as were TB tests and ANA.
At this point, levels of vitamin B1 were found to be low at 36 (normal range 78-185); vitamin B12 and folate levels were normal. The patient was more responsive and was responding to commands of opening eyes and squeezing fingers. Now as he could follow commands, when asked to laterally and medially move his gaze, bilateral nystagmus was seen on both lateral and medial gaze deviations. However, because he was responding quite well to current therapy, he was kept on corticosteroids for two more days before a taper started. A repeat lumbar puncture three days after admission showed glucose of 85, decrease in the protein to 97, 3 WBCs, and 103 RBCs. CSF VDRL, HSV-1/2 PCR, and Lyme titers were negative.
Over the next few days, the patient slowly became more responsive to commands and verbal cues and was soon extubated. A repeat MRI on the 7th day of admission showed marked resolution in the abnormal signals (Figures 1(b), 1(d), 2(b), 2(d), and 3(b)). He was transferred to the general medicine floors six days after initial admission. At this point, he was gingerly phonating but was still having problems with salivary secretions; he remained responsive to commands. His mental status gradually improved to the point that he started talking and tolerated a dysphagia diet without nausea. He was then transferred to inpatient rehabilitation 14 days after initial admission. Over the next few weeks, the patient continued to steadily improve to a fully conversational state and essentially normal diet, ambulating well with a walker and with intact cognition.