Age: 83.0
Sex: F
Vaccine: FLU3
Symptoms: Hoffmann's sign, Human herpes virus 6 serology negative, Hyperreflexia, Hypertension, Intensive care
Clinical narrative: Case number PHHY2013US000871 is an initial report from Vaccine Adverse Event Reporting System (VAERS reference number 470522) received on 02 Jan 2013 with a follow up literature report received on 28 Aug 2013. The author presented a report on an elderly woman with acute disseminated encephalomyelitis following seasonal influenza vaccination who recovered after plasma exchange. This report refers to an 83-years-old female patient. Her medical history included hypertension, type 2 diabetes and previous ischemic stroke. Concomitant medications included MVI (vitamins), simvastatin, lisinopril, citalopram, NORVASC, LASIX and glipizide and JANUVIA. She went for an annual evaluation to her primary care physician and had an intramuscular dose of FLUVIRIN (batch number: 1204521) intramuscularly in her right arm on 14 Sep 2012. It was reported that she was presented to the emergency room on 22 Sep 2012 with one day of altered consciousness, altered mental status, weakness and fever. During the previous 2 days, she experienced retro orbital pain which progressed to decreased verbal response and inability to follow commands along with weakness and fever on the day of admission. On admission, she was found to be obtunded with a Glasgow coma scale [GCS] score of 9, febrile with a body temperature of 103.5 F, tachycardic with 112 beats per minute, tachypneic with 32 breaths per minute and hypertensive with a blood pressure value of 140/64 mm Hg. She had no hypoxemia. On neurological examination, she had quadriparesis, brisk reflexes, bilateral extensor plantar response, bilateral Hoffman's sign and perioral fasciculations. A fundoscopic examination showed no evidence of papilledema, disc atrophy, neuritis or hemorrhages. The results of the rest of the physical examination were within normal limits. An initial computed tomography (CT) scan of the head was unremarkable. Cerebrospinal fluid (CSF) analysis revealed pleocytosis with 44 cells per liter, 60% lymphocytes, 30% monocytes. Her CSF protein was 136 g/dl and CSF glucose was 108 g/dl. Blood cell counts were reported to be normal. She had acute kidney injury with creatinine value of 1.5 mg/dl and mild transaminitis (aspartate aminotransferase [AST] was 98 mg/dl and alanine aminotransferase [ALT] was 89 mg/dl). A presumptive diagnosis of infectious meningoencephalitis was made and empirical treatment with ceftriaxone, ampicillin, vancomycin and acyclovir was started. Despite 48 hours of antibiotics, her mental status declined to a GCS score of 6 along with worsening focal findings and respiratory failure. She was emergently intubated and transferred to the intensive care unit for mechanical ventilation. Magnetic resonance imaging (MRI) of the brain and spine showed diffuse abnormal fluid attenuated inversion recovery (FLAIR) signals in the periventricular white matter (diffuse white matter lesions), splenium of the corpus callosum, bilateral thalami, bilateral mesial temporal lobes, midbrain, fornices, mammillary bodies, dorsal brain stem, and middle cerebellar peduncles as well as within the medulla oblongata. A 5-day course of intravenous methylprednisolone was given without clinical response. Further tests on the CSF, including bacterial and fungal cultures, PCR for herpes simplex virus (HSV), varicella-zoster virus (VZV), human herpes virus 6 (HHV-6), entero virus, JC and BK viruses, anti-NMDA (N-methyl-D-aspartate) receptor antibody and 14-3-3 protein, and flow cytometry for lymphoma were reported as negative. Serum studies for West Nile, Western equine, Eastern equine, and encephalitis virus; Cryptococcus and Histoplasma antigens and human immunodeficiency virus (HIV) viral load and a Quantiferon- TB test were all negative. Repeated MRI on day 12 showed progression and coalescence of T2 hyperintensity throughout the white matter of the corona radiata and centrum semiovale. After having ruled out infectious etiologies and due to the lack of clinical and radiological response, all antibiotics were discontinued on day 14 of admission. Her clinical presentation, fever, altered consciousness, and multifocal neurological deficits combined with MRI findings that demonstrated extensive white matter lesions suggested the diagnosis of acute disseminated encephalomyelitis (ADEM). Therefore, plasma exchange was started on day 24, with 5 exchange sessions performed over 10 days. Dramatic clinical improvement was seen, and follow-up brain MRI taken at the end of therapy showed as significant decrease in T2 hyperintensity throughout the white matter. On day 42, she was discharged to a rehabilitation facility with preserved alertness, motor function, speech, and spontaneous breathing. After 3 months, she developed pneumonia with septic shock and died. There was no relapse of neurological symptoms in this time interval. This confirmed the suspicion of ADEM, as the patient met level 1 criteria for its diagnosis by the presence of multifocal neurological findings, MRI displaying diffuse white matter lesions and the monophasic course of the disease (no relapse at 3 months). A final diagnosis of post vaccination ADEM was made after exclusion of infection and noninfectious etiologies along with the recent exposure to influenza vaccination 8 days prior to admission. The author stated that in this patient, the clinical event showed a plausible time relationship with the vaccine administration and in the absence of other possible etiology the diagnosis of post vaccination ADEM was very likely. The author concluded the article stating that the above reported case was a rare scenario of post influenza vaccination acute disseminated encephalomyelitis, emphasizing the paramount importance of recognizing recent vaccination exposures. Follow up information received on 28 Aug 2013: Events (all events except ADEM, mental status changes and white matter lesion), lab data, treatment medication updated.