Age: 14.0
Sex: M
Vaccine: HPV9, HPV9, HPV9
Symptoms: Tremor, Unresponsive to stimuli, Urine ketone body present, Varicella virus test negative, Viral infection
Clinical narrative: Adverse event: Development of fulminant acute disseminated encephalomyelitis (ADEM), leading to severe cerebral edema, leading to brainstem herniation and death. Tx: As documented below Outcome: Death From HPI: Patient is a 14 y.o. male with history of asthma who is being admitted for altered mental status of unknown etiology. Patient was in his usual state of good health until ~10 days ago when he developed pharyngitis and headache. He was seen by urgent care 1 week PTA (8/1) and tested negative for Strep; symptoms were attributed to viral illness. His pharyngitis persisted and  3 days PTA (Mon 8/6) when he developed a severe headache. He went to football practice (drills only; no tackling) that night and since then has had significant fatigue. He roused for football practice again 2 days ago but went to bed immediately afterward (~1900). Yesterday, he was too tired to attend practice. Mom became concerned when he was no longer able to follow commands consistently and seemed confused (e.g., taking his shirt off when he meant to put it on), so she brought him to ED. At ED, he was found to have left sided weakness on exam. Urine tox screen was negative. CBC was WNL aside from mild relative monocytosis and mild monocytosis without anemia. BMP and LFTs were unremarkable. Lactate was normal. Noncon head CT was normal. Cath UA was concentrated (SG >1.030) and had small ketones but otherwise unremarkable. EKG was unremarkable. They considered LP but decided to transfer to higher level of care. In our ED, physical exam revealed disorientation (oriented to self and location, "hospital'), slightly decreased level of consciousness (arouses to voices, opens eyes spontaneously, moves on command), and mild neurologic deficits (slightly weaker grip on left, slightly weaker left leg notable on ambulation) with "tapping"/tremor of his right hand and both feet. LP was performed (w/ 2 mg IV Ativan) and CSF studies were sent, including Lyme and meningitis/encephalitis panel. Ammonia level was obtained and was normal. After consultation with Peds Neurology, MRI brain w/wo contrast was obtained and showed nonspecific diffuse abnormal FLAIR hyperintensity in the supratentorial white matter consistent with ADEM. From discharge summary: Patient was initially admitted to the neurology service on 8/9 due to altered mental status. His focal neurologic deficits correlated with MRI findings of acute disseminated encephalomyelitis (ADEM) and were supported by lymphocytic pleocytosis and recent viral illness (as well as recent HPV vaccine. Both viral infections and HPV vaccine have been documented as being the inciting event for ADEM in the literature). Additional CSF studies included normal protein/glucose and negative meningitis/encephalitis panel. Later that evening patient was noted to have increased agitation and was difficult to arouse with verbal stimuli. Sternal rub resulted in eye opening and moaning. He was noted to have significant left sided weakness. Due to his change in neurologic status and need for further monitoring he was transferred to the ICU for further care. Patient had been started on a 5 day course of methylprednisolone for treatment of ADEM. That night patient had episodes of bilateral should jerking and associated desaturations. He was started on levetiracetam (keppra) and continuous bedside video EEG.  Early in the morning on 8/11, starting around 0345 patient developed gasping respiratory pattern with desaturations to the 60s and tachycardia ~ 200 bpm. Patient was given a dose of lorazepam due to concern for seizures. Due to his worsening status and concern for impending respiratory failure patient was intubated. He had copious brown emesis. Anesthesia was called to the bedside, and after receiving RSI medications patient was successfully intubated. He underwent an emergent bronchoscopy for evacuation of gastric content. Shortly thereafter, around 0700, patient was noted to have fixed and dilated pupils and was unresponsive on neurologic exam. A stat head CT was obtained and show interval development of severe cerebral edema. Neurosurgery was emergently consulted and patient went Class A to the OR for right sided hemicraniectomy. Post-operatively IVIG was added as treatment for ADEM. Paralytics were discontinued on 8/12 and sedation discontinued the morning of 8/13. Patient's EEG showed electrocerebral silence and no evidence of brainstem function or higher cerebral function on clinical exam. The morning of 8/14 patient underwent a brain death exam. The exam started at 1010, and patient was confirmed to be euthermic. Patient was noted to have absent pupillary reflex, corneal reflex, oculovestibular reflex, gag reflex or cough with suctioning, had absent response to pain stimuli, and apnea as demonstrated by apnea test. EEG was confirmed to be flat. After family spent time with patient at the bedside his supportive cares were withdrawn. Time of death 1310. An autopsy has been performed, preliminary neuropathology consistent with acute hemorrhagic leukoencephalitis (aka Weston-Hurst disease, a fulminant variant of ADEM) with superimposed global hypoxic-ischemic injury and edema.