Hatharasinghe et al. HCA Healthcare Journal of Medicine (2020) 1:2 https://doi.org/10.36518/2689-0216.1038 Case Report Acute Disseminated Encephalomyelitis Ashan Hatharasinghe, DO,1 Hossein Akhondi, MD,1 Don Pepito, MD1 Author affiliations are listed at the end of this article. Abstract Correspondence to: Introduction Ashan Hatharasinghe, DO Acute Disseminated Encephalomyelitis (ADEM) is a rare autoimmune demyelinating disor- 2880 North Tenaya Way der of the central nervous system. Clinical manifestations include encephalopathy, motor Las Vegas, NV, 89128 deficits, ataxia, and meningeal signs. In most cases, ADEM is preceded by either vaccination (Ashan.Hatharasinghe@ or viral illness. Here, we present a case with neither of the two predisposing elements. HCAhealthcare.com) Discussion A 28-year-old Hispanic female presenting with substance use and suicidal ideation was placed on an involuntary psychiatric hold, started on olanzapine and scheduled for a psychi- atric facility transfer. The following day, she was noted to have neurological deficits when ambulating. Computed tomography of the brain showed a right frontal lesion. Magnetic resonance imaging of the brain was notable for multiple peripherally enhancing white mat- ter lesions. Multiple sclerosis and other etiologies were ruled out through supporting tests and lumbar puncture. ADEM was suspected, and the patient was treated with both a five- day course of intravenous methylprednisolone as well as immune globulins. She continued to have mild expressive aphasia after treatment; however, the majority of her symptoms improved. Conclusions Diagnosis of ADEM versus multiple sclerosis can be difficult given there are no current diag- nostic criteria for it in the adult population. In this case, we explain how we reached a diag- nosis of ADEM and provide further discussion regarding the disease course and treatment. Keywords encephalomyelitis, acute disseminated; ADEM; demyelinating autoimmune diseases, CNS; autoimmune diseases of the nervous system, leukoencephalitis, acute hemorrhagic; immu- noglobulins, intravenous Introduction viral illness, and discuss how we reached the Acute disseminated encephalomyelitis (ADEM) diagnosis. is an uncommon autoimmune demyelinating disorder of the central nervous system.1,2 It is Case Report more common in the pediatric population but A 28-year-old Hispanic female was brought can occur in adults.3 In most cases, ADEM is to the emergency department with a chief preceded by an acute viral illness and in ex- complaint of suicidal ideation. The patient, tremely rare cases by vaccination.4,5,6 Clinical who lived by herself, was on the phone with manifestations include encephalopathy, motor her mother, when she reported snorting an deficits, ataxia and meningeal signs. The com- unknown substance and verbalized suicidal bination of these non-specific symptoms, its ideation. Her mother then called emergency low incidence and lack of universally accepted medical services, who transported the patient diagnostic criteria make it challenging to di- to our facility. The patient had no previous his- agnose. Here we present a case of ADEM with tory of mental illness but had a history of do- no known prior vaccination or preceding acute mestic abuse and two spontaneous abortions. www.hcahealthcarejournal.com HCA Healthcare © 2020 HCA Physician Services, Inc. d/b/a Journal of Medicine Emerald Medical Education 77 HCA Healthcare Journal of Medicine Her family noted that she had been exhibiting Table 1. Negative and Normal Labs strange behavior for approximately one week. Acetaminophen level The behavior was first observed when her family was watching her play in a softball game. Ammonia level The patient, who was an avid softball player Anti-neutrophil cytoplasmic antibodies (ANCA) for the past fifteen years, did not know how Anti-nuclear antibody (ANA) to run around the bases properly. The family also stated that she was fired from her job two Ceruloplasmin days prior to presentation, and it is believed Complement C3 and C4 this was due to odd behavior. Psychiatry was Complete blood count (CBC) consulted by the emergency room physician for suicidal ideation. Urine drug screen was nega- Comprehensive metabolic panel (CMP) tive (Table 1). The initial examination revealed Corticotropin the patient to be responding to internal stimuli C-reactive protein (CRP) (talking to herself). She was placed on an invol- untary psychiatric hold, started on olanzapine Creatine kinase (CK) and planned to be transferred to an inpatient Cryoglobulin psychiatric facility. Cytomegalovirus IgG The following day the patient was noted to Erythrocyte sedimentation rate (ESR) be swaying in bed and having ataxic gait while Free T4 walking with a nurse. A computed tomography Haptoglobin (CT) of the brain without contrast was ob- tained (Figure 1A) which was notable for a right HCG frontal lesion. The initial transfer to a psychiat- Hemoglobin A1C ric facility was cancelled, and the patient was Hepatitis A admitted to an intermediate care bed (IMC) under the internal medicine service. Magnet- Hepatitis B core ic resonance imaging (MRI) of the brain with Hepatitis B surface contrast (Figure 2A) was obtained and revealed Hepatitis C multiple peripherally enhancing white matter lesions in the deep cortical matter with sparing HIV-2 Ab of the corpus callosum. CT angiography of the HIV screen head and neck, MR angiography of the brain, HSV 1 & 2 PCR and MRI of the cervical spine and thoracic spine were all negative. Lactic acid dehydrogenase (LDH) Lipid panel A lumbar puncture was performed, yielding an Lupus anticoagulant elevated cerebrospinal fluid (CSF) white blood cell count of 0.045 x 103 µL (normal 0-0.005 µL) Paraneoplastic antibody profile with elevated lymphocytes (97%) and elevat- Salicylate level ed myelin basic protein of 37.2 ng/mL (normal 0.0-1.2 ng/mL). Oligoclonal bands, JC virus and T-cell populations (CD3, CD4, CD8) toxoplasma antibody were negative. Further Thyroid-stimulating hormone (TSH) lab testing was performed over the next few Treponema pallidum antibody days and was negative (Table 1), except for Bor- relia burgdorferi IgG, Epstein Barr Virus PCR Troponin and Varicella-Zoster Virus IgG. Urine drug screen Urine analysis The electroencephalogram while the patient was awake showed a posterior dominant VDRL background rhythm consisting of low to normal Vitamin B12 level amplitude (6-8 Hertz) theta slowing. Occasion- West Nile al sleep spindles were seen towards the end of 78 Hatharasinghe et al. (2020) 1:2. https://doi.org/10.36518/2689-0216.1038 Figure 1. A) Computed tomography (CT) of the brain without contrast showing a 2 x 1.9 x 1.7 cm low-den- sity area in the right frontal lobe in the axial plane. Differential included encephalomalacia, sub- acute infarct or mass. B) A repeat CT of brain without contrast 3 days after the first CT showing increasing hypo-densi- ty to the lesion, which is suggestive of a subacute phase. the study. No electrographic seizures, focal/ Discussion lateralizing discharges, periodic discharges or The case demonstrates the importance of epileptiform activity was seen. The EEG was conducting a thorough history and physical consistent with mild background slowing, exam, especially when determining wheth- which is commonly seen in cases of mild en- er a patient’s symptoms are the result of an cephalopathy. acute psychiatric illness versus an underlying neurological disease. Initially, the patient was These overall findings were most character- thought to have an acute psychiatric illness. istic of acute disseminated encephalomyelitis However, the presence of a neurological abnor- (ADEM) although multiple sclerosis (MS) and mality on physical exam, noticed approximately other pathology needed to be ruled out. After twelve hours after her presentation, ultimate- reviewing the findings, ADEM best explained ly resulted in the discovery of an underlying the findings. Intravenous (IV) methylpredniso- neurological cause of the patient’s symptoms. lone at a dosage of 1 gram every 24 hours was Although new-onset schizophrenia can com- started. Over the next 48 hours the patient’s monly present in young females (20-30 years mentation worsened, and she became increas- old), there are several details within the history ingly aphasic with associated hypotension. and physical itself that make the psychiatric A repeat CT of the brain without contrast illness less likely.7,8 The most important detail (Figure 1B) showed increasing hypo-density is the fact that the patient had motor deficits of the previous lesions, indicating a subacute (i.e. not being able to run the bases at her soft- phase. Intravenous immunoglobulin (IVIG) was ball game properly) and ataxic gait.9 then started in addition to the IV methylpred- nisolone. After 24 hours of IVIG the patient’s The ramifications of misdiagnosis can be fatal, mental status, including her aphasia began to as mortality rates in cases of ADEM have been improve. reported to be as high as 20-25%.10,11 ADEM also has a hyperacute variant known as acute necro- The patient received both a five-day course of tizing hemorrhagic leukoencephalitis of Weston IV methylprednisolone and IVIG. The IV methyl- Hurst. This variant is a fulminant syndrome prednisolone was started on day 2 of admission representing approximately 2% of ADEM cases and the IVIG was started on day 5. Repeat MRI and is characterized by abrupt onset of sei- of the brain with contrast post-IVIG (Figure zures, neck stiffness and fever with the finding 2B) still showed multiple white matter le- of petechial hemorrhages on brain imaging.12, 13 sions with increasing surrounding edema, but Acute necrotizing hemorrhagic leukoencephali- the enhancing components had resolved. The tis has more than a 50% mortality rate.14 patient’s mentation improved, and she was speaking full sentences with mild expressive Acute disseminated encephalomyelitis most aphasia. The patient was then discharged to a often occurs 2-4 weeks after exposure to a skilled nursing and rehabilitation facility. viral illness or vaccination. Our patient did not 79 HCA Healthcare Journal of Medicine Figure 2.