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 Table of Contents  
Year : 2021  |  Volume : 8  |  Issue : 4  |  Page : 203-207

Acute hemorrhagic encephalomyelitis – Rare presentation of pediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2: A case report

1 KrishnaMaternityHomeandPediatricCenterPvt.Ltd., Tirunelveli, Tamil Nadu, India
2 PS Neuro Centre, Tirunelveli, Tamil Nadu, India

Date of Submission26-Mar-2021
Date of Decision13-Jun-2021
Date of Acceptance16-Jun-2021
Date of Web Publication10-Jul-2021

Correspondence Address:
Dr. Prasanna Narayanan Raju
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpcc.jpcc_25_21

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Acute hemorrhagic encephalomyelitis (AHEM) is a rare form of acute disseminated encephalomyelitis (ADEM) characterized by a fulminant course, poor response to immunomodulation, and unfavorable outcomes. Early clinical and radiological suspicion can enable aggressive use of immunotherapy, thereby improving outcomes. Pediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2 (PIMS-TS) is a novel disease, with protean clinical manifestations being reported from all over the world. Although multiple neurological manifestations of PIMS-TS have been reported previously, AHEM has been less commonly reported. We describe a case of PIMS-TS with AHEM and highlight very rare presentation of this novel disease.

Keywords: Acute disseminated encephalomyelitis, acute hemorrhagic encephalomyelitis, hemorrhagic acute disseminated encephalomyelitis, pediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2

How to cite this article:
Raju PN, Raju PS, Pooranalingam M, Subramanian R. Acute hemorrhagic encephalomyelitis – Rare presentation of pediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2: A case report. J Pediatr Crit Care 2021;8:203-7

How to cite this URL:
Raju PN, Raju PS, Pooranalingam M, Subramanian R. Acute hemorrhagic encephalomyelitis – Rare presentation of pediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2: A case report. J Pediatr Crit Care [serial online] 2021 [cited 2021 Sep 19];8:203-7. Available from: http://www.jpcc.org.in/text.asp?2021/8/4/203/321101

  Introduction Top

Pediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2 (PIMS-TS) has varied clinical manifestations. Neurological manifestations of PIMS-TS have been less frequently reported in children, although presentations such as headache, encephalitis, meningitis, and encephalopathy have been reported in adults.[1] Acute demyelinating encephalomyelitis (ADEM) has been commonly described after viral infections in children. Acute hemorrhagic encephalomyelitis (AHEM) is a rare variant of ADEM and pediatric case reports are few. To our knowledge, in India, there is only one other pediatric case of AHEM associated with PIMS-TS reported so far.[2]

  Case Report Top

A 1-year-old male child was referred with a 3-day fever, vomiting, and decreased oral intake. The child was irritable, presented in cold shock, heart rate 190/min, systolic blood pressure of 75 mm hg, absent peripheral pulses, and Glasgow come scale (GCS) score of 11/15. There was no significant medical history and no recent history of vaccination. Systemic examination showed mild hepatomegaly with the absence of meningeal signs or neck stiffness. He required fluid resuscitation-crystalloid and colloid boluses followed by inotropic support. He was started on high-flow nasal oxygen and other supportive care. Initial investigations showed leukocytosis (14,400/cumm) with neutrophilic predominance (75%) and thrombocytopenia (48,000/cumm). Inflammatory markers were elevated procalcitonin (9.9/μg/L) and ferritin (>2000 μg/L). Liver functions were deranged features suggestive of fulminant hepatic failure. His renal functions were normal [Table 1]. Arterial blood gas showed severe metabolic acidosis with hyperlactatemia (14.8/mg/dL). Dengue NS1 was positive. His COVID IgG/IgM antibodies were also positive.
Table 1: Laboratory investigations chart

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Possibility of septic shock, PIMS-TS with multi-organ dysfunction, dengue shock, or tropical infection-like scrub typhus was considered. He was started on broad-spectrum antibiotics – meropenem, clindamycin, and azithromycin. Screening echocardiogram was normal.

Once initial culture reports came negative, the possibility of PIMS-TS (as per the WHO criteria),[3] was considered as the child had following features:

  • Fever for >3 days
  • Hypotensive shock
  • Coagulopathy
  • Elevated inflammatory markers – procalcitonin
  • Other focus of infection or sepsis ruled out
  • Positive COVID IgM and IgG.

The child was initiated on pulse methylprednisolone 30 mg/kg along with intravenous immunoglobulin (2 g/kg). By day 3 of intensive care unit (ICU) stay, the child showed clinical improvement, his hemodynamic were stabilized, inotropes were tapered off, and liver functions showed improving trend with normalizing coagulation parameters and reducing trend of lactates. Serial ammonia levels also showed decreasing trend. Sensorium improved and the child was initiated on oral feeds. Fever subsided and serial platelet counts were increasing.

On day 4 of pediatric ICU stay, the child developed one episode of seizure (generalized tonic clonic) lasting around 10 min. Seizure subsided with levetiracetam, and he was continued on supportive care. His sensorium continued to fluctuate with intermittent episodes of irritability and drowsiness. Magnetic resonance imaging (MRI) brain [Figure 1] showed multiple well-defined rounded lesions in right frontal, left parafalcine parietal, left periventricular, and bilateral cerebellar lobes with minimal postcontrast enhancement, suggestive of ADEM. He was continued on pulse steroids and other supportive care.
Figure 1: Initial magnetic resonance image - T2, DWI, and postcontrast images showing ring like lesions in cerebellar, parietal, and frontal lobes with minimal enhancement

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The child also developed recurrence of fever spikes with dropping counts subsequently, for which cultures were repeated and invasive lines were removed. Repeat sepsis markers including procalcitonin and cultures were negative. Bone marrow examination ruled out possibility of hemophagocytosis. Cerebrospinal fluid (CSF) examination showed increased protein and polymorphic pleocytosis. CSF viral panel, pan bacterial PCR panel, and culture reports were negative.

In view of worsening sensorium, the child was intubated, mechanically ventilated and neuroprotective measures were initiated. Electroencephalography showed generalized slowing, suggestive of encephalopathy. MRI brain [Figure 2] was repeated which showed increase in size of lesions in the bilateral frontoparietal region and cerebellar lobes with blooming on SWI sequence, with mild midbrain shift and evolving obstructive hydrocephalus – suggestive of hemorrhagic ADEM. MRI spine screening was normal. Neurosurgeon opinion was taken, and he advised decompression. Parents were also counseled regarding further immunomodulation (plasma exchange) and high risk involved in the same. However, his general condition worsened and parents wanted to transfer to another facility, where the child succumbed to his illness.
Figure 2: Repeat magnetic resonance images – T2 and DWI and postcontrast images – increase in size of lesions with involvement of bilateral cerebellar, parietal, and frontal regions

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  Discussion Top

PIMS-TS is a novel disease with protean manifestations being reported frequently. ADEM is commonly reported following viral infections. Hemorrhagic ADEM or AHEM is a rare entity forming around 2% of ADEM cases.[4] Cases of SARS-CoV-2–associated AHEM have been reported in adults previously.[5] However, pediatric case reports are few. The distinction between ADEM and AHEM is not well established, but AHEM is considered to be the most severe condition in the same spectrum. AHEM has a fulminant clinical course and unfavorable outcome as compared to ADEM, where recovery is the general rule.[6] The cause of neurological deterioration in our case was probably AHEM based on the MRI findings, polymorphonuclear pleocytosis on CSF examination, and fulminant clinical course despite immunomodulation. The definitive diagnosis however warrants a pathological examination.[7] Liver and brain involvement could also be considered an extension of the hyperinflammatory state.

As presentation similar to inflammatory syndrome with CNS involvement in the early course of disease, the possibility of dengue encephalitis was considered unlikely in this case.[8] Postinfectious ADEM associated with dengue virus (DENV-ADEM) occurs later in course of the disease. DENV-ADEM was not considered in our patient as the patient presented with acute onset of fever with NS1 positivity and dengue IgM/IgG negativity, showing that it was very likely to be an acute infection.

However, the possibility of a co-infection or antigen cross-reactivity cannot be completely ruled out and has been reported previously in the literature.[9] Although our patient showed good initial response to immunoglobulin and pulse steroids, further deterioration probably warranted early escalation of other modalities of immunotherapy.

PIMS-TS presenting as AHEM is exceedingly rare with poor outcome and warrants aggressive use of combination of immunomodulatory agents.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given consent for images and other clinical information to be reported in the journal. The patient's parents understand that the names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Aghagoli G, Gallo Marin B, Katchur NJ, Chaves-Sell F, Asaad WF, Murphy SA. Neurological involvement in COVID-19 and potential mechanisms: A review. Neurocrit Care 2021;34:1062-71.  Back to cited text no. 1
Sarangi B, Walimbe A, Shankar GS, Oswal JS, Reddy KV, Prithvichandra KC. Pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 in Indian children: Pune experience. Int J Adv Med 2021;8;4:499-504.  Back to cited text no. 2
World Health Organization. Multisystem Inflammatory Syndrome in Children and Adolescents with COVID-19: Scientific Brief. Available from: https://apps.who.int/iris/handle/10665/332095. [Last accessed on 2021 Jun 12].  Back to cited text no. 3
Borlot F, da Paz JA, Casella EB, Marques-Dias MJ. Acute hemorrhagic encephalomyelitis in childhood: Case report and literature review. J Pediatr Neurosci 2011;6:48-51.  Back to cited text no. 4
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Poyiadji N, Shahin G, Noujaim D, Stone M, Patel S, Griffith B. COVID-19-associated acute hemorrhagic necrotizing encephalopathy: Imaging features. Radiology 2020;296:E119-20.  Back to cited text no. 5
Anlar B, Basaran C, Kose G, Guven A, Haspolat S, Yakut A, et al. Acute disseminated encephalomyelitis in children: Outcome and prognosis. Neuropediatrics 2003;34:194-9.  Back to cited text no. 6
Pinto PS, Taipa R, Moreira B, Correia C, Melo-Pires M. Acute hemorrhagic leukoencephalitis with severe brainstem and spinal cord involvement: MRI features with neuropathological confirmation. J Magn Reson Imaging 2011;33:957-61.  Back to cited text no. 7
Soares CN, Faria LC, Peralta JM, de Freitas MR, Puccioni-Sohler M. Dengue infection: Neurological manifestations and cerebrospinal fluid (CSF) analysis. J Neurol Sci 2006;249:19-24.  Back to cited text no. 8
Santoso MS, Masyeni S, Haryanto S, Yohan B, Hibberd ML, Sasmono RT. Assessment of dengue and COVID-19 antibody rapid diagnostic tests cross-reactivity in Indonesia. Virol J 2021;18:54.  Back to cited text no. 9


  [Figure 1], [Figure 2]

  [Table 1]


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