key: cord-0936280-n71dzk14
authors: Akçay, Nihal; Oğur, Mustafa; Emin Menentoğlu, Mehmet; İrem Sofuoğlu, Ayşe; Boydağ Güvenç, Kübra; Bakirtaş Palabiyik, Figen; Şevketoğlu, Esra
title: Acute Cerebellitis in MIS-C: A Case Report
date: 2021-12-08
journal: Pediatr Infect Dis J
DOI: 10.1097/inf.0000000000003358
sha: c8246d0fbc5ea3a8394030dfeccaaee323cc9222
doc_id: 936280
cord_uid: n71dzk14

BACKGROUND: Coronavirus disease-2019 (COVID-19) is characterized predominantly by respiratory symptoms and has affected a small subset of children. Multisystem inflammatory syndrome in children (MIS-C) has been reported in children following COVID-19. There is increasing report that COVID-19 may also lead to neurologic manifestations. Cerebellar lesions may be observed in viral infections. CASE REPORT: We report a child with MIS-C related to severe acute respiratory syndrome coronavirus 2, who developed cerebellar lesion during the disease course. Encephalopathy was the first central nervous system symptom. His consciousness improved but he developed clinical signs of cerebellar dysfunction including ataxia, dysarthria and nystagmus. Brain magnetic resonance imaging (MRI) revealed symmetrical pathological signal changes in both cerebellar hemispheres. CONCLUSION: We demonstrated the first child with MIS-C to develop cerebellar lesion on brain MRI, suggestive of cerebellitis.

C oronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) leads to substantial morbidity and mortality in adults. Although, COVID-19 is more likely to be asymptomatic or has a mild to moderate disease course in children, severe cases and multisystem inflammatory syndrome in children (MIS-C) were reported as well. Serious complications may occur in conjunction with both acute SARS CoV-2 infection and MIS-C. 1, 2 However, there is increasing report that COVID-19 may also lead to neurologic manifestations. Headache, meningism/meningitis, encephalopathy/encephalitis, seizures, acute disseminated encephalomyelitis, acute necrotizing hemorrhagic encephalopathy, cerebellar ataxia, postinfectious brainstem encephalitis, myositis, global proximal muscle weakness, Guillain-Barre syndrome, bulbar palsy and anosmia have been defined as the neurologic manifestations of SARS-CoV-2 in children. [3] [4] [5] [6] [7] Herein, we aim to report a child with cerebellitis associated with SARS-CoV-2.

A 3-year-old previously healthy child presented with fever for 10 days. He had rash, nausea-vomiting, diarrhea and altered mental status for the last 2 days. He did not have COVID-19 contact history. He was admitted to pediatric intensive care unit with a provisional diagnosis of MIS-C. His body temperature was 38.6°C, heart rate was 157 beats/min, blood pressure was 73/43 mm Hg, respiratory rate was 44 breaths/min and oxygen saturation was 95% at room air, on admission. He was unconscious and disoriented, Glasgow Coma Scale was 11 (E 3, V 4 and M 4), both pupils were reactive to light. Meningeal irritation signs were positive. He had a diffuse retiform purpura localized over the lower extremities and chilblain-like acral lesion, bilateral conjunctivitis, papillitis of the tongue, lip cracking and fissuring. He was tachycardic with a 2/6 systolic murmur and lung auscultation revealed bilateral widespread crackles and decreased breath sounds. He was intubated because of respiratory failure and mechanical ventilation commenced. SARS CoV-2 real-time reverse transcription polymerase chain reaction (RT-PCR) on nasopharyngeal swab was negative. Laboratory findings showed elevated inflammatory markers along with positive SARS-CoV-2 antibody testing, fulfilling the criteria for MIS-C (Table 1 ). Brain magnetic resonance imaging (MRI) was normal (Fig. 1 ). Background electroencephalogram (EEG) activity was abnormal. Diagnostic lumbar puncture was performed successfully. Cerebrospinal fluid (CSF) analysis did not reveal pleocytosis, protein level was 44 mg/dl, and glucose level was 35 mg/dl when serum glucose level was 55 mg/dl. SARS-CoV-2 PCR was negative in CSF. Echocardiography (ECHO) showed ventricular systolic dysfunction and left ventricular ejection fraction was 30% with mitral insufficiency. Fluid replacement therapy, milrinone (0.5μg/kg/min), adrenaline and noradrenaline infusions were started because of hypotension. Antimicrobial therapy was initiated as cefotaxime, vancomycin and acyclovir. He received intravenous immunoglobulin (1 g/kg for 2 days) and high-dose corticosteroids (30 mg/kg for 5 day) followed by a prednisone taper. On day 5, the respiratory and hemodynamical parameters were stabilized and inotrope infusions were weaned off. CSF culture was sterile. Meningitis/encephalitis panel (Cryptococcus neoformans/Cryptococcus gattii, Cytomegalovirus, Enterovirus, Escherichia coli K1, Haemophilus influenza, Herpes simplex virus 1, Herpes simplex virus 2, Human herpesvirus 6, Varicella zoster virus, Human parechovirus, Listeria monocytogenes, Neisseria meningitides, Streptococcus agalactiae and Streptococcus pneumonia) was negative. On day 6, he was extubated and control ECHO was normal. His consciousness improved but he developed clinical signs of cerebellar dysfunction including ataxia, dysarthria and nystagmus. Brain MRI was repeated and revealed symmetrical pathological signal changes in both cerebeller hemispheres, suggesting diffusion restriction (Fig. 1) . Prednisone continued as 2 mg/kg/day. The patient improved gradually and was discharged on day 16 with prednisone and aspirin (100 mg/day) treatments. He remained symptom-free and at his cognitive baseline at 1-month follow-up after discharge.

We, herein, report a previously healthy child who met the criteria for MIS-C and developed reversible encephalopathy with EEG disorganization and bilateral cerebellar lesions, which improved with intravenous immunoglobulin and steroid.

In a multinational, multicenter, collaborative study revealing neuroimaging manifestations in children with SARS-CoV-2 and encephalopathy; the most common findings were acute disseminated encephalomyelitis-like changes of the brain, myelitis and neural enhancement. Cerebrovascular complications in children were rare according to adults. Splenial lesions and myositis were predominantly observed in children with MIS-C. 8 Akcay et al 7 reported two children with acute disseminated encephalomyelitislike disease presented with encephalopathy. Bektas et al 9 published a case series on two children who had MRI changes involving the splenium of the corpus callosum and who presented with fever, rash and shock. Reversible lesions of the corpus callosum have been and DWI (C) sequences in the cranial MRI examination of the case taken during the first admission no pathology was observed. However, in the control cranial MRI taken 6 days later, symmetrical pathological signal changes were observed in T2W (D) and FLAIR (E) sequences in both cerebral hemispheres, and these lesions showed restriction in DWI (F). MRI indicates magnetic resonance imaging.

Novel coronavirus disease (COVID-19) in children

Multi-system inflammatory syndrome in children & adolescents (MIS-C): a systematic review of clinical features and presentation

Neurological issues in children with COVID-19

Neurological manifestations of COVID-19 associated multi-system inflammatory syndrome in children: a systematic review and meta-analysis

Caring for critically Ill children with suspected or proven coronavirus disease 2019 infection: recommendations by the Scientific Sections' Collaborative of the European Society of Pediatric and Neonatal Intensive Care

Axonal Guillain-Barre syndrome associated with SARS-CoV-2 infection in a child

COVID-19-associated acute disseminated encephalomyelitis-like disease in 2 children

Neuroimaging manifestations in children with SARS-CoV-2 infection: a multinational, multicentre collaborative study

Reversible splenial lesion syndrome associated with SARS-CoV-2 infection in two children

Acute fulminant cerebellitis in children with COVID-19 infection: a rare but treatable complication

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