CASE 17637 Published on 18.02.2022

COVID-19-related Guillain-Barré Syndrome variant with multiple cranial neuropathies in a child

Section

Neuroradiology

Case Type

Clinical Cases

Authors

Héber Samuel Colares Costa1, Pedro Neves Paiva de Castro1, Nina Ventura2, Lucas C. Leite1, Caio, Tasso Oliveira Rego1, Roberto Queiroz dos Santos1, Dequitier Carvalho Machado1, Eduardo José Berardo Zaeyen1

1. United Health Group, Americas Medical Services, Barra da Tijuca, Rio de Janeiro, Brazil

2. Federal University of Rio de Janeiro, Rio de Janeiro, Brazil

Patient

3 years, female

Categories
Area of Interest Neuroradiology brain, Neuroradiology spine ; Imaging Technique MR
Clinical History

A 3-year-old female with no relevant previous clinical history presented with flu-like symptoms. After one week, she manifested with lameness, ataxia, bilateral facial paralysis, ophthalmoplegia, and diplopia. The cerebrospinal fluid (CSF) analysis showed albumin-cytologic dissociation. There was positive IgG and IgM COVID-19 in the third week after the initial symptoms. 

Imaging Findings

Brain and spine magnetic resonance imaging (MRI) exhibited bilateral thickening and contrast enhancement of multiple cranial nerves, notably the olfactory (I), oculomotor (III), trigeminal (V), and facial (VII) nerves (Figs. 1, 2, and 3). In addition, thickening and contrast enhancement of the spinal roots was also observed, including adjacent to the medullary cone (Figs. 4 and 5). 

Discussion

Guillain-Barré syndrome (GBS) has been reported in association with Coronavirus disease-2019 (COVID-19) in several cases, mainly in adults [1,2]. However, the pediatric age group involvement is rare, with few reports in the literature [3,4].

GBS diagnosis depends on the clinical picture, electrophysiological and CSF analysis. The patients present symmetrical ascending motor manifestations with or without sensitive or cranial nerves involvement [5]. Electroneuromyography confirms the GBS subtype [6], in which our case demonstrates acute inflammatory demyelinating polyradiculoneuropathy.

MRI could support the diagnosis: the spinal root involvement was present, with thickening and intense gadolinium enhancement of anterior and posterior spinal roots, compatible with inflammatory radiculopathy [6]. Also, imaging demonstrated the involvement of the olfactory, oculomotor, trigeminal, abducens, and facial nerves indicating multiple cranial inflammatory neuropathies.

CSF fluid can show the classic albumin-cytologic dissociation [7], which reflects the CSF findings, demonstrating an elevation of protein level (231,3 mg/dL in the case) and a negative white cell count. Since several pathogens can trigger GBS, such as bacteria and viruses [8], excluding these infections is essential. There was no positive polymerase chain reaction (PCR) or serology positive findings in the CSF.

The diagnosis of COVID-19 was confirmed with positive IgG and IgM in the third week after the initial symptoms. After eight weeks of symptoms, there was an increase in serum IgG levels and a reduction in IgM, corroborating the serological pattern for SARS-CoV2 infection.

Two pathogenic mechanisms have been proposed for the COVID-19 and GBS associations. Since SARS-CoV-2 RNA could be undetectable in the CSF during neurological manifestations [9], the GBS spectrum's most likely etiological mechanism is the post-infectious immune-mediated [10]. Previous diseases could produce antibodies with molecular mimicry that could bind on the surface of neurons and myelin membrane, triggering the syndrome [1,11].

However, a case report demonstrated positive RT-PCR for SARS-CoV-2 in the CSF analysis [4], reinforcing the possibility of direct invasion in the SNC: the second COVID-19-neuropathy mechanism proposed [10]. The virus could infect respiratory epithelial cells and neuronal receptors, mainly through the olfactory bulbs, trigeminal nerves, and brain stem [10].

The most accepted treatment of GBS is intravenous immunoglobulin or plasma exchange [12]. Even in the context of GBS triggered by SARS CoV-2, literature shows improvement with a single course of intravenous immunoglobulin in eight weeks [13]. The child presented a partial response after the second dose of immunoglobulin and subsequent improvement.

Differential Diagnosis List
COVID-19-related Guillain-Barré Syndrome variant with multiple cranial neuropathies
Lymphoma and leukaemia
Chronic inflammatory demyelinating polyneuropathy
Lyme disease
Charcot-Marie-Tooth syndrome
Dejerine-Sottas disease
Final Diagnosis
COVID-19-related Guillain-Barré Syndrome variant with multiple cranial neuropathies
Case information
URL: https://www.eurorad.org/case/17637
DOI: 10.35100/eurorad/case.17637
ISSN: 1563-4086
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