Longitudinal extensive transverse myelitis with cervical epidural haematoma following dengue virus infection

doi:10.1016/j.ejpn.2016.01.012

European Journal of Paediatric Neurology

Volume 20, Issue 3, May 2016, Pages 449-453

https://doi.org/10.1016/j.ejpn.2016.01.012 Get rights and content

Highlights

•
First reported post-dengue paediatric longitudinal extensive transverse myelitis.
•
Post-dengue epidural haematoma can have a good outcome without surgical decompression.
•
Our report widens the neurological manifestation following dengue infection.
•
Prompt immunomodulatory treatment needs to be given in parainfectious dengue LETM.

Abstract

Background

Longitudinal extensive transverse myelitis associated with dengue infection is rare with no reported paediatric cases.

Methods

We report a 12-year-old girl who presented with flaccid quadriplegia 8 days after onset of acute dengue fever. MRI spine showed T2 hyperintensity associated with epidural hematoma at C3–C6 level of the spinal cord. Transcranial magnetic brain stimulation revealed absent motor evoked potentials bilaterally. We also summarise and compare the reported cases of transverse myelitis associated with dengue infection.

Results

Immunomodulatory treatment was given which included pulse methylprednisolone, intravenous immunoglobulin and plasmapharesis. Six months post-admission, there was a good (near-complete) clinical recovery with the repeat MRI showing mild residual hyperintensity at C4 level and complete resolution of epidural haematoma.

Conclusion

This is the first reported paediatric case of longitudinal extensive transverse myelitis following dengue infection. It is also the first to illustrate that in patients with concomitant epidural haematoma a good outcome is possible despite not having surgical decompression. Clinicians should be aware of parainfectious dengue-related longitudinal extensive transverse myelitis in children and consider prompt immunomodulatory treatment.

Introduction

Dengue is the most common mosquito borne disease in the world with an estimated 50 million people affected by dengue each year.¹ Dengue virus infection is known to be associated with neurological manifestations including encephalopathy, acute disseminated encephalomyelitis (ADEM), encephalitis, Guillain-Barre syndrome and transverse myelitis (TM).2, 3

Acute TM is a spinal cord syndrome with a relatively abrupt onset of motor, sensory, and sphincter disturbances that is usually attributed to an inflammatory demyelinating lesion. Approximately 20% of acute TM cases occur in children.⁴ Longitudinally extensive transverse myelitis (LETM) refers to a spinal lesion that extends at least three vertebral segments.⁵ TM is classified broadly as: (i) demyelination – including monofocal clinical isolated syndrome or part of multifocal demyelinating disease including ADEM or multiple sclerosis; (ii) associated with systemic connective tissue disease; (iii) infectious and (iv) idiopathic of which the majority are presumably parainfectious in aetiology.⁶

Spinal magnetic resonance imaging (MRI) plays a crucial role in making the diagnosis of TM. In addition, transcranial magnetic brain stimulation (TMS) used to measure central motor conduction time (CMCT) is also a useful non-invasive investigation to assess the integrity of the corticospinal tract. Prolonged CMCT has been demonstrated in patients with TM.⁷ There has only been one published report to date showing the utility of CMCT in a paediatric TM patient.⁸

TM associated with dengue infection is uncommon with only 10 published case reports to date of which only one was in a paediatric patient aged 14 years.9, 10, 11, 12, 13, 14, 15, 16, 17 Of these 10 published cases, LETM was even rarer with only 5 adult cases of LETM reported (age range 31–45-years old) and 2 of these having concomitant spinal epidural haematoma.9, 10, 11, 12 There were no reported paediatric cases of LETM or spinal epidural hematoma associated with dengue infection.

We present a 12-year-old girl with LETM and concomitant cervical epidural hematoma following dengue infection. We also show the utility of serial non-invasive TMS in this patient and summarise the reported cases of TM associated with dengue infection.

Section snippets

Case report

A previously healthy 12-year-old girl was transferred to our tertiary unit from a local hospital on day 9 of illness with acute lower limb paraparesis and dysesthesia of her chest wall. She was diagnosed as acute dengue fever at the local hospital after a 6-day history of pyrexia and myalgia. Her full blood count at day 6 showed haemoglobin of 18 g/dL (elevated haematocrit of 46.8), leucopenia (2.7 × 10⁹/L), thrombocytopenia (32 × 10⁹/L) and positive dengue IgM serology. There was no evidence

Discussion

This is the first reported paediatric case of dengue virus associated LETM and expands the parainfectious causes of paediatric LETM. Our case also reiterates the clinical importance of differentiating between TM and LETM as LETM is associated with a number of specific conditions which has important diagnostic, prognostic and therapeutic implications.¹⁸ The most frequent cause of LETM is neuromyelitis optica (NMO) which requires long-term immunosuppression to prevent relapses.¹⁸ In cases with a

Conclusion

Our case report is the first paediatric case of LETM with dengue infection and expands the parainfectious causes of paediatric LETM. Our report is also the first to illustrate that in post-dengue infection patients with concomitant spinal epidural haematoma surgical intervention may not always be required. Clinicians should be aware of parainfectious dengue-related LETM in children presenting with acute weakness beyond the acute febrile phase of dengue infection and consider prompt

Competing interests

All authors have nothing to declare.

Funding

All authors have nothing to declare.

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Longitudinally extensive transverse myelitis with positive aquaporin-4 IgG associated with dengue infection: a case report and systematic review of cases
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Citation Excerpt :
She was started on Rituximab for long-term management and remains relapse-free at 21 months. Of 570 unique articles from the literature, 22 publications describing 27 patients met inclusion criteria as summarized in Figure 2. (Chanthamat and Sathirapanya, 2010; Chaudhry et al., 2018; Fong et al., 2016; Kunishige et al., 2004; Lana-Peixoto et al., 2018a; Lana-Peixoto et al., 2018b; Landais et al., 2019; Larik et al., 2012; Leao et al., 2002; Malik et al., 2018; Miranda de Sousa et al., 2006; Mo et al., 2016; Mota et al., 2017; Puccioni-Sohler et al., 2017; Puccioni-Sohler et al., 2009; Seet et al., 2006; Singh et al., 2019; Soares et al., 2006; Tan and Lim, 2019; Tomar et al., 2015; Weeratunga et al., 2014; Yamamoto et al., 2002) Including our reported patient, our series was comprised of a total of 28 patients as detailed in Table 2.
: Neuromyelitis Optica Spectrum Disorder can be associated with parainfectious and post-infectious triggers. Dengue virus infection is one of the most common arbovirus infections in the world, and may present with neurological manifestations.
: We present a case of DENV-associated with LETM and positive aquaporin-4 IgG, and a systematic review of published cases.
: Medline (Ovid) and PubMed were search through June 2021, for case reports, series and observational studies that described patients with DENV-associated LETM and/or NMOSD.
: An adolescent girl who had recently immigrated from a Dengue-endemic region presented with a LETM with high positive AQP4-IgG titer and seropositive DENV IgM/IgG antibodies. She responded well to steroids and subsequently started maintenance rituximab for her NMOSD diagnosis. Literature review: 22 publications describing 27 patients met inclusion criteria. In addition to this case, three published cases met current criteria for NMOSD with serological evidence of acute DENV infection.
: It is unknown whether there is a pathophysiological association between DENV infection and NMOSD. Regardless, if an immune-mediated event is suspected, particularly NMOSD, appropriate immunotherapy should be considered early. Decision regarding long term immunotherapy may depend on index of suspicion of true NMOSD, and this is where AQP4-IgG status and follow-up is helpful.
Spinal Epidural Hematoma Post Evacuation of Spontaneous Spinal Intradural Hematoma
2020, World Neurosurgery
Citation Excerpt :
Both intradural and extradural hematomas can occur after dengue fever.2 Myelitis has a rare association with dengue fever, usually as transverse myelitis and rarely as longitudinal myelitis.3 To date, only 1 pediatric and 2 adult cases of dengue fever associated with spinal hematoma and concomitant longitudinal extensive myelitis or extensive cord edema have been reported.3
Spinal hematomas are rarely associated with dengue syndrome and usually occur at the time of active dengue fever. Late presentation after recovery from dengue fever, intradural hematoma, presentation as a multiloculated cystic lesion with longitudinal extensive myelitis, and recurrence after surgery are rarely or not described. Due to the peculiar association of all these findings, we report this case to provide insight into the existence of such a rare presentation.
A 79-year-old-male developed sudden-onset paraparesis after 1 week of recovery from dengue fever. The blood counts were normal. Magnetic resonance imaging of the thoracic spine was suggestive of intradural hematoma. The patient underwent emergency decompression and drainage of hematoma with recovery in the neurologic status over the next few weeks. He presented to our emergency department after 5 weeks of the first surgery with deterioration in the neurologic status to complete paraplegia. Repeat magnetic resonance imaging showed a posterior epidural collection bulging anteriorly, causing cord compression. The patient was reoperated on by decompression. There was no neurologic recovery. The patient was managed with multidisciplinary rehabilitation, and he was independent in most of the activities at the time of discharge.
Spinal hematoma should be kept in mind in patients who present with neurologic complications after dengue fever. It can have an atypical radiologic presentation and may present with recurrent hemorrhage after surgery. Attention should also be given to delayed presentation of neurologic complications, which may develop even after weeks of recovery from dengue fever.
Increased risk of autoimmune diseases in dengue patients: A population-based cohort study
2018, Journal of Infection
Citation Excerpt :
The immunopathogenesis of dengue includes antibody-dependent enhancement, cellular immune response, soluble factors (such as cytokines and complements), and autoantibodies,3,4 which also play significant roles in the development of other autoimmune diseases. Some cases of dengue-associated autoimmune diseases have been reported, such as systemic lupus erythematosus (SLE), systemic vasculitis, acute disseminated encephalomyelitis (ADEM), neuromyelitis optica, transverse myelitis and subacute thyroiditis.5–10 However, the clinical spectrum of autoimmune presentation and the risk of autoimmune diseases in dengue patients remains unclear.
To investigate the risk of autoimmune diseases in dengue patients.
We conducted a population-based cohort study by the Taiwan National Health Insurance Research Database, including a total of 12,506 newly diagnosed dengue patients and 112,554 control subjects between 2000 and 2010, matched by gender, age, income, urbanization, and comorbidities. Both cohorts were followed for a 3-year period to examine the incidence of autoimmune diseases. A Cox proportional hazards regression analysis was applied to calculate the risk of autoimmune diseases between both groups.
The dengue group showed an overall increased risk for 21 autoimmune diseases, with an adjusted hazard ratio (aHR) of 1.88 (95% confidence interval [CI], 1.49–2.37, p < 0.001). Compared with the control group, the dengue group had higher risks of Reiter's syndrome (aHR 14.03, 95 % CI 1.63–120.58), multiple sclerosis (aHR 11.57, 95 % CI 1.8–74.4), myasthenia gravis (aHR 5.35, 95 % CI 1.43–20.02), autoimmune encephalomyelitis (aHR 3.8, 95% CI 1.85–7.8), systemic vasculitis (aHR 3.7, 95 % CI 1.11–12.28), systemic lupus erythematosus (aHR 3.5, 95% CI 1.85–6.63), and primary adrenocortical insufficiency (aHR 2.05, 95% CI 1.25–3.35).
Dengue patients were associated with an increased risk of autoimmune diseases.
Myelitis and cauda equina involvement following dengue fever. A case report and review of the literature
2018, Multiple Sclerosis and Related Disorders
Dengue fever (DF) is a common arbovirosis in tropical and subtropical countries and may be associated with a wide range of neurological complications. We describe a 41-year-old man who developed weakness in the right arm and lower limbs, paresthesia in the upper and lower limbs, and sphincter disturbance four weeks following DF. Examination disclosed a wheel-chair bound patient with urinary catheter, areflexia in the lower limbs, and a sensation level at T10. Spinal magnetic resonance imaging showed diffuse lesions with contrast-enhanced areas extending from the medullary-cervical junction to the conus medullaris and cauda equina. A review of the literature reveals that this is the first report of clinical and imaging signs of myeloradiculitis with cauda equina involvement following DF infection.
Re-examination of the risk of autoimmune diseases after dengue virus infection: A population-based cohort study
2023, PLoS Neglected Tropical Diseases
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View all citing articles on Scopus

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Case StudyLongitudinal extensive transverse myelitis with cervical epidural haematoma following dengue virus infection

Highlights

Abstract

Background

Methods

Results

Conclusion

Introduction

Section snippets

Case report

Discussion

Conclusion

Competing interests

Funding

Travel Med Infect Dis

J Clin Virol

Clin Neurophysiol

Dengue guidelines for diagnosis, treatment, prevention and control

Neurological manifestations in dengue patients

Southeast Asian J Trop Med Public Health

Post-dengue encephalopathy and parkinsonism

Pediatr Infect Dis J

Evidence-based guideline: clinical evaluation and treatment of transverse myelitis. Report of the therapeutics and technology assessment subcommittee of the American Academy of Neurology

Neurology

Pediatric acute transverse myelitis overview and differential diagnosis

J Child Neurol

An approach to the diagnosis of acute transverse myelitis

Semin Neurol

Central motor conduction studies in patients with spinal cord disorders: a review

Spinal Cord

Neurophysiological studies in acute transverse myelitis

J Neurol

Case Study
Longitudinal extensive transverse myelitis with cervical epidural haematoma following dengue virus infection