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Table of Contents
CASE REPORT
Year : 2017  |  Volume : 54  |  Issue : 3  |  Page : 291-293

Longitudinally extensive transverse myelitis (LETM) in a case of Japanese encephalitis with an unexpected complication


1 Department of Medicine, All India Institute of Medical Sciences, New Delhi, India
2 Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India

Date of Submission02-Mar-2017
Date of Acceptance27-Jun-2017
Date of Web Publication7-Nov-2017

Correspondence Address:
Animesh Ray
Assistant Professor, Room No. 3070, III Floor, Department of Medicine, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-9062.217623

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Keywords: Diaphragmatic palsy; Flavivirus; quadriparesis


How to cite this article:
Mohta S, Ray A, Sharma S K, Vyas S. Longitudinally extensive transverse myelitis (LETM) in a case of Japanese encephalitis with an unexpected complication. J Vector Borne Dis 2017;54:291-3

How to cite this URL:
Mohta S, Ray A, Sharma S K, Vyas S. Longitudinally extensive transverse myelitis (LETM) in a case of Japanese encephalitis with an unexpected complication. J Vector Borne Dis [serial online] 2017 [cited 2019 Aug 17];54:291-3. Available from: http://www.jvbd.org/text.asp?2017/54/3/291/217623

Japanese encephalitis (JE) is a mosquito borne disease caused by a virus belonging to the Flaviviridae family. It is endemic in northern India and generally occurs in the monsoon season. Even though vaccination has been introduced in India, there’s not much reduction in the incidence[1]. It causes acute encephalitis syndrome and has been rarely associated with myelitis. The disease has a high mortality and is associated commonly with long term neurological sequelae.

Case definition of a suspected case of acute encephalitis syndrome (AES) is described as a case of acute onset fever (5–7 days) with change in mental status with or without seizures[2]. Longitudinally extensive transverse myelitis is a variant of acute transverse myelitis (ATM) which involves three or more vertebral segments. It is most commonly associated with neuromyelitis optica (NMO)[3].

We report a case of a young male with altered sensorium and quadriparesis, and diagnosed to have JE with longitudinally extensive transverse myelitis (LETM).

Case Report

A 35-yr-old male resident of Delhi presented with a history of fever and altered sensorium for five days, and two generalised tonic-clonic convulsions. There was no history of recent travel or vaccination. On examination, the pulse rate was 84/min, blood pressure was 130/90 mmHg and glasgow coma score (GCS) was E1VTM1. Neurological examination revealed generalised hypotonia, weakness and areflexia. Plantars were mute and there were no signs of meningeal irritation. No rash or eschar was visible. After a lumbar puncture, he was started on antibiotics (according to meningitis protocol), acyclovir; antimalarials (artesunate) and antiepileptics (levetirace-tam). A clinical diagnosis of suspected AES was made with pending investigations.

Routine haematological, biochemical and metabolic parameters were normal. Cerebrospinal fluid (CSF) showed 40 ×106 leucocytes/l (55% lymphocytes and 45% polymorphs), sugar and protein of 4.44 mmol/l (corresponding blood sugar of 6.11 mmol/l) and 1.10 g/l respectively. Serum tests were negative for malaria, dengue, chikungunya, scrub typhus and HIV. The CSF was found negative for herpes virus, cryptococcal antigen and tuberculosis (gene Xpert test). However, both the serum and CSF samples were found to be positive for IgM against Japanese B virus. Accordingly, the patient was managed for JE.

MRI of the brain revealed features of encephalitis [Figure 1]. Over the next two days, the patient’s fever subsided and sensorium gradually improved to a GCS of E4VTM1. His quadriplegia and areflexia however persisted. A nerve conduction velocity and electromyography were normal, but the MRI spine showed cord oedema and altered signal involving segments from C2 to C6 level [Figure 2]. With the diagnosis of transverse myelitis, the patient was given a pulse of methylprednisolone for five days.
Figure 1: MRI of brain showing altered signal intensity involving the basal ganglia and thalami (bilaterally) appearing hyperintense on T2W image (a); with restricted diffusion on diffusion weighted imaging (DWI) (b); and apparent diffusion coefficient (ADC) map (c).

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Figure 2: T2 weighted sagittal section of the MRI cervical spine with cord swelling and altered signal involving segments from C2 to C6 level.

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The patient remained on mechanical ventilation however, the muscle power did not show significant improvement and the patient succumbed to ventilator acquired pneumonia two weeks later.


  Discussion Top


Japanese encephalitis causes an array of neurological manifestations and does not have specific antiviral therapy. The mortality rate in India varies from 20–40%[4] . Clinical manifestations includes fever, altered sensorium, seizures, movement disorders and brainstem involvement[5]. Rare findings include paralysis, dystonia and dysarthria[6]. Multiple seizures, respiratory pattern changes, flexor or extensor posturing and abnormalities of the papillary and occulocephalic reflexes are associated with poorer prognosis[7].

The diagnosis was done by using JE specific IgM antibody capture enzyme linked immunosorbent assay (MAC-ELISA) as recommended by WHO[8]. IgM against the virus is detected in CSF by four days and in serum by

7–9 days after clinical illness[9]. It may show cross reactivity with other Flavivirus, but presence of CSF antibody is a more accurate way to confirm CNS infection of the virus; as in the present case. Also, the test for dengue was negative.

The patient’s sensorium improved, but the quadriparesis persisted likely due to myelitis. He could not be weaned-off mechanical ventilation, as he continued to have type-II respiratory failure, likely due to the bilateral diaphragmatic palsy caused by cervical cord involvement. Even with steroids, the patient did not show significant improvement in muscle power over the first week, and developed ventilator acquired pneumonia. The high dose of steroids may have predisposed the patient to super added infection. The role of steroids in idiopathic transverse myelitis is well documented[10], but there are few such records in case of acute infectious myelitis.

Acute transverse myelitis, an inflammatory myelitis, has been reported to be associated with autoimmune, infectious and inflammatory pathologies[11]. When associated with acute encephalitis or encephalopathy, the differential diagnoses include non-infectious causes like acute disseminated encephalomyelitis (ADEM), NMO, post-vaccination encephalomyelitis, neurolupus, extraglandular Sjogren’s syndrome and rarely neoplasms. Infectious causes which must be considered are mostly viruses (including herpes family, flaviviridae, enterovirus and retrovirus), cerebral malaria, neurocysticercosis, CNS aspergillosis and rarely bacterial infections. These causes were excluded in this case systematically, till a diagnosis was confirmed. Association of ATM with JE is rare, and to the best of our knowledge it is only the third case where it has been documented[6],[12]. Both the earlier described cases showed signal alternations in the cervical region of spinal cord and were treated by a short course of intravenous methylprednisolone. One of the cases was of the parainfectious variety likely due to autoimmunity, which occurred after three weeks of infection while the second was in the acute phase of illness which may indicate an alternate pathogenesis, possibly acute infectious myelitis. Hence, myelitis may present in the acute phase or few weeks later in the parainfectious form. Motor weakness in JE has been observed and even quadriparesis is known which has been shown to occur due to anterior horn cell damage[13]. In the present case anterior horn cell damage was ruled out by the normal electromyography (EMG) and nerve conduction velocity (NCV), and by the abnormalities seen in MRI.

The present case is also unique, for the way, the patient required prolonged mechanical ventilation due to his diaphragmatic involvement, and this complication led to a change in the patient’s clinical outcome.

In conclusion, Japanese B virus infection can cause spinal cord involvement in the form of LETM with a predilection for the cervical cord, even in the acute phase as observed in this as well as other reported cases[12]. Associated complications like diaphragmatic palsy may also occur. The current treatment for myelitis remains early initiation of high dose steroids (when specific antibiotic therapy is not available) but further research is required to determine its efficacy and role of other modalities like plasma exchange, cyclophosphamide, etc.

Conflict of interest

There is no conflict of interests to declare.

Ethical statement

Consent was taken from the patient’s kin for the treatment administered and for publication of the case.

 
  References Top

1.
Annual Report 2014-15. Delhi: National Vector Borne Disease Control Programme 2015. Available from: http://www.nvbdcp. gov.in/Doc/Annual-report-NVBDCP-2014-15.pdf (Accessed on February 20, 2017).  Back to cited text no. 1
    
2.
Clinical management of acute encephalitis syndrome including Japanese encephalitis. Delhi: National Vector Borne Disease Control Programme 2009. Available from: http://nvbdcp.gov.in/ Doc/Revised%20guidelines%20on%20AES_JE.pdf (Accessed on February 25, 2017).  Back to cited text no. 2
    
3.
Trebst C, Raab P, Voss EV, Rommer P, Abu-Mugheisib M, Zettl UK, et al. Longitudinal extensive transverse myelitis—It’s not all neuromyelitis optica. Nat Rev Neurol 2011; 7: 688–98.  Back to cited text no. 3
    
4.
Misra UK, Kalita J, Goel D, Mathur A. Clinical, radiological and neurophysiological spectrum of JEV encephalitis and other nonspecific encephalitis during post-monsoon period in India. Neurol India 2003; 51: 55–9.  Back to cited text no. 4
    
5.
Sarkari NBS, Thacker AK, Barthwal SP, Mishra VK, Prapann S, Srivastava D, et al. Japanese encephalitis (JE). Part I: Clinical profile of 1282 adult acute cases of four epidemics. J Neurol 2012; 259: 47–57.  Back to cited text no. 5
    
6.
Verma R, Praharaj HN, Patil TB, Giri P. Acute transverse myelitis following Japanese encephalitis viral infection: An uncommon complication of a common disease. BMJ Case Rep 2012; 2012: pii: bcr2012007094.  Back to cited text no. 6
    
7.
Solomon T. Flavivirus encephalitis. N Engl J Med 2004; 351: 370–8.  Back to cited text no. 7
    
8.
Manual for the laboratory diagnosis of Japanese encephalitis virus infections. Geneva, Switzerland: World Health Organization 2007. Available from: http//www.wpro.who.int/immunization/documents/Manual_lab_diagnosis_JE.pdf (Accessed on February 25, 2017).  Back to cited text no. 8
    
9.
Johnson BW, Goodman CH, Jee Y, Featherstone DA. Differential diagnosis of Japanese encephalitis virus infections with the inbios je detect(TM) and DEN detect(TM) MAC-ELISA kits. Am J Trop Med Hyg 2016; 94(4): 820–8.  Back to cited text no. 9
    
10.
Awad A, Stüve O. Idiopathic transverse myelitis and neuromyelitis optica: Clinical profiles, pathophysiology and therapeutic choices. Curr Neuropharmacol 2011; 9(3): 417–28.  Back to cited text no. 10
    
11.
Jacob A, Weinshenker BG. An approach to the diagnosis of acute transverse myelitis. Semin Neurol 2008; 28(1): 105–20.  Back to cited text no. 11
    
12.
Ankur Nandan V, Nilesh K, Dibyaranjan B, Ashutosh T, Ravi A, Arvind A. Acute transverse myelitis (ascending myelitis) as the initial manifestation of Japanese encephalitis: A rare presentation. Case Rep Infect Dis 2013; 2013: 1–3.  Back to cited text no. 12
    
13.
Misra UK, Kalita J. Anterior horn cells are also involved in Japanese encephalitis. Acta Neurol Scand 1997; 96(2): 114–7.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2]


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