Longitudinally extensive transverse myelitis and meningitis due to a rare infectious cause

Abstract

DESCRIPTIONA previously healthy 9-year-old girl was admitted to the emergency department of a district hospital due to persistent headache for 3 days. Somnolence, fever and meningismus were noticed. No previous infections or recent immunisations were reported. The child had leucocytosis with elevated C reactive protein. Cerebrospinal fluid (CSF) analysis showed pleocytosis of 96 cells, 73% of lymphocytes, with negative bacteriological, enterovirus and herpes simplex virus screen. Ceftriaxone was started. Owing to persistent fever and headache, a brain CT scan was performed, showing no abnormalities. Focal and generalised seizures started by the ninth day of disease, followed by right-sided Todd hemiparesis. A second lumbar puncture showed increased pleocytosis (316 cells, 66% of lymphocytes). Acyclovir and vancomycin were added and the patient was transferred to our hospital with the diagnostic hypothesis of meningoencephalitis. On admission, she presented a normal level of consciousness, and the physical examination showed flaccid paraparesis, pain and vibratory hypoesthesia below T2 and bilateral cervical adenopathy. Ciprofloxacin was added. Brain MRI revealed leptomeningeal enhancement with no basal ganglia or thalami involvement. Spinal cord MRI showed a longitudinally extensive transverse myelitis (figures 1 and 2). Neoplastic and autoimmune causes were ruled out, including negative neuromyelitis optica IgG (NMO-IgG). The extensive microbiological screening revealed positivity for Epstein-Barr virus (EBV) DNA in blood (6.5×10 3 copies of EBV DNA/mL) and in CSF (900 copies of EBV DNA/ mL), with positive early EBV IgG and borderline EBV IgM. Treatment included a 5-day course of immunoglobulin and 28-day course of endovenous ganciclovir followed by oral valganciclovir. The patient gradually improved, regaining ability to walk, though maintaining a neurogenic bladder and left foot drop. Figure 1 Spinal cord MRI, sagittal T2-weighted images showing a hyperintense lesion on T2-weighted sequence, centrally located, occupying more than two-thirds of the cross-sectional area and leading to spinal cord expansion, extending from D2 to D10. There was no significant enhancement with gadolinium. Figure 2 Spinal cord MRI, axial T2-weighted images showing a hyperintense lesion on T2-weighted sequence, centrally located, occupying more than two-thirds of the cross-sectional area and leading to spinal cord expansion, extending from D2 to D10. There was no significant enhancement with gadolinium.

Differential diagnosis of neuromyelitis optica spectrum disorders

“…Though rare, infections such as herpes virus (Herpes simplex, 165 Epstein–Barr virus, 166 and cytomegalovirus 167 ), human T-lymphotrophic virus 1 (HTLV-1), 168 dengue virus, 169 Borrelia burgdorferi (Lyme), 170 tuberculosis, 171 Mycoplasma pneumoniae , 172 and Streptococcus pneumoniae 15 can manifest as LETM and/or optic neuritis.…”

“…Interestingly, though most of the infectious myelitis cases reported absence of AQP4-Ab, 15 , 166 – 173 several cases have shown that NMOSD with AQP4 can develop several days after zoster infection. 174 – 176 These findings might imply at least some pathogenic overlap between these two distinctive diseases of NMOSD and herpes zoster.…”

Comprehensive review of neuromyelitis optica and clinical characteristics of neuromyelitis optica patients in Puerto Rico

“…Syphilis is a sexually transmitted infection that can cause ON that can mimic NMOSD. [ 24 197 210 252 ] Other infections that can mimic NMOSD include Epstein Barr virus,[ 11 ] herpes simplex virus,[ 190 ] cytomegalovirus,[ 267 ] human t-lymphocytic virus,[ 279 ] dengue fever,[ 147 ] lyme disease,[ 174 ] tuberculosis,[ 100 ] Mycoplasma pneumoniae ,[ 80 ] and Streptococcus pneumoniae . [ 238 ]…”

Review of the Etiological Causes and Diagnosis of Myelitis and Its Medical Orientation Protocol

“…HSV-2 produces Elsberg's syndrome [37], EBV can rarely cause TM and is associated with a poor prognosis [38]. Dengue viral infection could cause an ATM episode as a manifestation, despite only four cases having been reported in the literature [39].…”