Acute transverse myelitis is a rare clinical manifestation of Coxsackie virus infection which cause acute and progressive debilitating illness associated with loss of spinal cord function in the affected patients. A 62 year-old female developed symptoms of rapidly progressive paraplegia with sensory loss. On spinal MRI, T2 sagittal image showed increased signal intensity with cord swelling at T11-L2 level and 8 folds or greater rise of Coxsackie virus B4 neutralizing antibody titers was observed in the CSF. There is only one previous report of acute transverse myelitis caused by Coxsackie virus B4 infection to our knowledge. The presence of specific viral antibody titers change in the CSF and a corresponding spinal cord lesion are sufficient to suggest a causal relationship between the virus and the illness. This article is a case report of an unusual acute transverse myelitis caused by Coxsackie virus B4 infection.
Isolated involvement of the spinal cord is an uncommon presentation of neuro-Behçet's disease (NBD) and it is associated with a poor prognosis for functional recovery.
A case report of an 18-year-old Turkish man who presented with a progressive paraparesis and bladder dysfunction secondary to a longitudinally extensive transverse myelitis as the sole presentation of NBD.
Examination revealed a spastic paraparesis and a T7 sensory level. Magnetic resonance imaging revealed multiple enhancing lesions throughout the thoracic cord and cerebrospinal fluid showed intense neutrophilia. On further enquiry a family history of Behçet's disease was elicited. The patient subsequently reported a history of recurrent oral ulceration and intermittent occular inflammation. A diagnosis of NBD was made and intravenous high-dose steroids commenced with poor response. In view of the poor prognosis for functional recovery associated with spinal NBD the patient was treated with infliximab, an anti-tumour necrosis factor-alpha monoclonal antibody, leading to excellent recovery of function.
Early treatment with infliximab may facilitate a favourable functional recovery and should be considered in cases of NBD with spinal cord involvement.
Behçet's disease; Infliximab; Myelitis; Spinal cord; Paraparesis
Transverse myelitis (TM) extending from midbrain to the entire spinal cord accompanied by internuclear ophthalmoplegia is extremely rare but cause serious central nervous system complications in patients with systemic lupus erythematosus. We report a case of a 28-yr-old woman with TM extending from the midbrain to the conus medullaris longitudinally and internuclear ophthalmoplegia associated with systemic lupus erythematosus. Her neurological symptoms had an abrupt catastrophic onset and rapidly progressed to respiratory failure within 24 hr. Bilateral internuclear ophthalmoplegia was also followed by TM. Brain MR images showed definite brainstem lesions, which were deeply associated with internuclear ophthalmoplegia, and diffuse signal changes in the whole spinal cord, medulla, pons and midbrain. Clinical improvement of her ophthalmoplegia and of neurological dysfunction of the upper extremities was noted after prompt and aggressive treatment with intravenous pulsed methylprednisolone and cyclophosphamide. However, the neurological dysfunction of the lower limbs and bladder and colon paralysis were almost unchanged until six months passed.
Lupus Erythematosus, Systemic; Myelitis, Transverse; Internuclear Ophthalmoplegia; Ocular Motility Disorders
Melioidosis has become an emerging infection in Sri Lanka; a country which is considered non endemic for it. Paraplegia due to Burkholderia pseudomallei is a very rare entity encountered even in countries where the disease is endemic. There are no reported cases of transverse myelitis due to melioidosis in Sri Lankan population thus we report the first case.
A 21 year old farmer presented with sudden onset bi lateral lower limb weakness, numbness and urine retention. Examination revealed flaccid areflexic lower limbs with a sensory loss of all modalities and a sensory level at T10 together with sphincter involvement. MRI of the thoracolumbar spine showed extensive myelitis of the thoracic spine complicating left psoas abscess without definite extension to the spinal cord or cord compression. Burkholderia pseudomallei was isolated from the psoas abscess pus cultures and the diagnosis of melioidosis was confirmed with high titers of Burkholderia pseudomallei antibodies and positive PCR. He was treated with high doses of IV ceftazidime and oral cotrimoxazole for one month with a plan to continue cotrimoxazole and doxycycline till one year. Patient’s general condition improved but the residual neurological problems persisted.
The exact pathogenesis of spinal cord melioidosis is not quite certain except in the cases where there is direct microbial invasion, which does not appear to be the case in our patient. We postulate our patient’s presentation could be due to ischemia of the spinal cord following septic embolisation or thrombosis of spinal artery due to the abscess nearby. A neurotrophic exotoxin causing myelitis or post infectious immunological demyelination is yet another possibility. This emphasizes the necessity of further studies to elucidate the exact pathogenesis in this type of presentations.
Health care professionals in Sri Lanka, where this is an emerging infection, need to improve their knowledge regarding this disease and should have high degree of suspicion to make a correct and a timely diagnosis to reduce the morbidity and mortality due to Burkholderia pseudomallei infection. It is highly likely that this infection is under diagnosed in developing countries where diagnostic facilities are minimal. Therefore strategies to improve the awareness and upgrade the diagnostic facilities need to be implemented in near future.
Melioidosis; Transverse myelitis; Burkholderia pseudomallei; Flaccid paraplegia; Psoas abscess
Few cases of Epstein-Barr virus myelitis have been described in the literature. Multi-centric Castleman's disease is a lymphoproliferative disorder that is well known for its associations with the human immunodeficiency virus, human herpes virus 8, and Kaposi's sarcoma. The concurrent presentation of these two diseases in a patient at the same time is extremely unusual.
We describe the case of a 43-year-old Caucasian man with acquired immune deficiency syndrome who presented with fever, weight loss and diffuse lymphadenopathy, and was diagnosed with multi-centric Castleman's disease. He presented three weeks later with lower extremity weakness and urinary retention, at which time cerebrospinal fluid contained lymphocytic pleocytosis and elevated protein. Magnetic resonance imaging demonstrated abnormal spinal cord signal intensity over several cervical and thoracic segments, suggesting the diagnosis of myelitis. Our patient was ultimately diagnosed with Epstein-Barr virus myelitis, as Epstein-Barr virus DNA was detected by polymerase chain reaction in the cerebrospinal fluid.
To the best of our knowledge, this is the first case of multi-centric Castleman's disease followed by acute Epstein-Barr virus myelitis in a human immunodeficiency virus-infected patient. Clinicians caring for human immunodeficiency virus-infected patients should be vigilant about monitoring patients with increasing lymphadenopathy, prompting thorough diagnostic investigations when necessary.
Longitudinally extensive transverse myelitis (LETM) is a neurological condition characterised by a contiguous inflammatory lesion of the spinal cord. LETM is often associated with the autoimmune central nervous system disease neuromyelitis optica (NMO) and rarely with multiple sclerosis. The discovery of the NMO-IgG antibody, provides a useful serological marker of LETM associated with the NMO disease spectrum (LETM and/or optic neuritis). Here, the authors report two cases of LETM, which differ in disease severity and NMO-IgG antibody serological status.
An occurrence of acute localised myelitis was recently
seen in four adult patients with atopic dermatitis who had
hyperIgEaemia and mite antigen specific IgE. The total and mite antigen
specific IgE was therefore studied in serum samples from 19 consecutive patients with acute localised myelitis of unknown aetiology, 56patients with clinically definite multiple sclerosis, and 40 healthy controls. The total IgE concentration was significantly higher in acute
localised myelitis (median=360 U/ml) than in multiple sclerosis
(median=52 U/ml, p<0.0001) and the controls (median=85 U/ml,
p=0.0002). The specific IgE to Dermatophagoides
pteronyssinus was found more often in patients with acute
localised myelitis (95%) than in patients with multiple sclerosis
(34%, p<0.0001) and the controls (35%, p<0.0001) and the specific
IgE to Dermatophagoides farinae was similar (acute
localised myelitis 79%, multiple sclerosis 29% (p<0.0001), controls
30%, (p=0.0003). Atopic dermatitis coexisted more commonly in patients
with acute localised myelitis (37%) than in patients with multiple
sclerosis (0%, p<0.0001) and the controls (7.5%, p=0.0089).
Therefore, acute localised myelitis with hyperIgEaemia, in which atopy
to mite antigens seems to exist, may be a distinct subtype of allergic
myelitis—that is, atopic myelitis.
Recurrent zoster myelitis is quite rare. We present a previously healthy 27-year-old woman who developed recurrent attacks of myelopathy shortly after the characteristic skin rashes of herpes zoster. Magnetic resonance imaging studies demonstrated each lesion in the spinal cord at the same segments as the skin lesions. She had two attacks at opposite sites at the same spinal cord level and complete recovery after being treated with intravenous acyclovir. We suspect that direct invasion of varicella zoster virus was the cause of recurrent myelopathy in our patient.
Introduction: Transverse myelitis is a very rare neurologic syndrome with an incidence per year of 1-5 per million population. We are presenting an interesting case of subacute transverse myelitis with its MRI (magnetic resonance imaging) and CSF (cerebrospinal fluid) findings.
Case: A 46-year-old African-American woman presented with decreased sensation in the lower extremities which started three weeks ago when she had a 36-hour episode of sore throat. She reported numbness up to the level just below the breasts. Lyme disease antibodies total IgG (immunoglobulin G) and IgM (immunoglobulin M) in the blood was positive. Antinuclear antibody profile was within normal limits. MRI of the cervical spine showed swelling in the lower cervical cord with contrast enhancement. Cerebrospinal fluid was clear with negative Borrelia Burgdorferi IgG and IgM. Herpes simplex, mycoplasma, coxiella, anaplasma, cryptococcus and hepatitis B were all negative. No oligoclonal bands were detected. Quick improvement ensued after she was given IV Ceftriaxone for 7 days. The patient was discharged on the 8th day in stable condition. She continued on doxycycline for 21 days.
Conclusions: Transverse myelitis should be included in the differential diagnosis of any patient presenting with acute or subacute myelopathy in association with localized contrast enhancement in the spinal cord especially if flu-like prodromal symptoms were reported. Lyme disease serology is indicated in patients with neurological symptoms keeping in mind that dissociation in Lyme antibody titers between the blood and the CSF is possible.
transverse myelitis; Lyme disease
Vogt-Koyanagi-Harada (VKH) disease is characterized by bilateral granulomatous uveitis with neurologic, auditory, and dermatologic manifestations. However, acute myelitis complicating VKH disease has rarely been reported.
A 50-year-old Chinese Han woman presented with difficulty walking, numbness on the left side of the body, and difficulty with urination. The patient was diagnosed with incomplete VKH disease and received corticosteroid treatment prior to the neurological presentation. Acute myelitis was diagnosed based on both clinical and spinal-cord MRI findings.
Clinicians should consider acute myelitis as a rare possible neurological manifestation in VKH disease patients, and early systemic administration of corticosteroids will suppress the acute inflammatory process and prevent recurrences. This report raises the possibility that VKH disease and acute myelitis share common pathogenic pathways.
Vogt-Koyanagi-Harada disease; acute myelitis; pathogenesis
A case of meningovascular syphilis presenting with the rare spinal cord manifestations is reported. The angiographic, computed tomographic, and magnetic resonance images of the subsequent cerebrovascular lesions are shown. The presentation, evolution, and treatment of this unusual form of vascular syphilis are discussed.
Reovirus infection of the murine spinal cord (SC) was used as a model system to investigate innate immune responses during viral myelitis, including the activation of glia (microglia and astrocytes) and interferon (IFN) signaling and increased expression of inflammatory mediators. Reovirus myelitis was associated with the pronounced activation of SC glia, as evidenced by characteristic changes in cellular morphology and increased expression of astrocyte and microglia-specific proteins. Expression of inflammatory mediators known to be released by activated glia, including interleukin-1β (IL-1β), tumor necrosis factor alpha (TNF-α), chemokine (C-C motif) ligand 5 (CCL 5), chemokine (C-X-C motif) ligand 10 (CXCL10), and gamma interferon (IFN-γ), was also significantly upregulated in the SC of reovirus-infected animals compared to mock-infected controls. Reovirus infection of the mouse SC was also associated with increased expression of genes involved in IFN signaling, including IFN-stimulated genes (ISG). Further, reovirus infection of mice deficient in the expression of the IFN-α/β receptor (IFNAR−/−) resulted in accelerated mortality, demonstrating that IFN signaling is protective during reovirus myelitis. Experiments performed in ex vivo SC slice cultures (SCSC) confirmed that resident SC cells contribute to the production of at least some of these inflammatory mediators and ISG during reovirus infection. Microglia, but not astrocytes, were still activated, and glia-associated inflammatory mediators were still produced in reovirus-infected INFAR−/− mice, demonstrating that IFN signaling is not absolutely required for these neuroinflammatory responses. Our results suggest that activated glia and inflammatory mediators contribute to a local microenvironment that is deleterious to neuronal survival.
Atopic myelitis is defined as myelitis with atopic diasthesis but the cause is still unknown. Toxocariasis is one of the common causes of hyperIgEaemia that may lead to neurologic manifestations. The purpose of this study was to evaluate the sero-prevalence of Toxocara specific IgG Ab among the atopic myelitis patients. We evaluated the medical records of 37 patients with atopic myelitis whose conditions were diagnosed between March 2001 and August 2007. Among them, the 33 sera were analyzed for specific serum IgG Ab to Toxocara excretory-secretory antigens (TES). All of 37 patients had hyperIgEaemia. Specific IgE to D. pteronyssinus and D. farinae was detected in 22 (64.7%) and 34 (100%) patients, respectively, of the 34 patients. Thirty-one of 33 patients (93.9%) were found to be positive by TES IgG enzyme-linked immunosorbent assay (ELISA). Based on the image findings of eosinophilic infiltrations in the lung and liver, 8 patients had positive results. These results inferred that the prevalence of toxocariasis was high in patients with atopic myelitis. Our results suggest that toxocariasis might be an important cause of atopic myelitis and Toxocara ELISA is essential for evaluating the causes of atopic myelitis.
Myelitis; Atopy; Toxocariasis
The efficacy of spinal cord stimulation (SCS) for treatment of various chronic painful conditions is well established. Very few reports have documented the use of SCS for treatment of chronic pain after spinal cord injury. We present a case showing a good outcome after such treatment, and suggest that high cervical stimulation may be efficacious. A 53-year-old male underwent SCS on the C1-3 level for treatment of intractable neuropathic pain below the T3 level, and in the upper extremities, arising from spinal cord injury resulting from transverse myelitis caused by schistosomiasis. High cervical SCS significantly improved the pain in the upper extremities and at the T3-T10 dermatome level. The patient continues to report excellent pain relief 9 months later. The present case suggests that high cervical stimulation may improve chronic pain in the upper extremities and the T3-T10 dermatome level arising from spinal cord injury.
Spinal cord stimulation; Schistosomiasis; Spinal cord injury; Transverse myelitis
Transverse myelitis (TM) is an immune-mediated spinal cord disorder associated with inflammation, demyelination, and axonal damage. We investigated the soluble immune derangements present in TM patients and found that IL-6 levels were selectively and dramatically elevated in the cerebrospinal fluid and directly correlated with markers of tissue injury and sustained clinical disability. IL-6 was necessary and sufficient to mediate cellular injury in spinal cord organotypic tissue culture sections through activation of the JAK/STAT pathway, resulting in increased activity of iNOS and poly(ADP-ribose) polymerase (PARP). Rats intrathecally infused with IL-6 developed progressive weakness and spinal cord inflammation, demyelination, and axonal damage, which were blocked by PARP inhibition. Addition of IL-6 to brain organotypic cultures or into the cerebral ventricles of adult rats did not activate the JAK/STAT pathway, which is potentially due to increased expression of soluble IL-6 receptor in the brain relative to the spinal cord that may antagonize IL-6 signaling in this context. The spatially distinct responses to IL-6 may underlie regional vulnerability of different parts of the CNS to inflammatory injury. The elucidation of this pathway identifies specific therapeutic targets in the management of CNS autoimmune conditions.
To describe clinical, MRI and cerebrospinal fluid (CSF) features of a varicella zoster virus (VZV) related meningo-encephalo-myelitis (MEM) without rash in an immunocompetent female.
An 85 year old immunocompetent woman with mild hyperthermia and acute, severe MEM.
Serum antibodies and CSF PCR were searched for several viruses. Brain and spinal cord MRI were performed. Immunological profile.
i.v. acyclovir 30 mg/kg/day; i.v. 6-MP 125 mg/day.
Marked CSF lymphomonocytic pleocytosis, blood-brain-barrier damage, and PCR detection of 3.05 X 10 6 copies of VZV DNA. MRI revealed lesions of the meninges, brain and spinal cord. No evidence of immunosuppression.
We highlight the importance of considering the possibility of VZV related MEM, even in immunocompetent patients. We also provide a MRI description of VZV related multifocal myelitis, not previously available. As supported by other reports, we underline the necessity of a prompt therapeutic intervention in this life-threatening condition
varicella-zoster; meningitis; encephalomyelitis; immunocompetence; vasculitis
Meningitis and myelitis represent common and very infrequent viral infections of the central nervous system (CNS), respectively. Indeed, the number of cases of viral meningitis that occurs annually exceeds the total number of meningitis cases caused by all other etiologies combined. Focal CNS infections, on the other hand, such as occur in the spinal cord with viral myelitis, are much less common and may be confused with non-infectious disorders that cause acute flaccid paralysis (AFP). This chapter will review some of the important clinical features, epidemiology, diagnostic approaches, and management strategies for patients with aseptic meningitis and viral myelitis. Particular focus will be placed on the diseases caused by enteroviruses (EVs), which as a group account for the vast majority of all aseptic meningitis cases as well as many focal infections of the spinal cord.
aseptic meningitis; myelitis; acute flaccid paralysis; enterovirus; cerebrospinal fluid; neurovirology
We report a case of myelitis after plasma-derived hepatitis B vaccination. The patient was a 31-year-old man who presented with progressive sensory symptoms in extremities that developed 2 weeks after a third vaccination. MRI of the cervicothoracic region revealed swelling and T2 high signal at the level of C4 to C5 cord, and isolated enhancement in the posterior columns between C4 and C5 cord. The significance of MRI findings and HLA haplotype of the patient will be briefly discussed.
Acute transverse myelitis is a rare manifestation of dengue viral infection. Four cases have been previously reported in the literature.
To report a case of a 61-year-old woman who developed acute transverse myelitis 6 days after the onset of a dengue viral infection.
Magnetic resonance imaging of spinal cord showed hypersignal intensity on T2W at T9-T10. Laboratories studies revealed a high titer of hemagglutination inhibition of dengue virus. Treatment with intravenous pulse methylprednisolone and physiotherapy yielded a partial recovery, followed by complete resolution at 1 year postinfection.
Acute transverse myelitis is a rare manifestation of dengue infection that can occur in either the peri-infectious or postinfectious phases.
Acute transverse myelitis, paraplegia; Dengue fever; Dengue hemorrhagic fever; Methylprednisolone; Flavivirus
We report the case of a middle aged Tanzanian man who developed a spinal cord syndrome over 6 weeks, along with a mild encephalopathy. Investigations ruled out the usual major causes of such a syndrome in our setting in northern Tanzania. Examination of his cerebrospinal fluid revealed trypanosomes, and he made a slow but dramatic improvement after a full course of suramine and melarsoprol. We postulate that he had a transverse myelitis due to African trypanosomiasis, a rare and barely recognised cause.
; Tanzania; transverse myelitis; trypanosomiasis
Allogeneic and autologous haematopoietic stem cell transplantation are established treatment options for haematological malignancies and may possibly be employed to treat a range of genetic and autoimmune diseases.
We report two patients who developed an acute myelitis within their thoracic spinal cord after allogeneic stem cell transplantation. Myelitis in these patients was not related to graft versus host disease or immune reconstitution and was responsive to intravenous methylprednisolone and cyclophosphamide.
Myelitis is a possibly disabling consequence of haematopoietic stem cell transplantation.
Transverse myelitis is a focal inflammatory disorder of the spinal cord which may arise due to different etiologies. Transverse myelitis may be idiopathic or related/secondary to other diseases including infections, connective tissue disorders and other autoimmune diseases. It may be also associated with optic neuritis (neuromyelitis optica), which may precede transverse myelitis. In this manuscript we review the pathophysiology of different types of transverse myelitis and neuromyelitis optica and discuss diagnostic criteria for idiopathic transverse myelitis and risk of development of multiple sclerosis after an episode of transverse myelitis. We also discuss treatment options including corticosteroids, immunosuppressives and monoclonal antibodies, plasma exchange and intravenous immunoglobulins.
Transverse myelitis; neuromyelitis optica; epidemiology; pathology; pathogenesis; treatment.
Transverse myelitis (TM) is an inflammatory process involving a restricted area of the spinal cord. The usual dramatic presentation makes TM a medical emergency. Early detection and aggressive therapy are required in order to improve the prognosis. The association of this unique clinical phenotype and autoantibody provides circumstantial evidence that an autoimmune aetiology might be involved. We describe two cases of TM associated with anti-Ro (SSA) autoantibodies without connective tissue disease manifestations. The two patients were treated successfully with IV steroids and cyclophosphamide.
Post-infectious autoimmune demyelination of the central nervous system is a rare neurological disorder typically associated with exanthematous viral infections. We report an unusual presentation of the condition and a previously undocumented association with Streptococcus pneumonia meningitis.
A 50-year-old Caucasian woman presented to our facility with an acute myelopathy three days after discharge following acute Streptococcus pneumoniae meningitis. Imaging studies of the spine ruled out an infective focus and no other lesions were seen within the cord. Diffuse, bilateral white matter lesions were seen within the cerebral hemispheres, and our patient was diagnosed as having a post-infective demyelination syndrome that met the diagnostic criteria for an acute transverse myelitis. Our patient clinically and radiologically improved following treatment with steroids.
The novel association of a Streptococcus pneumoniae infection with post-infectious autoimmune central nervous system demyelination should alert the reader to the potentially causative role of this common organism, and gives insights into the pathogenesis. The unusual dissociation between the clinical presentation and the location of the radiological lesions should also highlight the potential for the condition to mimic the presentation of others, and stimulates debate on the definitions of acute transverse myelitis and acute disseminated encephalomyelitis, and their potential overlap.
Post-infective demyelination; Transverse myelitis; Acute disseminated encephalomyelitis; Pneumococcal meningitis; Streptococcus pneumoniae
A 25-year-old man with Behçet's disease was admitted because of weakness of the lower limbs and difficulty in urination. He had received a rabies vaccination 2 months previous because he had been bitten by a dog.
Clinical and laboratory findings supported acute transverse myelitis. A hyperintense lesion and expansion at the level of conus medullaris was detected on spinal magnetic resonance imaging.
Although neurologic involvement is one of the main causes of mortality and morbidity in Behçet's disease, the factors that aggravate the involvement of the nervous system are still unclear. Vaccination may have been the factor that had activated autoimmune mechanisms in this case. To our knowledge, involvement of the conus medullaris in Behçet's disease after rabies vaccination has not been reported.
Rabies; Vaccination; Acute transverse myelitis; Behçet's disease; Conus medullaris; Neuro-Behçet syndrome