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PubMed1990

Transverse myelitis: a manifestation of systemic lupus erythematosus strongly associated with antiphospholipid antibodies.

All 4 patients with transverse myelitis (TM) included in a prospective study of 500 patients with systemic lupus erythematosus (SLE) tested for anticardiolipin (aCL) antibodies were found to be positive. To determine if the apparently strong association between transverse myelitis and antiphospholipid antibodies (aPLA) in patients with SLE continued to hold, we chose 12 patients with SLE with transverse myelitis from 2 institutions. Eleven of the 12 patients with SLE were tested for aCL and all but one was positive. Most of them (n = 8) had both IgG and IgM isotype aCL. The one who was negative had had a positive VDRL and prolonged APTT 15 months earlier, coinciding with the episode of transverse myelitis. One patient died before she was tested for aCL but she also had a false positive VDRL. Thus, all 12 patients with SLE with transverse myelitis had evidence of aPLA. We conclude that there is strong association between transverse myelitis in SLE and the presence of aPLA.

Adolescent↗
PubMed1998

Clinical and laboratory features of myelitis patients with anti-neutrophil cytoplasmic antibodies.

Although perinuclear anti-neutrophil cytoplasmic antibodies (pANCA) are associated with vasculitic neuropathy, their association with central nervous system (CNS) disorders has not been studied except for one report on optic-spinal type of multiple sclerosis associated with serum pANCA. We examined pANCA in sera from 98 patients with various CNS disorders, such as 58 MS, 17 myelitis, 12 HTLV-1 associated myelopathy, and 11 other CNS diseases using indirect immunofluorescence methods. The results showed serum pANCA to be positive in five patients with a peculiar type of myelitis, including two with MS and three with etiology unknown myelitis. All of these ANCA-positive patients were women and had acute or subacute myelopathy with various severities. MRI revealed segmental swelling of the spinal cord with T2 hyperintensity in the acute stage of the disease. Marked pleocytosis (227.8+/-101/mm3) and elevated protein level (128.8+/-52 mg/dl) in CSF were noted. Four of the patients had anti-nuclear antibodies and two had previous histories of symptoms suggesting autoimmune disorders. In a search for target antigens of pANCA, myeloperoxidase reactivity was found in the sera from two myelitis patients. Clinical and laboratory features of myelitis patients with pANCA in the present study are different from those of typical MS patients. Further study will be needed to delineate the role of pANCA in the pathogenesis of a specific type of myelitis.

Adolescent↗
PubMed1998

Parainfectious conus myelitis.

We describe electroclinical and imaging features of a peculiar type of parainfectious myelitis that selectively involves the conus/epiconus region of the spinal cord. Twelve patients of parainfectious myelitis with MRI evidence of inflammatory lesions in the conus/epiconus region of the spinal cord were studied. All patients underwent full clinical and electrophysiological evaluation along with MRI of the spine. MRI included axial images at the site of lesion. All patients had a unique clinical presentation with urinary symptoms. Careful clinical examination revealed minimal sensorimotor dysfunction in the lower lumbar and sacral segments, which remained unnoticed by most of the patients; three female patients had no sensorimotor deficit. The motor paralysis recorded in four patients was flaccid and areflexic. The sensory level was inconspicuous as it was in the leg area corresponding to the lumbar and sacral spinal segments. Sensory loss was significantly more in the perineal region in those seven patients who had MRI evidence of inflammatory lesion in conus medullaris; two patients had maximum sensory loss in lumbar dermatomal distribution, which corresponded with the focal segmental myelitis involving 'epiconus'. MRI done in the sagittal plane was either normal or only 'suggestive' of myelitis in most of the patients and the inflammatory lesions were much more visible in the axial plane. The lesions predominantly involved central gray matter with spread to adjoining white matter in nine patients; in three patients with pure bladder involvement, lesions were confined to lateral gray matter of the conus medullaris. Our findings indicate that parainfectious myelitis (PIM) selectively involving conus medullaris is an important cause of unexplained acute or sub-acute urinary symptoms in adolescent and adult patients. In suspected cases, MRI must include axial images of the conus-epiconus region, as sagittal images may not always reveal the lesion. Due to initial presentation with urinary symptoms, absent or minimal sensory-motor signs, no transverse level over the trunk and unique MRI features, this condition may be called parainfectious conus myelitis (PICM).

Acute Disease↗
PubMed2000

[Acute myelitis and Behcet's disease: three case reports].

INTRODUCTION: Neurological manifestations in Behçet's disease usually consist of meningoencephalitis and cerebral thrombophlebitis. Myelitis is rare, especially when it is the only neurological manifestation. EXEGESIS: The authors report three cases of acute myelitis complicating Behçet's disease demonstrated by magnetic resonance imaging. In two cases, Behçet's disease was revealed by myelitis. The three cases were transverse myelitis, two of which were juvenile Behçet's disease and two were meningomyelitis. CONCLUSION: Magnetic resonance imaging can help guide diagnosis of myelitis in Behçet's disease. It can also be useful to assess the results of the treatment. Clinical symptoms of Behçet's disease must be searched for in the case of myelitis, especially for patients from Mediterranean countries.

Acute Disease↗
PubMed2000

Myelitis.

Acute transverse myelitis (ATM) with moderate symptomatology and smaller multiple magnetic resonance imaging lesions is often caused by multiple sclerosis. Severe ATM with extensive magnetic resonance imaging lesions with or without associated meningitis often has a viral cause, particularly in the younger age groups, whereas vascular disorders may prevail among older patients. Previously, one had to rely on indirect evidence such as viral serology or viral identification in throat washings to confirm a diagnosis of myelitis. Thus, mycoplasma myelitis may occur coincident with a mycoplasma pneumonia. Viral myelitis is now often diagnosed by specific polymerase chain reaction of the cerebrospinal fluid, for echovirus, Coxsackie virus, mumps virus, herpes simplex virus or varicella-zoster virus, but an autoimmune component may still be important. An anterior horn syndrome may be produced by the tick-borne encephalomyelitis virus. Severe ATM may also be a postinfectious or postvaccinal disorder [i.e. a partial acute disseminated encephalomyelitis (ADEM)]. Neuromyelitis optica, a combination of severe myelitis and optic neuritis, is often a manifestation of ADEM or systemic lupus erythematosus. Many of these disorders are potentially treatable with specific antiviral agents or immunosuppression. 'Idiopathic' ATM is probably a consequence of inadequate examination and follow up. The differential diagnoses-viral myelitis, multiple sclerosis, ADEM, neuromyelitis optica, spinal arteriovenous malformation and arteritis-should be considered and are usually identified by a rapid diagnostic work-up, leaving few ATM cases undiagnosed.

Acute Disease↗
PubMed2003

Recurrent transverse myelitis after lumbar spine surgery: a case report.

STUDY DESIGN: A case of recurrent idiopathic transverse myelitis occurring after surgery is reported. OBJECTIVES: To present a case of idiopathic transverse myelitis recurring after surgery and to heighten awareness for the diagnosis and management of this disorder. SUMMARY AND BACKGROUND DATA: Transverse myelitis presenting with acute spinal pain and neurologic deficit must be considered along with structural causes of myelopathy by the spine specialist. This intramedullary spinal cord disorder may be caused by parainfectious and postvaccinal sequelae, multiple sclerosis, spinal cord ischemia, autoimmune disorders, and paraneoplastic syndromes. These various etiologies are often difficult to differentiate. However, a patient's history, clinical course, MRI studies, and laboratory findings often allow such classification. Determination of etiology provides pertinent information regarding potential recurrence, treatment, and prognosis. METHODS: The patient history, physical examination, radiologic and laboratory studies, and pertinent literature were reviewed. RESULTS: Thoracolumbar myelitis developed in the reported patient 6 weeks after lumbar spine surgery during an otherwise uncomplicated postoperative recovery. The workup did not identify a specific cause, and the patient recovered to ambulatory status. However, 4 months after surgery, acute transverse myelitis developed again, this time affecting the cervical spinal cord. Despite aggressive intervention with corticosteroids, the patient has remained nonambulatory with severe neurologic residua. In spite of an extensive workup, a definitive cause was not determined, although an autoimmune etiology was suspected. The patient has stabilized without recurrence using immunosuppressant therapies. CONCLUSIONS: Acute transverse myelitis is an intramedullary spinal cord disorder that may present to the spine specialist during the postoperative period. This diagnosis requires swift and aggressive diagnostic and treatment intervention. Although sometimes difficult, establishment of causation may help to determine therapy and prognosis.

Aged↗
PubMed1997

Parainfectious myelitis: three distinct clinico-imagiological patterns with prognostic implications.

Seventeen parainfectious myelitis patients were studied for site, extent and severity of lesions. Three patterns were observed each having distinct clinical, electrophysiological and MRI features: 1) focal segmental myelitis--focal cord lesion with long tract signs and good prognosis; 2) ascending myelitis--continuous lesion from conus to mid-cord with upper and lower motor neuron signs (not necessarily spinal shock), dysautonomia and poor outcome; 3) disseminated myelitis--discrete lesions scattered throughout the cord with subtle signs in spinal segmental distribution, above and below the transverse level and moderate outcome. Severe autonomic dysfunction, denervation of paraspinal muscles, "dense" lesion on imaging and often (but not always) the absent somatosensory evoked potentials carried poor outcome. In conclusion "parainfectious myelitis" is a better term to describe transverse myelitis, as the lesion extends to a large vertical extent. Further classification into 3 subgroups may improve understanding of anatomical and physiological dysfunction and prediction of outcome.

Adolescent↗
PubMed1993

Hyperbaric oxygen as prophylaxis or treatment for radiation myelitis.

This animal study was designed to investigate HBO as a treatment or prophylaxis for radiation myelitis. All animals received identical spinal cord radiation doses of 69 Gy in 10 daily fractions. Group I received no HBO; group II began HBO at the onset of signs of myelitis; group III received HBO with prophylactic intent beginning 6 wk after irradiation; and group IV received both modalities on the same day, but radiation always preceded HBO by at least 4 h. HBO consisted of 90 min oxygen at 2.4 atm abs for 20 daily treatments. Animals were objectively assessed for the loss of certain neurologic reflexes indicative of four levels of myelitis. Although all animals progressed to severe myelitis, group III animals had group-averaged levels of myelitis consistently less than control. The differences were statistically significant for several weeks. Group IV animals progressed to severe myelitis much more rapidly than any other group. Additional study is justified by this trial. Key questions to be answered include the optimal timing of HBO to produce a beneficial rather than detrimental effect.

Analysis of Variance↗
PubMed2000

Th1 dominance in HAM/TSP and the optico-spinal form of multiple sclerosis versus Th2 dominance in mite antigen-specific IgE myelitis.

To clarify the Th1/Th2 balance in spinal cord inflammation, we used ELISA to measure the total and allergen-specific IgE in 69 patients with clinically definite multiple sclerosis (MS), including 24 patients with the optico-spinal form of MS, 45 with HAM/TSP, 30 HTLV-I carriers without HAM/TSP, 40 patients with acute myelitis, 43 with neurodegenerative disorders, and 42 healthy subjects, and flow cytometry to study the intracellular IFNgamma-positive versus IL-4-positive cell ratio (intracellular IFNgamma/IL-4 ratio) in peripheral blood CD4(+) T cells in 40 patients with MS, including 17 patients with the optico-spinal form of MS, 23 with HAM/TSP, 22 with acute myelitis, 23 with neurodegenerative disorders, and 36 healthy subjects. Patients with HAM/TSP showed a significantly higher intracellular IFNgamma/IL-4 ratio, lower IL-4(+)/IFN-gamma(-) cell percentages, lower total IgE level, and lower frequency of cedar pollen-specific IgE than did the controls. The patients with optico-spinal MS showed a significantly higher intracellular IFNgamma/IL-4 ratio and higher IL-4(-)/IFN-gamma(+) cell percentages than the controls even at remission or in the convalescence phase. In contrast, in the patients with acute myelitis, the total serum IgE level and the frequency of mite antigen-specific IgE were significantly elevated in comparison to the controls, while those having mite antigen-specific IgE myelitis showed a significantly lower IFNgamma/IL-4 ratio in the CD4(+) T cells in comparison to the controls. These findings suggest that the Th1 cell response is predominant in HAM/TSP and optico-spinal MS, whereas the Th2 cell response is predominant in mite antigen-specific IgE myelitis.

Adult↗
PubMed2005

Acute transverse myelitis in a 7-month-old boy after diphtheria-tetanus-pertussis immunization.

STUDY DESIGN: Case report of a 7-month-old boy, who developed acute transverse myelitis after diphtheria-tetanus-pertussis immunization. OBJECTIVES: To describe the clinical course of acute transverse myelitis in an infant and to review the literature regarding the association of acute transverse myelitis and vaccinations. SETTING: Department of Pediatrics, University of Kentucky, Lexington, Kentucky, USA. METHODS: Case report. RESULTS: Magnetic resonance imaging (MRI) on admission demonstrated diffuse spinal cord edema with increased signal on T-2 weighted images and faint enhancement with gadolinium infusion. Urologic symptoms improved with steroids but motor function was never fully regained. Repeat MRI of the spinal cord several months later showed diminution of cord diameter with resolution of edema and signal abnormality. CONCLUSION: Based on the clinical course and MRI findings, the daignosis of acute transverse myelitis was made. The association of previously received DPT immunization and the genesis of transverse myelitis is explored.

Diphtheria-Tetanus-Pertussis Vaccine↗
PubMed2003

Cord damage elicits brain functional reorganization after a single episode of myelitis.

OBJECTIVE: To assess, using fMRI, the brain pattern of movement-associated cortical activations in patients with a previous remitting episode of acute cervical myelitis of possible demyelinating origin and to investigate whether the extent of cortical reorganization is associated with the extent of cervical cord pathology measured using magnetization transfer (MT) MRI. METHODS: From 14 right-handed patients in a chronic and stable phase after an isolated myelitis (male/female = 7/7, mean age = 35.3 years, median disease duration = 21.2 months) involving the cervical cord and 15 sex- and age-matched healthy control subjects, we obtained 1) fMRI during repetitive flexion-extension of the last four fingers of the right hand, 2) brain diffusion tensor MRI, and 3) brain and cervical cord conventional and MT MRI. FMRI data were analyzed using Statistical Parametric Mapping software. Brain mean diffusivity, fractional anisotropy, and MT ratio (MTR) histograms of the normal-appearing white and gray matter and cervical cord MTR histograms were produced. RESULTS: Patients with myelitis had lower average cord MTR (p < 0.0001) and cord MTR histogram peak position (p = 0.002) than control subjects. Compared with healthy volunteers, patients with myelitis showed increased recruitment of the ipsilateral hemisphere in the primary sensorimotor cortex (p < 0.0001), supplementary motor area (p = 0.002), and middle frontal gyrus (MFG) (p < 0.0001). Average cervical cord MTR was inversely correlated with relative activations of the ipsilateral MFG (r = -0.80) and of the ipsilateral postcentral gyrus (r = -0.80). The relative activation of the ipsilateral MFG was also correlated with cervical cord MTR peak position (r = -0.92). CONCLUSIONS: An abnormal pattern of movement-associated cortical activations was found in patients with a previous episode of cervical myelitis. These functional cortical changes might have an adaptive role in limiting the clinical outcome of structural cord damage.

Acute Disease↗
PubMed1986

Unusual viral causes of transverse myelitis: hepatitis A virus and cytomegalovirus.

Twenty to 40% of cases of acute transverse myelitis are attributed to viral infections, although the specific viral etiology is only rarely identified. We studied two patients with transverse myelitis in association with acute hepatitis A virus (HAV) infection and acute primary cytomegalovirus (CMV) infection. This is the first well-documented report of an association between HAV infection and transverse myelitis, and only the fourth documented case of transverse myelitis in association with CMV infection in an immunocompetent adult. Both viruses should be considered as rare causes of transverse myelitis in immunologically normal adults.

Adult↗
PubMed2004

[Diagnosis and treatment of CNS parasite infection with special reference to parasitic myelitis].

The occurrence of visceral larva migrans due to Ascaris suum (A. suum) and Toxocara canis (T. canis) has occasionally been reported in Japan, although parenchymatous involvement of the CNS is extremely rare in A. suum/T. canis visceral larvae migrans. Recently we experienced 7 cases with myelitis caused by visceral larva migrans due to A. suum/T. canis (parasite myelitis). The characteristics of this myelitis are: (1) sensory disturbances (Lhermitte's sign, paresthesia, and hypesthesia) are predominant symptoms, while severe motor weakness is rare, (2) spinal cord lesions on T2-weighted MRI show more extensive lesions compared with mild symptoms, (3) Gadolinium enhancement of spinal cord lesions are limited as compared with spinal cord lesions on T2-weighted MRI lesions, (4) Some cases show the presence of eosinophils in CSF, while others show Th2 deviation in CSF supernatant, and (5) Tests for anti-A. suum/T. canis IgG antibody are strongly positive in serum and CSF. Moreover, 6 percent of 108 consecutive cases with non-compression myelopathy presenting at the Department of Neurology at Kyushu University Hospital from January, 1998 to December, 2002 had parasitic myelitis. Myelitis from visceral larva migrans due to A. suum/T. canis might be overlooked because of its mild neurologic impairment without systemic symptoms, but should be considered as one of the differential diagnoses in non-compression myelopathy.

Adult↗
PubMed2006

[Two cases of myelitis associated with Sjögren syndrome without xerosis: characteristic MRI findings].

We report two cases of myelitis associated with Sjögren syndrome without xerosis. Case 1: A 30-year old woman developed dysesthesia on both upper extremities and weakness of the right arm. A T2-weighted MRI examination showed a high-intensity signal and a swollen lesion between the first and seventh cervical vertebral levels. She was diagnosed as having primary Sjögren syndrome based on the positive finding of a Saxon test, typical salivary gland scintigraphy findings, and an elevated anti-SS-A antibody titer. We suspected that her myelitis was associated with Sjögren syndrome and treated her using steroid therapy. Although her symptoms were alleviated, her myelitis relapsed at the same location after the cessation of steroid therapy. Case 2: A 31-year-old woman developed dysesthesia on her neck and both upper extremities and exhibited tonic spasm. A T2-weighted MRI examination showed a high-intensity signal and a swollen lesion between the first and sixth cervical vertebral levels. She was diagnosed as having primary Sjögren syndrome based on the positive findings of a Rose Bengal test, a Schirmer's test, and a Saxon test as well as typical salivary gland scintigraphy findings and elevated titers of anti-SS-A and anti-SS-B antibodies. We suspected that her myelitis was associated with Sjögren syndrome and treated her using steroid therapy. Her symptoms improved after steroid therapy. Based on these two cases, we concluded that MRI findings for myelitis associated with Sjögren syndrome are characterized by a swollen lesion of more than three vertebral segments in length, and the relapse tends to occur at the same location.

Adult↗
PubMed1997

[Myelitis and optic neuritis caused by varicella].

INTRODUCTION: Varicella mainly affects children between 1 and 14 years old. It is the initial infection caused by the Varicella-Zoster virus. It is characterized by a vesicular cutaneous eruption, fever and generally good prognosis. The neurological complications caused by the Varicella-Zoster virus are infrequent and include: meningitis, encephalitis, cerebellar ataxia, Reye's syndrome, myelitis, optic neuritis, mononeuropathy, polyneuropathy, necrosis of the retina and cerebral arteritis. CLINICAL CASE: We present the unusual case of a woman patient aged 18 who presented with myelitis 15 days after having a varicella rash. Analysis of the cerebrospinal fluid showed intrathecal production of antibodies against the Varicella-Zoster virus. Fourteen days after resolution of the myelitis, she presented with unilateral optic neuritis which remitted without sequelae, (as did the myelitis). Cerebral and medullary MR showed no alterations. CONCLUSIONS: The pathogenesis leading to involvement of the nervous system is still not well defined. Direct invasion by the virus has been postulated, particularly in Herpes-Zoster (reinfection by Varicella-Zoster), as immunological phenomena which may be more frequent with Varicella (initial infection by Varicella-Zoster virus). In our case there were two short episodes of neurological involvement: optic neuritis and myelitis, with a satisfactory clinical course after giving corticosteroids. This makes one think of immunological mechanisms rather than direct invasion of the central nervous system by the Varicella-Zoster virus.

Adolescent↗
PubMed2006

Atopic myelitis: a clinical, biological, radiological and histopathological diagnosis.

We describe a young patient with an unusual intramedullary lesion filled with eosinophils. The 21-year-old man developed chronic myelitis without optic neuritis or signs of systemic or infectious disease. A spinal biopsy was conducted because of the progressive extension and pseudo-tumoural aspect of the lesion. Histopathological analysis of the biopsy specimen revealed a severe inflammatory process with macrophages and numerous eosinophils. The eosinophil count in the blood and cerebrospinal fluid (CSF) were normal. Clinical, laboratory and radiological data did not correspond with the usual causes of eosinophilic myelitis. Abnormal mite antigen-specific IgE levels and features similar to Japanese cases of atopic myelitis suggested an allergic origin. Despite normal total IgE levels, this case may be the second case of atopic myelitis reported in a Caucasian patient. Striking differences with the first reported case are the absence of a typical history of atopy and normal total IgE levels. This case highlights that atopic myelitis should be considered in myelopathy occurring in Caucasian patients even in the absence of hyperIgEaemia.

Adult↗
PubMedDate not supplied

Demonstration of acute post-viral myelitis with magnetic resonance imaging.

Post-infectious transverse myelitis is an important clinical entity in childhood, but the diagnosis is difficult to confirm by conventional radiologic investigations. We report a patient with acute, cervical, post-infectious transverse myelitis whose diagnosis had been established by the distribution and evolution of lesions on serial magnetic resonance imaging (MRI). Autopsy results confirmed the diagnosis of post-infectious transverse myelitis and the presence of MRI-identified lesions. To our knowledge, this patient is the first child reported with MRI findings in post-infectious transverse myelitis and suggests that MRI may be valuable in the diagnosis and management of children with this syndrome.

Acute Disease↗
PubMed2001

Acute severe spinal cord dysfunction in bacterial meningitis in adults: MRI findings suggest extensive myelitis.

BACKGROUND: Bacterial meningitis is rarely complicated by acute spinal cord involvement (eg, myelitis, ischemic infarction, spinal abscess, or epidural hemorrhage). In spinal cord dysfunction, magnetic resonance imaging (MRI) is the imaging modality of choice. Still, MRI findings of myelitis due to bacterial meningitis in adults have not been reported. METHODS: Spinal MRIs were obtained during the acute stage of meningitis and on follow-up in 3 adults with bacterial meningitis that was complicated by paraparesis or tetraparesis and bowel and bladder incontinence. The causative pathogens were Streptococcus pneumoniae and Neisseria meningitidis; in 1 patient, the pathogen was not identified. RESULTS: In all cases, spinal MRI ruled out a compression of the cord by an extramedullary mass but demonstrated hyperintensities on T2-weighted images that predominantly involved the gray matter and extended from the cervical to the lumbar cord. Leptomeningeal and discrete nodular intramedullary enhancement on T1-weighted images was detected only in 1 patient. Follow-up examinations revealed that hyperintensities resolved completely in 1 patient, while a central cavitation developed in the cervical spinal cord of another, and the MRI findings were progressive during the first 4 weeks in the third patient. In all cases, severe paresis and bowel and bladder incontinence persisted. CONCLUSION: We demonstrate for the first time the MRI findings of adults with acute spinal cord involvement during bacterial meningitis. Magnetic resonance imaging showed central intramedullary hyperintensities on T2-weighted images that extended from the cervical to the lumbar cord, indicating myelitis. Clinical follow-up examinations suggest that myelitis during bacterial meningitis has an unfavorable prognosis.

Adolescent↗