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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

[Acute transverse myelitis associated with ECHO-25 virus infection].

A case of acute transverse myelitis associated with ECHO-25 virus infection was reported here with a brief review of the literature. The patient was a 42-year-old house wife. Without any antecedent symptoms, weakness and paresthesia of bilateral lower extremities, and sphincter disturbance developed suddenly. Neurological examination revealed paraplegia of both legs, hyperreflexia of lower extremities, bilateral positive Babinski sign and impairment of all sensory modalities below Th4 level. No abnormality was found in myelography and CT myelography. Abnormal intensity area was found in lower cervical spinal cord on MRI. Serum antibody titer to ECHO-25 virus was elevated in the convalescent stage (X4096), and 7 month later from the onset of neurological symptoms, high value was continued (X128). We diagnosed her illness as acute transverse myelitis complicating ECHO-25 virus infection. In the literature, only 3 cases of transverse myelitis following ECHO virus infection (type 2, 5, 19) were found. This case seemed to be the first case of transverse myelitis associated with ECHO-25 virus infection.

Acute Disease

Radiation myelitis following craniospinal irradiation with concurrent actinomycin-D therapy.

Two patients are presented who developed radiation myelitis as a complication of treatment for medulloblastoma. These patients had been entered onto a pilot study to evaluate the therapeutic efficacy of combined treatment with radiation therapy and actinomycin-D (AMD). AMD is a known radiation enhancer in all tissues but the nervous system. Both of these patients received a full course of cranio-spinal irradiation plus AMD 15 mcg/kg for the first five days of therapy. One patient developed myelitis at 4 months, and the other at 12 months after treatment. Both patients are surviving three years posttreatment without any evidence of recurrent disease. The myelitis in one patient has improved, and it is stable in the other patient. It is concluded that AMD enhances the effect of radiation therapy on the central nervous system. This has never been reported but is important to note now, since radiation enhancers are included in multimodal treatment regimens for a variety of malifnant conditions. Reduction of radiation dose may be necessary when large volumes of the nervous system are treated.

Adolescent

Recurrent herpes zoster myelitis treated with human interferon alpha: a case report.

Recurrent herpes zoster myelitis is very rare. However, a case was recently observed in our hospital. A 43-year-old woman developed myelitis 2 weeks after development of shingles. Her condition was improved by methylprednisolone. Seven months later, she developed myelitis after development of shingles again. Antibody against varicella-zoster (VZV), increased in the serum, but was negative in the cerebrospinal fluid. Methylprednisolone was not sufficiently effective against this attack. The refractory sensory disturbance was improved by human interferon alpha (IFN-alpha). Natural killer cell activity, the helper T-cell/suppressor T-cell ratio and the kappa/lambda ratio of B-cells increased with clinical improvement. In this case, delayed-type hypersensitivity after VZV infection played a role in the occurrence of myelopathy and clinical improvement resulted from the immunosuppressive effects of IFN-alpha.

Adult

Relapsing transverse myelitis.

Acute transverse myelitis is a monophasic disorder, the recurrence of which raises the question of multiple sclerosis (MS) or other multifocal CNS disease. We now report three patients with a previously undescribed syndrome of relapsing isolated acute transverse myelitis. Each had two to five attacks over periods of 3 to 8 years, characterized by ascending paresthesias, urinary retention, sensory loss with a thoracic or cervical level, paraparesis, hyperreflexia, and bilateral Babinski signs. MRI demonstrated areas of increased signal intensity on T2- and proton density-weighted scans and decreased signal intensity on T1-weighed scans of the cervical or thoracic spinal cord consistent with an inflammatory or demyelinating process. All patients had normal complete myelograms, oligoclonal IgG bands were consistently absent from the cerebrospinal fluid, cranial MRIs were normal, and there was no other clinical or laboratory evidence of MS, collagen-vascular disease, or active viral infection. They were treated with high doses of intravenous corticosteroids, stabilized between episodes, and had partial or complete recovery. The recognition of these three patients at a single medical center in a 1-year period suggests that relapses of acute transverse myelitis may not be rare.

Adolescent

Comparative analysis of the therapy of acute transverse myelitis.

Three methods of treatment of acute transverse myelitis are presented. The first group of 16 patients were treated with antibiotics (average age 32.1). Three patients died, 11 remained unrecovered, while in two cases remarkable improvement was recorded. The second group of 15 patients, whose average age was 32.4, were treated with corticosteroid per os or instrumuscularly. Four of the patients died (three of them with ascendent course of disease), there was no change in five cases, while marked improvement ("cured" and "walking with assistance") was recorded in the remaining six cases. The third group of ten patients, eight women two men, ranging from 15 to 47 years of age (average age 26.9) was treated with methylprednisolone acetate intrathecally. Five patients were practically cured after 3 to 4 weeks. Two patients were cured to an extent that they could walk with assistance. In two cases no improvement was recorded, while one of the patients, who in addition suffered from serum myelitis, died of bilateral bronchopneumonia. In all these cases the drug was administered comparatively late, on the 6th or 7th day ofthe disease.

Acute Disease

Acute anterior myelitis complicating West Nile fever.

A healthy young man developed acute anterior myelitis resembling the "polio syndrome." He had visited an area (the Gulf of Suez) in which West Nile fever is endemic prior to his illness. The course of his febrile illness, the spinal fluid findings, and complement fixation antibody titers established the diagnosis of West Nile fever. Meningoencephalitis is a serious, and previously the only known, complication of this usually benign febrile illness. This is the first report, to our knowledge, of another neurological complication, namely acute anterior myelitis.

Acute Disease

Recurrent transverse myelitis, myasthenia gravis, and autoantibodies.

A 45-year-old man with a longstanding diagnosis of myasthenia gravis presented with four episodes of transverse myelitis in 5 years. Each episode improved after treatment with steroids. Laboratory studies revealed no evidence of multiple sclerosis or a structural spinal lesion. He had antinuclear and anti-DNA antibodies and the HLA-A1, B8, DR3 haplotype known to be associated with certain autoimmune diseases. We propose an autoimmune cause for the recurrent episodes of myelitis.

Antibodies, Antinuclear

Borrelia burgdorferi myelitis presenting as a partial stiff man syndrome.

Eight weeks after a tick bite, a 33-year-old male patient presented with stiffness of one leg together with spasmodic painful jerks resembling stiff man syndrome. Isolated myelitis of lumbosacral segments of the spinal cord, apparently confined to the grey matter, was diagnosed and its spirochaetal aetiology confirmed by serology and CSF findings. Oligoclonal IgG bands in CSF specific for Borrelia burgdorferi were found. Thus, there is evidence that B. burgdorferi ist able to cause a localized myelitis, probably of spinal interneurons, presenting as a partial stiff man syndrome.

Adult

Acute ascending necrotizing myelitis in Okinawa caused by herpes simplex virus type 2.

A case of rapidly progressing ascending myelitis was necropsied. Necrosis was present throughout the whole length of the spinal cord and involved both the grey and white matter randomly. The perivascular lymphocytic infiltration in the spinal cord in the present case was more pronounced than that in the previously reported two cases of necrotizing myelopathy associated with malignancy. Using immunoperoxidase staining the presence of herpes simplex virus type 2 (HSV 2) antigen was demonstrated. Electron microscopic examinations revealed large numbers of HSV particles in the spinal cord. HSV 2 may be a common aetiological agent of necrotizing myelopathy and myelitis in Okinawa, an HSV 2 endemic area. In the present case, the necrosis was mainly found in the spinal cord but was also observed, to a very limited extent, in the brain.

Acute Disease

Radiation myelitis: a complication of concurrent cisplatin and 5-fluorouracil chemotherapy with extended field radiotherapy for carcinoma of the uterine cervix.

Radiation myelitis is a rare but serious complication of radiation therapy. The total dose of radiation to the spinal cord required to cause myelopathy is greater than 50 Gy when the treatment is administered in 25 or more fractions; however, recent evidence has suggested that the concurrent use of chemotherapy may decrease the tolerance of the spinal cord to radiation. This report describes a case of radiation myelitis in a patient after concomitant fluorouracil/cisplatin chemotherapy and extended field radiotherapy for stage IIA adenosquamous cell carcinoma of the uterine cervix metastatic to the para-aortic lymph nodes.

Adenocarcinoma

The incidence of myelitis after irradiation of the cervical spinal cord.

To further define the tolerance of the cervical spinal cord, the dose of radiation to the cervical spinal cord was calculated for all 2901 patients with malignancies of the upper respiratory tract treated at the University of Florida between October 1964 and December 1987. To further define the population evaluated, certain criteria were used: (a) a minimum of 3000 cGy to at least 2 cm of cervical spinal cord and (b) a minimum of 1 year of follow-up, unless a neurological complication occurred before 1 year. A total of 1112 patients were evaluable, of which 2 (0.18%) developed radiation myelitis. One received 4658 cGy to the cervical cord at 172.5 cGy per day, and the other patient received 4907 cGy to the cord at 169.2 cGy per day. The risk of myelitis at each dose level was 0/124 at 3000-3999 cGy, 0/442 at 4000-4499 cGy, 2/471 at 4500-4999 cGy, and 0/75 at a cord dose of 5000 cGy or greater.

Dose-Response Relationship, Radiation

Transverse myelitis in systemic lupus erythematosus: two cases with magnetic resonance imaging.

Transverse myelitis is one of the most serious neurological complications occurring in the course of systemic lupus erythematosus. We describe two lupus patients, with transverse myelitis, one of whom had associated optic neuritis. In both, magnetic resonance imaging of the spinal cord showed an abnormal signal. In one case a good response to steroid and immunosuppressive therapy was observed; the other case failed to improve despite the therapy applied.

Adrenal Cortex Hormones

Transverse myelitis following mumps in children.

Although acute transverse myelitis is a rare complication of mumps, it is relatively well documented. We describe a child who developed mumps associated acute transverse myelitis and who subsequently recovered completely. To our knowledge, only 13 cases have been reported in children. This case is compared with 13 previously reported patients.

Acute Disease

Acute transverse myelitis in a 15-month-old girl: report of a case with MRI findings.

A 15-month-old girl demonstrated progressive weakness in all limbs. Magnetic resonance imaging (MRI) on admission revealed (1) diffuse and symmetric cervical cord swelling, (2) diffuse decrease and increase in signal intensity within the affected cord on T1- and T2-weighted images, respectively, (3) preserved anatomic structure of gray and white matter of the cord, and (4) lack of gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA) enhancing effect. She showed rapid clinical improvement in response to steroid therapy, and repeat MRI showed marked reduction in the degree of cord swelling and abnormal signal intensity. Based on the above clinical and MRI findings, the diagnosis of acute transverse myelitis was made. Although various pathologic conditions also could produce similar cord swelling and abnormal signal intensity on MRI, the third and fourth findings mentioned above suggested that the lesion was inflammatory rather than neoplastic. Our case indicates that MRI may be informative in differentiating acute transverse myelitis from other intramedullary disorders.

Acute Disease

[Contribution to the subacute necrotic myelitis (Foix-Alajouanine) (author's transl)].

A case of a 54 years old man with an acute lumbalgia in result of heavy labour is described. With in 7 years he developed a progressive paralysis of the upper and lower motor neuron type and an insensibility of the inferior extremities. The protein content of the cerebrospinal fluid was increased. The cells were insignificantly increased in number. No tumour was detected. The man died at the age of 62 on intoxication by infected decubitus and focal pneumonia. Autopsy revealed subacute necrotic myelitis (Foix-Alajouanine) with enlarged, varicose, and thickened extramedullary veins of the sacral, lumbal, and lower thoracic spinal cord, which was soft and diminished in size. In the grey and white matter of the cord, there were found more numerous and more prominent vessels like an angioma capillare et venosum; complete and incomplete necrosis was observed, partially with plasmatic infiltration. Some nerve cells were still present. Nerve fibres without myelin mantle were found in the spinal cord as well as in the spinal roots. The authors support the suggestion that the subacute necrotic myelitis results from a dysgenesis of the spinal venous vessels.

Autopsy

[An adult case of transverse myelitis with erythema infectiosum].

We reported an adult case of transverse myelitis with erythema infectiosum. A 33-year-old female was admitted to Kyoto University Hospital because of a weakness in the lower extremities and "cloth-wearing" sensation of the trunk and legs. One month before admission, she became febrile and developed a symmetrical erythema on the extremities. At the same time she noticed a slight weakness of the legs and numbness in her fingers and toes, which disappeared next few days. A week later, she again developed a fever, severe weakness of the legs and "cloth-wearing" sensation on the trunk, and erythema appeared on the cheek. Physical examination on admission revealed a weakness and hyperreflexia in the extremities, in particular, knee and ankle jerk, and hypesthesia of the trunk and legs below the level of Th6. Cerebrospinal fluid (CSF) examination revealed 181/mm3 cells (mononuclear cell dominant) and 30 mg/dl protein. Magnetic resonance imaging, CT and electrophysiological studies indicated no abnormalities. IgM antibody against human parvovirus (B19) was detected in the serum and CSF. She was diagnosed as transverse myelitis with parvovirus infection and was medicated with prednisolone 40-60 mg/day, and improved gradually with the residua of a mild weakness of the legs and hypesthesia on the trunk between the level of Th6 and Th10.

Adult

The role of magnetic resonance imaging in acute transverse myelitis.

Eighteen adult patients presenting with acute transverse myelitis (ATM) were evaluated using magnetic resonance imaging. Only 7 had abnormal scans showing an area of increased signal intensity within the cord solely on T2 weighted images; T1 weighted images were normal. The MRI abnormality did not correlate with the cause of the transverse myelitis, the extent of maximum neurological deficit, or the prognosis. A scan performed more than 5 days after the onset of disease was most likely to be positive. Even though the prognostic value of MRI in ATM may be limited, it remains a valuable technique for ruling out other causes of noncompressive spinal cord lesions, such as hemorrhage, vascular malformation, or tumor.

Adolescent