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Longitudinal involvement of the spinal cord in a patient with lupus related transverse myelitis.

Transverse myelitis is a rare and serious complication of systemic lupus erythematosus (SLE). Magnetic resonance imaging is the investigation of choice for diagnosis and followup. This typically shows T1 and T2 signal prolongation, cord widening, and contrast enhancement over several spinal segments. We describe a 21-year-old woman with SLE who developed very extensive SLE related transverse myelitis with longitudinal involvement of the spinal cord from C3 to T2 and from T7 to the conus medullaris. Clinically, this was manifest as leg weakness, bladder dysfunction, severe low back pain, and patchy lower limb sensory loss. She responded to treatment with pulse cyclophosphamide and high dose corticosteroids with complete recovery in 3 months. To our knowledge, this is the first case report of such an extensive "longitudinal" myelitis.

Adult↗

Transverse myelitis.

Transverse myelitis is a known complication of radiation treatment for carcinoma of the head and neck. Otolaryngologists treat patients who receive radiotherapy and should be familiar with this complication. During the past ten years there has been little or no mention made of this problem in the ENT literature. In a five year period, 1970 to 1975, 120 patients with head and neck cancer received radiation as part of their treatment in this hospital. A review of the records of these patients showed only two cases of myelitis, an incidence of about 2%. This paper reviews the clinical syndrome; treatment and preventive measures are discussed and a survey of the literature is presented.

Aged↗

Immunopathogenesis of acute transverse myelitis.

Acute transverse myelitis is a group of disorders characterized by focal inflammation of the spinal cord and resultant neural injury. Acute transverse myelitis may be an isolated entity or may occur in the context of multifocal or even multisystemic disease. It is clear that the pathological substrate--injury and dysfunction of neural cells within the spinal cord--may be caused by a variety of immunological mechanisms. For example, in acute transverse myelitis associated with systemic disease (i.e. systemic lupus erythematosus or sarcoidosis), a vasculitic or granulomatous process can often be identified. In idiopathic acute transverse myelitis, there is an intraparenchymal or perivascular cellular influx into the spinal cord, resulting in the breakdown of the blood-brain barrier and variable demyelination and neuronal injury. There are several critical questions that must be answered before we truly understand acute transverse myelitis: (1) What are the various triggers for the inflammatory process that induces neural injury in the spinal cord? (2) What are the cellular and humoral factors that induce this neural injury? and (3) Is there a way to modulate the inflammatory response in order to improve patient outcome? Although much remains to be elucidated about the causes of acute transverse myelitis, tantalizing clues as to the potential immunopathogenic mechanisms in acute transverse myelitis and related inflammatory disorders of the spinal cord have recently emerged. It is the purpose of this review to illustrate recent discoveries that shed light on this topic, relying when necessary on data from related diseases such as acute disseminated encephalomyelitis, Guillain-Barré syndrome and neuromyelitis optica. Developing a further understanding of how the immune system induces neural injury will depend upon confirmation and extension of these findings and will require multicenter collaborative efforts.

Acute Disease↗

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↗

Acute transverse myelitis.

Acute transverse myelitis is a relatively uncommon neurological disease in which affected patients exhibit acute dehabilitating symptoms associated with the loss of spinal cord segment function. During January 1996, a 23-year-old black female college student, who had been previously healthy, presented to our clinic with acute onset of symptoms typical of acute transverse myelitis. These symptoms included bilateral lower extremity paresthesias, back pains in the thoracic and lumbar areas and pronounced lower extremity weakness. This article is a review of literature to learn about the presenting symptoms, postulated etiologies, diagnostic studies, laboratory findings, management, and variable prognosis for acute transverse myelitis.

Acute Disease↗

Spinal cord impairment: acute transverse myelitis.

Acute transverse myelitis (ATM) is an unusual inflammatory disease involving the entire thickness of the spinal cord. It results in loss of motor and sensory function below the level of injury. The prognosis is variable. The following paper reviews the literature, clinical consideration, and nursing interventions of this puzzling condition.

Adolescent↗

[Acute transverse myelitis].

Acute transverse myelitis may be caused by many factors, however, in most cases the cause cannot be clinically found, which justifies the diagnosis of "Myelitis transversa acuta" or "myelopathia transversa acuta" in such cases. The disease is inflammatory spinal demyelination, differing morphologically from multiple sclerosis. Magnetic resonance is the examination which discloses the injury of several spinal segments. The upper limit of the lesion is higher than the clinical symptoms indicate. There is protein increase and pleocytosis in the cerebrospinal fluid. In most cases the prognosis is favourable; in 33% of patients complete regression of symptoms takes place; 33% present significant improvement and 33% show permanent disability. The frequency of relapses is high and then multiple sclerosis must be suspected. There also occur cases of monophasic multiple sclerosis and relapses of the disease without other symptoms of multiple sclerosis. The treatment of choice are steroids administered in high doses.

Anti-Inflammatory Agents↗

Proposed diagnostic criteria and nosology of acute transverse myelitis.

Acute transverse myelitis (ATM) is a focal inflammatory disorder of the spinal cord, resulting in motor, sensory, and autonomic dysfunction. A set of uniform diagnostic criteria and nosology for ATM is proposed to avoid the confusion that inevitably results when investigators use differing criteria. This will ensure a common language of classification, reduce diagnostic confusion, and lay the groundwork necessary for multicenter clinical trials. In addition, a framework is suggested for evaluation of individuals presenting with signs and symptoms of ATM. Best treatment often depends on a timely and accurate diagnosis. Because acute transverse myelopathies are relatively rare, delayed and incomplete work-ups often occur. Rapid and precise diagnosis will ensure not only that compressive lesions are detected and treated but also that idiopathic ATM is distinguished from ATM secondary to a known underlying disease. Identification of etiologies may suggest medical treatment, whereas no clearly established medical treatment currently exists for idiopathic ATM. Establishment of a diagnostic algorithm will likely lead to improved care, although it is recognized that the entire evaluation may not be performed for each patient.

Acute Disease↗

Residual bladder dysfunction 2 to 10 years after acute transverse myelitis.

OBJECTIVE: Acute transverse myelitis (ATM) is a relatively rare condition in children. The recovery rate is reported to be generally complete. In the current study, the long-term urological outcome of children with ATM was assessed. METHODOLOGY: The medical records of children with ATM admitted to Queen Mary Hospital, Hong Kong, over the last 15 years, were reviewed. RESULTS: The median age of the five children with ATM at the time of onset was 6 years (range = 2-12 years). The median length of follow up was 5 years (2-10 years). Four children recovered completely from paraparesis; two had no urinary symptoms with normal micturition. However, video-urodynamic studies 3 years after the acute onset revealed that four out of the five children, including one without any urinary symptom, suffered from residual bladder dysfunction - two from contractile neurogenic bladder and two from intermediate type of neurogenic bladder. CONCLUSION: Residual bladder dysfunction is common in children suffering from ATM despite improvement of paraparesis and apparent lack of urological symptoms. Long-term follow up of urological function in these patients is recommended.

Acute Disease↗

Benzene poisoning as a possible cause of transverse myelitis.

A case of transverse myelitis in a 25-year-old patient was described. The transverse myelitis was caused by toxic cause, probably as a result of benzene poisoning. This diagnosis was based on: The differential diagnosis. The patient's occupation. The abnormal high urinary phenol levels. The coincidence of decreasing urinary phenol values with an amelioration of the clinical condition of patient. After consulting the literature, we think that this case of transverse myelitis based on a benzene poisoning is the first ever described.

Adult↗

Decrease in multiple sclerosis with acute transverse myelitis in Japan.

Acute transverse myelitis (ATM) may be a manifestation of multiple sclerosis (MS) and was reported to be more common among Japanese MS patients than in Caucasian MS patients. Recently there are arguments whether clinical manifestations of MS may have changed. Therefore, we studied the frequency of ATM in MS and the clinical subtypes of MS in 86 clinically definite MS patients whose onsets were in 1970-1979, 1980-1989, and 1990-1998 in Sendai City, Japan. Fifty-six of the patients were women and 30 were men. Forty-four patients had the conventional form of MS (C-MS) commonly seen in Western countries, and 42 had optic-spinal or spinal forms of MS (OSS-MS). Twenty MS patients had ATM, and all of them were belong to optic-spinal form of MS. ATM was not seen in any cases of C-MS. The mean onset age (years) of the clinical subtypes was 25.5 in C-MS, 34.1 in OSS-MS without ATM, and 30.9 in OSS-MS with ATM. Among the patients whose onset of the disease was in 1970-1979, 60.0% of them were cases of OSS-MS with ATM, but such cases were markedly decreased to 5.3% in 1990-1998. In contrast, the frequency of C-MS increased to 63.2% in 1990-1998 compared with 20.0% in 1970-1979. Analysis of the data by the year of birth of the patients showed similar results. Our data suggest that the frequency of ATM in MS markedly decreased, and that of C-MS increased during the last 30 years in Sendai, Japan. Since the genetic background of Japanese has not changed, some exogenous factors, such as food, infectious microorganisms, and chemicals in our environment, may be responsible for the change.

Adult↗

Usefulness of immunoadsorption therapy for systemic lupus erythematosus associated with transverse myelitis. A case report.

Transverse myelitis (TM) is a very rare complication of systemic lupus erythematosus (SLE) and its prognosis is poor. It therefore needs to be treated aggressively. We describe a patient suffering from SLE associated with TM, who responded well to a combination of immunoadsorption therapy and steroid mini-pulse therapy. His serum interleukin 6 levels as well as clinical indicators fell to normal after this treatment.

Adult↗

Determinants of prognosis of acute transverse myelitis in children.

BACKGROUND: Acute transverse myelitis (ATM) is a severe disorder; recovery requires several months and often leaves neurologic residua. To determine what features of patients with acute transverse myelitis significantly influence prognosis, the authors reviewed reports of ATM in Japanese children published in the last 15 years (from 1987 to 2001). METHODS: The authors studied reports of 50 Japanese patients (17 boys, 26 girls, 7 children of unspecified sex; mean age +/- SD, 8.0 +/- 3.8 years). Acute-phase and demographic features including age, increased deep tendon reflexes, Babinski reflex, sex, preceding infection, decreased deep tendon reflexes, time course of peak neurologic impairment, treatment with prednisolone and/or high-dose methylprednisolone, and the day of illness when treatment was started were used as independent variables in a regression analysis. The dependent variable was long-term persistence of neurologic deficits. RESULTS: Younger patients and those without increased deep tendon reflexes or a Babinski reflex were more likely to have residual neurologic deficits such as paraplegia or tetraplegia, sensory loss and sphincter disturbance. No relationship was seen between prognosis and sex, preceding infections, decreased deep tendon reflexes, time course of peak neurologic impairment, treatment with prednisolone or high-dose methylprednisolone, or timing of treatment initiation. CONCLUSIONS: Age at onset and neurologic features were important for outcome prediction in ATM. Steroid therapy did not associate with better outcome.

Adolescent↗

CT myelography and MR imaging of acute transverse myelitis.

Two patients with acute transverse myelitis were evaluated by both CT myelography and magnetic resonance. Computed tomographic myelography showed fusiform cord enlargement involving several levels; one patient had a subtotal block in the thoracic spine. Magnetic resonance displayed similar findings of cord enlargement. The demonstration of cord swelling in acute transverse myelitis is an uncommon radiologic manifestation of the disease.

Acute Disease↗

[Favorable response to corticoid therapy in a patient with transverse myelitis in systemic lupus erythematosus].

Transverse myelitis is one of the most unusual neurologic complications of systemic lupus erythematosus. Its pathogenetic mechanisms are controversial. Several therapeutic regimens have been attempted with contradictory results. Corticotherapy appears to improve prognosis, although some authors question its beneficial effects. The case of a patient with systemic lupus erythematosus and transverse myelitis, who presented a favourable clinical course following early treatment with high-dose corticoids, is reported.

Administration, Oral↗

Idiopathic recurrent transverse myelitis.

OBJECTIVE: To determine whether idiopathic recurrent transverse myelitis (RTM) can be distinguished from multiple sclerosis-associated RTM (MSRTM) on the basis of clinical manifestations of myelopathy, or findings from magentic resonance imaging or cerebrospinal fluid examination. DESIGN: A retrospective analysis of 37 cases was conducted. Patients were classified as having idiopathic RTM on the basis of recurrent myelitis confirmed by clinical manifestations of myelopathy and magnetic resonance imaging findings. On review patients with idiopathic RTM had normal cranial magnetic resonance imagings and did not demonstrate paraclinical evidence of spatial dissemination beyond the spinal cord of the disease process. Patients were classified as having MSRTM on the basis of criteria of Poser et al for clinically definite multiple sclerosis involving the central nervous system. Fifteen patients met study criteria for idiopathic RTM. Twenty-two patients had MSRTM. SETTING: Asan Medical Center, Seoul, South Korea, from January 1, 1992, through December 31, 2001. MAIN OUTCOME MEASURES: Presenting symptoms and clinical manifestations, relapsing times, magnetic resonance imaging features (involved spinal cord segments in T2-weighted images and gadolinium 64-enhanced lesions on T1-weighted images), IgG index, and oligoclonal bands in cerebrospinal fluid were compared. RESULT: Idiopathic RTM occurred preponderantly in male patients and presented more often with acute transverse myelitis than did MSRTM. More than 2 relapses occurred in 6 cases (40%) of idiopathic RTM. The involved segments of spinal cord on T2-weighted images were not significantly different in idiopathic RTM and MSRTM, with enhancing lesions mostly in the posterior columns, and the spinothalamic and spinocerebellar tracts of white matter. Additionally, almost all patients with idiopathic RTM had normal cerebrospinal fluid indexes. CONCLUSION: Idiopathic RTM might be a disease entity distinct from MSRTM, differing in its male preponderance, absence of oligoclonal bands, frequent multiple relapses, and frequent presentation as acute transverse myelitis.

Acute Disease↗

Characteristics and course of urinary tract dysfunction after acute transverse myelitis in.

Bladder involvement is common in transverse myelitis but its characteristics and natural history, particularly in relation to neurological outcome, have not been described in a paediatric population previously. Ten children, with ages ranging from 8 months to 16 years, who had a diagnosis of acute transverse myelitis were studied with video urodynamics and followed up in a tertiary paediatric neurourology clinic. Nine of ten children had obstructive urinary tract symptoms at presentation and all went on to develop 'irritative' urinary tract symptoms (frequency and urgency) about 1 month after initial presentation. Video urodynamics showed a combination of irritative (detrusor hyperreflexia) and obstructive (detrusor-sphincter dyssynergia) abnormalities in most patients and enabled management to be specifically directed towards these. The patients progress was followed up for a median duration of 36 months. All had residual bladder dysfunction, only four were asymptomatic on treatment. The degree of recovery of bladder function was not related to the degree of motor recovery.

Adolescent↗

MR imaging of acute transverse myelitis and AIDS myelopathy.

Acute transverse myelitis (ATM) is a well recognized clinical entity, though its etiology remains obscure. Only a few reports of magnetic resonance imaging of ATM appear in the literature. These reports describe conflicting findings with respect to the signal intensity of the spinal cord on long repetition time (TR) sequences. The purpose of this study is to present our experience with five cases of ATM in which long TR sequences demonstrated abnormal increase in signal intensity of the cord. Magnetic resonance imaging also demonstrated extension of abnormal cord signal intensity over at least six spinal segments and above the clinically determined sensory level in four of five cases. Cord expansion was noted in two of five cases with normal myelograms. A case of acquired immunodeficiency syndrome (AIDS) myelopathy that demonstrated a similar high signal intensity of the cord is also presented. Our findings suggest that both ATM and AIDS myelopathy should be considered in the list of conditions that may result in a diffuse increase in the signal intensity of the cord on long TR sequences.

Acquired Immunodeficiency Syndrome↗