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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↗

Dural puncture and iatrogenic pneumocephalus with subsequent transverse myelitis in a parturient.

PURPOSE: To report a case of transverse myelitis following inadvertent dural puncture and iatrogenic pneumocephalus and any possible causal relationship is explored. CLINICAL FEATURES: A 32-yr-old primigravida developed a severe headache associated with pneumocephalus following accidental dural puncture when the loss of resistance to air technique was used to locate the epidural space. She was treated with oxygen 100% to promote resorption of the air and the headache resolved. One month later she developed a sensory disturbance in her feet. Neurological examination revealed reduced sensation to cold and pain to ankle level and bilateral suppressed knee and ankle reflexes but was otherwise normal. A spinal cord lesion (epidural abscess/hematoma) was excluded with an emergency T1 and T2 weighted magnetic resonance imaging (MRI) scan of the lumbar spine. Over the next 48 hr the sensory disturbance worsened to involve her legs and waist. Examination revealed a sensory loss to waist level, reduced joint position sense and vibration sense in her lower limbs and absent knee and ankle reflexes bilaterally, but normal power in both her legs. A further full length T2 weighted MRI scan of the spine showed a small area of high signal at the level of T3 compatible with transverse myelitis. This was treated with high dose corticosteroids and her symptoms resolved over the next three months. CONCLUSION: The etiology of transverse myelitis after dural puncture in a parturient could not be identified nor could any causal link be established between the dural puncture, pneumocephalus, and subsequent transverse myelitis.

Adult↗

Recurrent transverse myelitis in primary antiphospholipid syndrome--case report and literature review.

Transverse myelitis is an uncommon but well-known neurologic complication of systemic lupus erythematosus, and antiphospholipid antibodies have been implicated as having a pathogenic role of myelopathy in primary and secondary antiphospholipid syndrome. Transverse myelitis usually is monophasic, but recurrent transverse myelitis was reported in association with systemic lupus erythematosus, multiple sclerosis, and spinal arteriovenous malformations. It has been reported extremely rarely in patients with primary antiphospholipid syndrome. Herein we describe a patient with both conditions and propose that recurrent transverse myelitis should be included as a very rare feature of primary antiphospholipid syndrome.

Adult↗

Extensive myelitis associated with Mycoplasma pneumoniae infection: magnetic resonance imaging and clinical long-term follow-up.

Myelitis is a severe neurological complication associated with Mycoplasma pneumoniae infections. Little is known about the extent and the reversibility of this myelitis, and reports on the value of spinal imaging are inconclusive. To obtain more information on the diagnostic and prognostic value of spinal magnetic resonance imaging (MRI) we studied the clinical course and MRI long-term follow-up of two patients with extensive myelitis associated with M. pneumoniae infection. The neuroradiological findings were correlated with the clinical extent of the spinal syndrome, but their reversibility preceded clinical improvement. These preliminary findings indicate that follow-up spinal MRI may give valuable prognostic information in cases of M. pneumoniae associated myelitis and warrant further more systematic studies to ascertain the relationship between spinal MRI findings and prognosis.

Adult↗

Absence of cervical radiation myelitis after hyperfractionated radiation therapy with and without concurrent chemotherapy for locally advanced, unresectable, nonmetastatic squamous cell carcinoma of the head and neck.

PURPOSE: To evaluate the risk of developing radiation myelitis after a cervical spinal cord dose of 50.6 Gy given via 1.1 Gy b.i.d. fractionation during a prospective, randomised trial of hyperfractionated radiation therapy (HFX RT) to a total dose of 77 Gy given in 70 fractions of 1.1 Gy b.i.d., with and without concurrent low-dose, daily cisplatin (CDDP) for head and neck cancer. METHODS: Of 130 patients with locally advanced, unresectable, nonmetastatic squamous cell carcinoma of the head and neck (SCC H&N) who entered a prospective, randomised trial, 101 patients received 50.6 Gy to a portion of their spinal cord and survived > 1 year following the beginning of therapy. Forty-five patients were treated with HFX RT alone and fifty-six patients also received CDDP. RESULTS: None of these 101 patients developed cervical radiation myelitis. Therefore, it was not possible to investigate the influence of potentially contributing factors on the occurrence of radiation myelitis, such as interfraction interval, cord length, and administration of concurrent CDDP. CONCLUSIONS: Given the increasing number of studies with both altered fractionated regimens and concurrent radio-chemotherapy in SCC H&N, new studies with more patients are needed to gain better insight into the risks of developing cervical radiation myelitis.

Adult↗

Idiopathic severe recurrent transverse myelitis: a restricted variant of neuromyelitis optica.

Recurrent idiopathic transverse myelitis occur in multiple sclerosis (MS) and neuromyelitis optica (NMO). In NMO, acute optic neuritis and myelitis occur, either monophasic or relapsing, without clinical manifestations of involvement of other parts of the central nervous system (CNS). Recent evidence suggests that NMO is different from multiple sclerosis. The authors reported two patients having severe recurrent transverse myelitis sparing the optic nerves and cerebral hemispheres. Both patients had longitudinally extensive myelitis in some attacks with poor neurological outcome despite aggressive immunomodulatory therapy. One patient had prominent clinical features of brainstem injury with radiological and histological confirmation of brainstem involvement, and the other patient had trigeminal neuralgia suggestive of possible brainstem dysfunction. Histologically, prominent necrosis and neutrophilic infiltration of spinal cord tissue without eosinophils or hyalinized vessels were observed, and oligoclonal bands were absent in their cerebrospinal fluid. It is likely to be a distinct idiopathic inflammatory demyelinating disorder restricted to the spinal cord and brainstem different from MS, but within the spectrum of NMO with probably an autoimmune basis.

Adult↗

Recurrent demyelinating myelitis associated with hepatitis C viral infection.

We report a 46-year-old-man who developed recurrent myelitis associated with hepatitis C viral (HCV) infection. Spinal cord biopsy showed acute demyelination without evidence of vasculitis. Antibodies to HCV were present in the CSF; HCV RNA was not detected in the CSF. Neither HCV antigens nor RNA were detected in the spinal cord biopsy, whereas they were found in the liver biopsy. Evaluation for other infectious or autoimmune causes was unrevealing. These observations suggest that recurrent myelitis in this patient is etiologically related to HCV infection, possibly via an immune-mediated mechanism. This is the first report of pathologically proven myelitis associated with HCV infection and we suggest that HCV be considered in the differential diagnosis of the transverse myelitis syndrome.

Antigens, CD↗

Effect of immunotherapy in myelitis with atopic diathesis.

OBJECTIVE: A recent nationwide survey of myelitis with atopic diathesis in Japan disclosed that the disease frequently shows a chronic persistent course. A neuropathological study of the spinal cord also revealed chronic active inflammation. Since the effects of various immunotherapies have not been studied extensively in this condition, we evaluated the efficacies of various immunotherapies in patients with myelitis with atopic diathesis. PATIENTS AND METHODS: Forty-two treatments in 26 patients with myelitis with atopic diathesis were retrospectively analyzed. One of the following therapies was administered: (1) corticosteroids (CS) (pulse therapy followed by oral administration with gradual tapering); (2) intravenous immunoglobulin (IVIG) (400 mg/kg/day for 5 consecutive days); (3) plasma exchanges (PE); or (4) PE followed by IVIG or CS (PE+IVIG/CS). The therapeutic efficacies were evaluated by thorough neurological examination and laboratory tests including MRI, somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs). RESULTS: Objective neurological findings improved in 89% of the PE group and 90% of the PE+IVIG/CS group, compared with only 72% of the CS and 60% of the IVIG groups. Improvement determined by laboratory tests was seen in 57% of the PE and 57% of the PE+IVIG/CS groups, compared with only 15% of the CS and none of the IVIG groups. Thus, the improvement rate determined by laboratory tests was significantly greater for therapies including PE than for those without PE (p=0.0187). CONCLUSIONS: These data suggest that immunotherapy is effective in myelitis with atopic diathesis despite a chronic persistent course, and that PE is the most beneficial immunotherapy.

Adolescent↗

[Cytomegalovirus myelitis in immunocompetent adult].

INTRODUCTION: Cytomegalovirus (CMV) infection occurs in 40 to 100% of general population. It is often asymptomatic in immunocompetent subject but may induce neurological syndromes such as encephalitis and myelitis. CASE RECORD: We reported a case of a 64-years-old woman immunocompetent, with acute proximal upper and lower limb weakness, paresthesias and two episodes of urinary retention. MRI of the spine showed abnormal enhancement from cervical to lumbar spine indicative for myelitis. Diagnosis of CMV associated myelitis was confirmed by a positive CMV serology. Administration of ganciclovir was followed by a partial improvement in five months. DISCUSSION: Few cases of CMV acute myelitis in immunocompetent patients have been reported in the literature. The pathogenesis is not well known, however, immune-mediated central nervous system damage may be attributed to the pathogenesis of the disease. Early diagnosis and treatment improves the prognosis.

Antiviral Agents↗

Epidemiology and prognosis of acute myelitis in Southern Finland.

In this study we analyzed all acute adult (>15 years) myelitis cases in the province of Uusimaa in Southern Finland during the years 1981-1993. Only cases with acute infectious myelitis were included. Demyelinating diseases, and medullopaties due to degeneration, traumatic, toxic, hereditary, nutritional or metabolic causes were excluded. A total of 45 patients fulfilled the criteria. The mean incidence was 3.5 cases/million inhabitants/year. The mean latency time from the initial infection to the beginning of neurological symptoms was 11 days. Motor paraparesis was found in 62% and tetraparesis in 13%. Sensory symptoms were found in 82% and bowel disturbances were experienced by 71% of patients. Normal cerebrospinal fluid (CSF) leukocytes were seen in 18% of patients, and CSF protein was elevated in 70% of patients. Case fatality was 6.7%. Permanent care in hospital needed by 13% of patients, and after 24 months 88% were ambulatory. Prognosis is quite good in myelitis, and normal CSF leukocytes do not exclude myelitis.

Acute Disease↗

Transverse myelitis in children: long-term urological outcomes.

PURPOSE: Urological complications are well documented in patients with traumatic spinal cord injury. We examined the long-term urological outcomes in a large population of children affected by transverse myelitis. MATERIALS AND METHODS: We retrospectively reviewed the medical history, imaging studies and urodynamic findings in 22 children with transverse myelitis. Age at disease onset ranged from 3 months to 18 years (average 8.8 years). RESULTS: At a mean followup of 7.1 years 19 patients (86%) had persistent bladder dysfunction and 17 (77%) had persistent bowel dysfunction. Initial evaluation at least 1 month after disease onset revealed detrusor overactivity in 59% of patients, detrusor external sphincter dyssynergia in 41%, low compliance in 47% and detrusor leak point pressure greater than 40 cm water in 12%. Functional motor recovery and absence of lower extremity spasticity did not reflect normal urodynamic findings. Of the 19 patients with imaging available for review 5 (26%) had upper tract changes. One patient had development of chronic renal insufficiency. Low compliance (p = 0.02) and upper tract changes (p = 0.1) were more frequent in patients who started clean intermittent catheterization more than 2 years after disease onset. CONCLUSIONS: Persistent bowel and bladder dysfunction is common in transverse myelitis. Urodynamic abnormalities may be present despite normal neurological examination and absence of urinary symptoms. All pediatric patients with transverse myelitis require baseline renal ultrasound and urodynamic evaluation to guide treatment. Early institution of clean intermittent catheterization appears to preserve bladder compliance and decrease upper tract disease, and should be instituted at disease onset.

Adolescent↗

Experimental myelitis in BALB/cN and C57BL/6N mice caused by herpes simplex virus type 1 compared with herpes simplex virus type 2.

Intraperitoneal and footpad inoculations of herpes simplex virus type 1 (HSV) into BALB/cN (HSV-susceptible) and C57BL/6N (HSV-resistant) mice were carried out to induce experimental myelitis. Standard laboratory strains (McIntyre, F, RK, and recently Okinawa strain R1) were inoculated in mice. As a control, the HSV 2 standard laboratory strain SAV was also inoculated. The McIntyre strain was the most virulent, while the F strain was the least. RK and R1 were both moderately virulent. Myelitis was induced in BALB/cN mice after intraperitoneal and footpad inoculations of low to high doses of the McIntyre strain, and intraperitoneal inoculation of moderate and high doses of the RK and R1 strains. Symptoms of paraplegia of the hind legs and rectal and urinary incontinence were observed, but not until 3-5 hours before death. The symptoms caused by footpad inoculation were slightly different from those following intraperitoneal inoculation; rectal incontinence, in particular, was inconspicuous in the former. In the case of footpad inoculation of RK and R1, only one mouse inoculated with R1 showed symptoms and histology of myelitis. The F strain caused no symptoms. In the case of C57BL/6N mice, high dose intraperitoneal and footpad inoculations of the McIntyre strain also caused myelitis, and the symptoms were observed about 6-7 hours before death. In only one C57BL/6N mouse intraperitoneally inoculated with a high dose of R1 did symptoms appear about 6 hours before death. The same symptoms caused by intraperitoneal and footpad inoculations of HSV 2 (SAV) were observed more clearly and for a longer period (half to one day) than those caused by HSV 1 inoculation. Spinal cord necrosis was noted with McIntyre, RK and R1 inoculations, but it was not marked with randomly located foci, when compared with that caused by SAV. Further, the foci of necrosis in C57BL/6N mice were smaller than in BALB/cN mice, even when high dose McIntyre strain was used. Nuclear pyknosis and edema of the brain in the dead mice following HSV 1 inoculation were more marked than in those killed by SAV.

Animals↗

Absence of thoracic radiation myelitis after hyperfractionated radiation therapy with and without concurrent chemotherapy for Stage III nonsmall-cell lung cancer.

PURPOSE: To investigate whether thoracic spinal cord dose of 50.4 Gy given via 1.2 Gy b.i.d. fractionation carries a risk of developing radiation myelitis during studies using hyperfractionated radiation therapy (HFX RT) with and without concurrent chemotherapy (CHT). METHODS AND MATERIALS: Of 300 patients with Stage III nonsmall-cell lung cancer (NSCLC) who were treated on two consecutive Phase III studies, 158 patients received 50.4 Gy to a portion of their spinal cord and survived > 1 year after the beginning of the therapy. RESULTS: None of these 158 patients developed thoracic radiation myelitis. Therefore, influence of potentially contributing factors on the occurrence of radiation myelitis, such as interfraction interval, or those unproven yet, such as cord length or administration of concurrent CHT, was not possible to investigate. CONCLUSION: Given the continuing interest in HFX RT and encouraging results obtained in studies in lung cancer, further investigation is needed to get more informations about risks of developing thoracic radiation myelitis with this cord dose.

Adult↗

Polyradiculoneuritis with myelitis: a rare differential diagnosis of Guillain-Barré syndrome.

AIM: To describe the symptoms, signs, findings and prognosis in children suffering simultaneously from polyradiculoneuritis and myelitis. METHODS: Retrospective review of eight patients aged 2 to 13 years out of 210 patients with polyradiculoneuritis reported from 70 hospitals. Diagnostic criteria for polyradiculoneuritis were: flaccid paresis with loss of tendon reflexes, increased CSF protein and slowing of nerve conduction velocity. Criteria for myelitis were: severe and persistent bladder dysfunction, a sharply defined sensory level and/or evolving spastic paresis, with or without myelitic changes in spinal MRI. RESULTS: In the disease's earliest stage it was difficult to differentiate polyradiculoneuritis with myelitis from classical GBS. However, onset was often unusually rapid compared to GBS. Five patients developed a sensory level and seven suffered from severe bladder dysfunction. Four of the six children studied showed focal myelitic changes in MRI. All seven children with sufficient follow-up remained with residual paresis and significant long-term motor deficits. CONCLUSION: Due to its severe long-term prognosis, polyradiculoneuritis with myelitis must be differentiated from classical GBS. In the disease's early stage, the detection of a sensory level, severe bladder dysfunction and an unusually rapid onset can be helpful. The effect of high-dose corticosteroids is not yet clear. After the acute phase, most children require extended rehabilitation.

Adolescent↗

Myelitis due to varicella-zoster virus in two patients with AIDS: successful treatment with acyclovir.

Only a few cases of varicella-zoster virus (VZV) myelitis have been described, and nearly all have been diagnosed post-mortem. There have been no reports in the literature of successful treatment of VZV myelitis with antiviral medications. We report on two patients with AIDS who had acute severe myelitis accompanied by herpes zoster. The presence of VZV DNA in cerebrospinal fluid (CSF) was documented by the polymerase chain reaction (PCR) technique. Early treatment with acyclovir was followed by a slow but complete recovery after a phase of initial aggravation. After a follow-up of > 1 year, the two patients remained asymptomatic. We conclude that (1) VZV should be considered a curable cause of myelitis in patients with AIDS, (2) PCR assay of CSF will assist in early diagnosis, and (3) early treatment with acyclovir should aid in recovery.

AIDS-Related Opportunistic Infections↗

Longitudinal myelitis as an initial manifestation of systemic lupus erythematosus.

Transverse myelitis is a rare and serious complication of systemic lupus erythematosus (SLE). A longitudinal involvement of the spinal cord with lupus-related transverse myelitis is more unusual. Only 7 cases have been reported. We describe a 53-year-old woman presenting with short-term paraplegia as an initial manifestation of SLE with longitudinal myelitis. She had a partial response to treatment with pulse cyclophosphamide and high-dose corticosteroids after follow-up more than 2 years. To the best of our knowledge, this is the first case report of "longitudinal" myelitis as an initial presentation of SLE. Magnetic resonance imaging typically shows increased signal intensity in T2-weighted images, cord swelling, and contrast enhancement over several spinal segments. The possibility of SLE should be kept in mind in women presenting with paraplegia with no apparent cause.

Diagnosis, Differential↗

Evidence for a causal association between oral polio vaccine and transverse myelitis: A case history and review of the Literature.

A 6-month-old boy developed transverse myelitis 7 days after the receipt of oral polio vaccine (OPV). A paediatric neurologist confirmed the diagnosis when the boy was aged 9 years. The boy had received his first scheduled OPV at the age of 4 months and had developed immunity to serotypes 1 and 2 but not to serotype 3. A poliovirus type 3 was isolated from stool and throat specimens collected from the boy in the first 2 days after symptom onset. This was shown, in a World Health Organization accredited laboratory, to be a vaccine strain by nucleic acid probe hybridiztion and enzyme-linked immunosorbent assay. The boy subsequently developed immunity to poliovirus serotype 3. It is accepted that poliovirus infection can present occasionally as transverse myelitis. This is estimated to occur in 1:125-1:800 cases. It is also accepted that OPV can cause vaccine-associated paralytic polio with a frequency of approximately one case per 2.5 million doses of OPV distributed. It seems feasible therefore that OPV could cause transverse myelitis with a frequency of 1 in 300 million to one in two billion doses distributed. In a 1993 report from the Institute of Medicine of the National Acadamies of the United States pertaining to vaccine safety, theoretical criteria were advanced for the establishment of a causal relationship between a vaccine and a clinical outcome. The clinical history and laboratory results in this case satisfy these criteria, providing plausible evidence for the causal link between OPV and transverse myelitis.

Causality↗

Rapid onset transverse myelitis in adolescence: implications for pathogenesis and prognosis.

Five adolescents with transverse myelitis were reviewed. All presented with a rapid onset paralysis of the lower limbs and impairment of bladder control. The maximum disability developed between 10 minutes and six hours. There was no history of trauma, asthma, or prodromal illness. Investigations failed to demonstrate a vascular cause. Extensive spinal cord abnormalities were observed on magnetic resonance imaging. Electrophysiological investigations, performed in four cases, were all consistent with anterior horn cell damage. In all five adolescents there was poor recovery. The underlying pathogenesis of this rapid onset condition remains a subject of debate. Similarities with both transverse myelitis and fibrocartilaginous emboli are evident, widening the spectrum of conditions within the transverse myelitis umbrella. These observations suggest that in rapid onset "transverse myelitis" the combination of extensive hyperintensity on spinal cord neuroimaging with electrophysiological evidence of anterior horn cell involvement might have adverse prognostic significance.

Adolescent↗