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Acute myelitis with hyperIgEaemia and mite antigen specific IgE: atopic myelitis.

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, 56 patients 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.

Adult↗

Clinical, immunological and MRI features of myelitis with atopic dermatitis (atopic myelitis).

In order to clarify the characteristic features of myelitis with atopic dermatitis (AD), we compared the clinical, immunological and MRI findings between 14 myelitic patients with AD and 12 myelitic patients without AD. The myelitic patients with AD showed the following distinct features, compared with those without AD. (1) A preferential involvement of the cervical cord, as shown by neurologic as well as MRI examinations (14/14 vs. 5/12; P=0.0012), (2) paresthesia/dysesthesia as the predominant symptoms and a rare occurrence of definite muscle weakness (0/14 vs. 5/12; P=0.0120) and dysuria (1/14 vs. 8/12; P=0.0029), (3) a lower Expanded Disability Status Scale score (mean, 1.5 vs. 3.5; P=0.0018), (4) normal cerebrospinal fluid (CSF) findings including those for the IgG index and oligoclonal IgG bands and (5) a persistence of neurologic symptoms and MRI lesions during the follow-up periods (mean, 17 months). In addition, both the serum total IgE level and the frequency of specific IgE to Dermatophagoides farinae were significantly higher in the myelitic patients with AD (median IgE=1266 U/ml, specific IgE 14/14) than in those without AD (145 U/ml, P=0.0034 and 8/12, P=0.0331, respectively) and in 40 healthy controls (86 U/ml, P<0.0001 and 12/40, P<0.0001, respectively). Since myelitis with AD has distinct features and atopy to mite antigens appears to be the underlying cause of this condition, it may therefore be a distinct subtype of myelitis.

Acute Disease↗

Herpes simplex myelitis as a cause of acute necrotizing myelitis syndrome.

We report a patient with herpes simplex virus (HSV) myelitis with a clinical history of rapidly progressive spinal cord dysfunction and extensive necrosis of the lumbosacral spinal cord at autopsy. The etiologic agent was HSV type 2. The close resemblance of the clinical features of this case to the syndrome of acute necrotizing myelitis emphasizes the need to recognize HSV myelitis as a cause of this syndrome, since it is diagnosable during life by culturing the CSF and is treatable.

Acute Disease↗

Multifocal relapsing-remitting myelitis in a patient with atopic dermatitis: multiple sclerosis or atopic myelitis?

We describe a patient with multifocal relapsing-remitting myelitis. This case had abnormal high intensity lesions in the spinal cord at the cervical and thoracic levels on magnetic resonance imaging (MRI). She had complicated atopic dermatitis and high serum IgE levels, which showed strong sensitivity to mite antigen. These features closely resemble those of atopic myelitis, suggesting that atopic myelitis may develop a relapse with multifocal involvement of the spinal cord, which can mimic multiple sclerosis.

Adult↗

[Adenovirus myelitis and Epstein-Barr myelitis: two unusual viral causes with similar presentations].

We report two cases of viral myelitis, one associated with adenovirus and the other with Epstein-Barr virus. The clinical presentation and course were similar, with viral illness, paraplegia with a D4 sensory level, and fever. Spinal magnetic resonance imaging showed a T2 cervico-dorsal high intensity signal. Cerebrospinal fluid examination showed pleocytosis without oligoclonal bands. Both patients recovered after treatment with high-dose methylprednisolone. These two cases suggest a similar clinical and paraclinical pattern in viral transverse myelitis. Moreover, treatment with corticosteroids seems to be useful in viral myelitis.

Adenoviridae Infections↗

Spinal cord lesions of myelitis with hyperIgEemia and mite antigen specific IgE (atopic myelitis) manifest eosinophilic inflammation.

We report the neuropathological findings of spinal cord specimens obtained from two patients who had localized myelitis with hyperIgEemia and mite antigen specific IgE (atopic myelitis). Both cases showed mild spinal cord dysfunction, and the gadolinium-enhanced area of the isolated spinal cord lesion observed on MRI was biopsied, respectively. Neuropathologically, both cases showed many perivascular lymphocyte cuffings associated with disrupted vessels, and the infiltration of eosinophils in the spinal cord lesions. Both myelin and axons were lost in the lesions, which were associated with astrogliosis. These findings suggest that an allergic mechanism may play a role in this condition.

Adult↗

High incidence of subclinical peripheral neuropathy in myelitis with hyperIgEaemia and mite antigen-specific IgE (atopic myelitis): an electrophysiological study.

OBJECTIVE: To study subclinical involvement of the peripheral nerves in myelitis with hyperIgEaemia and mite antigen-specific IgE (atopic myelitis: AM). MATERIAL AND METHODS: We carried out a nerve conduction study of the median, ulnar, tibial, and sural nerves in 21 patients with AM and in 28 patients with clinically definite or laboratory-supported definite multiple sclerosis (MS). RESULTS: The patients with AM showed a significantly higher frequency of abnormal records than the MS patients in the sensory nerve conduction study (52.4% vs. 14.3%, p = 0.0106). The frequency of abnormal records in the motor nerve conduction study in AM patients was twice as high as in MS patients (38.1% vs. 17.9%), but the difference was not statistically significant. Abnormality in the F-wave-evoked frequency in the median nerve was also significantly more common in AM patients than in MS patients (57.9% vs. 10.7%, p = 0.0016). CONCLUSIONS: These findings suggest that subclinical peripheral neuropathy is frequent in patients with AM.

Adult↗

Myelitis associated with atopic disorders in Japan: a retrospective clinical study of the past 20 years.

OBJECTIVE: To clarify the clinical features of myelitis associated with atopic disorders in Japanese patients. SUBJECTS AND METHODS: We retrospectively studied the clinical, immunological and electrophysiological features of 68 consecutive patients with myelitis of acute or subacute onset diagnosed at Kyushu University Hospital during the past 20 years. RESULTS: While only 2 of 28 (7%) patients with myelitis diagnosed between 1979 and 1993 had either atopic dermatitis (AD) or bronchial asthma (BA), 19 of 40 (48%) patients with myelitis diagnosed between 1994 and 1998 did. Among the 40 patients with myelitis diagnosed between 1994 and 1998, 19 patients with either AD or BA as well as 21 patients without either disease showed a significantly higher level of serum total IgE, higher frequency of hyperIgEaemia and higher frequency of mite antigen-specific IgE than 82 healthy controls. Myelitis patients with AD presenting as persistent paresthesia/dysesthesia in all four limbs showed cervical cord lesions on MRI and abnormalities in upper limb motor evoked potentials but no abnormalities in the cerebrospinal fluid (CSF), while myelitis patients with BA showed preferential involvement of the lower motor neurons clinically and electromyographically. In addition, 12 patients with myelitis who had hyperIgEaemia and mite antigen-specific IgE but neither AD nor BA showed incomplete transverse myelitis with mild motor disability and few CSF abnormalities. CONCLUSION: The clinical features of myelitis associated with atopic disorders were in part distinguished by the type of preceding atopic disorder, and also were different from those of hyperIgEaemic myelitis with no preceding atopic disorders.

Adult↗

Magnetic resonance imaging findings in 22 cases of myelitis: comparison between patients with and without multiple sclerosis.

We reviewed the magnetic resonance imaging (MRI) database of the Dent Neurologic Institute to study the abnormal findings in myelitis. We identified 22 patients, and compared non-MS-related acute transverse myelitis (ATM, n = 9), to myelitis associated with multiple sclerosis (MS-myelitis, n = 13). The ATM patients were significantly older than MS patients at the time of the myelitis diagnosis (mean age 46 vs 35, p < 0.05). ATM appeared as a "longitudinal myelitis", with fusiform cord expansion on T1-weighted images and intramedullary increased signal on T2-weighted images, each involving multiple spinal levels (mean = 7-8). However, MS-myelitis lesions appeared focal, involving significantly fewer spinal levels (mean = 1-2, p < 0.001), although the lesions were equally likely to expand the cord. Four (42%) of the ATM lesions showed abnormal, variable enhancement, whereas none of the MS myelitis lesions enhanced. Cranial MRI was more likely to be normal in ATM (78%) than in MS-myelitis patients (15%, p < 0.001). Although readily distinguishable from lesions due to MS, the various etiologies for ATM, including post-infectious (n = 2), post-vaccination (n = 3), and idiopathic (n = 4) were indistinguishable on MRI. The MRI findings of an extensively lesioned, swollen cord, suspicious for an intramedullary tumor and providing a temptation for a biopsy, may reflect a non-neoplastic inflammatory disorder.

Journal Article↗

Lupus-related myelitis: serial MR findings.

PURPOSE: To correlate the MR findings in transverse myelitis secondary to systemic lupus erythematosus with clinical findings during disease exacerbation and remission. METHODS: Four patients (ages 33 to 47 years) with episodes of transverse myelitis secondary to systemic lupus erythematosus were identified. Three patients had recurrent transverse myelitis episodes (one patient with two recurrences), for a total of eight episodes. MR examinations (six after contrast administration) were performed during each transverse myelitis episode, as well as during four periods of remission (in three patients) after therapy with steroids and/or immunosuppressive agents. MR examinations were reviewed for the presence of spinal cord enlargement, intramedullary signal abnormality, and contrast enhancement. RESULTS: Prolongation of T1 or T2 signal (or both) was seen in eight episodes (100%). Spinal cord enlargement was seen in six (75%) of eight transverse myelitis episodes, although it was mild during two episodes. Contrast enhancement was seen in three of six transverse myelitis episodes (dense, inhomogeneous enhancement during two episodes in one patient, and a small focus of enhancement in one patient). During periods of remission, spinal cord diameter returned to normal, and no contrast enhancement was seen, although abnormal signal was present in three examinations performed within 2 months of a transverse myelitis episode. CONCLUSION: Spinal cord widening and signal abnormalities are common MR findings during episodes of transverse myelitis related to systemic lupus erythematosus, and contrast enhancement is less frequently seen. Improvement or resolution of these findings correlates with clinical improvement.

Adrenal Cortex Hormones↗

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↗

[Three cases of herpes simplex virus type 2 myelitis--detection of HSV2 DNA in cerebrospinal fluid].

Polymerase chain reaction (PCR) technique has been successfully used to detect herpes simplex virus (HSV) from patients with HSV encephalitis. By PCR assay capable of differentiating HSV1 and 2, we detected HSV 2 DNA in cerebrospinal fluid (CSF) from patients with HSV myelitis and discussed the clinical findings. Three cases of HSV myelitis (a 49-year-old female, two 38- and 44-year-old males) were studied. All cases were characterized by transverse myelopathy of the thoracic cord, and two patients had recurrence. In all cases HSV1 antibodies were significantly elevated in serum and CSF. We used 500 microliters of CSF for PCR, and prepared one common upstream primer and two type specific downstream primers for HSV1 and HSV2. Using three primers simultaneously different sizes of PCR products were amplified from HSV1 and HSV2 DNA. PCR products subjected to electrophoresis on 1.2% agarose and stained with ethidium bromide. Still more southern blot hybridization was performed to detect DNA by 35S-end-labelled oligonucleotide prove. HSV2 DNA was amplified from CSF in all cases by PCR, and HSV2 DNA was detected at both first and second episode in two relapsing myelitis. No case of relapsing myelitis by HSV2 has been reported. The PCR technique is useful for diagnosis of HSV1 and 2 myelitis, and its would suggest that some patients of idiopathic myelopathy could be due to HSV2 myelitis and HSV2 myelitis may not be rare.

Adult↗

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↗

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↗

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↗

[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↗