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Query: UMLS:C0014070 (encephalomyelitis)
 13,017 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Infectious agents have been postulated as causes of multiple sclerosis for over a century. The possible role of a virus or viruses is supported by data that (1) a childhood exposure is involved and "viral" infections may precipitate exacerbations of disease, (2) experimental infections in animals and natural infections in humans can cause diseases with long incubation periods, remitting and relapsing courses, and demyelination, and (3) patients with multiple sclerosis have abnormal immune responses to viruses. The pathogenesis of three human demyelinating diseases of known viral etiology is discussed. In progressive multifocal leukoencephalopathy, a papovavirus selectively infects oligodendrocytes and causes focal areas of demyelination. In postmeasles encephalomyelitis, the virus is lymphotrophic and disrupts immune regulation that can result in an autoimmune perivenular demyelinating illness without evidence of infection of the central nervous system. In human immunodeficiency virus-encephalopathy and myelopathy virus is present in macrophages and microglia and the myelin abnormalities apparently are caused by soluble factors such as viral proteins, cytokines, or neurotoxins. These findings may have implications on how, when, and where to seek viruses in multiple sclerosis.
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PMID:The virology of demyelinating diseases. 801 89

Clinically, the Schumacher Panel criteria remain the best set of diagnostic criteria. Two subsets therein are definable, i.e., exacerbating-remitting (ER) and chronic progressive (CP), with the latter subdivided into progressive from onset and secondarily progressive. A clinically stable stage can also be recognized. It has been customary in treatment trials to separate ER and CP patients. End point for the latter is a comparison of neurologic status at the end of the trial with that entry. A similar assessment can be made for ER patients. With this criterion both types could be included in a single study. One could also, though, treat the exacerbation in an acute study or assess whether exacerbations can be prevented in a long-term trial. Most clinicians no longer consider monophasic disease as multiple sclerosis (MS). Depending on clinical extent, such patients are divisible into acute disseminated encephalomyelitis, Devic disease, transverse myelopathy, or optic neuritis. Each subgroup could be studied as with an acute exacerbation or in long term as to whether future and different neurologic insults can be prevented. One measure of neurologic status is the Disability Status Scale (DSS), which grades clinical impairment due to MS on a 0 (normal) to 10 (death due to MS) basis. The expanded DSS (EDSS) subdivides each step 1 through 9 into two. Type and severity of neurologic impairment is defined by graded involvement in the following eight functional systems (FS): pyramidal, cerebellar, brainstem, sensory, bowel and bladder, visual, cerebral, and other. Frequency and severity of each FS correlates strongly with DSS scores.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Clinical definition for multiple sclerosis treatment trials. 801 92

A 33-year-old man was admitted to our hospital because of tetraplegia and dyspnea in November 4, 1991. He had been healthy until October 22, 1991, when he developed difficulty in urination, ascending paralysis, respiratory paralysis and oscillopsia. On admission, neurological examination showed transverse myelopathy below the level of fourth cervical cord segment, opsoclonus and respiratory paralysis, which developed apnea. Cell numbers and protein concentration in CSF were elevated 37/mm3 and 380 mg/dl respectively. MRI disclosed increased signal intensity area at the upper cervical cord and brainstem. From these findings, the patient was diagnosed as acute disseminated encephalomyelitis (ADEM). Cases of ADEM associated with respiratory paralysis in the acute stage are rare. An extended lesion of respiratory upper motor neuron descending from the medulla oblongata to the upper cervical cord may be involved, and resulted in respiratory paralysis in this case.
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PMID:[Acute disseminated encephalomyelitis presented with apnea in the acute stage]. 802 28

An interesting neurological syndrome, characterized by recurrent optic neuritis, cervical myelopathy from syringomyelia, paraparesis, amenorrhea-galactorrhea, and other endocrine problems, has been described among young black women in the French West Indies. The etiology remains unknown, but possible links with Devic's disease, acute disseminated encephalomyelitis, and neurotoxicity from quinolines in Annona muricata teas have been postulated. The largest epidemic of neuropathy in this century occurred in Cuba in 1991-1994. Clinical features and etiologic studies are reviewed. Its primary cause was nutritional. A similar epidemic was recently described in Tanzania. A number of infectious neuropathies and myopathies are reviewed, including leprosy, tuberculosis, hemorrhagic fevers (Ebola and Marburg filoviruses, Lassa, Argentinean and Bolivian arenaviruses), the human retrovirus human T-cell lymphotropic virus type I, Lyme disease and postimmunization neuropathies. The tropics continue to contribute interesting and important clinical conditions that may illuminate the etiopathiogenesis of other common disorders.
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PMID:Tropical myeloneuropathies revisited. 984 4

Paraneoplastic syndromes are disorders associated with cancer but without a direct effect of the tumor mass or its metastases on the nervous system. Small cell carcinoma of lung associated with paraneoplastic sensory neuronopathy and/or paraneoplastic encephalomyelitis with the presence of anti-Hu antibodies has been termed "anti-Hu syndrome." Anti-Hu associated PSN-PEM is an immune disorder in which both cell-mediated and humoral mechanisms are involved. Patients are considered affected by Anti-Hu associated PSN-PEM when they develop clinical signs and symptoms of CNS dysfunction and/or sensory neuropathy not caused by metastases or other disorders, and serum or cerebrospinal fluid is positive for Hu abs. SCLC is found in more than 90% of patients with cancer and positive Hu abs. Individual patients with Hu abs associated to SCLC may suffer PSN-PEM, limbic encephalitis, brainstem encephalopathy, opsoclonus-myoclonus, paraneoplastic cerebellar degeneration or myelopathy. Hu abs have a specificity of 99% and sensitivity of 82% in detecting paraneoplastic neurological syndromes. There are two types of treatment: the first is to treat the cancer, the second is to suppress the immune reaction with the use of corticosteroids, cyclophosphamide, azathioprine, plasma exchange, intravenous immunoglobulin and immunoadsorption; however, treatment of paraneoplastic syndromes is generally unsatisfactory.
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PMID:Paraneoplastic syndromes associated with anti-Hu antibodies. 1134 32

Although infectious myelopathies are rare, appropriate aetiological diagnosis is of crucial importance to improve outcome. Lyme disease causes a myelomeningoradiculitis during the second stages and a progressive encephalomyelitis during the third stage. Serum and cerebrospinal fluid antibody tests are the basis of diagnosis. Myelopathy usually responds to treatment with ceftriaxone or doxycycline. Vacuole myelopathy occurs in patients with human immunodeficiency virus infection. It is characterised by a progressive spastic and ataxic paraparesis. About half of patients have symptoms of dementia. No specific treatment is available. Human T-cell lymphotropic virus type I is endemic in tropical areas and particularly in French West Indies. It causes a chronic spastic paraparesis with sexual and bladder dysfunction. Diagnosis depends on the positive antibody response in both serum and cerebrospinal fluid, and on the exclusion of other causes of spinal cord disease. There is no specific treatment.
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PMID:[Spinal cord infections]. 1150 90

We report a 63-year-old man with acute disseminated encephalomyelitis (ADEM), initially showing depression for one and a half months but subsequently meningoencephalitis followed by acute-onset myelopathy. Neuroradiological examinations of the brain demonstrated no focal lesion causative for his depression, while cerebrospinal fluid revealed elevated levels of inflammatory cytokines in parallel with disease activity. Because depression is usually a rare initial symptom for patients with ADEM, an increased production of inflammatory cytokines in the central nervous system as well as age-related alterations of immune response might have played an important role in the development of depression in this elderly patient.
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PMID:Severe depression as an initial symptom in an elderly patient with acute disseminated encephalomyelitis. 1175 74

In recent years, advances in the diagnosis and treatment of herpes simplex encephalitis (HSE) have been achieved due to the prevalence of antiviral drugs and the introduction of the polymerase chain reaction (PCR) to test the cerebrospinal fluid. The several clinical forms of herpes simplex virus type 1 (HSV-1) infections of the central nervous system (CNS), including acute disseminated encephalomyelitis and brainstem encephalitis, have been clarified. However, fatal, prolonged, or relapsed cases are still observed, and early detection and appropriate treatment is necessary to lead to a good prognosis for these intractable HSE cases. In adult HSV-2 infections, meningitis and myelitis associated with genital herpes are common. In the past, HSV-2 myelitis has been reported as a form of fatal necrotizing myelopathy; however, using PCR and magnetic resonance imaging studies, mild surviving cases are increasingly likely to be identified. Meanwhile, various CNS syndromes resulting from the herpes group viruses, including varicella-zoster virus and Epstein-Barr virus have also been reported. These herpesviruses have several characteristics in common, e.g., they exist in the latent state and they occur in both mucocutaneous and CNS infections. Adult HSV-1 and -2 infections of the CNS are discussed together with other herpes group virus infections of the CNS.
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PMID:Herpesvirus infections of the central nervous system. 1197 Nov 55

A patient with exanthem and fever showed progressive disturbance of consciousness and flaccid quadriplegia predominantly in the lower extremities. Antibiotics, aciclovir, high-dose methylprednisolone (1 g/day for 3 consecutive days) and IVIG (400 mg/kg/day for 5 consecutive days) were not effective. Nerve conduction study and SEP in the lower extremities showed peripheral and central conduction block. EEG showed irregular sharp and slow waves predominantly in the left hemisphere. ABR and SEP in the upper extremities were normal. Consecutive studies of cranial and spinal MRIs showed no abnormalities. A diagnosis of acute disseminated encephalomyelitis (ADEM) was made. We started administration of ultra-high-dose methylprednisolone (5.4 mg/kg/h for 47 hours), the dose for acute spinal cord injury based on the randomized controlled trial of The Third National Acute Spinal Cord Injury Study in the USA. After this, she regained consciousness and the quadriplegia improved. The abnormalities in the electrophysiological studies also normalized. It is thought that the neuroprotective mechanism of ultra-high-dose methylprednisolone could be attributed to its inhibition of lipid peroxidation, secondary, ischemia, energy failure and so on. If the usual treatment is not effective for severe encephalomyelitis cases, we can consider the administration of ultra-high-dose methylprednisolone as one of the new treatment options.
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PMID:[A patient of ADEM with central and peripheral conduction block improved with ultra-high-dose methylprednisolone]. 1247 95

Acute encephalomyelitis in children refers to an insult of cortical white matter leading to acute disseminated encephalomyelitis, insult of the spinal cord leading to multifocal myelopathy, or a combined form of encephalomyelitis. We report here the clinical presentations and outcome of 16 children with severe acute encephalomyelitis analyzing the effect of high-dose methylprednisolone or intravenous immunoglobulins, administered separately or in combination. Five children developed acute disseminated encephalomyelitis alone, eight developed severe multifocal myelopathy accompanied in two of them by radiculoneuropathy, and three developed the most severe form of combined encephalomyeloradiculoneuropathy. The indications for treatment with either high-dose methylprednisolone, intravenous immunoglobulin, or a combination of the two were severe acute disseminated encephalomyelitis, visual loss, or severe flaccid weakness accompanied by bladder and bowel incontinence. Overall, 10 children had remarkably responded to high-dose methylprednisolone alone and recovered within 10 days. One patient with severe myelopath, developing paraplegia, who failed oral corticosteroids completely recovered following intravenous immunoglobulin. Of the isolated acute disseminated encephalomyelitis group, all patients were initially treated with high-dose intravenous methylprednisolone and recovered within 10 days, including visual remission in the child with severe optic neuritis. All six children with solitary severe multifocal myelopathy were treated with high-dose methylprednisolone alone and recovered within the first week. Two patients had severe myeloradiculoneuropathy and were therefore treated with combined high-dose methylprednisolone and intravenous immunoglobulin: one remains paraplegic, whereas the second was ventilated for 3 weeks and recovered after 2 months. The three children with the most severe form of encephalomyeloradiculoneuropathy were treated with combined high-dose methylprednisolone and intravenous immununoglobulin; two remain severely handicapped, of whom one is paraplegic, and the third unexpectedly recovered within 3 months. Therefore, our experience indicates that either high-dose methylprednisolone or intravenous immunoglobulin, given separately or combined, may be efficacious in severe debilitating pediatric-onset acute encephalomyelitis. In children with the most severe form of encephalomyeloradiculoneuropathy, we suggest initially administering high-dose methylprednisolone and intravenous immunoglobulin combined, given the poorer outcome of our patients with combined severe central and peripheral demyelination.
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PMID:Outcome of severe encephalomyelitis in children: effect of high-dose methylprednisolone and immunoglobulins. 1258 19


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