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

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

A 34-year-old man with human immunodeficiency virus type 1 (HIV-1) presented with axial rigidity, painful spasms, and delayed hemiparesis and dementia. Cerebrospinal fluid analysis showed no antiglutamic acid dehydrogenase antibodies but viral genome from Epstein-Barr virus was detected by polymerase chain reaction. Clinical features and possible viral aetiology of progressive encephalomyelitis with rigidity are briefly discussed.
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PMID:Encephalomyelitis with rigidity complicating human immunodeficiency virus infection. 1183 67

Recent discoveries suggest that the resident cells of the central nervous system (CNS) the nerve cells and glia, play a more immunologically active role than was previously assumed. Neuroglial communication is of central interest in virtually all types of pathological conditions that affect the brain and several features of the activation that results from nerve cell damage resemble the type of innate immune reactions that occur in other parts of the body In particular, the characteristics of the activation of these CNS cells will affect both the interaction with cells of the immune system as well as processes related to neurodegeneration and regeneration. We here review data regarding 3 different aspects of local inflammatory activation in the rat nervous system: (i) the genetic heterogeneity of glial activation across inbred strains after nerve injury, (ii) expression of MHC class I genes in the CNS and (iii) neuroprotective effects of CNS antigen autoreactive immune reactions. Apart from neuroimmune diseases such as experimental autoimmune encephalomyelitis/multiple sclerosis, these features are also of relevance for a wider range of neurological diseases which present pathological signs of inflammation, such as Alzheimer's dementia, cerebrovascular diseases and CNS trauma.
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PMID:Neuroinflammation in the rat--CNS cells and their role in the regulation of immune reactions. 1208 14

Viliuisk encephalomyelitis (VE) is a neurodegenerative disorder expressed as subacute meningo-encephalitis progressing to a more prolonged pan-encephalitic syndrome with a fatal outcome within 1 to 10 years. Some patients survive to a steady state of global dementia and severe spasticity that may last for over 20 years. Multiple micronecrotic foci surrounded by inflammatory infiltrates are observed throughout the cerebral cortex and other gray matter areas. Infectious etiology of VE is strongly suspected, but the causative agent has not been identified. We conducted a search for assays that might be helpful for VE diagnosis and established for the first time that the majority of patients with definite VE show evidence for intrathecal IgG synthesis correlating with the clinical manifestations of the disease. This indicates that the detection of oligoclonal IgG banding in the cerebrospinal fluid is a valuable diagnostic assay for VE. Implications of these findings for a possible etiology of VE are discussed.
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PMID:Viliuisk encephalomyelitis: intrathecal synthesis of oligoclonal IgG. 1281 1

Hashimoto's encephalopathy may present with a wide variety of different neurological symptoms and signs. These include recurrent severe migrainous headache, psychoses, seizures, ataxia, dementia, stupor and coma. We present a personal series of 18 adult patients with Hashimoto's encephalopathy and a review of the literature in this paper. The natural history, laboratory abnormalities and neuroimaging data in these cases favour an immunopathological basis for this syndrome similar to relapsing acute disseminated encephalomyelitis. We suggest that Hashimoto's encephalomyelitis should be considered in the differential diagnosis of seizures, coma, atypical migraine and reversible dementia. Serological screening for anti-thyroid antibody should form part of the initial investigations in all relapsing and reversible encephalopathies.
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PMID:The clinical spectrum, diagnosis, pathogenesis and treatment of Hashimoto's encephalopathy (recurrent acute disseminated encephalomyelitis). 1287 Oct 97

About a third of patients with intravascular lymphoma (IVL) present to the neurologist with symptoms mimicking thromboembolic events. Diagnosis is difficult, and often made postmortem. As remission may be induced in almost half of patients with combination chemotherapy, early diagnosis of this rare disease is essential. We report two cases of IVL. A 62-year-old male presented with hyperacute myelopathy followed by cortical ischaemic events. The diagnosis was reached with frontal cortical and meningeal biopsy. A 56-year-old female had symptoms of transient ischaemic events, subacute dementia, weight loss and fever. As the disease progressed, she developed nephrotic syndrome and thrombocytopenia. Diagnosis was made postmortem. Our cases illustrate that IVL should be considered in the differential diagnosis of cerebral and systemic vasculitis and subacute bacterial endocarditis. Literature suggests IVL can also mimic Creutzfeld-Jakob disease and paraneoplastic encephalomyelitis.
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PMID:Neurological presentation of intravascular lymphoma: report of two cases and discussion of diagnostic challenges. 1612 73

A seventy-four-male with disorientation and convulsion was transferred to this hospital after three days fever which was unknown origin. Because the examination of cerebrospinal fluid were; cell count 1,560/3 (N : L = 4 : 1), protein 305 mg/dl, sugar 91 mg/dl, he was treated as encephalitis. However, MRI of the 18th hospital day revealed bilateral thalamic lesion and disseminated white matter lesions, suggesting acute disseminated encephalomyelitis. He left dementia after treatment and transferred to another hospital. Since, it is difficult to make a differential diagnosis between encephalitis and acute disseminated encephalomyelitis, early establishment of diagnostic criteria for acute disseminated encephalomyelitis is required.
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PMID:[A case of difficulty for differential diagnosis between encephalitis and acute disseminated encephalomyelitis]. 1685 21

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis of childhood and young adolescence due to persistent measles virus infection of the central nervous system. In majority of cases onset occurs from 5-15 years of age. In a nonimmunized population the average onset is 8 years. Children with SSPE had experienced natural infection with the rubeola virus at an early age, half before age 2 years. SSPE generally occurs 5-10 years after measles infection. In the early stages of the disease behavioral and personality changes is followed by myoclonic jerks and convulsions. In late stages dementia, stupor and coma develops. Diagnosis is achieved by typical clinical findings, measles antibody titer increase in cerebrospinal fluid (CSF) and serum, high amplitude, slow, sharp waves in EEG. Prognosis is poor and death ensues in about 3 yr after the diagnosis. Here it is presented a 7-years-old boy with involuntary movements in both hands, drop attacks while walking, ataxia and stupor. Due to suggestive radiological and clinical findings and a history of recent mumps infection he was thought to have acute disseminated encephalomyelitis initially and given treatment. But due to clinical deterioration and detection of anti measles IgG in serum and CSF, SSPE diagnosis was confirmed. With this SSPE case presenting initially as ADEM, the authors tried to emphasize that presentation of SSPE may clinically and radiologically be diverse and a thorough differential diagnosis is mandatory for a definite diagnosis.
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PMID:Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis. 1720 44

Susac's syndrome (SS) consists of the triad of encephalopathy, branch retinal artery occlusions (BRAO), and hearing loss. It usually affects women aged 20 to 40, but men are also affected, and the age range extends from 9 to 72 years. It tends to be unrecognized, even in major academic centers. The complete triad may not be present at the onset, which makes diagnosis more difficult. However, since this disorder is treatable, early diagnosis is important. The encephalopathy is usually associated with headaches, multifocal neurologic manifestations, and psychiatric features (particularly paranoia). MRI shows a white matter disturbance that is frequently confused with multiple sclerosis and acute disseminated encephalomyelitis. During the encephalopathy, the corpus callosum is always affected and shows central involvement--small to large "snowballs" and linear defects, "spokes." As the acute changes (microinfarcts) resolve, central callosal "holes" develop, a pathognomonic finding. The deep gray matter (70%) and leptomeninges (33%) also may be involved. Dilated fundus examination will reveal branch retinal artery occlusions. Fluorescein angiography may disclose pathognomonic staining of the arterioles proximal to the occlusions and of nonoccluded arterioles. The cochlear hearing loss, sometimes associated with vertigo, is usually bilateral, and deafness becomes a major disabling problem. Brain biopsies, anatomic observations, and responses to immunosuppressive therapy suggest that SS represents an autoimmune endotheliopathy in the microvasculature of the brain, retina, and cochlea. Treatment requires immunosuppression. High-dose corticosteroid therapy is the mainstay, but additional therapies such as intravenous immunoglobulin, mycophenolate mofetil, and cyclophosphamide are often necessary. Rituximab is the newest therapy to consider. Treatment should be prompt, aggressive, and sustained to avoid the dreaded residuals of dementia, deafness, and blindness.
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PMID:Treatment of Susac's Syndrome. 1832 1

A 65-year-old man was admitted to our hospital complaining of diplopia, dysarthria, difficulty in walking and progressive dysesthesia that developed in his left hand and leg. Brain MRI revealed high signal intensity regions on T2-weighted and FLAIR images of the hippocampus and the corpus amygdaloideum. After admission, the patient's neurological symptoms progressed to delirium and dementia with hallucinations. When he eventually developed severe respiratory failure requiring ventilatory support, brain MRI revealed new high signal intensity regions on T2-weighted images of the medulla oblongata and pons. Chest CT scans showed a mass under the aortic arch, and based on subsequent histopathological examination of a transesophageal endoscopic ultrasonography-guided fine needle aspiration biopsy of the tumor, a diagnosis of small cell lung cancer was made. In addition, anti-Hu antibody was found in the patient's serum, leading to a diagnosis of paraneoplastic encephalomyelitis/sensory neuropathy. One course of chemotherapy (carboplatin + etoposide) was administered; however, the protocol was not completed because the patient developed severe pneumonia. Given that neurological symptoms usually precede a diagnosis of malignancy in paraneoplastic neurological syndromes, it is important that these are considered carefully, as they may contribute to early diagnosis and treatment. Here we report a rare case of severe central hypoventilation in paraneoplastic encephalomyelitis/sensory neuropathy.
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PMID:[Paraneoplastic neurological syndrome accompanied by severe central hypoventilation and expression of anti-Hu antibody in a patient with small cell lung cancer]. 1851 96


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