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

The clinical and pathological features of a fourth patient with progressive encephalomyelitis with rigidity are reported and compared with those previously described. It is suggested that the muscular rigidity, abnormal postures, painful muscular spasms, and myoclonus are a product of excessive and abnormal discharges of alpha motor neurones caused by their release from control by spinal internuncial neurones. A count of neuronal perikarya in the ventral horns confirmed that the disease selectively destroyed small and medium sized neurones, most of which were spinal internuncial neurones. Experimental, clinical, and pathological evidence concerning spinal internuncial neurones is reviewed and found to conform to this theory. The pathogenesis of opsoclonus may be similar.
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PMID:Spinal internuncial neurones in progressive encephalomyelitis with rigidity. 50 76

We describe a case of continuous motor unit potential (MUP) activity of central origin (unlike stiff man syndrome and progressive encephalomyelitis) characterized clinically by rigidity, painful muscle spasms, abnormal postures and spinal myoclonus. The topography of the manifestations, the subacute and benign course, the presence of stable sequels 2 years after onset and a searching process of differential diagnosis lead us to attribute the condition to an inflammation of the cord, which makes the case of particular clinical interest.
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PMID:Rigidity and painful muscle spasms in a patient with probable myelitis. 178 38

Paraneoplastic cerebellar degeneration (PCD) is a rare complication of systemic cancer. PCD may present as a "pure", severe pan-cerebellar syndrome of subacute progression or be only one clinical feature in the setting of extensive CNS disease. The most characteristic form of "pure" PCD is associated with the presence of an anti-Purkinje cell antibody (AB), called anti-Yo, in patients with breast or ovarian cancer. The primary tumor is very often unknown when the cerebellar signs occur, and extensive investigations, including laparotomy or prolonged follow-up may be required to demonstrate its presence. More rarely, others AB than anti-Yo are discovered during PCD. Almost 50% of patients with "pure" PCD do not have circulating anti-neuronal AB. In the cases, the primary cancer is more often known and the clinical course of the cerebellar syndrome may be slower. Cerebellar degeneration may also occur during paraneoplastic encephalomyelitis. In this setting, the cerebellar signs which may be isolated at the onset, become associated with other signs of neuraxis involvement (limbic encephalitis, brainstem encephalitis, myelitis and particularly, subacute sensory neuronopathy) during the course of the disease. When a paraneoplastic encephalomyelitis is associated with a small cell lung cancer, an antineuronal AB called anti-Hu is frequently found. Finally PCD may be associated with the opsoclonus-myoclonus syndrome with the Lambert-Eaton syndrome.
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PMID:[Paraneoplastic cerebellar degeneration]. 786 50

A 57-year-old woman without a known neoplasia developed opsoclonus, myoclonus, and ataxia. Positive anti-Ri antibodies were present in both serum and CSF. The patient also had progressive encephalomyelitis with rigidity, an association not previously described.
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PMID:Anti-Ri antibodies associated with opsoclonus and progressive encephalomyelitis with rigidity. 805 63

In the last decade, anti-neurons autoantibodies have been found in serum and cerebro spinal fluid of patients suffering from neurological paraneoplastic syndrome. This discovery has made possible to improve the knowledge of these syndromes as well as to characterize some proteins specific to the nervous system, unknown until now. Paraneoplastic encephalomyelitis can manifest together with an ataxic sensitive neuropathy, a limbic encephalitis, a brainstem or cerebellum syndrome. This encephalomyelitis is almost always associated with a small cells lung cancer and auto-antibodies, called anti-Hu, which recognize all the neurons of the nervous system. Patients with paraneoplastic cerebellar degeneration present an antibody, called anti-Yo, directed against Purkinje cells. In this case, the tumor is a gynecologic cancer. Patients presenting with an opso-myoclonus and a breast cancer have an antineurons anti-body, called anti-Ri, which is absent when the opso-myoclonus is associated with a lung cancer or a neuroblastoma. These three antibodies are the most frequently found and the best studied, but others, rarer, have been described. The genes coding for the proteins recognized by these three antibodies have been cloned. These proteins seem to play a major role in the neuronal maturation and homeostasis. These antibodies prove to be irreplaceable tools to study the phenomenons subtending the neuronal degeneration and the cellular proliferation.
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PMID:[Value of the detection of anti-nervous system autoantibodies in neurologic paraneoplastic syndromes]. 805 19

The clinical, biochemical, neuroimaging and neurophysiological findings of eight patients with stiff man syndromes (SMS) [four of six being tested with autoantibodies against glutamic acid decarboxylase (GAD)] are presented. In two patients (one GAD-positive, one GAD-negative), transient oculomotor disturbances suggested progressive encephalomyelitis with rigidity and myoclonus (PERM) as differential diagnosis. The catalogue of characteristic clinical symptoms of SMS is extended by three new symptoms: (1) an aura-like feeling reported by five patients to precede spontaneous spasmodic attacks; (2) a stereotyped motor pattern seen in seven patients during spasmodic jerks, consisting of brief opisthotonos, stiffening of the slightly abducted legs and inversion of the plantar-flexed feet; (3) a paroxysmal fear when crossing a free space unaided, or even thinking of it. Clinical findings did not enable us to discriminate between patients tested GAD-positive or GAD-negative. Cerebrospinal fluid contained elevated immunoglobulin levels or cell counts, or both, in the majority of patients. Autopsy of one patient revealed scattered lymphocyte cuffs around leptomeningeal, intracerebral and particularly intraspinal vessels, suggesting a mild inflammatory process. Whether SMS and PERM are closely related is discussed; they are possibly both manifestations of a spectrum of encephalomyelopathies having autoimmunity against GABAergic neurons in common.
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PMID:Stiff man syndrome: clinical and laboratory findings in eight patients. 2154 85

The medical records of 220 dogs with inflammatory/infectious diseases of the central nervous system (CNS) were retrospectively examined. The aims of the study were to determine if clinical and clinicopathologic data (not including biopsy or necropsy examination) could distinguish inflammatory CNS diseases from diseases of other types, and to search for criteria allowing differentiation of specific inflammatory diseases. The signalment, historical findings, extraneural and neurological signs, and the lesion site contributed marginally to a specific diagnosis. Multifocal signs were only noticed in one third of the dogs with inflammatory/infectious diseases. Particular neurological abnormalities were more frequent in certain diseases than in others (eg, myoclonus was frequent in dogs with distemper, but it was also found in those with other meningoencephalomyelitides). Hematologic findings contributed to the diagnosis in certain conditions (eg, canine distemper encephalitis, protozoal encephalomyelitis, steroid-responsive meningitis-arteritis). Cerebrospinal fluid examinations, including immunoglobulin G index and cytology were useful to separate meningoencephalomyelitides from the other CNS diseases and to distinguish certain conditions from others. In most cases a specific diagnosis depended on a combination of clinical signs and ancillary diagnostic aids. Still, a specific diagnosis remained very difficult, if not impossible, in at least one third of the dogs.
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PMID:Diagnosis of inflammatory and infectious diseases of the central nervous system in dogs: a retrospective study. 853 Nov 75

Paraneoplastic encephalomyelitis developed as the presenting feature of small-cell lung carcinoma in 3 patients. Two patients with paraneoplastic encephalomyelitis manifested predominantly as subacute sensory neuronopathy did not improve after prednisone treatment and chemotherapy. The third patient had severe axial and limb rigidity and myoclonus, which partially improved after chemotherapy and treatment with intravenous immunoglobulin and prednisone. Serum from each patient immunocytochemically stained the neuropil and to a lesser degree the neuronal cytoplasm in human cerebral and cerebellar cortex. On immunoblots of human neuronal extracts, each patient's serum contained high-titer IgG antibodies reacting with a protein band of apparent molecular mass 125 kd. This autoantibody pattern is indistinguishable from antibodies recently identified in several women with breast carcinoma and stiff-man syndrome. Screening of a human brain complementary DNA expression library with patient serum yielded clones whose sequence is identical to that of the synaptic vesicle-related protein amphiphysin. Reverse transcriptase-polymerase chain reaction demonstrated expression of amphiphysin in 8 of 10 small-cell lung carcinomas and in 5 of 14 breast carcinomas. These observations highlight the clinical and serological heterogeneity of paraneoplastic central nervous system disorders: Patients with a given clinical syndrome may have different antineuronal antibodies, and patients with a given autoantibody specificity have differing clinical presentations.
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PMID:Antiamphiphysin antibodies with small-cell lung carcinoma and paraneoplastic encephalomyelitis. 861 52

A neurologic paraneoplastic syndrome may be the first sign of an occult and treatable cancer. Some syndromes are associated with autoantibodies against neuronal antigens. Patients with cerebellar degeneration and ovarian or breast cancer have antibodies against 34 and 62 kilodalton (kDa) proteins in Purkinje cell cytoplasm: anti-Yo antibodies. Patients with encephalomyelitis or sensory neuronopathy and small cell lung cancer have antibodies against 35-40 kDa neuronal nuclear proteins: anti-Hu antibodies. Patients with opsoclonus-myoclonus and breast cancer have antibodies against 55 and 80 kDa neuronal nuclear proteins: anti-Ri antibodies. Patients with Lambert-Eaton myasthenic syndrome and small cell lung cancer have antibodies against voltage-gated calcium channels (anti-VGCC) in motor nerve terminals. The presence of anti-neuronal antibodies strongly indicates that a neurological syndrome is paraneoplastic, and often identify the site of an occult neoplasm. However, the absence of detectable antibodies does not rule out the presence of an underlying tumour.
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PMID:[Neurologic paraneoplastic syndromes and anti-neuronal antibodies]. 863 63

In a case of acute disseminated encephalomyelitis displaying opsoclonus-myoclonus syndrome, magnetic resonance images (MRI) disclosed pontine lesions involving part of the pontine paramedian reticular formation and the raphe nucleus. Shrinkage of the lesions correlated with the patient's clinical state. This observation supports the hypothesis that a pontine lesion can cause opsoclonus-myoclonus syndrome.
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PMID:Correlation of clinical improvement with pontine lesions in opsoclonus-myoclonus syndrome. 890 57


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