Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0014070 (encephalomyelitis)
 13,017 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In an attempt to associate the clinical neurological syndromes with the neuropathological features of canine distemper (CD), 19 spontaneous cases with neurological involvement were examined, before and after euthanasia. Seventeen dogs were less than one year of age and all except two (89.4%) were unvaccinated against CD. Various extraneural signs associated with CD encephalomyelitis (CDE) were seen in 15 dogs. Generalized or localized myoclonus was the most common sign observed (13/19). Seventeen of the dogs presented with signs suggestive of one neuroanatomical location of lesions. Of these animals, seven had signs of cerebral, two of cerebellar, four of cervical, one of cervicothoracic, two of thoracolumbar and two of lumbosacral syndrome. The diagnosis of CD was confirmed immunohistochemically (detection of CD viral antigen), serologically (neutralizing serum antibody titre > or = 16) and histopathologically (CDV inclusion bodies, type of central nervous system lesions). An association of the neuroanatomical lesion location and the histopathological findings was noted in 14 out of 17 dogs (82.3%). Myoclonus could be attributed to lower motor neuron damage in eight out of 13 dogs (61.5%).
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PMID:Relation of clinical signs to pathological changes in 19 cases of canine distemper encephalomyelitis. 1181 21

The stiff man syndrome (SMS) and its variants, focal SMS, stiff limb (or leg) syndrome (SLS), jerking SMS, and progressive encephalomyelitis with rigidity and myoclonus (PERM), appear to occur more frequently than hitherto thought. A characteristic ensemble of symptoms and signs allows a tentative clinical diagnosis. Supportive ancillary findings include (1) the demonstration of continuous muscle activity in trunk and proximal limb muscles despite attempted relaxation, (2) enhanced exteroceptive reflexes, and (3) antibodies to glutamic acid decarboxylase (GAD) in both serum and spinal fluid. Antibodies to GAD are not diagnostic or specific for SMS and the role of these autoantibodies in the pathogenesis of SMS/SLS/PERM is the subject of debate and difficult to reconcile on the basis of our present knowledge. Nevertheless, evidence is emerging to suggest that SMS/SLS/PERM are manifestations of an immune-mediated chronic encephalomyelitis and immunomodulation is an effective therapeutic approach.
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PMID:Stiff man syndrome and related conditions. 1236 May 34

We conducted the current review of the paraneoplastic neurologic syndromes (PNSs) associated with gynecologic and breast carcinomas to describe their clinical and immunologic characteristics and their relative frequency. We retrospectively reviewed 92 patients whose serum was sent to our laboratories to detect onconeural antibodies and who were diagnosed as having PNSs associated with breast or gynecologic tumors. PNSs were defined as "definitive" and "possible" (atypical PNS, no onconeural antibodies, and no improvement after tumor treatment). Forty-nine patients had breast and 43 had gynecologic cancer. Sixty-three patients had onconeural antibodies (50 Yo-ab, 5 Hu-ab, 5 Ri-ab, and 3 amphiphysin-ab). Cerebellar ataxia represented 57 (62%) of all PNSs and was associated with anti-Yo in 88%. All Yo-abnegative patients had breast cancer; 4 of them had a mild cerebellar syndrome that improved after tumor treatment. Sensorypredominant neuropathies were present in 17 (18%) patients. Seven of them had Hu-ab (5) or amphiphysin-ab (2). Other PNSs were opsoclonus-myoclonus syndrome (4 cases, Ri-ab in 2), sensorimotor neuropathy (4 cases), paraneoplastic encephalomyelitis (4 cases, Ri-ab in 3), paraneoplastic retinopathy (2 cases), amyotrophic lateral sclerosis (2 cases), stiff-person syndrome (1 with amphiphysin-ab), and limbic encephalitis (1 case). All patients with gynecologic cancer presented definitive PNS, and onconeural antibodies were diagnosed in 93% of them. In contrast, 20% of PNSs associated with breast cancer were defined as possible and the incidence of onconeural antibodies was 51%, excluding the 2 patients with paraneoplastic retinopathy in whom antiretinal antibodies were not analyzed. In patients with possible PNS, a coincidental association between the tumor and the neurologic disorder cannot be excluded.
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PMID:Spectrum of paraneoplastic neurologic disorders in women with breast and gynecologic cancer. 1279 7

The head retraction reflex (HRR) is a vestigial withdrawal reflex of the face and is suppressed in healthy subjects. We investigated the prevalence and electrophysiological patterns of the HRR in patients suffering from stiff-man syndrome (SMS, n = 28) and related disorders, stiff-limb syndrome (SLS, n = 2), and progressive encephalomyelitis with rigidity and myoclonus (PERM, n = 20). In patients with a positive HRR, the electromyographic (EMG) pattern was analyzed with surface recordings from the orbicularis oculi, sternocleidomastoid, trapezius, and the paraspinal extensor muscles at midthoracic and lumbar levels. On clinical testing, 17 of 28 SMS patients, 10 of 20 PERM patients, and 0 of 2 SLS patients had a positive HRR, ranging from a brief contraction of the neck extensors to violent retropulsion of the upper body. In all muscles, EMG reflex patterns elicited by gentle taps to the face or by electrical stimulation of the trigeminal nerve branches consisted of two components: an early, synchronous, brief burst with the shortest latency in trapezius (12.5-20.0 msec) and a late, asynchronous, longer response with latencies between 44 and 70 msec. We conclude that the HRR is an abnormal cutaneomuscular brainstem reflex that occurs in a considerable proportion of patients with SMS and related disorders. Although neither specific nor particularly sensitive for SMS, presence of the HRR in a setting with otherwise unexplained stiffness and spasms might help to avoid the otherwise frequent misdiagnosis of a psychogenic motor disturbance in patients with SMS.
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PMID:Head retraction reflex in stiff-man syndrome and related disorders. 1288 80

The susceptibility of red foxes (Vulpes vulpes) to European bat lyssavirus type 1 (EBLV-1) infection was examined. Eight foxes were inoculated intramuscularly (i.m.) with 10(4.9) foci-forming units (FFU) (n = 4) and 10(5.1) FFU (n = 4) and observed for up to 90 days. All foxes showed manifestations of a neurologic disorder (e.g. seizures, myoclonus, agitation), starting as early as 5 days post-infection (p.i.). Subsequently, all animals showed improvement followed by one or more relapses. One fox was killed 3 days after it recovered, 26 days post-infection. Two other foxes were also killed 38 and 54 days post-infection after severe neurologic signs returned. All foxes developed a humoral immune response against EBLV-1 as determined in serum and brain tissues. However, no rabies virus antigen was detected in the brain, other tissues and secretions examined (e.g. salivary gland, saliva, tonsils, lungs) by using different standard diagnostic techniques [fluorescent antibody test, reverse transcription polymerase chain reaction (RT-PCR), rabies tissue culture inoculation test], with the exception of one fox in which EBLV-1 RNA was detected by RT-PCR in only the spinal cord. Brain tissues showed moderate to severe multifocal, mononuclear encephalomyelitis in the three foxes that were killed during the observation period, although no EBLV-1 virus was detectable in these tissues.
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PMID:Rabies in red foxes (Vulpes vulpes) experimentally infected with European bat lyssavirus type 1. 1552 59

Segmental myoclonus is described as the involuntary contractions of contiguous muscles innerved by the brain stem or by spinal cord. The underlying causes of segmental myoclonus in children are demyelinating diseases and intrinsic tumors. Here, we report a case who was presented with segmental myoclonus on his left arm and later diagnosed as atypical monosymptomatic presentation of acute disseminated encephalomyelitis (ADEM). The case represents the first in the literature in which ADEM is considered as the possible cause of segmental myoclonus. Our findings demonstrate that: (i) in focal movement disorders such as segmental myoclonus, a careful neuroradiological examination of the neuroanatomical region for the possible presence of organic lesions might be rewarding, (ii) ADEM might be one of the potentially reversible causes of myoclonus.
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PMID:Segmental myoclonus as the presenting symptom of an acute disseminated encephalomyelitis: a case report. 1653 Apr 37

Generalized 1Hz, burst-and-slow-wave complexes were observed in a comatosed patient with acute disseminated encephalomyelitis (ADEM) when she showed extremely intractable, generalized convulsions and fragmented myoclonus in the whole body. Two types of short-latency SEPs were obtained separately during the burst and slow phase of the EEG (SEP-burst and SEP-slow, respectively), which showed a two fold greater amplitude of N20 in the former than in the latter. This suggests enhanced responsiveness to the peripheral stimuli during the burst phase as compared with the slow phase. CSF and serum were positive for autoantibodies to NMDA receptors. The "burst and slow complexes" reported here are considered to be an atypical EEG pattern of a generalized epileptic phenomenon.
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PMID:"Burst and slow complexes" in nonconvulsive epileptic status. 1656 28

In this study, we have investigated the expression of the nuclear transcription factor (c-Fos, NFkB), growth factors (nerve growth factor--NGF, brain-derived neurotrophic factor--BDNF), peptides (enkephalin, galanin) and glutamate transporter (AA 504-523 rat EAAC1) in 6 dogs sacrificed immediately after seizure attack during encephalomyelitis due to canine distemper virus (CDV) (as assessed by clinical examination, RT-PCR and viral RNA detection either in blood or brain tissue and CDV immunohistochemistry in brain slices). In all these CDV affected dogs, the observed neurological signs included untreatable seizures, leading to cluster seizure activity and status epilepticus. In the inter-ictal phase abnormal mentation, postural and gait deficits and sometimes involuntary movements such as myoclonus were recorded. The same investigation was carried out in 5 control dogs affected by different disorders, all characterized by the absence of seizures. Brains were dissected out immediately after euthanasia and fixed; sections collected from the dorsal hippocampus were processed for immunohistochemistry. By comparing hippocampus sections obtained from dog with and without seizure, the following regulations were observed. A strong up-regulation of glutamate transporter throughout the cell layers was found together with the onset of nuclear Fos and NFkB-IR in the pyramidal cell layer X. Among the investigated peptides, we observed a slight increase in enkephalinergic fibers and a strong up-regulation of mu-opioid receptors, whereas galanin-IR seemed to be weaker. Finally, both NGF and BDNF expression was strongly up-regulated. BDNF-IR was mainly localized in the apical dendrite in pyramidal neurons. To our knowledge, these data offer the first indication that molecular events described in experimental kindling also occur during spontaneous pathology in animal species sharing close similarities to human neuropathology.
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PMID:A molecular study of hippocampus in dogs with convulsion during canine distemper virus encephalitis. 1676 33

In dogs with neurological disturbances without myoclonus and extraneural signs, the clinical diagnosis of distemper is difficult perform. Considering the great infectious potential of the disease, the possibility of carrying out an antemortem diagnosis of distemper is important, particularly in hospitalized patients with neurological disease. The present study was carried out to evaluate RT-PCR for antemortem CDV detection in hospitalized dogs with neurological disturbances without the typical findings of distemper. We investigated five dogs with canine distemper virus (CDV) encephalomyelitis, in which the clinical diagnosis was not performed owing to the absence of characteristic signs of the disease, such as myoclonus and systemic signs. We observed an apparent high sensitivity of RT-PCR in urine samples for detection of CDV: four out of five urine samples were RT-PCR positive. The results of the present study suggest that urine is a good biological sample for antemortem CDV detection by RT-PCR in dogs with distemper encephalomyelitis in which the clinical diagnosis is likely to be difficult owing to the absence of suggestive distemper signs. The use of two different body fluids (urine and CSF) may increase the RT-PCR sensitivity for antemortem diagnosis of distemper in such cases.
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PMID:Antemortem diagnosis of CDV infection by RT-PCR in distemper dogs with neurological deficits without the typical clinical presentation. 1683 10

The clinical diagnosis of distemper is difficult in dogs presented with nervous deficits in the absence of extraneural signs and myoclonus. The aim of this study is to verify how the clinicopathological findings may suggest distemper encephalomyelitis in such cases. We prospectively investigated 20 necropsied dogs presented with neurological signs without those characteristic signs of distemper at the time of hospital admission. Eight out of 20 dogs were diagnosed with distemper encephalomyelitis at post mortem by reverse transcription-polymerase chain reaction (RT-PCR) and histological examination. Cerebellar and/or vestibular signs progressing to tetraparesis/plegia were frequent neurological signs. Abnormalities in hematologic findings were non-specific, nevertheless the cerebrospinal fluid evaluation could suggest canine distemper virus (CDV) infection by a lymphocytic pleocytosis. At post mortem chronic CDV encephalomyelitis was predominant. Our clinical results, as well as the predominance of chronic encephalomyelitis, differ from other studies about CDV encephalomyelitis with naturally infected dogs presenting extraneural signs and myoclonus.
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PMID:Clinicopathological findings in dogs with distemper encephalomyelitis presented without characteristic signs of the disease. 1708 26


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