Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Case reports of 28 neuroblastoma patients who had opso-myoclonus as their presenting feature are reviewed. As compared with the 30%-34% two-year survival rate for the overall population of patients with neuroblastoma, those who exhibited the opso-myoclonus/neuroblastoma combination had a tumor-free two-year survival rate of 89.3%. This excellent prognosis may be explained partially by earlier diagnosis and a higher percentage (71% vs. 33%) of patients with Stage I, II, and IV-S disease in the opso-myoclonus sub-group. However, these factors are not, of themselves, sufficient to explain totally the differences in survival rate since five of seven patients with Stage III-IV disease also exhibited long-term survival. This raises the question as to whether the neurologic dysfunction in these patients is pathogenetically related to an unknown factor (possibly autoimmune) which also controls growth and spread of the tumor.
Cancer 1976 Feb
PMID:Favorable prognosis for survival in children with coincident opso-myoclonus and neuroblastoma. 125 9

Atypical dementias confront the adequacy of current diagnostic concepts. The two patients with atypical dementia syndromes described here shared common postmortem features of numerous neocortical neuritic (senile) plaques and microvascular amyloid, sparing of hippocampus and substantia nigra, and the virtual absence of neurofibrillary tangles. Microscopically, the two differed only by the presence of a few subcortical Lewy bodies in case 1. These similar morphologic features were associated with dramatically different clinical presentations. In the first patient, visual hallucinations, Capgras' syndrome, cognitive slowing, myoclonus, parkinsonism, and primitive reflexes evolved over 3 years. Memory and language were relatively spared. In the second, dysphagia, nonfluent aphasia, hypophonia, motor perseveration, and a severe disorder of attention developed during this 18-month illness. At autopsy, an unrecognized colon malignancy was found. Despite high neuritic plaque counts in cortex, neither the clinical nor the pathologic criteria for Alzheimer's disease adequately describe either case. The cases will be examined first as clinical, then as neuropathologic, entities. From this approach, we conclude that a specific clinical dementia syndrome may be expressed by several neuropathologic "diseases" and that a variety of clinical syndromes may represent a single neuropathologic diagnosis. This strategy identifies a conceptual dichotomy between Alzheimer's syndrome and postmortem Alzheimer's disease. Meticulous clinical and neuropathologic observation is essential in advancing an understanding of the relationship between the two.
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PMID:Alzheimer's disease or plaque disease? Two cases at the frontier of a definition. 141 64

Paraneoplastic neurological syndromes are well established causes of neurological dysfunction in cancer patients. Recently an auto-immune origin has been found for the Lambert-Eaton myasthenic syndrome, the subacute sensory neuronopathy of Denny-Brown, the paraneoplastic cerebellar degeneration, the paraneoplastic opsoclonus-myoclonus as well as a few other minor syndromes. Indeed patients suffering from these diseases have been found to produce antibodies directed against the central nervous system. These antibodies can be detected as much as a year before the cancer is found and they are therefore interesting for neurological and oncologic diagnosis.
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PMID:[Autoimmune paraneoplastic neurological syndromes]. 144 41

Immune-mediated myasthenia gravis occurred in a 13-month-old girl who had sequelae of an opsoclonus-myoclonus syndrome. She had an abnormal Raji cell assay along with elevated antiacetylcholine receptor and antithyroglobulin antibodies. No evidence of an associated malignancy was found with either condition. Clearly, the co-occurrence of two diseases within a single patient does not establish a common pathogenesis. Nevertheless, this is a provocative association and may point to an immunologically mediated process in the opsoclonus-myoclonus syndrome.
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PMID:Myasthenia gravis in a child with sequelae of opsoclonus-myoclonus syndrome. 156 15

Paraneoplastic syndromes are complexes of symptoms and signs that occur in association with cancer and that are unexplained by the known anatomic and physiologic characteristics of the tumor. Many of these syndromes are neurologic in nature or have consequences for the central or peripheral nervous system. These syndromes have been well characterized in adults. With the exception of opsoclonus-myoclonus, little has been written about the occurrence of such syndromes in children. This review looks at published reports of paraneoplastic syndromes in children and concludes that paraneoplastic syndromes in childhood differ from those seen in adulthood because of differences in both the host and the kinds of neoplasms most prevalent in each age group. Paraneoplastic syndromes may be underreported in childhood because of the difficulty in eliciting specific neurologic complaints from children and because a thorough neurologic examination is often not undertaken as a matter of routine.
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PMID:Nervous system dysfunction in children with paraneoplastic syndromes. 163 46

A German family with 21 members affected by Alzheimer disease (AD) was studied clinically and genetically. The diagnosis was histologically verified in three affected family members. Ancestors were traced through seven generations to a couple residing in East-Westfalia during the middle of the 19th century. Dementia was often accompanied by extrapyramidal features and myoclonus. No cases of Down syndrome or hematologic malignancy occurred in this family. Clinical manifestations, temporal progression, neurological testing, and neuropathological features do not differ from the more common sporadic form of AD. The inheritance pattern is most consistent with autosomal-dominant transmission.
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PMID:Familial Alzheimer disease: a large, multigeneration German kindred. 202 23

2'-Deoxycoformycin (DCF) is an experimental drug with specific lymphocytotoxic activity which has proved effective in the treatment of some lymphoproliferative syndromes (LPS). This paper deals with the results achieved in 6 patients with LPS, five of them with T-cell and one with B-cell malignancies, who received DCF as initial (2 cases) or salvage (4 cases) therapy. One patient with adult T-cell leukaemia-lymphoma achieved complete remission maintained for 36 months of follow-up; partial remission was attained in three others and the remaining two patients failed to respond to the treatment. Kidney toxicity was seen in three cases, and in two of them it was necessary to stop the treatment. Kidney toxicity was seen in three cases, and in two of them it was necessary to stop the treatment. Other untoward effects included nausea and vomiting (2 patients), myoclonus, dysaesthesia of the limbs and conjunctivitis arida (one case each). No haematologic or infectious complications were present in association with DCF treatment.
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PMID:[Treatment of various lymphoproliferative syndromes with deoxycoformycin: results in 6 patients]. 208 59

The clinical features and neuropathological findings of two patients suffering from progressive multifocal leukoencephalopathy (PML) are reported. These patients had cancer and died two months and one month after onset of their neurological symptoms, respectively. In both demyelination was discovered only as a result of autopsy due to different reasons: the CAT-scan findings were misinterpreted in the first patient, while in the second diagnosis was overshadowed by the sudden progress towards a fatal outcome preceded by myoclonus and other encephalitis-like manifestations. The major findings were the extreme paucity of the mesodermal elements on the one hand, and the modest spread of the affected areas on the other. It is pointed out that, whatever the size of the lesions, characters were the same and that their formation could hardly be traced in time. The intriguing similarities between PML and several types of demyelination obtained experimentally using certain virus strains are remarked.
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PMID:Progressive multifocal leukoencephalopathy (PML): clinical and pathological findings in two short-duration patients. 241 98

Paraneoplastic syndromes are heterogeneous in their clinical presentations and their associations with particular tumor types and are an important part of the differential diagnosis of neurologic dysfunction in patients with or without a known neoplasm. Patients presenting with one of the more distinctive syndromes, such as subacute cerebellar degeneration, opsoclonus-myoclonus, and the Lambert-Eaton syndrome, should undergo a careful evaluation for the presence of an occult malignancy. The importance of looking for a monoclonal gammopathy in patients with certain polyneuropathies and motor neuron syndromes is also becoming clear. At this time, an autoimmune pathogenesis has been clearly demonstrated only for the Lambert-Eaton syndrome. Specific autoantibodies in other syndromes appear to be valuable diagnostic markers for the presence of an underlying malignancy, but the actual role of these antibodies in producing tissue damage and clinical disease is still unknown.
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PMID:The remote effects of cancer on the nervous system. 254 61

A 66-year-old man with hepatic metastases from gastric adenocarcinoma was treated on two occasions with 5-fluoro-2-deoxyuridine (FUdR) via hepatic artery infusion (HAI). The patient developed neurologic signs and symptoms including disorientation, oculomotor defects, ataxia and multifocal myoclonus during both attempts at HAI. Systemic drug toxicity is unusual when FUdR is given via HAI, and neurologic toxicity has not previously been reported. We postulate individual hypersensitivity to FUdR or selective concentration of FUdR in brainstem structures to explain the toxicity in this case.
Cancer Chemother Pharmacol 1986
PMID:Neurologic toxicity associated with hepatic artery infusion HAI of FUdR. 294 26


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