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)

We studied somatosensory evoked potentials (SSEPs) in eight Creutzfeldt-Jakob disease (CJD) patients presenting with subacute progressive dementia, generalized myoclonus, and characteristic periodic sharp wave complexes in EEG. Somatosensory evoked potentials were elicited by median nerve stimulation at the wrist. We compared SSEP findings with EEG and the clinical stage proposed by the Japanese Slow Virus Infection Research Committee (stage 1: early stage to stage 5: terminal stage). Until clinical stage 3, short-latency SSEPs showed normal findings despite the severely abnormal EEG. With the progression to clinical stages 4 and 5, however, the amplitude of N20 began to decrease and finally disappeared without prolongation of the latency of N20, whereas other short-latency components were preserved. We recorded giant SSEPs in two of three patients in stage 4, when the periodic sharp wave complex in EEG began to decrease in amplitude. The giant SSEPs decreased in amplitude with the progression of the illness. These findings suggest that the short-latency SSEP is relatively preserved until the middle phase of the disease but that it is eventually affected in the terminal phase. We conclude that our results are compatible with the CJD pathologic findings and that the amplitude of N20 reflects the extent of cortical damage in CJD patients.
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PMID:Decrement of N20 amplitude of the median nerve somatosensory evoked potential in Creutzfeldt-Jakob disease patients. 1177 72

We describe a 58-year-old patient with relapsing high-grade non-Hodgkin's lymphoma who exhibited exacerbation of posthypoxic action myoclonus during high-dose intravenous trimethoprim-sulfamethoxazole (TMP-SMX) treatment for highly suspicious Pneumocystis jiroveci pneumonia (PCP). Three months previously the patient had experienced a hypoxic insult caused by respiratory arrest due to an anaphylactic reaction to antibiotic therapy. He had developed posthypoxic action myoclonus (Lance-Adams syndrome), which was well controlled by oral treatment with piracetam. However, after TMP-SMX therapy (115 mg/kg daily) was started for suspicion of newly developed PCP, posthypoxic action myoclonus worsened dramatically resulting in complete disability. Anti-myoclonic therapy with increased doses of piracetam and valproic acid did not significantly improve his clinical condition. Only when TMPSMX doses were reduced (38 mg/kg daily) on day 12 did action myoclonus cease within 2 to 3 days. We suggest that TMP-SMX can exacerbate posthypoxic action myoclonus.
Infection 2004 Jun
PMID:Trimethoprim-sulfamethoxazole exacerbates posthypoxic action myoclonus in a patient with suspicion of Pneumocystis jiroveci infection. 1518 79

Chikungunya virus was initially thought to be a non-neurotropic virus, but recently neurological complications have been reported in patients with chikungunya virus infection. Here, we report a rare case of stimulus-sensitive myoclonus following chikungunya meningoencephalitis. The cranial MRI scan of the patient was normal, the cerebrospinal fluid contained 200 lymphocytes/mm(3), and the serum immunoglobulin M ELISA was positive for chikungunya. The patient improved completely after 1 month of treatment. This case study illustrates that chikungunya virus should also be considered in a febrile patient with myoclonus, especially in an endemic area.
Infection 2013 Jun
PMID:Stimulus-sensitive myoclonus and cerebellar ataxia following chikungunya meningoencephalitis. 2335 53

Myoclonus describes a movement disorder characterised by brief, abrupt and involuntary contractions of muscles or groups of muscles, usually associated with intracranial lesions, with limited evidence linking it to spinal pathologies. The pathophysiology of spinal myoclonus is extensive and multifactorial. Infection, intramedullary and extramedullary space-occupying lesions, trauma, vascular abnormalities, degenerative processes and cervical spondylosis have been implicated with the disease, the latter been associated with cervical stenosis with no reported cases linking it to an underlying cervical disc herniation. Although medical therapy with clonazepam, levetiracetam, valproate, tetrabenazine hydrochloride and spinal block injections has been equivocal, spinal myoclonus secondary to disc herniation requires surgical intervention. This report describes a case of segmental spinal myoclonus, secondary to a herniated cervical intervertebral disc. After corpectomy and a cage-augmented fusion technique, the myoclonic symptoms resolved. To our knowledge, this was the first report to describe the successful management of discogenic spinal myoclonus with spinal surgery.
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PMID:Spinal myoclonus: a rare presentation of cervical myelopathy. 3006 Nov 34

Opsoclonus, an uncommon clinical sign, and is often described in the context of opsoclonus myoclonus ataxia syndrome (OMAS). OMAS may be paraneoplastic or postinfectious. However, opsoclonus with or without OMAS may occur in association with a wide gamut of infections. Infection-associated opsoclonus/OMAS (IAO) needs recognition as a separate entity, since it demands relatively brief immunosuppression, symptomatic treatment, and has a better outcome. Case records of children, who presented with opsoclonus to a tertiary-care teaching hospital of North India over a period of 1 year (2017-2018), were reviewed. Those with opsoclonus in the setting of an acute infection/febrile illness (symptomatic opsoclonus; IAO) were included. Of 15 children with opsoclonus, 6 children [median age: 42 months (range: 8 months to 7 years); 2 boys] had opsoclonus associated with an infective or febrile illness. Additional clinical findings in these children included myoclonus (n = 2), ataxia (n = 4) and behavioral abnormalities (n = 4). All these patients had an associated neurologic or nonneurologic illness- scrub typhus (n = 1), tuberculous meningitis (n = 1), mumps encephalitis (n = 1), brainstem encephalitis (n = 1), acute cerebellitis (n = 1), and subacute sclerosing panencephalitis (SSPE, n = 1). Children with acute cerebellitis, brainstem encephalitis, and mumps encephalitis were treated with steroids while those with scrub typhus, tuberculosis, and SSPE were treated with antibiotics, antitubercular therapy, and Isoprinosine, respectively. None of them needed long-term maintenance immunotherapy. The evaluation for tumor was negative in all. Three of the 6 children are functionally normal at the last follow-up. Acute neuro infections may trigger opsoclonus. A careful analysis of clinical data and suitable investigations can help differentiate these children from those with OMAS. This distinction may avoid unwarranted long-term immunosuppression.
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PMID:Infection-Associated Opsoclonus: A Retrospective Case Record Analysis and Review of Literature. 3221 1