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 present a case of a 39-year-old patient, who was diagnosed and treated for a tick-borne meningoencephalitis. Three months after the treatment he started to complain of annoying, cracks-resembling, rhythmical sounds, coming from the inside of his head to both his ears. Physical examination revealed rhythmical oscillations of the soft palate with a frequency of 100-120 per minute and a clock ticking noise synchronic with the palate tremor. Electromyography revealed continuous motor unit activity at rest in the tensor veli palatini muscle. Palatal myoclonus (PM) as a result of tick-borne meningoencephalitis was diagnosed. Treatment with several medications was started with no effect, then botulinum toxin was administered under EMG guidance to both sides of the patient's soft palate with great improvement. A 5-year follow-up and continuation of botulinum toxin injections with only minor and reversible side effects proved the treatment efficacy and safety. In the article we present a case of symptomatic palatal myoclonus with ear click and shortly discuss its aetiology, types and treatment options. We also stress the efficacy and safety of PM treatment with repetitive injections of botulinum toxin.
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PMID:Symptomatic palatal myoclonus with ear click after tick-borne meningoencephalitis. 1670 86

A 3-year-old female pug presented with general seizure following a partial seizure. During the remaining 48 months till death, the dog showed various neurological signs such as disturbance of consciousness, myoclonus and various types of partial seizure after each occurrence of the seizure clusters, and the dog eventually exhibited inability to stand and dementia. Magnetic resonance imaging findings revealed atrophy of the brain over the course of the disease. On histopathological analysis, the dog was diagnosed with necrotizing meningoencephalitis. This case of a canine necrotizing meningoencephalitis observed over the long term is valuable.
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PMID:A canine case of necrotizing meningoencephalitis for long-term observation: clinical and MRI findings. 1805 39

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.
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PMID:Stimulus-sensitive myoclonus and cerebellar ataxia following chikungunya meningoencephalitis. 2335 53

Sporadic Creutzfeldt-Jakob disease is a rare neurodegenerative disorder of unknown etiology that causes rapidly progressive dementia. This disease is uniformly fatal and most patients die within 12 months. Clinical findings include myoclonus, visual disturbances, and cerebellar and pyramidal/extrapyramidal signs in addition to rapidly progressive cognitive and functional impairment. These findings are all non-specific and it is often difficult and challenging to diagnose premortem because of low awareness and clinical suspicion. We present a 66-year-old woman with a 5-month history of rapidly progressive dementia. After a series of extensive diagnostic examinations and continuous follow-up, she was diagnosed with probable sporadic Creutzfeldt-Jakob disease based on Centers for Disease Control and Prevention (CDC) criteria, with key findings of rapidly progressive dementia, blurry vision, extrapyramidal signs (cogwheel rigidity), and abnormal hyperintensity signals on diffusion-weighted MRI. Her symptoms progressively worsened and she died 7 months after the onset. The postmortem brain autopsy demonstrated the presence of abnormal protease-resistant prion protein by Western Blot analysis. A literature review was performed on differential diagnoses that present with rapidly progressive dementia and thereby mimic sporadic Creutzfeldt-Jakob disease. These include Alzheimer's disease, dementia with Lewy Bodies, frontotemporal dementia, meningoencephalitis, corticobasal degeneration, progressive supranuclear palsy, CADASIL, and paraneoplastic encephalomyelitis.
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PMID:Creutzfeldt-Jakob disease: a case report and differential diagnoses. 2379 14

West Nile virus (WNV) is an arthropod borne neurotropic single stranded RNA flavivirus with <1% developing presenting with neurological disease. Immunocompromised and elderly patients are more prone to developing WNV meningitis or encephalitis. Definitive diagnosis of WNV meningoencephalitis is a combination of clinical suspicion and cerebrospinal fluid (CSF) serology. Forty-eight year old Caucasian female presented with a sudden onset of altered mental status after being found unresponsive. She was confused with intermittent bouts of alertness/lethargy and unintelligible responses to questioning. Her medical problems included endometrial cancer that was in remission after undergoing a total abdominal hysterectomy with bilateral salpingectomy and postoperative chemotherapy with paclitaxel and carboplatin. Pertinent physical examination revealed muscle strength that was significantly decreased, nuchal rigidity and +2 pitting edema of both lower extremities. Computed tomography and magnetic resonance imaging of the brain were negative for any intracranial pathology. CSF analysis was consistent with aseptic meningitis with all CSF serology being negative except for positive WNV antibody. A few days after being admitted she developed involuntary random movements of her eyes and generalized jerking movements (myoclonus). This was determined to be opsoclonus myoclonus syndrome (OMS) induced by the WNV meningoencephalitis. She then received five consecutive days of plasmapheresis with a significant improvement in her neurological status. Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder associated with chaotic multidirectional eye movements, myoclonus and less frequently cerebellar ataxia. OMS affects as few as 1 in 10,000,000 people per year. The pathogenesis is not fully understood with the majority of cases of opsoclonus-myoclonus syndrome being idiopathic. According to current medical literature there have only been two previous case reports of opsoclonus myoclonus syndrome associated with WNV encephalitis.
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PMID:West nile virus encephalitis induced opsoclonus-myoclonus syndrome. 2498 3