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

The clinical features of subacute sclerosing panencephalitis (SSPE) include the following : stage I, behavioral changes; stage II, myoclonus and motor deterioration; stage III, mental and motor deterioration to coma; stage IV, a vegetative state. Anti-measles titers of serum and cerebrospinal fluid (CSF) are elevated in the patients with SSPE. CSF IgG levels and IgG index are also elevated in SSPE. The characteristic electroencephalography in stage II of SSPE shows periodic high voltage slow wave bursts. In the early stage, psychiatric diseases and epilepsy should be ruled out. Neurodegenerative diseases and other encephalitis/encephalopathy should be also excluded.
 ...
PMID:[Clinical course and findings in and differential diagnosis of subacute sclerosing panencephalitis]. 1769 87

A 19-year-old man was admitted to our hospital with tremor and myoclonus that appeared after several episodes of consciousness disturbance and generalized convulsions. While steroid therapy resolved these symptoms, epilepsia partialis continua (EPC) and action myoclonus developed. Clobazam improved the EPC, but action myoclonus persisted. Oral tandospirone (30 mg/day) was given because 5-hydroxyindole acetic acid (5-HIAA) was markedly decreased in the cerebrospinal fluid (CSF). After 10 days of this therapy, most action myoclonus disappeared and he could perform fine motor skills. Although the MR structural images were unremarkable, cerebral SPECT showed decreased uptake in the left thalamus and bilateral frontal lobes. The antibody against glutamate receptor subunit epsilon2 was positive in the CSF. This is the first report of autoimmune encephalitis with anti-glutamate receptor antibody presenting as low level of 5-HIAA in the CSF. Tandospirone was effective for action myoclonus.
 ...
PMID:[Autoimmune encephalitis with anti-glutamate receptor antibody presenting as epilepsia partialis continua and action myoclonus: a case report]. 1771 Aug 87

Hashimoto's encephalopathy is a term used to describe an encephalopathy of presumed autoimmune origin characterised by high titres of antithyroid peroxidase antibodies. In a similar fashion to autoimmune thyroid disease, Hashimoto's encephalopathy is more common in women than in men. It has been reported in paediatric, adult and elderly populations throughout the world. The clinical presentation may involve a relapsing and remitting course and include seizures, stroke-like episodes, cognitive decline, neuropsychiatric symptoms and myoclonus. Thyroid function is usually clinically and biochemically normal.Hashimoto's encephalopathy appears to be a rare disorder, but, as it is responsive to treatment with corticosteroids, it must be considered in cases of 'investigation negative encephalopathies'. Diagnosis is made in the first instance by excluding other toxic, metabolic and infectious causes of encephalopathy with neuroimaging and CSF examination. Neuroimaging findings are often not helpful in clarifying the diagnosis. Common differential diagnoses when these conditions are excluded are Creutzfeldt-Jakob disease, rapidly progressive dementias, and paraneoplastic and nonparaneoplastic limbic encephalitis. In the context of the typical clinical picture, high titres of antithyroid antibodies, in particular antithyroid peroxidase antibodies, are diagnostic. These antibodies, however, can be detected in elevated titres in the healthy general population. Treatment with corticosteroids is almost always successful, although relapse may occur if this treatment is ceased abruptly. Other forms of immunomodulation, such as intravenous immune-globulin and plasma exchange, may also be effective. Despite the link to autoimmune thyroid disease, the aetiology of Hashimoto's encephalopathy is unknown. It is likely that antithyroid antibodies are not pathogenic, but titres can be a marker of treatment response. Pathological findings can suggest an inflammatory process, but features of a severe vasculitis are often absent. The links between the clinical pictures, thyroid disease, auto-antibody pattern and brain pathology await further clarification through research. It may be that Hashimoto's encephalopathy will be subsumed into a group of nonvasculitic autoimmune inflammatory meningoencephalopathies. This group may include disorders such as limbic encephalitis associated with voltage-gated potassium channel antibodies. Some authors have suggested abandoning any link to Hashimoto and renaming the condition 'steroid responsive encephalopathy associated with autoimmune thyroiditis' to better reflect current, if limited, understanding of this condition.
 ...
PMID:Hashimoto's encephalopathy : epidemiology, pathogenesis and management. 1785 Jan 70

This article describes the clinical presentation, diagnostic workup, and neurologic outcome of 3 immunocompetent pediatric patients diagnosed with human herpesvirus 6 (HHV6) rhombencephalitis. Presentation of HHV6 rhombencephalitis included new onset seizures, ataxia, encephalopathy, and opsoclonus-myoclonus. Neurologic examination revealed cranial neuropathies, cerebellar dysfunction, and extremity weakness. Magnetic resonance imaging abnormalities located in the cerebellum, basal ganglia/thalamus, and cerebral hemispheres were detected in 2 patients. Diagnosis of HHV6 encephalitis was made by real-time and nested polymerase chain reaction of serum and cerebrospinal fluid. The HHV6 variant A was detected in 2 patients by sequence analysis, and HHV6 protein was detected by immunomicroscopy in a patient who underwent biopsy secondary to progressive clinical and neuroradiographic findings. Therapy with intravenous ganciclovir did not correlate with resolution of neurologic symptoms, despite eventual non-detectable HHV6. Human herpesvirus 6 should be considered in the differential diagnosis of unexplained cases of rhombencephalitis in immunocompetent children. Features may be rapidly progressive and include profound encephalopathy, seizures, ataxia, and opsoclonus-myoclonus.
 ...
PMID:Human herpesvirus 6 rhombencephalitis in immunocompetent children. 1800 54

The objective of this study was to describe the clinical features of cases hospitalized with West Nile virus (WNV) infections and identify clinical parameters that could potentially predict poor outcome (death). Retrospective medical chart reviews were completed for 172 confirmed cases of WNV infection hospitalized in the Houston, Texas, metropolitan area between 2002 and 2004. Of the 172 patients, 113 had encephalitis which resulted in 17 deaths, 47 had meningitis, and 12 had uncomplicated fever. Risk factors associated with progression from encephalitis to death were absence of pleocytosis in the cerebrospinal fluid, renal insufficiency, requiring intubation and mechanical ventilation, presence of myoclonus or tremors, and loss of consciousness. These findings can aid physicians in evaluating their patients suspected of WNV infection and determining outcomes in their patients with confirmed WNV neuroinvasive disease.
 ...
PMID:Clinical investigation of hospitalized human cases of West Nile virus infection in Houston, Texas, 2002-2004. 1839 81

Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection of immune-resistant measles virus. Diagnostic hallmarks include widespread cortical dysfunction on EEG, myoclonus, white matter abnormalities on neuroradiological examination and the presence of IgG anti-measles antibodies in the cerebrospinal fluid. We present the first case of SSPE with signs of peripheral nerve hyperexcitability, observed as extra discharges following the compound motor action potential at motor nerve stimulation. In addition we demonstrate the importance of SSPE in the differential diagnosis of adult patients with psychiatric and neurological symptoms.
 ...
PMID:Indication of peripheral nerve hyperexcitability in adult-onset subacute sclerosing panencephalitis (SSPE). 1848 11

The treatment and disposition of patients with neurological disorders is commonplace in the emergency setting, but atypical and uncommon presentations can prove to be especially challenging. In this article we discuss the case of a 31-year-old woman who presented with a rare disorder known as opsoclonus myoclonus syndrome (OMS). OMS is characterized by involuntary, multidirectional movement of the eyes, myoclonus, and truncal ataxia. The etiology is thought to be autoimmune, and is most commonly associated with encephalitis or paraneoplastic syndrome. After an 8-day hospital course, which included several different treatment modalities including plasmapheresis, the patient was discharged after making a complete recovery. Unusual presentations such as the one described in this article illustrate the point that it is crucial to have a systematic approach that can be applied to identify and treat potentially life-threatening neurological conditions.
 ...
PMID:Emergency department presentation of a rare neurological disorder. 1848 9

Movement disorders of various types may occur in relation to viral infections of the central nervous system. They manifest themselves as obvious signs at clinical onset or during the acute phase of an encephalitis (myoclonus, tremor, or parkinsonism), or appear as later sequelae decades after the illness. We describe here a man who developed an unusual movement disorder after a probable viral encephalitis in his childhood. This consisted of a tremor (3-4Hz frequency and 100-150ms duration) of the neck, left shoulder and arm, which persisted unchanged during the ensuing years. The patient regarded this abnormal movement as annoying, but otherwise it did not impair his lifestyle. He subsequently developed the clinical picture of parkinsonism many decades after the onset of tremor, and we speculate that both tremor and parkinsonism can be considered sequelae of encephalitis, but each with a different time-course. The left-sided jerky tremor was an immediate sequela in the childhood; whereas, the rigid-akinetic parkinsonian picture represented a later sequela of the infection in old age. The injured site responsible for both the segmental tremor and the parkinsonism presumably involved the brainstem.
 ...
PMID:Post-encephalitic tremor and delayed-onset parkinsonism. 1859 Nov 31

A 50-year-old woman presented with confusion, fever and drowsiness following an episode of headache and dizziness. On admission, neurological examination found positive pyramidal tract signs, meningeal irritation, and bilateral myoclonus in her arms. Laboratory tests revealed liver dysfunction, positive inflammatory reaction, elevated serum IgM antibody against cytomegalovirus, and increased cerebrospinal fluid protein of 67 mg/dl. MRI of brain by diffusion weighted imaging showed a wide spread hyperintense lesion in white matter and limbic areas. We administered aciclovir, ganciclovir and steroid pulse therapy that showed a limited effect in the initial stage. In spite of all these therapies, she suffered from status epilepticus, followed by persistent disturbance of consciousness for about 2 months. However, her level of consciousness and motor deficit were gradually improved by continuous administration of ganciclovir. The present case indicates that prolonged disturbance of consciousness due to cytomegalovirus encephalitis could be restored with continuous ganciclovir administration.
 ...
PMID:[A case of probable cytomegalovirus encephalitis who restored from persistant disturbance of consciousness]. 1871 81

The clinical and EEG data of a 49-year-old man with myoclonic and generalized tonic-clonic seizures resulting from early childhood encephalitis are described. He experienced no tonic-clonic seizure for 10 years before brief exposure first to 60 mg/day duloxetine and then to 20mg/day paroxetine for depressive symptoms. These drugs were separately prescribed at a 9-month interval, and after beginning each drug, the patient experienced tonic-clonic seizures of worsening intensity and myoclonus of increasing frequency. Clinical features correlated with subcontinuous, generalized spike-wave discharges on the EEG. Discontinuation of antidepressant treatment resulted in rapid disappearance of clinical and electrophysiological manifestations of myoclonic status. We suggest care must be taken when using serotonin-noradrenaline reuptake inhibitors (SNRIs) or selective serotonin reuptake inhibitors (SSRIs), as these drugs pose the risk of complications in the specific population of people with myoclonic seizures.
 ...
PMID:Antidepressant-associated myoclonic status in a patient with symptomatic generalized epilepsy: does risk occur with therapeutic doses? 1943 83


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>