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)

Ten patients with involuntary leg movements due to myelopathy were studied clinically and polysomnographically. The clinical manifestation and polysomnographical findings of involuntary leg movements were identical to sleep-related periodic leg movement (PLM) (nocturnal myoclonus). Since 2 patients had complete transection of spinal cord due to injury or vascular accident, the spinal cord deprived of supraspinal influences was considered to generate the rhythm of PLM. Suppression of the leg movements during REM sleep was not obvious in the patients with complete transection of spinal cord. In addition, PLM alternated from one side to the other 1-4 times a night with intervals of 1-4 h in all patients. This alternation also seemed to be from the spinal cord. This PLM of spinal cord origin was different from spinal myoclonus in their clinical features although both were generated within the spinal cord. PLM of spinal cord origin showed a triple flexion of the ankle, knee and hip, and this was very similar to a flexor withdrawal reflex which all patients exhibited. Therefore, it was suggested that PLM of spinal cord origin has a common mechanisms with spinal automatism. Although all patients had extensor plantar responses, PLM preceded the paresis in three patients and the severity of paresis was variable. There was no laterality of left and right PLMs even in patients with weakness of the leg on one side. This suggested that PLM of spinal cord origin might be induced by the interruption of the tract which was separate from, but runs near the corticospinal tract.
 ...
PMID:Sleep-related periodic leg movements (nocturnal myoclonus) due to spinal cord lesion. 191 96

This paper describes the few case reports of neurological effects of acute (primary) HIV infection. Following a typical primary illness (fever, sore throat, headache, rash, lymphadenopathy, superficial oral ulcers, conjunctivitis, leukopenia and thrombocytopenia) aseptic meningitis, myelopathy, spinal myoclonus, peripheral or cranial neuropathy, neuralgia and ganglioneuronitis may occur, usually within 3 weeks. Encephalopathy with spontaneous recovery also occurs, usually without other features of acute HIV infection. Diagnosis depends on demonstration of seroconversion which may be delayed by weeks. No therapy is yet available.
 ...
PMID:The neurological features of acute HIV infection. 304 55

A 46-year-old male patient was irradiated following left orchiectomy for seminoma. Three months after radiation, segmental myoclonus appeared involving all skeletal muscle groups up to the level of D10, the area of radiation. Urodynamic testing showed the presence of segmental myoclonus in the urethral sphincter. An around-the-clock 'continuous segmental myoclonus' during sleep and wakefulness with a frequency of 1 c/s and an amplitude of 400 microV selectively inhibited by REM sleep was not found in the literature. This continuous segmental myoclonus caused by a radiation myelopathy at D10-D11 was followed up for 2 years. It was partially controlled by carbamazepine.
 ...
PMID:Postradiation segmental myoclonus selectively inhibited by REM sleep (sleep-wake myoclonus). 321 6

We observed 37 patients (mean age at onset, 48.5 years; range, 13 to 84 years) with segmental myoclonus (18 branchial, 19 spinal). Etiologies for branchial myoclonus included brain-stem demyelination, cerebrovascular disease, Meige's syndrome, cerebral arteritis secondary to bacterial meningitis, central nervous system Whipple's disease, acute cervicomedullary trauma, and cerebellar degeneration. Spinal myoclonus was associated with laminectomy, remote effect of cancer, spinal cord injury, post-operative pseudomeningocele, laparotomy, thoracic sympathectomy, poliomyelitis, herpes myelitis, lumbosacral radiculopathy, spinal extradural block, and myelopathy due to demyelination, electrical injury, acquired immunodeficiency syndrome, and cervical spondylosis. The latency between the predisposing condition and the onset of myoclonus ranged from immediate to 33 years (mean, 2.9 years). In six patients, the myoclonus was the presenting symptom of a serious underlying disease. Treatment with clonazepam, tetrabenazine hydrochloride, or other medications provides a satisfactory control in most patients.
 ...
PMID:Segmental myoclonus. Clinical and pharmacologic study. 375 63

We studied seven patients with AIDS or AIDS-related complex (ARC) and movement disorders. Three had hemichorea-ballismus, two had segmental myoclonus, one had postural tremor with dystonia, and one had paroxysmal dystonia. Besides the hyperkinesias, two patients had parkinsonism, and one had cerebral Whipple's disease. In two, the movement disorder preceded other evidence of AIDS; in three others, the diagnosis of AIDS was not considered until there was a movement disorder. The movement disorders were attributed to toxoplasmosis in four patients (one confirmed at autopsy), viral encephalitis, vacuolar myelopathy, and CNS Whipple's disease.
 ...
PMID:Movement disorders and AIDS. 379 36

Four cases of spinal myoclonus are described, three males and one female. The mean age was 51 years (28-75 years). The mean time between the onset of the myelopathy and the myoclonic jerks was 4.3 months (1-8 months). The involuntary movements were determined by trauma, Devic's disease, tuberculous myelopathy and tumor. Three patients had spastic paraplegia with bilateral myoclonus more evident on the right side. The fourth patient had a flaccid paraplegia with symmetrical jerks. The data suggest that different processes (trauma, demyelinating, infection and tumor) affecting the spinal cord may cause the same type of involuntary movements.
 ...
PMID:Spinal myoclonus: report of four cases. 814 51

K complex is the characteristic wave of stage II of sleep. The relationship between periodic limb movements (PLM) and the restless legs syndrome (RLS), and the incidence of K complexes and alpha activity has been previously described. The aim of the present study was to evaluate the effect of an acute physical activity upon K complex, PLM, and myoclonus during sleep in individuals who were paraplegic. We evaluated 84 polysomnograms from 28 volunteers with a spinal cord injury at the level of T7-T12, obtained during three consecutive nights. On day 3, the volunteers were submitted to a test of maximum effort (manual cycloergometer, with the equipment Cybex Met 300, with a progressive load increase of 12.5 w, every 2 min). The analysis of the polysomnographic recordings showed a positive correlation between the incidence of K complex and limb movements on nights 1, 2 and 3. Similarly, a correlation between the incidence of K complex and myoclonus was observed on nights 1, 2 and 3. An increased incidence of the total K complex was seen on night 3, 36 h after the test maximum effort. Both total K complex and K complex/h were reduced on night 2, compared to basal recording (night 1). There was a reduction of sleep latency on night 2, whereas total sleeping time increased progressively on night 3, as well as REM phase on nights 2 and 3. These findings indicate that physical activity can effect or modulate the incidence of K complex and suggest that a positive correlation between PLM and K complex may occur in those who are paraplegic from a spinal cord injury. In conclusion, sleep can be consolidated after physical activity.
Spinal Cord 1997 Apr
PMID:Correlation between K complex, periodic leg movements (PLM), and myoclonus during sleep in paraplegic adults before and after an acute physical activity. 914 89

Type 1 antineuronal nuclear autoantibody (ANNA-1, also known as "anti-Hu") is a marker of neurologic autoimmunity that is highly associated with small-cell lung carcinoma (SCLC). To determine the spectrum of symptoms and signs as well as the frequency of cancer in adult patients who are seropositive for ANNA-1, we reviewed 162 sequential patients (67% female) identified as ANNA-1-positive in a comprehensive immunofluorescence screening test. In 21% of these patients, the antibody test requested by the physician was not ANNA-1. By the end of the follow-up period, cancer had been found in 142 patients (88%). Ten of these lacked evidence of SCLC (4 had prostate carcinoma, 3 breast carcinoma, 1 both prostate carcinoma and melanoma, 1 lymphoma, and 1 squamous-cell lung carcinoma). Of the 132 patients (81%) with proven SCLC, 17 had one or more coexisting malignant neoplasms (6 had renal carcinoma, 4 another lung primary carcinoma, 3 prostate carcinoma, 3 breast carcinoma, and 4 assorted neoplasms). The diagnosis of SCLC in 128 patients (97%) followed the onset of paraneoplastic symptoms. SCLC was identified in 10 patients by chest MRI after an equivocal chest radiograph or CT; in 28 by bronchoscopy, mediastinoscopy, or thoracotomy; and in 7 at autopsy. Neurologic signs in decreasing frequency were neuropathy (sensory > mixed somatic > autonomic > cranial [especially cranial nerve VIII] > motor), cerebellar ataxia, limbic encephalitis, polyradiculopathy, associated Lambert-Eaton myasthenic syndrome, myopathy, myelopathy, opsoclonus/myoclonus, motor neuronopathy, brachial plexopathy, and aphasia. Nineteen patients had a solely gastrointestinal initial presentation, including gastroparesis, pseudo-obstruction, esophageal achalasia, or other dysmotility. We conclude that seropositivity for ANNA-1 can expedite the diagnosis and treatment of otherwise occult cancer in patients, especially tobacco abusers, with varied neurologic and gastroenterologic presentations. The search for SCLC should not end on discovering a different neoplasm.
 ...
PMID:Paraneoplastic and oncologic profiles of patients seropositive for type 1 antineuronal nuclear autoantibodies. 952 Dec 51

Paraneoplastic syndromes are disorders associated with cancer but without a direct effect of the tumor mass or its metastases on the nervous system. Small cell carcinoma of lung associated with paraneoplastic sensory neuronopathy and/or paraneoplastic encephalomyelitis with the presence of anti-Hu antibodies has been termed "anti-Hu syndrome." Anti-Hu associated PSN-PEM is an immune disorder in which both cell-mediated and humoral mechanisms are involved. Patients are considered affected by Anti-Hu associated PSN-PEM when they develop clinical signs and symptoms of CNS dysfunction and/or sensory neuropathy not caused by metastases or other disorders, and serum or cerebrospinal fluid is positive for Hu abs. SCLC is found in more than 90% of patients with cancer and positive Hu abs. Individual patients with Hu abs associated to SCLC may suffer PSN-PEM, limbic encephalitis, brainstem encephalopathy, opsoclonus-myoclonus, paraneoplastic cerebellar degeneration or myelopathy. Hu abs have a specificity of 99% and sensitivity of 82% in detecting paraneoplastic neurological syndromes. There are two types of treatment: the first is to treat the cancer, the second is to suppress the immune reaction with the use of corticosteroids, cyclophosphamide, azathioprine, plasma exchange, intravenous immunoglobulin and immunoadsorption; however, treatment of paraneoplastic syndromes is generally unsatisfactory.
 ...
PMID:Paraneoplastic syndromes associated with anti-Hu antibodies. 1134 32

Clinically relevant movement disorders are identified in 3% of patients with HIV infection seen at tertiary referral centres. In the same setting, prospective follow-up shows that 50% of patients with AIDS develop tremor, parkinsonism or other extrapyramidal features. Hemiballism-hemichorea and tremor are the most common hyperkinesias seen in patients who are HIV positive, but other movement disorders diagnosed in these patients include dystonia, chorea, myoclonus, tics, paroxysmal dyskinesias and parkinsonism. Patients with movement disorders usually present with other clinical features such as peripheral neuropathy, seizures, myelopathy and dementia. In the vast majority of patients, hyperkinesias result from lesions caused by opportunistic infections, particularly toxoplasmosis, which damage the basal ganglia connections. On the other hand, parkinsonism and tremor can result from dopaminergic dysfunction resulting from HIV itself or the use of antidopaminergic drugs. The management of patients who are HIV positive who present with movement disorders involves recognition and treatment of opportunistic infections, symptomatic treatment of the movement disorder and the use of highly active antiretroviral therapy (HAART). The most effective treatment of cerebral toxoplasmosis in patients with HIV infection is the combination of sulfadiazine and pyrimethamine. Symptomatic treatment of the movement disorder is often disappointing: hemiballism improves with antipsychotics, but tremor, parkinsonism and other phenomena usually fail to respond to available therapies. Preliminary data suggest that HAART may be helpful in the symptomatic control as well as prevention of movement disorders in patients who are HIV positive.
 ...
PMID:HIV-related movement disorders: epidemiology, pathogenesis and management. 1226 60


1 2 Next >>