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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sydenham's chorea (chorea minor, St. Vitus dance, rheumatic encephalitis), described by Thomas Sydenham in 1686, is considered one of the major manifestations of rheumatic fever (1, 2, 3, 4). Clinically it is characterized by involuntary movements, hypotonia, dysarthria, emotional disorders, and less frequently, by other neurological manifestations such as weakness, headache, seizures and sensory abnormalities (1,4). The motor disorders may be generalized or unilateral, in this case constituting a hemichorea (3). Chorea may present associated to other rheumatic fever manifestations during an acute episode, or in isolated form, characterizing the so-called "pure" chorea (5, 6, 7). Its etiology and pathophysiological mechanisms are still unclear, although its relation with a previous pathophysiological group A Beta-hemolytic streptococcus infection is well established (8). There is also evidence of the participation of immunological mechanisms in its pathogenesis, such as the finding of serum anti-nucleus caudatus and anti-subthalamic antibodies (9) and increase in IgG levels in cerebrospinal fluid of patients with chorea (10). In developed countries due to the reduction in rheumatic fever incidence and decrease in frequency of chorea as its manifestation (3, 11), the latter has become rare. However, in developing countries rheumatic fever remains a public health problem. In Brazil, in the last years an increase in the incidence of chorea has been observed as part of the clinical picture of rheumatic fever (12). The present study reports the clinical and laboratory findings of 187 cases of Sydenham's chorea followed-up during the period of January 1980 to December 1990 in two university centers in the city of Sao Paulo, Brazil.
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PMID:Sydenham chorea: clinical and laboratory findings. Analysis of 187 cases. 134 Oct 4

Lacunar infarcts in the basal ganglia are known to cause various movement disorders, such as chorea, focal dystonia, and hemichorea-hemiballismus. We report here a case of putaminal lacunar infarction which presented with "painful tonic spasms" of the contralateral limbs. This consisted of paroxysmal brief, painful, flexor contractures of the upper, and occasionally the lower limb. These were not focal seizures but were controlled with carbamazepine, which has been used for the "painful tonic spasms" well-associated with multiple sclerosis. The putaminal infarct we describe is probably related to a lupus anticoagulant and systemic lupus erythematosus.
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PMID:Painful tonic spasms caused by putaminal infarction. 381 Jul 37

A 23-year-old woman, who had nonbullous congenital ichthyosiform erythroderma since her childhood, was diagnosed as nephrotic syndrome caused by systemic lupus erythematosus (SLE). She was pregnant but experienced fetal loss at the age of 25. Although 10 mg/day of oral prednisolone was administered, low levels of serum complement, proteinuria, thrombocytopenia (6.0 x 10(4)/mm3) and biological false positive for STS continued. When she was 27 years old, right hemichorea developed. She was admitted to our hospital at the age of 28 because of low levels of serum complement, high titers of anti ds-DNA antibody, profuse proteinuria, gingival bleeding and thrombocytopenia (1.5 x 10(4)/mm3). The nephrotic syndrome gradually improved after 1 g/day of methylprednisolone for 2 days and the oral prednisolone dosage was then increased up to 40 mg/day, and was tapered to 10 mg/day. Epileptic attack (minor seizure) occurred at the age of 29. Continuous low levels of serum complement and high titers of anti ds-DNA antibody were improved by adding 50 mg/day of cyclophosphamide. However, high levels of beta 2 GPI dependent anticardiolipin antibody and lupus anticoagulant activity were observed throughout the study. Our patient was a very rare case of congenital ichthyosis with typical antiphospholipid antibody syndrome and SLE. A few cases of acquired ichthyosis associated with SLE has been reported, and ichthyosis developed only in active stage of SLE. However, our patient's ichthyosiform lesions were not changed throughout the course.
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PMID:[Systemic lupus erythematosus and antiphospholipid antibody syndrome in a patient with congenital ichthyosiform erythroderma]. 748 67

Fahr's disease associates various degrees of neuropsychological impairment and calcium deposits in the basal ganglia. We report 3 cases. The first case was a 54-year-old man with hemichorea of one-year duration. Laboratory results demonstrated idiopathic hypoparathyroidism. In the second case, a 23-year-old man treated for epilepsia for 8 years was hospitalized for subintrant episodes and hemichorea. Dysmorphism and laboratory results led to the diagnosis of pseudo-hypothyroidism. The third case was a 62-year-old woman with generalized seizures of epilepsia and dementia of two-month duration. Physical examination revealed extra-pyramidal rigidity. Hyperparathyroidism due to an adenoma was confirmed histologically. In all three patients, correction of phosphocalcium levels led to clinical improvement, particularly with disappearance of the epileptic seizures and abnormal movements. Clinical expression of Fahr's syndrome varies greatly. Symptoms include psychiatric disorders, epileptic seizures, extra-pyramidal syndrome and various neurological conditions. Diagnosis requires CT brain scan which identifies calcium deposits in the basal ganglia. The main cause is hypoparathyroidism, whether primary or post-operative. Cases due to other causes of dysparathyroidism are rare. The pathophysiology of this condition remains unknown and results of treatment are often unsatisfactory. Since correcting the impaired calcium phosphorus metabolism often leads to considerable improvement, it is essential to systematically search for dysparathyroidism in patients presenting with neuropsychologic manifestations associated with calcifications of the basal ganglia.
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PMID:[Fahr syndrome and dysparathyroidism. 3 cases]. 750 22

Only 12 AIDS cases with hemichorea were reported in the literature. We report the first case of hemichorea associated with AIDS and cerebral toxoplasmosis in our country. A 26-year-old man had 3 episodes of focal seizures on the left side with subsequent loss of consciousness. A few weeks later, he noticed progressive left-sided weakness. Examination revealed a left hemiparesis. MRI of the head showed a round mass in the right frontal lobe and a smaller lesion in the left temporo-occipital area. Laboratory showed positive serum ELISA and Western Blot analysis for HIV antibodies. Serum tests for Toxoplasma showed elevated titers. He was treated with pyrimethamine and sulfadiazine. His weakness improved and he had no further seizures. Two weeks later, choreic movements appeared in the left foot, finally involving the entire left hemibody. A second MRI showed a new small lesion in the right cerebral peduncle. The patient completed 6 weeks of treatment, with further reduction in the size of the lesions. Nevertheless, the left hemichorea persisted. We believe that the hemichorea our patient had was caused by the contralateral peduncular lesion. Lesions involving the subthalamic nucleus or its connections may cause contralateral hemiballismus or hemichorea. In spite of the favorable response to antitoxoplasmic therapy, the hemichorea persisted. The present report illustrates an uncommon neurological complication in AIDS. We believe that a combination of a focal cerebral lesion and the HIV infection caused the movement disorder presented by the patient.
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PMID:[Hemichorea associated with cerebral toxoplasmosis and AIDS]. 854 Aug 36

Records of 108 patients with lupus erythematosus beginning in childhood (1953-1990) were reviewed; 25 had recorded neurologic findings. This is the largest group of childhood lupus erythematosus patients with neurologic disease that has been reported. The average age of children at the time of diagnosis of lupus was 154 months. There were 22 girls and 3 boys in the group. All patients met at least four of the 1982 American Rheumatism Association criteria for the classification of systemic lupus erythematosus. Average age at onset of neurologic difficulties was 168 months. In 4 patients, the neurologic symptoms preceded the diagnosis: 1 month (spastic diplegia), 1 month (bilateral weakness and spasticity), 24 months (chorea), and 26 months (chorea), respectively. Four patients had neurologic symptoms coincident with the diagnosis of lupus erythematosus. In those patients whose symptoms followed the diagnosis of lupus erythematosus, the average elapsed time until symptoms appeared was 33 months; the single lowest and highest outliers were discounted. Most frequent findings were headache (16/25) and behavioral aberrations (10/25). All behavioral manifestations were depression except in 1 patient. Other prevalent findings included hemichorea or chorea (7/25), cerebrovascular accident with hemiplegia or diplegia (7/25), seizures (5/25), visual loss (3/25), and cranial neuropathy (2/25). Vertigo and myelopathy occurred in 1 patient each. All patients were treated primarily with corticosteroids and azathioprine; in the presence of active disease, the drug dosages were increased with significant improvement in neurologic symptoms. Resolution usually occurred from days to months; most improved in a few days to a few weeks; 3-4 months was the longest period until symptoms subsided.
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PMID:Neurologic characteristics of childhood lupus erythematosus. 855 56

In a cross-sectional study of 21 children with Systemic Lupus Erythematosus, 15 (71%) were found to have neuropsychiatric manifestations. The most common finding was generalised seizures (42.8%) followed by encephalopathy (19%) and hallucinations (19%). One child (4.76%) had hemichorea. In 3 children neurological manifestations were the first symptom of SLE. Computerised Axial Tomograms (CAT scans) showed cerebral atrophy in 7 of 12 scans available for review. Ten children had abnormal EEGs. Although none of the children had clinical evidence of a peripheral neuropathy, 8 had neurophysiological evidence of a neuropathy. One child died of intracranial haemorrhage. Six children had residual neuropsychiatric sequalae.
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PMID:Neurological manifestations of children with systemic lupus erythematosus. 1122 Nov 57

A 6-year-old female presented with right hemichorea, initially thought to be post-streptococcal, which subsequently progressed to a right dystonic hemiplegia. At 7 1/2 years she developed right focal and secondary generalized tonic-clonic seizures. These became intractable. A brain biopsy was consistent with Rasmussen's encephalitis (RE). At 9 1/2 years the child underwent a left hemispherectomy making a good recovery with resolution of seizures. At 12 years she presented with recurrent episodes of abdominal pain, fever, and malar rash with serological evidence of systemic lupus erythematosus (SLE). A possible link between RE and SLE is discussed.
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PMID:Rasmussen's encephalitis followed by lupus erythematosus. 1220 25

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.
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PMID:HIV-related movement disorders: epidemiology, pathogenesis and management. 1226 60

A single small enhancing computerized tomographic lesion is a common finding in Indian patients with seizures, particularly focal seizures. A small single enhancing computed tomography lesion also presents with varied non-epileptic manifestations viz. focal neurological deficits, episodic vascular headache, syndrome of increased intracranial pressure, etc. Here we present a case of hemichorea resulting from single enhancing CT lesion. A 12-year-old female presented with acute onset abnormal movements involving right side of body. A clinical diagnosis of right hemichorea was made and patient was subjected to neuroimaging along with other investigations. CT scan showed a single ring-enhancing lesion with perifocal edema in left thalamic area suggestive of neurocysticercosis. Patient was treated with albendazole, steroids and haloperidol therapy. Patient showed marked improvement at follow-up after one month. Repeat CT scan revealed resolution of lesion. This case has been reported because hemichorea in young female is usually caused by post-streptococcal infection and it is quite rare presentation of small ring enhancing lesion.
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PMID:Hemichorea resulting from single enhancing computed tomography lesion. 1721 25


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