Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0040822 (tremor)
 18,428 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The natural history, management, and long-term outcome for patients with benign, intrinsic tectal plate gliomas remain controversial in spite of their propensity to cause late-onset hydrocephalus. A 10-year retrospective review has identified 11 consecutive children with tectal plate lesions. Headache, vomiting, a decline in school performance, tremor, and complex partial seizures were common presenting symptoms. All patients presented with signs and symptoms of hydrocephalus. Magnetic resonance (MR) imaging delineated an intra-axial mass lesion of the midbrain primarily localized to the tectal plate which uniformly was hyperintense on T2-weighted imaging and had a more variable appearance on T1-weighted imaging and rare enhancement with gadolinium. No patient underwent surgical resection, chemotherapy, or radiotherapy. Three of 11 patients (27%) showed evidence of progression in size or a new focus of enhancement on MR imaging, which was clinically asymptomatic. In this series, no patient with a tectal plate lesion less than 1.5 cm in maximal diameter and without gadolinium enhancement showed any evidence of clinical or radiological progression. Although intrinsic tectal lesions in children are clinically indolent and the initial management consists of CSF diversion, these lesions may eventually progress and still warrant long-term follow-up with serial MR imaging.
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PMID:Management of intrinsic gliomas of the tectal plate in children. A ten-year review. 1070 25

Clinical data, neuroimaging, and neuropathology of 17 patients with central nervous system tuberculosis were reported. Of this population, 12 were men, 5, women; ages ranged from 23 to 75 years (mean, 46.9). There were three HIV positive patients among them. More than a half of patients had disturbance of consciousness as initial symptom. Neurological signs were variable such as visual acuity loss, hemiparesis, paraparesis, cerebellar ataxia, and tremor, though disturbance of consciousness was the most frequent (36%). Neuroimaging (X-ray CT and MRI) revealed meningeal enhancement (53%), tuberculoma (50%), hydrocephalus, infarction or bleeding and spinal cord tuberculoma. There were three patients who showed paradoxical progression. Eleven patients were performed CSF examination, all of them revealed increased cell count (mean, 206 counts/mm3) and protein (mean, 225 mg/dl), but only 4 patients were positive on bacteriological examination including PCR. Seven patients died and 5 patients were performed autopsy. Neuropathologically, all patients showed a stage of meningitis prominent on basal brain (basal cistern and/or Sylvian fissure). Cell infiltrations including lymphocyte, monocyte, and eosinocyte were most severe around blood vessels, and observed in all cases except one which showed only fibroblast and collagen fibers indicating healed stage. In some cases, there existed epithelioid cells and Langhans giant cells, and in some cases, fibrin exudate. There were three cases having tuberculoma, one HIV case and two non-HIV cases. Center of tuberculoma in non-HIV case was formed by caseous necrosis, and tuberculoma was surrounded by granuloma constituted by epithelioid cells and Langhans giant cells with lymphocyte cell infiltration and proliferation of blood vessels. In contrast, tuberculoma of HIV case did not include granuloma, and was formed with small cells with large nucleus which surrounded arteries. Our studies, as other studies, failed to show any differences between HIV and non-HIV patients clinically, as well as on neuroimaging study. But neuropathological study suggests that mechanism of tuberculoma formation may be different between in HIV positive patients and in non-HIV patients.
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PMID:[Central nervous system tuberculosis with and without HIV infection--clinical, neuroimaging, and neuropathological study]. 1088 29

Hashimoto's encephalopathy is a poor understood neurological disorder associated with thyroid disease. We describe a 12 years old woman with a relapsing clinical picture characterized by acute onset confusion, tremor, convulsive seizures and a stroke-like episode associated with autoimmune thyroid disease. High titers of antithyroid antibodies were detected in her serum. Neurologic investigation showed a marked slowing of the basic activity in EEG, diminished perfusion of left hemisphere in brain SPECT, mild CSF protein level without pleocytosis, and normal brain CT and cerebral angiogram. The clinical course was favorable without corticosteroid therapy. Antithyroid antibodies and EEG were normal after one-year follow-up. We suggest that antithyroid antibody levels should be checked in any unexplained acute or subacute encephalopathy, especially when a relapsing course, stroke-like exacerbation, seizures, tremor or elevation of the CSF protein are found.
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PMID:[Hashimoto's encephalitis: a new case with spontaneous remission]. 1107 81

The authors report a kindred in which GTP-CH deficiency resulted in a myoclonus-dystonia syndrome. The proband, a 17-year-old boy, presented with early-onset myoclonus and later, dystonia and bradykinesia. Blood prolactin was increased and CSF homovanillic acid, 5-hydroxyindoleacetic acid, and biopterin were all reduced. L-Dopa/carbidopa administration resulted in clinical improvement. In the paternal branch, the grandfather and three relatives had myoclonus-dystonia and resting or postural tremor of limbs. The authors found a missense mutation in the exon 6 of GCH-1 gene (K224R).
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PMID:Autosomal dominant GTP-CH deficiency presenting as a dopa-responsive myoclonus-dystonia syndrome. 1239 38

The most common cause of subdural hematomas (SDH) in infants is shaken-baby syndrome (SBS). The pathogenesis and natural history of infantile SDH (ISDH) are poorly documented, because in SBS, the date of shaking is usually imprecise and the assault is often repeated. Victims of traffic accidents (TA) form a study group close to experimental conditions, because the trauma is unique, witnessed and dated. We reviewed 18 cases of SDH due to TA in infants under the age of 24 months. Our goal was to investigate the clinical and radiological data and natural history of SDH. A subdural collection was found on the day of trauma in 7 cases. In 3 of these, the collection was already hypodense. The perifalcine region was the most frequent site of intracranial bleeding. Blood hyperdensity was always found on CT scans performed during the first week, and turned hypodense on about the 9th day. Three patients had retinal hemorrhage, of a type distinct from that found in SBS. Drainage of the SDH was required in 14 cases after a mean delay of 13.5 +/- 5.8 days after trauma. Four patients also required a ventriculoperitoneal shunt because of associated hydrocephalus. Our data suggest that impaired CSF drainage plays a large role in the pathogenesis of ISDH. The fact that a single and recent trauma can result in mixed-density ISDH can be of great importance in forensic medicine.
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PMID:Infantile subdural hematomas due to traffic accidents. 1241 16

In response to the increase of resistant depressive disorders and in spite of improved treatments, numerous studies were conducted in the last thirty Years aiming at assessing the pre-morbid thyroid state of depressed patients resistant to well conducted tricyclic treatments. "Minimal" thyroid abnormalities were evidenced as well as central thyroid disorders which may not be detected by peripheral-i.e plasmatic- dosages. Regarding the premorbid thyroid status, the hypothesis of subclinical hypothyroidism was considered by many Authors. It is marked by four grades including T3 and T4 decreased levels, basal TSH concentration abnormalities as well as increased TSH response to TRH stimulation, and the presence of antimicrosomal and antithyroglobulin antibodies. Although, there are different views on the existence or not of these abnormalities, we'll focus our attention on a metaanalysis including six studies. It shows in a population with a resistant depression, 52% of patients with subclinical hypothyroidism, against 8 to 17% in patients with simple depression and 5% in the overall population.Similarly, antithyroid antibody levels (group IV hypothyroidism) were significantly higher in depressed patients (9% to 20% against 7,5% in the overall population). For many Years, a central hypothyroidism was hypothesized on the basis of an exhausted T3-T4 transference mechanism and a lowered TRH hypothalamic biodisponibility.In the last Years, new data emerged on the role of transthyretin, a cerebral carrier T4 protein, whose concentration in the CSF was found significantly lower in depressed patients than in a control group, the lowest levels being observed in the most severely depressed. This decreased level of transthyretin would result in a lower central T4 biodisponibility-hence, in view of a T4-T3 desiodation insufficiency, a T3 deficit is observed. A low transthyretin level associated or not to subclinical hypothyroidism could be a factor of depressive vulnerability on one hand, of resistance to tricyclic treatment on the other one. Conversely, subclinical hypothyroidism could be a predictive factor of a good response to a potentializing strategy. The pharmacological mechanisms involved in this potentializing phenomenon are now well known: they consist in an interaction between depression, adrenergic receptors and thyroid hormones biodisponibility. The decreased norepinephrine level observed in depressive patients is associated, in case of increased thyroid hormones biodisponibility, with a higher sensitivity of adre-nergic receptors, mostly betaadrenergic. This seems to underly the recovery process. According to some Authors, the serotoninergic system might be involved in the potentialization of tricyclics by thyroid hormones. We know that in animals with hypothyroidism, the serotonin synthesis is decreased and that the administration of T3 increases the brain levels of serotonin and its 5HIA catabolite. In addition, T3 could correct the down-regulation induced by serotoninergics on beta-adrenergic receptors. On the basis of numerous studies carried out on the potentializing of tricyclics, we suggest practical modalities of treatment - which until today did not materialize in every day practice in the absence of a clear consensus based on statistically reliable data: after four to six weeks of inefficient tricyclic or serotoninergic treatment on a correct dosage testified by plasmatic dosages, it is recommended to initiate a T3 treatment on a effective posology (25 to 50 micrograms per day), which must be reached in 2 or 3 days, except in case of rare and transitory side effects (sweating, shaking, tachycardia, nervousness, anxiety). If the treatment is not rapidly efficient, it must be discontinued in case there is no improvement after 3 weeks. Until today, there is no consensus about the duration of a T3 treatment. It is important to take into account the predictive criteria of good or bad response to a T3 potentialization, since they have direct consequences on the management of depressed patients. For example, a high degree of chronic evolution with resistance to numerous treatments, associated disorders according to the DSM IV axis I and a comorbidity of addiction, point to a bad prognosis of a potentialization treatment. In addition, we'll examine the few recent studies on the potentializing of serotoninergic antidepressant drugs by thyroid hormones.
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PMID:[Potentializing of tricyclics and serotoninergics by thyroid hormones in resistant depressive disorders]. 1523 25

An outbreak of acute encephalopathy among 6 patients with renal dysfunction after eating "Sugihiratake" mushroom in the northern area of Niigata Prefecture was reported. All of the patients had varying degrees of renal dysfunction, and 3 of them were on dialysis treatment. Patients initially presented with shaking limbs and difficulty in walking. Several days later, disturbances in consciousness developed, which were followed by status epileptics. Four patients were put on mechanical ventilation for seizure control. Two of the 6 cases died. CSF examination showed elevated protein without pleocytosis; bacterial, rickettsial and viral screenings was negative. Brain CT and MRI studies showed edema in the claustrum and external capsule, and in the white matter of the base of the frontal, parietal, and temporal lobes. No common drug administration was recognized. All of the patients had a history to have ingested "Sugihitarake" mushroom (angels' wing mushroom, pleurocybella porrigens) in varying quantities and frequencies prior to the onset of the illness. This mushroom may have induced toxic encephalopathy although no similar case have been reported in the past.
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PMID:[Acute encephalopathy among patients with renal dysfunction after ingestion of "sugihiratake", angel's wing mushroom--study on the incipient cases in the northern area of Niigata Prefecture]. 1583 96

An outbreak of acute encephalopathy has occurred among patients with renal dysfunction after ingestion of "sugihiratake" mushroom (angel's wings mushroom) in the northern area of Japan between the end of September and the middle of October in 2004. Most of the patients had varying degree of renal dysfunction. Patients initially presented with asthenia in legs, shaking limbs, and difficulty in ambulation. Several days later, tremor-like involuntary movements or myoclonus developed, which were frequently followed by intractable status epilepticus. Eleven patients were dead. CSF examination showed elevated protein levels without pleocytosis. Brain CT and MRI studies revealed abnormal signal intensities in bilateral external capsule and claustrum, and in the cortical white matter. All of the patients had a history to have ingested sugihiratake in varying quantities and frequencies prior to the onset of the illness. Although no similar patients have been reported in the past, this edible mushroom must have induced acute toxic encephalopathy. The characteristic features of clinical signs and symptoms, and laboratory findings of this encephalopathy were briefly summarized.
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PMID:[Acute encephalopathy after ingestion of "sugihiratake" mushroom]. 1644 34

Paraneoplastic cerebellar degeneration (PCD) is a rare syndrome associated with systemic malignancies, most in lung and ovarian cancer. Cerebellar ataxia has previously been associated with the presence of anti-Purkinje cell antibodies (anti-Yo) in the serum and cerebrospinal fluid and responses to therapy are uncommon. We reported two patients were identified with delayed onset of PCD associated with high titer of CSF anti-Yo (1:30,000, 1:320 U/ml) and a marked elevation of tumor markers for ovarian cancer (CA-125 17,700 ng/ml, 43 ng/ml) titer 1 year and 6 months prior to discovery of the carcinoma. Both developed subacute onset of severe ataxia, dysarthria, tremor, nystagmus with progression to severe debilitation (wheelchair bound or bedridden status). One of these patients also developed dysphagia that required PEG tube feeding. They were treated with six cycles of intravenous immunoglobulin (IVIG) 0.4 gm/kg/day x 5 days, every 4-6 weeks in conjunction with combination chemotherapy of Taxol and Carboplatin after the surgical resection of ovarian cancer. In each case, a significant improvement of neurological deficits were seen after the third cycle of IVIG, approximately 4 months after initiation of treatment. This type of delayed response is contrary to the previous reports. Both patients could ambulate without assistance in correlation with dramatic decrease in anti-Yo titer (1:80, 1:320 U/ml) and CA-125 (11 ng/ml, 8 ng/ml). This is a first report of benefit from IVIG in patients with late onset of PCD, which showed a delayed response with significant neurological improvement.
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PMID:Neurologic improvement after high-dose intravenous immunoglobulin therapy in patients with paraneoplastic cerebellar degeneration associated with anti-Purkinje cell antibody. 1677 14

We describe the case of a 36-year-old woman who developed acute encephalo-myelitis after acute viral hepatitis type B. She was admitted to the hospital with a history of general malaise and nausea of 5 days duration. Her serum showed high transaminase levels and positive HBs-Ag and increased IgM HBc-Ab titers. She had urinary dysfunction, myoclonus and postural tremor of her extremities. Several days later, she developed bilateral limb ataxia and alteration of consciousness. The cerebrospinal fluid examinations showed pleocytosis and increased protein. Treatment with high-dose methylprednisolone resulted in a marked improvement of the clinical and CSF examination. Magnetic resonance imaging of the brain and the spinal cord did not disclose abnormal lesions. The symptoms and clinical course were quite similar to those of acute disseminated encephalomyelitis.
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PMID:Acute encephalomyelitis associated with acute viral hepatitis type B. 1921 76


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