Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the case of a man who had an insidious onset of asymmetrical distal muscle weakness of the upper extremity at the age of 17. Objective findings were 1) muscular atrophy of calf and forearm flexor muscles and intrinsic hand muscles; 2) fasciculations; and 3) hand tremor. EMG and muscle biopsy showed neurogenic changes. MRI of the medulla and plasma creatine kinase were normal. Genetic testing for SMA-III was negative.
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PMID:[Juvenile asymmetrical segmental spinal muscular atrophy]. 1222 8

Ankylosing spondylitis (AS) is the prototypical form of the spondyloarthropathies, which at a prevalence of 2 % is among the most frequent rheumatic diseases. Spondyloarthropathy comprises the following five disorders: AS, reactive arthritis, psoriatic arthritis, enteropathic arthritis in Crohn's disease, and ulcerosing colitis as well as undifferentiated spondyloarthropathy. In 99 % of the patients with AS initial abnormal findings affect the sacroiliac joints. The radiographic changes required for diagnosing AS occur as late as 5 - 9 years after the onset of clinical symptoms. MRI of the sacroiliac joints reliably demonstrates both chronic inflammatory changes (erosions, sclerotic changes, bone bridges) and acute inflammatory changes (synovitis, capsulitis, osteitis) and allows for grading the chronicity and acuity of such changes. Enthesitis of the interosseous ligaments of the retroarticular space is a manifestation of AS. Spondylodiscitis (Andersson 1937) may occur as an inflammatory or non-inflammatory process (transdiscal fatigue fracture). Inflammations of the facet and costospinal joints developing into ankylosis are typical of AS. Changes of the vertebral bodies occur as anterior (Romanus 1952), posterior, and marginal spondylitis. All forms of spondyloarthropathies are furthermore characterized by asymmetrical synovitis of the large joints, particularly of the legs (gonarthritis, coxitis, tarsitis, peripheral oligoarthritis), rheumatic fibroosteitis (pelvic enthesitis, rheumatic calcaneopathy), and peri- and synchondritis of the pubic symphisis and sternal synchondrosis. Since early inflammatory changes of the spinal column and of the extravertebral localizations in AS are demonstrated by MRI before they become apparent on radiographs, and thereby the diagnostic gap could be closed, the early use of MRI for diagnostic and follow-up is commendable, when new therapeutical options like the so-called "biologicals" are employed.
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PMID:[Magnetic resonance imaging in ankylosing spondylitis (Marie-Struempell-Bechterew disease)]. 1247 19

We document the MRI features in six patients aged 5-14 years with acute encephalitis following measles. The diagnosis was made on a characteristic morbiliform rash and detection of specific IgM and IgG antibodies. The symptoms of encephalitis occurred 1-11 days after the appearance of the rash. All patients underwent MRI within 1-4 days of the onset of neurological symptoms. Diffusion weighted images (DWI) were obtained in three patients. In all patients, T2-weighted images showed widely distributed, multifocal high signal in both cerebral hemispheres with swelling of the cortex, with bilateral, symmetrical involvement of the putamen and caudate nucleus. The lesions had showed low apparent diffusion coefficients. Three patients showed subacute gyriform haemorrhage, and asymmetrical gyriform contrast enhancement on follow-up MRI.
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PMID:Acute encephalitis associated with measles: MRI features. 1259 93

Hyperactivity in the subthalamic nucleus (STN) is seen in animal models of Parkinson's disease, and lesioning of the STN dramatically relieves the animal's parkinsonism. Deep brain stimulation (DBS) of the STN is an effective treatment for patients with advanced Parkinson's disease. We have studied the effects of a unilateral lesion placed in the STN in predominantly hemi-parkinsonian patients. Twenty-one patients with advanced idiopathic Parkinson's disease were studied. Seventeen had asymmetrical tremor-dominant Parkinson's disease and four had bilateral disease. All patients underwent radiofrequency lesioning of the dorsolateral part of the STN under stereotactic guidance. The four patients with bilateral disease had, in addition, an electrode implanted contralaterally in the STN. Twenty-one patients have been followed for a minimum of 12 months. Clinical evaluation included the use of the Unified Parkinson's Disease Rating Scale (UPDRS) before and after surgery. Post-operative high-resolution MRI was performed in each patient to confirm lesion location, and this was correlated with clinical outcome. There was improvement in contralateral tremor, rigidity and bradykinesia in all patients followed for 6, 12 and 24 months, with the effect on tremor being greatest. L-dopa equivalent daily intake was approximately halved, and this resulted in a significant reduction in dyskinesia. Psychometric test scores were mostly unchanged or improved. All lesions were successfully located in the dorsolateral STN. Nineteen of the 21 lesions extended beyond the STN to involve pallidofugal fibres (H2 field of Forel) and the zona incerta (ZI). Lesion-induced dyskinesias were not a management problem except in one patient, whose lesion was confined to the STN. This patient was successfully treated with deep brain stimulator placement in the region of H2/ZI. Unilateral STN lesions can be made safely and are an effective alternative to thalamotomy, pallidotomy and unilateral STN DBS for the treatment of asymmetrical tremor-dominant advanced Parkinson's disease. Com bined lesioning of the dorsolateral STN and H2/ZI is particularly effective.
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PMID:Unilateral subthalamotomy in the treatment of Parkinson's disease. 1269 53

We described the clinical and neuroradiological findings together with a transcranial magnetic stimulation study in two patient with hemiparkinson-hemiatrophy syndrome (HP-HA). In both patients the neuroradiological findings (MRI) and the central motor conduction were normal whereas the functional imaging studies (SPECT) showed asymmetrical perfusion in the basal ganglia; the intracortical inhibition at short interstimulus intervals and the silent period duration in the motor cortex contralateral to hemiparkinsonism were significantly increased only in one of the patient which has a poor response to L-Dopa therapy. These studies suggest that intracortical or thalamo-cortical neuronal inhibition may be increased in HP-HA. The etiopathogenetic considerations, the diagnostic criteria and the prognostic value of our finding to evaluate the clinical evolution of parkinsonism are discussed in the context of current models of basal ganglia-thalamo-cortical connectivity. Transcranial magnetic stimulation will provide valuable information for the differential diagnosis of the parkinsonian disorders and may predict the efficacy of L-Dopa therapy.
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PMID:Hemiparkinson-hemiatrophy syndrome: a transcranial magnetic stimulation study. 1283 89

The Peterborough ENT department receives many referrals for MoD personnel who have suffered hearing loss from occupational noise exposure. Those patients with asymmetrical sensorineural hearing loss are routinely screened for vestibular schwannomas by MRI scanning. Scan reports from the past 5 years have been reviewed and out of 152 scans, four revealed vestibular schwannomas giving a pick-up rate of 2.5%, which compares favourably with other published pick-up rates. Review of the audiograms in these cases suggests that they can be misleading in this context. The conclusion is that patients with noise-induced asymmetrical hearing loss should be screened for acoustic neuromas.
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PMID:Should patients with asymmetrical noise-induced hearing loss be screened for vestibular schwannomas? 1514 83

Odorant identity is believed to be encoded in the olfactory bulb (OB) by glomerular activity patterns. It has not yet been possible to visualize and compare entire patterns for different odorants in the same animal because of technical limitations. For this purpose we used high-resolution functional MRI at 7 T, combined with glomerular-layer flat maps, to reveal responses to aliphatic homologues in the mouse OB. These odorants elicited reproducible patterns in the OB, with the medial and lateral regions containing the most intense signals. Unexpectedly, in view of the symmetrical projections of olfactory receptor neurons to medial and lateral glomeruli, the activity patterns in these regions were asymmetrical. The highly activated medial and lateral areas were shared by homologous members, generating a conserved "family signature" for a homologous series. The moderately active areas, including the dorsal region that has been extensively studied by optical imaging, were more sensitive to the length of the carbon chain, producing more subtle features of individual members and different changing trends among homologues. The global mapping with functional MRI not only extended previous studies but also revealed additional rules for representation of homologues in the OB. Insights into possible relations between the functional patterns, molecular projections, and odor perception may now be obtained based on the global from the olfactory epithelium to the OB glomerular activity patterns.
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PMID:Odor maps of aldehydes and esters revealed by functional MRI in the glomerular layer of the mouse olfactory bulb. 1296 19

A 7-year-old boy from an area endemic to Japanese encephalitis (JE) manifested with acute febrile illness, left hemiplegia and preserved consciousness during the prodromal phase of illness. The child developed features of encephalitis 48 hours after the onset of hemiplegia. IgM MAC ELISA for JE virus revealed high titers in the serum and cerebrospinal fluid suggestive of JE. MRI of the brain showed asymmetrical bilateral thalamic hyperintense lesions on T2 weighted image, considered diagnostic of JE. Hemiplegia during the prodromal phase or as an initial symptom of JE is rather unusual.
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PMID:Hemiplegia: an initial manifestation of Japanese encephalitis. 1465 52

We report on a case of polymicrogyria with benign childhood epilepsy and amyotrophic lateral sclerosis (ALS). While performing dexterity tasks with either hand, strong unsustained mirror movements of the unintended hand were observed. The patient was seen over a period of three years and, as often seen in ALS, there was a moderate progress of the motor neuron disease affecting the upper and lower motor neuron in an asymmetrical manner. In addition, more rapidly progressive bulbar symptoms could be observed by the clinical and neurophysiological examination. MRI revealed polymicrogyria of the right frontal lobe with irregular bumpy inner and outer surface and abnormally thick and smooth cortex, dysplastic insular cortex and asymmetrically widened Sylvial fissure. No signs of atrophy, especially of the motorcortex and corticospinal tract were observed. The corpus callosum was completely developed and of normal size. We hypothesize an enrolment of the dysplastic right frontal lobe pathophysiology of the observed mirror movements.
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PMID:Amyotrophic lateral sclerosis (ALS) and mirror movements in a patient with polymicrogyria. 1475 62

Fibroadenoma is the main cause of unilateral breast mass in teenagers and adolescents. 4% of these are a special form described as giant or juvenile fibroadenoma. For primary diagnosis, ultrasound is the method of choice. The MRI allows exact evaluation of size and location. The fibroadenoma must be distinguished from the phylloid tumour, which can be malignant. The latter occurs in patients of all ages, but peaks between the ages 40 and 50 years. Only 2% of all primary malignant breast lesions are found in women aged under 25. Metastases of other primary tumours must be excluded, especially with a history of prior malignancies. When planning the surgical excision, the final cosmetic result is important. Although the main reason of an asymmetrical breast enlargement of young girls is a benign mass, an early surgical excision is efficient with regard to the best possible cosmetic outcome.
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PMID:Fibroadenoma of the breast in an 11-year-old girl. 1502 81


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