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11/151. Thalamic hand dystonia: an MRI anatomoclinical study.

    Focal dystonia has been attributed to lesions involving the basal ganglia and/or thalamus. Hand dystonia was studied in a patient with a unilateral thalamic infarction documented by MRI. A 18-year-old girl presented with severe isolated dystonia of the right hand as a sequel of perinatal infarction. MRI scan revealed infarction affecting part of the dorsomedian, lateral posterior, ventral lateral, ventral posterior lateral nuclei, and centromedian-parafascicular nucleus of the contralateral thalamus. The unique MRI anatomoclinical presentation of this case, taken together with the literature data, could provide evidence that a lesion affecting one or several thalamic nuclei, including the centromedian nucleus, can induce hand dystonia.
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12/151. Physeal arrest due to laser beam damage in a growing child.

    A laser beam applied directly to epiphyseal cartilage may damage the cartilage selectively without affecting adjacent bone. The damaged physis is replaced by bone, which forms a bone bridge between the metaphysis and epiphysis, similar to the bone bridges that follow fracture of any long bone. This case report documents damage to two distal phalangeal epiphyseal plates as a sequela of laser beam injury. This resulted in premature partial physeal closure (physeal bars), which in turn caused progressive angular deformity and relative shortening of the digits, requiring multiple osteotomies for correction. This report suggests that special care must be exercised when using laser therapy near physes in growing children.
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ranking = 24880.020783033
keywords = deformity
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13/151. Proximal interphalangeal joint surface replacement arthroplasty.

    A consecutive series of 20 joints in 13 patients underwent arthroplasty with the RMS PIP joint surface replacement implant. Twelve joints were treated for painful osteoarthritis (all females). Two joints were implanted for rheumatoid arthritis, two for post-traumatic pain and stiffness, two for post-traumatic stiffness and one each for post-traumatic pain and pain-free post-traumatic instability. Excellent, often total long-term pain relief was obtained in 18 joints. The other two patients with (compensible work-related) post-traumatic pain and stiffness reported "50-70% pain reduction". No patients lost movement and 14 out of 20 joints were pain-free with a 73.1 degrees average arc of motion. Six joints from the first half of the series had poor motion (average arc of 19.6 degrees ), even after open extensor tenolysis or manipulation under anaesthesia. As experience was gained, reliably better results were achieved with a more intensive regimen of hand therapy, particularly within the first post-operative week.
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14/151. Treatment of hand deformities in a long-term survivor with dermolytic bullous dermatosis-recessive (DBD-R).

    Treatment of hand deformities in a long-term survivor with dermolytic bullous dermatosis-recessive is described. An "open method" is sufficient for dealing with volar skin defects. There is still no effective treatment for perfect control of blister formation. But appropriate surgery, followed by careful post-operative rehabilitation can give reasonable hand function.
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15/151. Camptodactyly in a neurology outpatient clinic.

    Camptodactyly is a non-neurogenic flexion deformity of the proximal interphalangeal joint, most commonly affecting the little finger. The frequency of camptodactyly in patients referred to three general neurology outpatient clinics, two in district general hospitals and one in a regional neuroscience centre, over the course of one year (2000) was measured. Five out of 978 consecutive new outpatients (0.5%) had camptodactyly (2 M, 3 F); four had bilateral deformity. In only one was camptodactyly the reason for referral. In two cases, camptodactyly was entirely incidental to the neurological symptoms; in two others it was incidental but might have been confused with the neurological features. In three cases other family members were affected, in a manner consistent with autosomal dominant inheritance with variable penetrance. Camptodactyly is not infrequently observed in the general neurology outpatient clinic. It merits attention in order to avoid confusion with other, neurogenic, causes of clawing, and so avoid unnecessary investigation.
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ranking = 49760.041566066
keywords = deformity
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16/151. melorheostosis: unusual presentation in a girl.

    melorheostosis is a rare bone disease of unknown etiology. It is characterized by tense and erythematous skin, contractures and shortening of the affected limbs. Radiographs reveal hyperostosis in long bones resembling melting wax, and spotty or patchy endostic deposits in short bones. An 11-year-old girl showed, since birth, contractural deformities in the right hand without bone abnormalities. At the age of 12 months, she complained of soft tissue tightness in the right buttock. At 3 years, limitation of flexion and extension of the right knee was evident. Radiographs revealed findings typical of melorheostosis.
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keywords = hand
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17/151. Finger sucking digital deformities.

    We report our experience with severe digital deformities caused by prolonged finger sucking. Our analysis of nine patients demonstrated that the deformity was mainly located in the proximal and middle phalanges of the affected digit(s). We have reviewed the literature, enumerated the common patterns of "finger sucking" and explained the resulting deformities. Corrective osteotomy, preferably at the metacarpal base level, is required in severe deformities.
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keywords = deformity
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18/151. Localized hypertrophy of hand in syringomyelia.

    Segmental amyotrophy is a common feature resulting from extension of the syrinx into the anterior horns. Segmental hypertrophy in the form of chiromegaly/podomegaly or limb and hemi-hypertrophy are rare but well recognized clinical feature of syringomyelia. We report a case of hand hypertrophy secondary to syringomyelia. hypertrophy of the hands is a rare but interesting feature of syringomyelia and in all cases of enlargements of hands syringomyelia should be considered in the differential diagnosis.
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19/151. Extension contracture of the wrist caused by fibrosis of the extensor carpi radialis longus muscle.

    A patient with bilateral extrinsic contracture of the wrists is described. The management of this deformity is presented in detail.
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keywords = deformity
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20/151. Deforming arthritis of the hands in polymyositis.

    Arthritis of the hands with erosions, periosteal calcification, and interphalangeal thumb joint instability was seen in 6 patients with polymyositis. "Overlap" features such as Raynaud's phenomenon, positive LE clot test, and positive antinuclear antibody test were present, but clinically the primary disease was clearly polymyositis. This rather unusual constellation of roentgenographic findings strongly suggests the possibility of polymyositis.
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