Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Raynaud's phenomenon, uncommon in childhood, often heralds connective tissue disorder. Since microvascular abnormalities can be detected at an early stage of the connective tissue disease, especially in scleroderma, a specific diagnosis can be made in patients presenting with Raynaud's phenomenon alone or Raynaud's phenomenon associated with symptoms suggestive of connective tissue disease. Raynaud's phenomenon was studied in 11 consecutive children, 10 girls and 1 boy, ages 6 to 15. One child had a definite diagnosis of cutaneous polyarteritis nodosa. In 6 others connective tissue disease was suspected: 4 had arthritis, 2 has telangiectasia, leg ulcers and antinuclear antibodies. Of the remaining 4, one had hemiplegia and 3 Raynaud's phenomenon only. Oscillometry of the radial artery was reduced in 7 of 9. Decreased capillary resistance was found in 2 of 6, while abrupt thinning in conjunctival vessels was seen in 3 of 7. On nailfold capillaroscopy, reduced vascularity was noted in 5 of 11, dilated capillaries in 4 of 11, tortuousity in 2 of 11, capillary thinning in 1 of 11, capillary spasm in 1 of 11 and normal pattern in 3 of 11. Two patients presenting with Raynaud's phenomenon were found to have "scleroderma-like pattern" on nailfold capillaroscopy. One of them died 2 years later of cardiopulmonary sclerosis, and another developed esophageal stricture and Barrett's esophagus. Neither has sclerodermatous skin. In childhood Raynaud's phenomenon, nailfold capillaroscopy is a non-invasive examination enabling early diagnosis of "systemic scleroderma sine scleroderma".
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PMID:Raynaud's features in childhood. Clinical, immunological and capillaroscopic study. 149 54

Progressive multifocal leukencephalopathy (PML) is a very rare complication of immunosuppressive or cytostatic treatment of rheumatic diseases. We describe a patient with a mixed connective tissue disease (MCTD) existing for more than 15 years, who had polyarthritis, myositis, scleroderma-like symptoms, pulmonary involvement, and psychosis of undetermined origin. After a 12-year low-dose corticosteroid therapy and a 4-year azathioprin therapy a quickly progressive PML developed; symptoms included: difficulty in urinating, bladder, articulation, spastic hemiplegia, eye movement malfunction. The patient died after after a few months. PML is an important consideration in the differential diagnosis when a patient with MCTD develops neurologic symptoms.
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PMID:[Progressive multifocal leukoencephalopathy as a cause of neurologic symptoms in Sharp syndrome]. 174 73

A woman with CREST syndrome since the age of 35, had 11 and 13 years respectively after her disease onset, two episodes of CVA with residual right side hemiplegia. The angiography revealed segmented stenosis in the left common carotid, right subclavian and left renal arteries. At the age of 49 she developed gangrene of the right foot, requiring below the knee amputation. Pathological examination of the surgical specimen, showed extensive intimal fibrosis of the vessel walls in large and medium size arteries. Involvement of large and medium size arteries is infrequent in scleroderma. The case described illustrates this severe and unusual complication.
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PMID:Hemiplegia and peripheral gangrene secondary to large and medium size vessels involvement in C.R.E.S.T. syndrome. 718 36

We describe a systemic sclerosis and cerebral vascular accident case in which the cutaneous manifestation and the distal acroosteolysis occurred in an asymmetrical way in the non-paretic limb. The subsequent sclerodermic alterations and the acroosteolysis acquired an asymmetric pattern, sparing the patient's hemiparetic side. Although a number of definitions of this protective effect may be found in other rheumatic diseases, such as rheumatoid arthritis and gout, we found in the literature only one previous case describing the protective effect of the hemiplegia in scleroderma.
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PMID:Asymmetric scleroderma in a CVA patient. 1847 12