| progressive tapetochoroidal dystrophy | An x chromosome-linked abnormality characterised by atrophy of the choroid and degeneration of the retinal pigment epithelium causing night blindness. (12 Dec 1998) |
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| progressive torsion spasm | A genetic, environmental, or idiopathic disorder, usually beginning in childhood or adolescence, marked by muscular contractions that distort the spine, limbs, hips, and sometimes the cranial-innervated muscles. The abnormal movements are increased by excitement and, at least initially, abolished by sleep. The musculature is hypertonic when in action, hypotonic when at rest. Hereditary forms usually begin with involuntary posturing of the foot or hand (autosomal recessive form ) or of the neck or trunk (autosomal dominant form ); both forms may progress to produce contortions of the entire body. Synonym: progressive torsion spasm, torsion disease of childhood, torsion dystonia, Ziehen-Oppenheim disease. (05 Mar 2000) |
| progressive vaccinia | A severe or even fatal form of vaccinia occurring chiefly in subjects with an immunologic deficiency or dyscrasia and characterised by progressive enlargement of the initial and also of secondary lesions. Synonym: vaccinia gangrenosa. (05 Mar 2000) |
| hereditary progressive arthro-ophthalmopathy | Autosomal dominant arthro-ophthalmopathy associated with progressive multiple dysplasia of the epiphyses, overtubulation of long bones, cleft lip and palate, hypermobility of joints, flattened vertebral bodies, pelvic bone deformities, and deafness. Synonym: Stickler's syndrome. (05 Mar 2000) |
| supranuclear palsy, progressive | A progressive neurological disease usually of the fifth decade characterised by supranuclear ophthalmoplegia especially paralysis of downward gaze, pseudobulbar palsy, gait disturbance, dysathria, truncal dystonia, memory and personality deterioration, and dementia. (12 Dec 1998) |
| diaphyseal dysplasia, progressive | Progressive thickening of diaphyseal cortex of long bones. (12 Dec 1998) |
| infantile progressive spinal muscular atrophy | Transmitted as autosomal recessive on chromosome 5q. Progressive dysfunction of the anterior horn cells in the spinal cord and brainstem cranial nerves with profound weakness and bulbar dysfunction occurring in the first two years of life. Three groups, based on age of clinical onset, are recognised. Synonym: familial spinal muscular atrophy, Hoffmann's muscular atrophy, infantile muscular atrophy, infantile progressive spinal muscular atrophy, progressive infantile spinal muscular atrophy, Werdnig-Hoffmann disease, Werdnig-Hoffmann muscular atrophy. (05 Mar 2000) |
| ophthalmoplegia, chronic progressive external | One of the mitochondrial encephalomyopathies characterised by slowly progressive paralysis of the extraocular muscles. Muscle biopsies disclose the characteristic ragged red fibres and large numbers of mitochondria with deleted DNA. (12 Dec 1998) |
| ovine progressive pneumonia | A chronic, progressive, contagious interstitial pneumonitis of sheep in Europe and the U.S. Caused by a "slow virus" (family Lentiviridae); it is now believed that maedi and visna are two histopathological and clinical manifestations of the same viral infection. Synonym: ovine progressive pneumonia. Origin: Icelandic, dyspnea (05 Mar 2000) |
| traumatic progressive encephalopathy | A chronic progressive brain damage resulting from multiple brain injuries, e.g., dementia pugilistica. (05 Mar 2000) |
| essential progressive atrophy of iris | Progressive atrophy of the iris without inflammatory signs, characterised by patchy loss of all layers of the iris with hole formation, migration of the pupil, degeneration of the corneal endothelium, peripheral anterior synechiae, and secondary glaucoma; usually unilateral, predominantly affecting women in their middle years. (05 Mar 2000) |
| lenticular progressive degeneration | <gastroenterology, neurology> An inherited (autosomal recessive) disorder where there is excessive quantities of copper in the tissues, particularly the liver and central nervous system. Wilson's disease causes the body to absorb and retain copper. The copper deposits in the liver, brain, kidneys and eyes. Complications include dementia and liver failure. Symptoms include jaundice, vomiting, tremors, weakness and slow stiff movements. Blood tests show serum ceruloplasmin is low. Medications are given to remove the excess copper from the body. Even with life-long treatment, disabling (and life-threatening) side effects are common. Inheritance: autosomal recessive. (27 Sep 1997) |
| leukoencephalopathy, progressive multifocal | Rare demyelinating disease of the central nervous system which develops in immunocompromised patients secondary to lymphoproliferative disease, immunosuppressive therapy, autoimmune disorders, or aids. It is caused by the jc virus, a polyomavirus. (12 Dec 1998) |