Urbach-Wiethe Sindroom (beter bekend as lipo"ied prote"inose of hyalinosis cutis et mucosae) is 'n seldsame, outosomaal-oorerflike siekte. Die kenmerkendste simptome van die siekte is vel- en slymvliesveranderinge wat deur 'n neerslag van ekstrasellulere hialienagtige materiaal van onbekende oorsprong veroorsaak word. Die vel word maklik beseer, genees stadig en lelike, pokagtige letsels ontstaan. 'n Fyn, korrelagtige neerslag op die ooglede, die sogenaamde "kralestringvoorkoms" kenmerk die siekte, sowel as heesheid, die prominentste en lastigste simptoom, wat sedert geboorte teenwoordig kan wees. Radiografie en tomografie toon bilaterale,boontjievormige verkalking op die temporale lobbe van die brein, wat tot epileptiese aanvalle en ander neuropsigiese simptome soos geremde geheue en aggressie lei. Heesheid veroorsaak kommunikatiewe beperkinge vir die lyer,terwyldie opsigtelike velletsels aversiewe- en die verkalkings onsigbare beperkinge meebring. Die lyer aan Urbach-Wiethe Sindroom kan volgens die beginsels en kriteria van die medies-kliniese, die persoonsgeorienteerde en die sosio-omgewingsperspektiewe gestremdheid ondervind. Teoreties kan die lyer se belewinge van sy andersheid en die nie-aanvaardingdeurdie gemeenskap, soos by gestremdes,die handhawing van sy selfagting rem, sodat 'n negatiewe selfkonsep tot skuldgevoelens, angsbelewinge en depressie kan lei. 'n Betekenisvolle verband is in die empiriese ondersoek tussen die graad van aantasting en wanaanpassing in die lyer se leefwereld gevind. Die lyers wat ernstig aangetas is, identifiseer moeilik met hulle fisieke voorkoms. ldentiteitsvorming word gerem en die selfagting is negatief. Hulle openbaar 'n negatiewe selfkonsep en depressiewe gevoelens met selfmoordgedagtes. Hulle relasies en sosialisering is problematies en hulle voel hulle word nie deur die gemeenskap aanvaar nie. Die lyers ondervind 'n algemene wanaanpassing in hulle leefwereld. 'n Geval van paranoia is ook gevind. Alhoewel die ouers vrae oor die toestand het en sekere emosies beleef, kan die meeste van hulle die situasie hanteer. Riglyne vir 'n terapeutiese begeleidingsprogram vir lyers aan Urbach-Wiethe Sindroom, wat op die verbetering van die selfkonsep; die hantering van depressie, aggressie en woedebuie, asook die verbetering van relasies en sosialisering gerig is, is saamgestel. 'n Ondersteuningsgroep waarby lyers en hulle ouers kan inskakel, is gestig. / Urbach-Wiethe Syndrome, also known as lipoid proteinosis or hyalinosis cutis et mucosae, is a rare, recessively inherited, autosomal disorder characterized by lesions of the skin and mucosae, caused by widespread deposition of hyaline material of unknown etiology. The skin injures easily and heals slowly with "pock­ like" lesions. Bead-like deposits on the eyelids, called "string of pearls",are often found. Present since early infancy, hoarseness is the first, and most striking, irritating symptom. Bean-shaped intracranial calcification within the temporallobes of the brain in the area of the hippocampus, shown up by radiography and tomography, may cause epileptic seizures and other neuropsychological complications like impaired memory and aggression. The sufferer experiences communicative impairment through hoarseness, aversive impairment because of the conspicuous lesions, and concealed impairment as a result of the calcifications. According to the medical-clinical,person-orientated and socio-environmental perspectives on disability, a sufferer of Urbach-Wiethe Syndrome may experience disability. Like disabled persons, the sufferer finds it difficult to identify with his physical appearance. Impaired identity formation and low self-esteem cause a negative self­ concept. Feelings of guilt,anxiety and depression result from perceived dissimilarity, social rejection and low self-esteem. Socialising is adversely affected. A significant relationship was found in the empirical study between the extent to which the sufferer is affected and the degree of maladjustment in his life-world. Seriously affected sufferers display a negative self-concept and feelings of depression with suicidal thoughts. Socialisation and relationships are problematic and sufferers feel unaccepted by the community. General maladjustment in the life-world is experienced. A case of paranoia was reported. Except for some questions and unresolved feelings about the disease,most parents are able to cope with the situation. Guidelines have been set for a therapeutic programme for sufferers from Urbach­ Wiethe Syndrome aimed at enhancing self-concept, coping with depression and aggression, and improving relationships and socialising. A support group has been ounded for sufferers and their parents. / Psychology of Education / D. Ed. (Sielkundige Opvoedkunde)

Urbach-Wiethe Sindroom (beter bekend as lipo"ied prote"inose of hyalinosis cutis et mucosae) is 'n seldsame, outosomaal-oorerflike siekte. Die kenmerkendste simptome van die siekte is vel- en slymvliesveranderinge wat deur 'n neerslag van ekstrasellulere hialienagtige materiaal van onbekende oorsprong veroorsaak word. Die vel word maklik beseer, genees stadig en lelike, pokagtige letsels ontstaan. 'n Fyn, korrelagtige neerslag op die ooglede, die sogenaamde "kralestringvoorkoms" kenmerk die siekte, sowel as heesheid, die prominentste en lastigste simptoom, wat sedert geboorte teenwoordig kan wees. Radiografie en tomografie toon bilaterale,boontjievormige verkalking op die temporale lobbe van die brein, wat tot epileptiese aanvalle en ander neuropsigiese simptome soos geremde geheue en aggressie lei. Heesheid veroorsaak kommunikatiewe beperkinge vir die lyer,terwyldie opsigtelike velletsels aversiewe- en die verkalkings onsigbare beperkinge meebring. Die lyer aan Urbach-Wiethe Sindroom kan volgens die beginsels en kriteria van die medies-kliniese, die persoonsgeorienteerde en die sosio-omgewingsperspektiewe gestremdheid ondervind. Teoreties kan die lyer se belewinge van sy andersheid en die nie-aanvaardingdeurdie gemeenskap, soos by gestremdes,die handhawing van sy selfagting rem, sodat 'n negatiewe selfkonsep tot skuldgevoelens, angsbelewinge en depressie kan lei. 'n Betekenisvolle verband is in die empiriese ondersoek tussen die graad van aantasting en wanaanpassing in die lyer se leefwereld gevind. Die lyers wat ernstig aangetas is, identifiseer moeilik met hulle fisieke voorkoms. ldentiteitsvorming word gerem en die selfagting is negatief. Hulle openbaar 'n negatiewe selfkonsep en depressiewe gevoelens met selfmoordgedagtes. Hulle relasies en sosialisering is problematies en hulle voel hulle word nie deur die gemeenskap aanvaar nie. Die lyers ondervind 'n algemene wanaanpassing in hulle leefwereld. 'n Geval van paranoia is ook gevind. Alhoewel die ouers vrae oor die toestand het en sekere emosies beleef, kan die meeste van hulle die situasie hanteer. Riglyne vir 'n terapeutiese begeleidingsprogram vir lyers aan Urbach-Wiethe Sindroom, wat op die verbetering van die selfkonsep; die hantering van depressie, aggressie en woedebuie, asook die verbetering van relasies en sosialisering gerig is, is saamgestel. 'n Ondersteuningsgroep waarby lyers en hulle ouers kan inskakel, is gestig. / Urbach-Wiethe Syndrome, also known as lipoid proteinosis or hyalinosis cutis et mucosae, is a rare, recessively inherited, autosomal disorder characterized by lesions of the skin and mucosae, caused by widespread deposition of hyaline material of unknown etiology. The skin injures easily and heals slowly with "pock­ like" lesions. Bead-like deposits on the eyelids, called "string of pearls",are often found. Present since early infancy, hoarseness is the first, and most striking, irritating symptom. Bean-shaped intracranial calcification within the temporallobes of the brain in the area of the hippocampus, shown up by radiography and tomography, may cause epileptic seizures and other neuropsychological complications like impaired memory and aggression. The sufferer experiences communicative impairment through hoarseness, aversive impairment because of the conspicuous lesions, and concealed impairment as a result of the calcifications. According to the medical-clinical,person-orientated and socio-environmental perspectives on disability, a sufferer of Urbach-Wiethe Syndrome may experience disability. Like disabled persons, the sufferer finds it difficult to identify with his physical appearance. Impaired identity formation and low self-esteem cause a negative self­ concept. Feelings of guilt,anxiety and depression result from perceived dissimilarity, social rejection and low self-esteem. Socialising is adversely affected. A significant relationship was found in the empirical study between the extent to which the sufferer is affected and the degree of maladjustment in his life-world. Seriously affected sufferers display a negative self-concept and feelings of depression with suicidal thoughts. Socialisation and relationships are problematic and sufferers feel unaccepted by the community. General maladjustment in the life-world is experienced. A case of paranoia was reported. Except for some questions and unresolved feelings about the disease,most parents are able to cope with the situation. Guidelines have been set for a therapeutic programme for sufferers from Urbach­ Wiethe Syndrome aimed at enhancing self-concept, coping with depression and aggression, and improving relationships and socialising. A support group has been ounded for sufferers and their parents. / Psychology of Education / D. Ed. (Sielkundige Opvoedkunde)