Isifo somuntu esiqinile

-Delile

• Izimpawu
• Ukwelashwa

Esikhathini se-rigid person syndrome, lowo muntu unokuqina okukhulu okungazibonakalisa emzimbeni wonke noma emilenzeni kuphela, ngokwesibonelo. Lapho lokhu kuthinteka, umuntu angahamba njengesosha ngoba akakwazi ukunyakazisa imisipha namalunga akhe kahle.

Lesi yisifo se-autoimmune esivame ukuvela phakathi kweminyaka engama-40 nengama-50 ubudala futhi saziwa nangokuthi yi-Moersch-Woltmann syndrome noma ngesiNgisi, iStiff-man syndrome. Cishe ama-5% wamacala avela ebuntwaneni noma ebusheni.

Isifo sesifo somuntu esiqinile singabonakala ngezindlela ezi-6 ezihlukene:

1. Ifomu elijwayelekile lapho kuthinta kuphela isifunda se-lumbar nemilenze;
2. Ifomu ehlukile lapho kukhawulwe emlenzeni owodwa nje nge-dystonic noma ukuma emuva;
3. Ifomu elijwayelekile lapho ukuqina kwenzeka emzimbeni wonke ngenxa ye-encephalomyelitis ezenzakalelayo yokuzimela;
4. Lapho kunokuphazamiseka kokuhamba okusebenzayo;
5. Nge-dystonia kanye ne-parkinsonism ejwayelekile futhi
6. Ngefa spastic paraparesis.

Imvamisa umuntu onalesi sifo akanalesi sifo kuphela, kodwa futhi unezinye izifo ezizimele ezifana nohlobo 1 sikashukela, isifo segilo noma i-vitiligo, isibonelo.

Lesi sifo singelapheka ngemithi ekhonjiswe ngudokotela kodwa ukwelashwa kungadla isikhathi.

Izimpawu

Izimpawu zesifo somuntu esiqinile zinzima futhi zifaka:

• Ukuqina kwemisipha okuqhubekayo okuqukethe izinkontileka ezincane emisipheni ethile ngaphandle kokuthi umuntu akwazi ukulawula, futhi
• Ukuqina okuphawulwe emisipheni engadala ukuqhekeka kwemicu yemisipha, ukuhlukaniswa kwamathambo kanye nokwaphuka kwamathambo.

Ngenxa yalezi zimpawu umuntu angaba ne-hyperlordosis nobuhlungu emgogodleni, ikakhulukazi lapho kuthinteke imisipha yangemuva futhi ingawa njalo ngoba akakwazi ukunyakaza nokulinganisa kahle.

Ukuqina kwemisipha okukhulu kuvame ukuvela ngemuva kwesikhathi sokuxineka njengomsebenzi omusha noma ukwenza imisebenzi esidlangalaleni, futhi ukuqina kwemisipha akwenzeki ngesikhathi sokulala nokukhubazeka ezingalweni nasemilenzeni kujwayelekile ngenxa yokuba khona kwalezi zinhlungu, uma isifo aselashwa.

Ngaphandle kokwanda kwethoni yemisipha ezifundeni ezithintekile, ama-tendon reflexes ajwayelekile ngakho-ke ukuxilongwa kungenziwa ngokuhlolwa kwegazi okubheka amasosha omzimba athile kanye ne-electromyography. Ama-X-ray, ama-MRIs nama-CT scan nawo kufanele ayalelwe ukukhipha amathuba ezinye izifo.

Ukwelashwa

Ukwelashwa komuntu oqinile kufanele kwenziwe ngokusetshenziswa kwezidakamizwa ezifana ne-baclofen, vecuronium, immunoglobulin, gabapentin kanye ne-diazepam ekhonjiswe yisazi sezinzwa. Kwesinye isikhathi, kungadingeka ukuhlala e-ICU ukuze kuqinisekiswe ukusebenza kahle kwamaphaphu nenhliziyo ngesikhathi sesifo kanti nesikhathi sokwelapha singahluka kusuka emasontweni kuye ezinyangeni.

Ukufakwa kwegazi kanye nokusetshenziswa kwe-anti-CD20 monoclonal antibody (rituximab) nakho kungakhonjiswa futhi kube nemiphumela emihle. Iningi labantu okutholakale ukuthi linalesi sifo liyelapheka lapho lithola ukwelashwa.