Isifo seCharcot-Marie-Tooth

Isifo seCharcot-Marie-Tooth liqela lokuphazamiseka okudluliselwe kwiintsapho ezichaphazela imithambo-luvo engaphandle kwengqondo kunye nomqolo. Ezi zibizwa ngokuba yimithambo yoluvo.

ICharcot-Marie-Tooth yenye yezona ngxaki zixhaphakileyo zinxulumene ne-nerve ezidluliselwe kwiintsapho (ezizuzwe njengelifa). Utshintsho ubuncinci kwimfuza engama-40 lubangela iintlobo ezahlukeneyo zesi sifo.

Esi sifo sikhokelela ekonakaleni okanye ekutshatyalalisweni (i-myelin sheath) ejikeleze iintsinga zemithambo-luvo.

Imithambo ekhuthaza intshukumo (ebizwa ngokuba yimithambo-luvo yeemoto) ichaphazeleka kakhulu. Iimbilini emilenzeni zichaphazelekayo kuqala kwaye zinzima kakhulu.

Iimpawu zihlala ziqala phakathi kobuntwana kunye nokuba mdala. Banokubandakanya:

• Ukukhubazeka kweenyawo (i-arch ephezulu kakhulu ukuya kwiinyawo)
• Ukuhla kweenyawo (ukungakwazi ukubamba unyawo ngokuthe tyaba)
• Ukuphulukana nemisipha yomlenze esezantsi, ekhokelela kumathole abhityileyo
• Ukuba ndindisholo elunyaweni okanye emlenzeni
• "Ukubetha" ukuhamba (iinyawo zibetha phantsi nzima xa uhamba)
• Ubuthathaka besinqe, imilenze, okanye iinyawo

Emva kwexesha, iimpawu ezifanayo zinokubonakala ezingalweni nasezandleni. Oku kunokubandakanya isandla esifana neenzipho.

Uvavanyo lomzimba lunokubonisa:

• Kunzima ukuphakamisa inyawo nokwenza iintshukumo zokuphuma kweenzwane (ukuhla kweenyawo)
• Ukungabikho kolwazelelelo kwimilenze
• Ukuphulukana nolawulo lwezihlunu kunye ne atrophy (ukuncipha kwezihlunu) elunyaweni okanye emlenzeni
• Iinyanda ezijijekileyo phantsi kolusu lwemilenze

Uvavanyo lokuqhutywa kwemithambo luhlala lwenziwa ukuchonga iindlela ezahlukeneyo zokuphazamiseka. I-biopsy ye-nerve inokuqinisekisa ukuxilongwa.

Uvavanyo lwemfuza lukwakhona nakwezinye iintlobo zesifo.

Akukho lunyango lwaziwayo. Utyando lwamathambo okanye izixhobo (ezinje ngezihlangu zezilima okanye izihlangu zamathambo) zinokwenza kube lula ukuhamba.

Unyango ngokwasemzimbeni kunye nasemsebenzini lunokunceda ukugcina amandla emisipha kunye nokuphucula ukusebenza ngokuzimeleyo.

Isifo seCharcot-Marie-Tooth siye siba mandundu. Amanye amalungu omzimba anokuba ndindisholo, kwaye iintlungu zingahluka ukusuka kubumnene ukuya kubunzima. Ekugqibeleni esi sifo sinokubangela ukukhubazeka.

Iingxaki zinokubandakanya:

• Ukuqhubela phambili ukungakwazi ukuhamba
• Ubuthathaka obuqhubekayo
• Ukwenzakala kwiindawo zomzimba eziye zancipha uvakalelo

Fowunela umboneleli wakho wezempilo ukuba kukho ubuthathaka obuqhubekayo okanye ukuncipha komoya kwiinyawo okanye emilenzeni.

Ukucebisa ngemfuza kunye nokuvavanywa kuyacetyiswa ukuba kukho imbali eyomeleleyo yosapho kwesi sifo.

Progressive neuropathic (peroneal) muscular atrophy; Ukungasebenzi kakuhle kwemithambo-luvo; Neuropathy - peroneal (ilifa); Imoto yelifa kunye ne-neuropathy yeemvakalelo

• Inkqubo ye-nervous central kunye ne-peripheral system

Katirji B.Ukuphazamiseka kwemithambo yoluvo. Ku: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, ii-eds. I-Neurology kaBradley kwiKlinikhi yokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier Saunders; Ngo-2016: isahluko 107.

I-Sarnat HB. I-neuropathies yeemoto ezizuzwa njengelifa. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahluko 631.