Neurodegeneration yokhala ndi iron iron kudzikundikira (NBIA)

Neurodegeneration yokhala ndi chitsulo chamaubongo (NBIA) ndi gulu la zovuta zamanjenje zosowa kwambiri. Amadutsa m'mabanja (obadwa nawo). NBIA imakhudza kusuntha, matenda amisala, ndi zizindikilo zina zamanjenje.

Zizindikiro za NBIA zimayamba muubwana kapena munthu wamkulu.

Pali mitundu 10 ya NBIA. Mtundu uliwonse umayambitsidwa ndi vuto lina la majini. Vuto lofala kwambiri la majini limayambitsa matenda otchedwa PKAN (pantothenate kinase-associated neurodegeneration).

Anthu omwe ali ndi mitundu yonse ya NBIA ali ndi chitsulo chambiri mu basal ganglia. Awa ndi malo omwe ali mkati mwa ubongo. Zimathandiza kuyendetsa kayendedwe.

NBIA imayambitsa mavuto osuntha. Zizindikiro zina zitha kuphatikiza:

• Kusokonezeka maganizo
• Kulankhula kovuta
• Zovuta kumeza
• Mavuto am'mimba monga kukhwimitsa kapena kutsekeka kwamagulu (dystonia)
• Kugwidwa
• Kugwedezeka
• Masomphenya kutayika, monga retinitis pigmentosa
• Kufooka
• Kusuntha kolemba
• Kuyenda chala

Wothandizira zaumoyo adzayesa thupi ndikufunsa za zidziwitso ndi mbiri yazachipatala.

Mayeso achibadwa amatha kuyang'ana za jini yolakwika yomwe imayambitsa matendawa. Komabe, mayeserowa sakupezeka kwambiri.

Mayeso monga kusanthula kwa MRI atha kuthana ndi zovuta zina zoyenda ndi matenda. MRI nthawi zambiri imawonetsa mayikidwe azitsulo mu basal ganglia, ndipo amatchedwa "diso la kambuku" chifukwa chazomwe zimayang'ana pakuwunika. Chizindikiro ichi chikuwonetsa kuti ali ndi matenda a PKAN.

Palibe mankhwala enieni a NBIA. Mankhwala omwe amamanga chitsulo amatha kuthandiza kuchepetsa matendawa. Chithandizo chimayang'ana kwambiri kuwongolera zizindikirazo. Mankhwala omwe amagwiritsidwa ntchito kwambiri poletsa zizindikiro monga baclofen ndi trihexyphenidyl.

NBIA imakulirakulira ndipo imawononga mitsempha pakapita nthawi. Zimayambitsa kusayenda, ndipo nthawi zambiri amafa akadali wamkulu.

Mankhwala omwe amagwiritsidwa ntchito pochiza zitha kubweretsa zovuta. Kulephera kuchoka pa matenda kungayambitse:

• Kuundana kwamagazi
• Matenda opuma
• Kuwonongeka kwa khungu

Itanani omwe akukuthandizani ngati mwana wanu akukula:

• Kuchuluka kuuma kwa mikono kapena miyendo
• Mavuto owonjezeka kusukulu
• Kusuntha kosazolowereka

Upangiri wa chibadwa ungalimbikitsidwe kwa mabanja omwe akhudzidwa ndi matendawa. Palibe njira yodziwika yopewera izi.

Matenda a Hallervorden-Spatz; Pantothenate kinase-yokhudzana ndi neurodegeneration; PKAN; NBIA

Gregory A, Hayflick S, Adam MP, ndi al. Neurodegeneration yokhala ndi vuto lamaubongo achitsulo. 2013 Feb 28 [yasinthidwa 2019 Oct 21]. Mu: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews [Intaneti]. Seattle, WA: Yunivesite ya Washington; 1993-2020. Kufufuza kwa PMID:

Matenda a Jankovic J. Parkinson ndi zovuta zina zoyenda. Mu: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, olemba. Neurology ya Bradley mu Kuchita Zachipatala. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2016: chap 96.

Bungwe la NBIA Disrupt Association. Chidule cha zovuta za NBIA. www.nbiadisorders.org/about-nbia/overview-of-nbia-disorders. Idapezeka pa Novembala 3, 2020.