Neurodegeneration tare da tara baƙin ƙarfe kwakwalwa (NBIA)

Neurodegeneration tare da tarin baƙin ƙarfe na kwakwalwa (NBIA) rukuni ne na rikice-rikice masu saurin rikicewar tsarin. An wuce dasu ta hanyar dangi (sun gaji). NBIA ya shafi matsalolin motsi, rashin hankali, da sauran alamun tsarin jijiyoyi.

Kwayar cututtukan NBIA tana farawa ne tun yarinta ko girma.

Akwai nau'ikan NBIA guda 10. Kowane nau’i yana faruwa ne ta hanyar nakasuwar kwayar halitta daban Mafi yawan lalacewar kwayar halitta na haifar da cutar da ake kira PKAN (pantothenate kinase-hade neurodegeneration).

Mutanen da ke da duk nau'ikan NBIA suna da ƙarfe a cikin ƙananan ganglia. Wannan yanki ne mai zurfin cikin kwakwalwa. Yana taimakawa sarrafa motsi.

NBIA yafi haifar da matsalolin motsi. Sauran cututtuka na iya haɗawa da:

• Rashin hankali
• Matsalar magana
• Matsalar haɗiyewa
• Matsalolin tsoka kamar taurin kai ko ƙuntatawar ƙwayar tsoka (dystonia)
• Kamawa
• Tsoro
• Rashin hangen nesa, kamar na retinitis pigmentosa
• Rashin ƙarfi
• Jujjuyawar motsi
• Kafan kafa

Mai ba da sabis na kiwon lafiya zai yi gwajin jiki kuma ya yi tambaya game da alamomi da tarihin lafiya.

Gwajin kwayar halitta na iya neman kwayar halittar da ke haifar da cutar. Koyaya, waɗannan gwaje-gwajen basu yadu ba.

Gwaje-gwaje kamar na MRI na iya taimakawa wajen kawar da wasu rikicewar motsi da cututtuka. MRI yawanci yana nuna baƙin ƙarfe a cikin basal ganglia, kuma ana kiransu alamar "eye of the tiger" saboda yadda abubuwan da aka saka a cikin hoton. Wannan alamar tana nuna ganewar asali na PKAN.

Babu takamaiman magani don NBIA. Magunguna waɗanda ke ɗaure baƙin ƙarfe na iya taimakawa jinkirin cutar. Jiyya ya fi mayar da hankali kan sarrafa alamun. Magungunan da aka fi amfani dasu don sarrafa alamun sun hada da baclofen da trihexyphenidyl.

NBIA tana ƙara lalacewa kuma yana lalata jijiyoyi akan lokaci. Yana haifar da rashin motsi, kuma galibi mutuwa ta hanyar balagar farko.

Magungunan da ake amfani da su don magance alamomin na iya haifar da rikitarwa. Rashin ikon motsawa daga cutar na iya haifar da:

• Jinin jini
• Cututtukan numfashi
• Rushewar fata

Kira mai ba ku sabis idan yaronku ya ci gaba:

• Stara taurin kai a cikin hannu ko ƙafa
• Problemsara matsaloli a makaranta
• Motsa jiki mara kyau

Za'a iya bada shawarar bada shawara akan kwayoyin halittar dangin da wannan cutar ta shafa. Babu wata sananniyar hanyar da za a iya hana ta.

Hallervorden-Spatz cutar; Pantothenate kinase-hade neurodegeneration; PKAN; NBIA

Gregory A, Hayflick S, Adam MP, et al. Neurodegeneration tare da kwakwalwar ƙwaƙwalwar ƙarfe tarin cuta. 2013 Feb 28 [sabunta 2019 Oct 21]. A cikin: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews [Intanet]. Seattle, WA: Jami'ar Washington; 1993-2020. PMID: 23447832 pubmed.ncbi.nlm.nih.gov/23447832/.

Jankovic J. Parkinson cuta da sauran rikicewar motsi. A cikin: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology a cikin Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016: babi na 96.

Disungiyar Rashin Lafiya ta NBIA. Bayani game da rikice-rikicen NBIA. www.nbiadisorders.org/about-nbia/overview-of-nbia-disorders. An shiga Nuwamba 3, 2020.