NG DNG LM SNG CA VIC PHN LOI BNH THN KINH NGOI BIN

BS L MINH Bnh thn kinh ngoi bin rt thng gp nhng cng c th gy nhiu kh khn cho vic chn on do bi tnh a dng v phc tp ca cc bnh thuc nhm ny. Chn on bnh thn kinh ngoi bin bi l c th chim nhiu thi gian v ngn nhiu tn ph nhng lm khi li dn n nhng kt qu tht bi khng ng. Vic thm khm lm sng k lng phi hp vi kho st in c k ng quy cch l nhng bc i u tin ca chn on nhm cc bnh ny. Trong qu trnh ny kiu cch din tin v loi tn thng ca h thn kinh ngoi bin cng l nhng thnh t rt gip ch cho chn on bnh thn kinh ngoi bin. Cc thm d cn lm sng cn thit cho chn on bnh thn kinh ngoi bin gm c in c k (kho st dn truyn thn kinh, in c kim), thm d chc nng thn kinh t ch, kho st dch no ty, v sinh thit dy thn kinh ngoi bin. n nay cha c nhng d liu dch t c quy m v bnh thn kinh ngoi bin Vit Nam, tuy nhin trong thc hnh lm sng thng ngy, cc bnh thng gp l bnh dy thn kinh i tho ng (diabetic neuropathy), bnh thn kinh ngoi bin do ri loi min nhim (dysimmune neuropathy), bnh thn kinh ngoi bin do thuc (iatrogenic neuropathy), bnh thn kinh ngoi bin do vi trng Hansen (leprous neuropathy), bnh thn kinh ngoi bin ngi nhim HIV, cc chn thng dy thn kinh v r thn kinh (traumatic neuropathy, compressive neuropathy), bnh thn kinh ngoi bin di truyn Charcot-Marie-Tooth. cc trung tm ln v bnh thn kinh ngoi bin ca cc nc pht trin, s bnh thn kinh ngoi bin khng xc nh c nguyn nhn chim t l t 13 n 22 phn trm. Nu kho st k tin s gia nh c khi nhiu trng hp ban u khng r nguyn nhn li bc l ra l mt bnh thn kinh ngoi bin di truyn (c th ln n 42 phn trm).

Tip cn chn on
Kiu phn loi thng dng v y bnh thn kinh ngoi bin thng tch hp cc kha cnh sau y (1) kiu hi chng lm sng, (2) kiu tn thng ca dy thn kinh l tn

thng si trc hay tn thng myelin, tn thng si ln hay tn thng si nh v (3) nguyn nhn ca bnh thn kinh ngoi bin l di truyn hay mc phi. t c cc mc tiu nu trn trc ht cn thc hin y v ti m khu tip cn lm sng trong gm c (1) Hi bnh s v tin s c nhn cng nh tin s gia nh tht chi tit, (2) khm chi tit pht hin cc triu chng chc nng v thc th ca tn thng thn kinh ngoi bin. Trong khai thc bnh s, cn ch nhng chi tit sau y: Kiu khi pht t ngt, cp hay bn cp, hay kiu din tin mn tnh nng dn? Kiu phn b ca cc triu chng lc mi pht bnh, i xng hay khng i xng, ngn chi hay gc chi? Triu chng chc nng c th v cm gic, vn ng v thn kinh t ch?

Trong thm khm cc tiu chng thc th, cn ch : Cc bin dng xng khp v da lng tc? Cch phn b ca cc triu chng cm gic (i gng i v, theo phn b ca dermatomes, phn b kiu mng)? Triu chng lit v teo c i xng hay khng i xng, ngn chi hay gc chi? Triu chng gim hay mt phn x gn c? Triu chng dy thn kinh ph i? Triu chng thn kinh t ch i km?

Vi cc d liu c c t thm khm k trn ngi ta c th khoanh vng v thu hp c cc kh nng chn on trng hp bnh thn kinh ngoi bin c th , v d: Khi pht t ngt hay cp tnh v triu chng kh tr, cc b (bnh mt dy thn kinh do tc mch, do chn p cp). Khi pht cp tnh v i xng (bnh a r dy thn kinh cp, v d Hc GuillainBarr).

Din tin ko di v phn b ri rc, khng i xng kiu ca bnh nhiu dy thn kinh (bnh nhiu dy thn kinh i tho ng, bnh nhiu dy thn kinh ca bnh phong).

Din tin ko di trn hai thng hoc ti i ti li nhiu ln vi triu chng phn b i xng (vim a r dy thn kinh mt myelin mn tnh).

Din tin ko di nhiu nm, triu chng phn b i xng, c km bin dng c xng khp (bnh thn kinh ngoi bin di truyn Charcot-Marie-Tooth).

Triu chng khu tr lng bn tay trong vng chi phi ca dy thn kinh gia, din tin ko di (Hc ng c tay).

Triu chng au v lit lan rng c mt chi trn, din tin bn cp (vim m ri cnh tay).

Kho st in c k (electromyography) l mt thm d cn lm sng khng th thiu c trong quy trnh chn on bnh thn kinh ngoi bin. Hai loi thm d quan trong y gm c kho st dn truyn (conduction study) v in c kim (needle electromyography) s gip ngi ta tr li c cc cu hi sau y: y c phi l mt bnh thn kinh ngoi bin hay khng? (ngoi tr trng bnh si nh, small fiber neuropathy). Tn thng si trc, hay tn thng myelin, hay tn thng hn hp? Tn thng cp, ang cn hot tnh hay tn thng mn tnh? Tn thng phn b kiu a r, a dy hay phn b theo kiu nhiu dy?

Kho st dch no ty s rt gip ch v cn thit phi c ch nh trong cc trng hp nghi ng mt bnh a r (polyradiculopathy) hay bnh nhiu r thn kinh (multiple radiculopathy). Sinh thit dy thn kinh (nerve biopsy) c th s gip ch trong mt s tnh hung chn on bnh thn kinh ngoi bin v s xc nh c tn thng thn kinh ngoi bin, gip nhn dng r c ch gy tn thng v nguyn nhn ca bnh thn kinh ngoi bin ny. Cng cn lu l trong thc hnh lm sng thng ngy, nhiu loi bnh thn kinh ngoi bin mc phi v di truyn c chn on chnh xc ch nh cn c vo lm sng v

in c cng nh mt s xt nghim cn lm sng khc c lin quan, nhng trng hp ny khng cn n sinh thit dy thn kinh. Bng 1. Cc nguyn nhn ca bnh thn kinh ngoi bin Bnh mt dy thn kinh Bnh dy thn kinh b mc kt (vd, Hc ng c tay) Chn thng trc tip hay gin tip Bnh nhiu dy thn kinh Bnh thn kinh ngoi bin do vim mch i tho ng Sarcoidosis Phong Bnh dy thn kinh vn ng nhiu Bnh Tangier Hi chng Lewis-Sumner Bnh dy thn kinh di truyn d b lit do chen p (HNPP) Bnh a dy thn kinh i xng Bnh thn kinh ngoi bin do ri lon min nhim Hc Guillain-Barre (AIDP, AMAN, AMSAN) Vim a r dy thn kinh mt myelin mn (CIDP) Bnh thn kinh ngoi bin trong sarcoidosis, lupus Bnh thn kinh ngoi bin do nhim c Do thuc Do c cht khc trong mi trng chung quanh Bnh thn kinh ngoi bin do thiu vitamin Vitamin B1, B6 Vitamin B12, Folate Bnh thn kinh ngoi bin c lin quan ung th Hi chng cn ung th Do ung th thm nhim vo dy thn kinh Bnh thn kinh ngoi bin c lin quan vi bnh paraprotein huyt Bnh a u tuy Cryoglobulin huyt Hi chng POEMS Amyloidosis AL Bnh thn kinh ngoi bin chuyn ha i tho ng Nhc gip Suy thn Bnh thn kinh ngoi bin trong bnh nhim HIV Phong Bnh Lyme Nguyn nhn khc Bnh thn kinh ngoi bin ca sn sc c bit Lymphoma Bnh thn kinh ngoi bin di truyn

Bng 2. Cc thuc c th gy bnh thn kinh ngoi bin Vinca alkaloids (vinblastne, vincristine, vindesine, vinorelbine) Almitrine Amiodarone Chloroquine Cisplatine Colchicine Dapsone Didanoside (iu tr HIV) Disulfiram Interferon Isoniazide Metronidazole Misonidasole Nitrofurantoin Nitrat oxid Perhexilline Phenytoin Pyridoxine liu cao gy ng c Mui vng Stavudine (iu tr HIV) Taxoid Thalidomide Zalcitabine (iu tr HIV)

Bng 3. Phn loi bnh thn kinh ngoi bin theo kiu cch khi pht v din tin Th bnh cp tnh Trong vng vi gi (ch yu l cc bnh vim mch) Trong vng vi ngy (t hn 4 tun): Vim a r cp tnh Bnh thn kinh ngoi bin porphyria Bnh thn kinh ngoi bin ca bch hu Bnh thn kinh ngoi bin do nhim c Bnh thn kinh ngoi bin chuyn ha (i tho ng, thiu dinh dng, nghin ru) Bnh thn kinh ngoi bin au n v gy teo c Th bnh bn cp/mn tnh (xut hin t t trong nhiu thng) a phn cc bnh thn kinh ngoi bin chuyn ha, nhim c, thiu dinh dng. Bnh thn kinh ngoi bin ca dysproteinemia (myeloma, plasmocytoma, cryoglobulinemia) Bnh thn kinh ngoi bin ca lymphoma Bnh thn kinh ngoi bin amyloid Bnh thn kinh ngoi bin trong cc bnh ton thn Bnh thn kinh ngoi bin cn ung th Th bnh mn tnh (khi pht v ko di nhiu nm, t khi cn l tr nh) Bnh thn kinh ngoi bin di truyn Bnh a r dy thn kinh mn tnh Bnh a dy thn kinh c lin quan vi monoclonal IgM gammopathy Th bnh ti pht Bnh a r dy thn kinh mt myelin mn Bnh dy thn kinh di truyn d b lit khi chn p (hereditary neuropathy with liability to pressure palsy)

Bng 4. Cc th bnh thn kinh ngoi bin au n nguyn pht V cn Bnh a dy thn kinh ngn chi si nh Vim Vim mch Vim bao ngoi vi ca dy thn kinh (perineuritis) Di truyn Bnh Fabry Bnh dy thn kinh cm gic-t ch di truyn type V Bnh Tangier Bnh amyloid gia nh Bnh chuyn ha Bnh a dy thn kinh i tho ng ngn chi i xng Bnh r thn kinh i tho ng bt i xng Bnh dy thn kinh ngoi bin i tho ng ca trc thn Nhim c Thallium, arsenic Bnh amyloid AL

Bng 5. Bnh thn kinh ngoi bin si ln vi triu chng tht iu Bnh ri lon min nhim Bnh ca hch thn kinh Bnh thn kinh ngoi bin cm gic cn ung th Bnh thn kinh ngoi bin i km vi hi chng Sjoegren Bnh thn kinh ngoi bin cm gic v cn (cp, bn cp, mn) Bnh thn kinh ngoi bin mt myelin Hi chng Guillai-Barr Vim a r mt myelin mn (CIDP) Bnh a dy thn kinh lin quan vi gammapathy n dng IgM (anti-MAG) Bnh nhim Tabes dorsalis Bnh thn kinh ngoi bin bch hu Bnh thn kinh ngoi bin lin quan HIV Bnh thn kinh ngoi bin tht iu ca nhim HTLV-1 Nhim c Do thng d vitamin B6 Cisplatine Metronidazole Vacor

Bng 6. Bnh thn kinh ngoi bin c triu chng thn kinh t ch ni bt Bnh thn kinh ngoi bin i tho ng (tn thng si nh) Bnh thn kinh ngoi bin amyloid gia nh v mc phi Bnh thn kinh t ch cn ung th Hi chng Guillain-Barr Bnh thn kinh ngoi bin do thuc: vincristine, taxol, cisplatine, amiodarone Bnh thn kinh ngoi bin do nhim c: vacor, thallium, arsenic, thy ngn, cht dung mi hu c, acrylamide Bnh thn kinh ngoi bin ca nghin ru porphyria

Bng 7. Bnh thn kinh ngoi bin n thun vn ng (hoc tri v vn ng) Bnh sng trc ty (st bi lit, teo c ty) Bnh dy thn kinh vn ng nhiu c bloc dn truyn (MMN) Vim a r dy thn kinh cp v mn Bnh thn kinh ngoi bin do ng c ch Bnh thn kinh ngoi bin ca porphyria Bnh CMT Bnh thn kinh ngoi bin gy teo c Bnh thn kinh ngoi bin gc chi i tho ng

Bng 8. Phn loi cc bnh thn kinh ngoi bin theo tn thng si trc hay tn thng myelin Bnh thn kinh ngoi bin si trc Phn ln bnh thn kinh ngoi bin chuyn ha, do thiu dinh dng, do nhim c, do ru Bnh thn kinh ngoi bin do vim mch hay thm nhim Phn ln bnh thn kinh ngoi bin cn ung th Phn ln bnh thn kinh ngoi bin trong cc bnh ton thn Bnh thn kinh ngoi bin amyloid gia nh hay hu cbnh thn kinh ngoi bin ca porphyria cp Mt s bnh thn kinh ngoi bin ca dysproteinemia (myeloma, cryoglobulinemia) Mt s bnh thn kinh ngoi bin di truyn (CMT2) Bnh a dy thn kinh nguyn nhn khng r u th cm gic Bnh a dy thn kinh vn ng mun CMT like Bnh thn kinh ngoi bin mt myelin Cc vim a r dy thn kinh cp v mn Bnh a dy thn kinh c lin quan vi monoclonal gammapathies lnh tnh Bnh a dy thn kinh ca cc bnh dysproteinemia khc (plasmocytoma, myeloma x ct, Hc POEMS) Bnh a dy thn kinh di truyn (CMT1, Dejerine-Sottas, Refsum, metachromatic leukodystrophy)

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