title: Combined treatment with LDL-apheresis, chenodeoxycholic acid and HMG-CoA reductase inhibitor for cerebrotendinous xanthomatosis.
abstract: The effects of combined treatment with low-density lipoprotein (LDL)-apheresis, chenodeoxycholic acid (CDCA) and 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) reductase inhibitor were studied in 2 patients with cerebrotendinous xanthomatosis. Patient 1 was initially treated with LDL-apheresis alone: serum cholestanol levels decreased by 50% after each apheresis, but returned to their initial levels within 2 weeks. After an addition of CDCA administration, the serum cholestanol levels steadily decreased, resulting in slight improvement of neurological symptoms. Patient 2 received a combined treatment with LDL-apheresis, CDCA and HMG-CoA reductase inhibitor. This combination showed less LDL-apheresis-dependent fluctuation and more rapid decrease of serum cholestanol levels than those in Patient 1, resulting in improvement and stabilization of the symptoms. Our results suggest that LDL-apheresis in combination with CDCA and HMG-CoA reductase inhibitor may have beneficial effects and can be one of the treatment options.
authors: Department of Neurology (D3), Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, 260-8670, Chiba, Japan. s_yonezu@ka2.so-net.ne.jp
authors: Shoichi
authors: S
authors: Ito
authors: Satoshi
authors: S
authors: Kuwabara
authors: Ryuji
authors: R
authors: Sakakibara
authors: Takeshi
authors: T
authors: Oki
authors: Hiroshi
authors: H
authors: Arai
authors: Shigeto
authors: S
authors: Oda
authors: Takamichi
authors: T
authors: Hattori
mesh_terms: D000328:Adult; D001781:Blood Component Removal; D002635:Chenodeoxycholic Acid / Q000494:pharmacology*; D004083:Cholestanol / Q000037:antagonists & inhibitors / Q000097:blood*; D002784:Cholesterol / Q000378:metabolism; D003131:Combined Modality Therapy; D004359:Drug Therapy, Combination; D006801:Humans; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors / Q000494:pharmacology* / Q000627:therapeutic use; D008077:Lipoproteins, LDL / Q000097:blood* / Q000187:drug effects; D008297:Male; D016896:Treatment Outcome; D019294:Xanthomatosis, Cerebrotendinous / Q000097:blood / Q000503:physiopathology / Q000628:therapy*
chemical_list: D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors; D008077:Lipoproteins, LDL; D002635:Chenodeoxycholic Acid; D004083:Cholestanol; D002784:Cholesterol