Results of quinacrine administration to patients with Creutzfeldt-Jakob disease.
Several chemicals inhibit the accumulation of abnormal prion proteins in vitro. We administered one, the antimalarial agent quinacrine, to three patients with sporadic Creutzfeldt-Jakob disease (CJD) and to one with iatrogenic CJD. Quinacrine at 300 mg/day was given enterally for 3 months. Within 2 weeks of administration, the arousal level of the patient with akinetic mutism improved. The other 3 patients, insensible before treatment, had integrative responses such as eye contact or voluntary movement in response to verbal and/or visual stimuli restored. Clinical improvement was transient, lasting 1-2 months during treatment. Quinacrine was well tolerated, except for liver dysfunction and yellowish pigmentation. Although its antiprion activity in the human brain has yet to be proved, these modest effects of quinacrine suggest the possibility of using chemical intervention against prion diseases.
Nakajima
Masashi
M
Department of Neurology, Fukuoka University, Fukuoka, Japan. masashi@tokyoh.rofuku.go.jp
Yamada
Tatsuo
T
Kusuhara
Tomohiko
T
Furukawa
Hisako
H
Takahashi
Mitsuo
M
Yamauchi
Atsushi
A
Kataoka
Yasufumi
Y
D000962:Antimalarials / Q000097:blood / Q000627:therapeutic use*; D007562:Creutzfeldt-Jakob Syndrome / Q000188:drug therapy* / Q000523:psychology; D004569:Electroencephalography; D005133:Eye Movements; D005260:Female; D019416:Head Movements; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009068:Movement; D009207:Myoclonus / Q000188:drug therapy / Q000209:etiology; D011796:Quinacrine / Q000097:blood / Q000627:therapeutic use*
D000962:Antimalarials; D011796:Quinacrine