Fig. 1

Transitions of CSF orexin levels in initial and follow up examinations of NPC patients. 6 cases with cataplexy exhibit low (< 110 pg/ml) or intermediate (110-200 pg/ml) orexin levels, while 4 cases without cataplexy exhibit normal orexin levels. In two cases with cataplexy without miglustat treatment (case 3, 4), orexin levels at the onset were intermediate, and became lower in the later period (174→54 pg/ml, 178→142 pg/ml). Among 5 cases with miglustat treatment, cataplexy of case 6 disappeared and orexin level increased (183→351 pg/ml). Three other cases without cataplexy remained normal orexin levels during miglustat treatment (203→216 pg/ml, 283→270 pg/ml, 408→297 pg/ml). *As for case 6, cataplexy disappeared with the course of miglustat treatment; thus it is indicated as Cataplexy - (orange)