1naresh
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Patient 1 2 3 4 5 6 7 8 9 Sex Male Male Female Male Male Male Male Female Female Age at diagnosis of LCH 1 y 10 mo 2 y 9 mo 1 y 10 mo 3 y 4 mo 2 y 2 y 8 mo 16 y 10 mo 2 y 10 mo 6 mo Organs involved at diagnosis of LCH Bone, skin, liver, lung, spleen, hematopoietic system Bone, skin Bone, skin, liver Bone, skin, DI Bone, skin Bone, skin, liver, spleen Bone, skin, DI Bone, skin, hematopoietic system, DI Skin, liver Course of LCH (outside the CNS) Chronic active 3 RA 2 RA no RA 4 RA 1 RA 2 RA 2 RA Chronic active Age at diagnosis of ND-LCH 6 y 6 mo 7 y 3 mo 5 y 7 mo 4 y 5 mo 2 y 6 mo 8 y 2 mo 17 y 11 mo 4 y 3 mo 4 y 3 mo Interval diagnosis LCH to the diagnosis of ND 4 y 7 mo 4 y 7 mo 3 y 10 mo 1 y 1 mo 5 mo 5 y 7 mo 1y 1 y 5 mo 3 y 9 mo MR imaging follow-up period 7 y 5 mo 10 y 3 mo 5 y 2 mo 7 y 6 mo 11 y 7 y 3 mo 5 y 11 y 8 mo 6 y 8 mo No. of evaluated MR imaging studies 4 4 3 3 5 4 3 5 3 Age at last MR imaging 13 y 10 mo 17 y 6 mo 10 y 10 mo 11 y 8 mo 13 y 1 mo 15 y 5 mo 22 y 11 mo 15 y 10 mo 10 y 10 mo Additional intracranial abnormalities HPR abnormalities** −/+ +/+ +/+ +/+ −/+ +/+ +/+ +/+ +/+ Space-occupying meningeal lesions** −/− −/+ −/− −/− −/+ −/− −/− +/+ −/− Craniofacial bone lesions** +/+ +/+ +/+ +/− +/+ +/− +/+ +/+ −/− Space-occupying intraparenchymal lesions** −/− −/− −/− −/− −/− −/− +/+ −/− −/− DI* −/+ +/+ −/+ +/+ −/+ +/+ +/+ +/+ +/+ Growth hormone deficiency* −/+ +/+ −/+ −/− −/− −/− +/+ −/+ −/− Neurologic symptoms* −/− −/− −/− Ataxia/severe cerebellar symptoms −/− −/− −/− −/ataxia, spastic diplegia −/−
Note:—ND indicates neurodegeneration; LCH, Langerhans cell histiocytosis; y, year; mo, month; DI, diabetes insipidus; RA, reactivation; HPR, hypothalamic pituitary region; −, absent; +, present.
* At diagnosis/at the last follow-up.
** before or at the diagnosis of ND-LCH/after the diagnosis of ND-LCH.