Table 1:

Key features of 9 patients with radiologic ND-LCH disease

Patient123456789
SexMaleMaleFemaleMaleMaleMaleMaleFemaleFemale
Age at diagnosis of LCH1 y 10 mo2 y 9 mo1 y 10 mo3 y 4 mo2 y2 y 8 mo16 y 10 mo2 y 10 mo6 mo
Organs involved at diagnosis of LCHBone, skin, liver, lung, spleen, hematopoietic systemBone, skinBone, skin, liverBone, skin, DIBone, skinBone, skin, liver, spleenBone, skin, DIBone, skin, hematopoietic system, DISkin, liver
Course of LCH (outside the CNS)Chronic active3 RA2 RAno RA4 RA1 RA2 RA2 RAChronic active
Age at diagnosis of ND-LCH6 y 6 mo7 y 3 mo5 y 7 mo4 y 5 mo2 y 6 mo8 y 2 mo17 y 11 mo4 y 3 mo4 y 3 mo
Interval diagnosis LCH to the diagnosis of ND4 y 7 mo4 y 7 mo3 y 10 mo1 y 1 mo5 mo5 y 7 mo1y1 y 5 mo3 y 9 mo
MR imaging follow-up period7 y 5 mo10 y 3 mo5 y 2 mo7 y 6 mo11 y7 y 3 mo5 y11 y 8 mo6 y 8 mo
No. of evaluated MR imaging studies443354353
Age at last MR imaging13 y 10 mo17 y 6 mo10 y 10 mo11 y 8 mo13 y 1 mo15 y 5 mo22 y 11 mo15 y 10 mo10 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.