Table 2:

Course of radiologic ND-LCH in the cerebellum, anterior pons, and basal ganglia in 9 patients

PatientFollow-Up
At Diagnosis3 years6 years9 years12 years
1
    Cerebellum++++++++
    Basal ganglia−+++++
    Anterior pons−−−−
2
    Cerebellum++++++++
    Basal ganglia++++++
    Anterior pons−−−−
3
    Cerebellum+++
    Basal ganglia++++
    Anterior pons−−−
4
    Cerebellum+++*+++*+++*
    Basal ganglia+*++*++*
    Anterior pons+*+*+*
5
    Cerebellum++++++++++++
    Basal ganglia−++++
    Anterior pons−−+++
6
    Cerebellum++++++++++++
    Basal ganglia++++++++++
    Anterior pons−−−−
7
    Cerebellum+++
    Basal ganglia−++
    Anterior pons−−−
8
    Cerebellum+++++++*+++*+++*
    Basal ganglia−+++*++*++*
    Anterior pons−+++*++*++*
9
    Cerebellum+++++++
    Basal ganglia−−++
    Anterior pons−−−

  • Note:—ND indicates neurodegeneration; LCH, Langerhans cell histiocytosis; Cerebellum: +, mild signal alterations limited to the dentate nucleus; ++, moderate signal alterations in dentate nucleus; +++, extensive signal alterations in the dentate nucleus, cerebellar white matter, middle cerebellar peduncles, and dorsal part of the pons. Anterior pons: −, normal; +, subtle signal alterations; ++, prominent signal alterations. Basal ganglia: −, normal; +, mild signal alterations limited to the pallidum; ++, moderate signal alterations limited to the pallidum; +++, extensive alterations involving the pallidum and the putamen.

  • * Radiologic ND-LCH associated with neurologic symptoms.