Serial Proton MRS of Neurometabolic Alterations in Hereditary Spastic Paraplegia with Thin Corpus Callosum (HSP-TCC)
Brockmann K, Helms G, Frahm J, Dreha-Kulaczeski S, Grtner J, Dechent P
HSP-TCC is a complicated form within the group of hereditary spastic paraplegias characterized by a marked thinning of the corpus callosum. We report a follow-up proton MRS study of a patient at age 20 and 25 yrs., in the latter case combined with DWI. Over that period the clinical course progressed. While MRI did not change significantly, MRS disclosed a progressive reduction of tNAA and a significant increase of Ins. The pattern is consistent with progressive neuroaxonal loss accompanied by astrogliosis which is in good agreement with histopathological findings. The ongoing neurometabolic alterations leading to clinical deterioration were best appreciated by MRS.