Tim Godel1, Philipp Bäumer2, Said Farschtschi2, Isabel Gugel2, Moritz Kronlage2, Barbara Hofstadler2, Sabine Heiland2, Mathias Gelderblom2, Martin Bendszus2, Victor-Felix Mautner2. 1. From the Department of Neuroradiology (T.G., P.B., M.K., B.H., S.H., M.B.), Neurological University Clinic, Heidelberg University Hospital; Center for Radiology dia.log (P.B.),Altötting; Department of Neurology (S.F., M.G., V.-F.M.), University Medical Center Hamburg-Eppendorf, Hamburg; and Department of Neurosurgery (I.G.), Tübingen University Hospital, Germany. Tim.godel@med.uni-heidelberg.de. 2. From the Department of Neuroradiology (T.G., P.B., M.K., B.H., S.H., M.B.), Neurological University Clinic, Heidelberg University Hospital; Center for Radiology dia.log (P.B.),Altötting; Department of Neurology (S.F., M.G., V.-F.M.), University Medical Center Hamburg-Eppendorf, Hamburg; and Department of Neurosurgery (I.G.), Tübingen University Hospital, Germany.
Abstract
OBJECTIVE: To examine the involvement of dorsal root ganglia and peripheral nerves in children with neurofibromatosis type 2 compared to healthy controls and symptomatic adults by in vivo high-resolution magnetic resonance neurography. METHODS: In this prospective multicenter study, the lumbosacral dorsal root ganglia and sciatic, tibial, and peroneal nerves were examined in 9 polyneuropathy-negative children diagnosed with neurofibromatosis type 2 by a standardized magnetic resonance neurography protocol at 3T. Volumes of dorsal root ganglia L3 to S2 and peripheral nerve lesions were assessed and compared to those of 29 healthy children. Moreover, dorsal root ganglia volumes and peripheral nerve lesions were compared to those of 14 adults with neurofibromatosis type 2. RESULTS: Compared to healthy controls, dorsal root ganglia hypertrophy was a consistent finding in children with neurofibromatosis type 2 (L3 +255%, L4 +289%, L5 +250%, S1 +257%, and S2 +218%, p < 0.001) with an excellent diagnostic accuracy. Moreover, peripheral nerve lesions occurred with a high frequency in those children compared to healthy controls (18.89 ± 11.11 vs 0.90 ± 1.08, p < 0.001). Children and adults with neurofibromatosis type 2 showed nonsignificant differences in relative dorsal root ganglia hypertrophy rates (p = 0.85) and peripheral nerve lesions (p = 0.28). CONCLUSIONS: Alterations of peripheral nerve segments occur early in the course of neurofibromatosis type 2 and are evident even in children not clinically affected by peripheral polyneuropathy. While those early alterations show similar characteristics compared to adults with neurofibromatosis type 2, the findings of this study suggest that secondary processes might be responsible for the development and severity of associated polyneuropathy.
OBJECTIVE: To examine the involvement of dorsal root ganglia and peripheral nerves in children with neurofibromatosis type 2 compared to healthy controls and symptomatic adults by in vivo high-resolution magnetic resonance neurography. METHODS: In this prospective multicenter study, the lumbosacral dorsal root ganglia and sciatic, tibial, and peroneal nerves were examined in 9 polyneuropathy-negative children diagnosed with neurofibromatosis type 2 by a standardized magnetic resonance neurography protocol at 3T. Volumes of dorsal root ganglia L3 to S2 and peripheral nerve lesions were assessed and compared to those of 29 healthy children. Moreover, dorsal root ganglia volumes and peripheral nerve lesions were compared to those of 14 adults with neurofibromatosis type 2. RESULTS: Compared to healthy controls, dorsal root ganglia hypertrophy was a consistent finding in children with neurofibromatosis type 2 (L3 +255%, L4 +289%, L5 +250%, S1 +257%, and S2 +218%, p < 0.001) with an excellent diagnostic accuracy. Moreover, peripheral nerve lesions occurred with a high frequency in those children compared to healthy controls (18.89 ± 11.11 vs 0.90 ± 1.08, p < 0.001). Children and adults with neurofibromatosis type 2 showed nonsignificant differences in relative dorsal root ganglia hypertrophy rates (p = 0.85) and peripheral nerve lesions (p = 0.28). CONCLUSIONS: Alterations of peripheral nerve segments occur early in the course of neurofibromatosis type 2 and are evident even in children not clinically affected by peripheral polyneuropathy. While those early alterations show similar characteristics compared to adults with neurofibromatosis type 2, the findings of this study suggest that secondary processes might be responsible for the development and severity of associated polyneuropathy.
Authors: Tim Godel; Philipp Bäumer; Said Farschtschi; Barbara Hofstadler; Sabine Heiland; Mathias Gelderblom; Martin Bendszus; Victor-Felix Mautner Journal: Diagnostics (Basel) Date: 2022-03-23
Authors: Moritz Kronlage; Thomas David Fischer; Rouven Behnisch; Daniel Schwarz; Philipp Bäumer; Veronique Schwehr; Sabine Heiland; Martin Bendszus; Tim Godel Journal: Diagnostics (Basel) Date: 2022-06-28