Long-term Follow-up and Histological Correlation of Peripheral Nervous System Alterations in Neurofibromatosis Type 2.
Clin Neuroradiol
; 32(1): 277-285, 2022 Mar.
Article
em En
| MEDLINE
| ID: mdl-34652463
ABSTRACT
PURPOSE:
To examine long-term alterations of the dorsal root ganglia (DRG) and the peripheral nerve in patients with neurofibromatosis type 2 (NF2) by in vivo high-resolution magnetic resonance neurography (MRN) and their correlation to histology.METHODS:
In this prospective study the lumbosacral DRG, the right sciatic, tibial, and peroneal nerves were examined in 6 patients diagnosed with NF2 and associated polyneuropathy (PNP) by a standardized MRN protocol at 3â¯T. Volumes of DRG L3-S2 as well as peripheral nerve lesions were assessed and compared to follow-up examinations after 14-100 months. In one patient, imaging findings were further correlated to histology.RESULTS:
Follow-up MRN examination showed a non-significant increase of volume for the DRG L3 +0.41% (pâ¯= 0.10), L4 +22.41% (pâ¯= 0.23), L5 +3.38% (pâ¯= 0.09), S1 +10.63% (pâ¯= 0.05) and S2 +1.17% (pâ¯= 0.57). Likewise, peripheral nerve lesions were not significantly increased regarding size (2.18â¯mm2 vs. 2.15â¯mm2, pâ¯= 0.89) and number (9.00 vs. 9.33, pâ¯= 0.36). Histological analyses identified schwannomas as the major correlate of both DRG hyperplasia and peripheral nerve lesions. For peripheral nerve microlesions additionally clusters of onion-bulb formations were identified.CONCLUSION:
Peripheral nervous system alterations seem to be constant or show only a minor increase in adult NF2. Thus, symptoms of PNP may not primarily attributed to the initial schwannoma growth but to secondary long-term processes, with symptoms only occurring if a certain threshold is exceeded. Histology identified grouped areas of Schwann cell proliferations as the correlate of DRG hyperplasia, while for peripheral nerve lesions different patterns could be found.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neurofibromatose 2
Tipo de estudo:
Guideline
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Humans
Idioma:
En
Revista:
Clin Neuroradiol
Assunto da revista:
NEUROLOGIA
/
RADIOLOGIA
Ano de publicação:
2022
Tipo de documento:
Article
País de afiliação:
Alemanha