Nerve ultrasound shows subclinical peripheral nerve involvement in neurofibromatosis type 2

2017 ◽  
Vol 57 (2) ◽  
pp. 312-316 ◽  
Author(s):  
Johan A. Telleman ◽  
Menno D. Stellingwerff ◽  
Geert J. Brekelmans ◽  
Leo H. Visser
Keyword(s):  
Peripheral Nerve ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Nerve Ultrasound ◽  
Nerve Involvement ◽  
Peripheral Nerve Involvement

scholarly journals Occurrence and characterization of peripheral nerve involvement in neurofibromatosis type 2

Brain ◽  
2002 ◽  
Vol 125 (5) ◽  
pp. 996-1004 ◽  
Author(s):  
A. D. Sperfeld ◽  
C. Hein ◽  
J. M. Schröder ◽  
A. C. Ludolph ◽  
C. O. Hanemann
Keyword(s):  
Peripheral Nerve ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Nerve Involvement ◽  
Peripheral Nerve Involvement

Malignant peripheral nerve sheath tumor: Transformation in a patient with neurofibromatosis type 2

2018 ◽  
Vol 66 (2) ◽  
pp. e27520 ◽  
Author(s):  
Laura Agresta ◽  
Ralph Salloum ◽  
Trent R. Hummel ◽  
Nancy Ratner ◽  
Francesco T. Mangano ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Tumor Transformation

scholarly journals Long-term Follow-up and Histological Correlation of Peripheral Nervous System Alterations in Neurofibromatosis Type 2

2021 ◽  
Author(s):  
Tim Godel ◽  
Philipp Bäumer ◽  
Said Farschtschi ◽  
Klaus Püschel ◽  
Barbara Hofstadler ◽  
...  
Keyword(s):  
Nervous System ◽  
Peripheral Nerve ◽  
Peripheral Nervous System ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Nerve Lesions ◽  
Peripheral Nerve Lesions

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 3 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% (p = 0.10), L4: +22.41% (p = 0.23), L5: +3.38% (p = 0.09), S1: +10.63% (p = 0.05) and S2: +1.17% (p = 0.57). Likewise, peripheral nerve lesions were not significantly increased regarding size (2.18 mm2 vs. 2.15 mm2, p = 0.89) and number (9.00 vs. 9.33, p = 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.

Nerve ultrasound

Neurology ◽  
2017 ◽  
Vol 88 (17) ◽  
pp. 1615-1622 ◽  
Author(s):  
Johan A. Telleman ◽  
Menno D. Stellingwerff ◽  
Geert J. Brekelmans ◽  
Leo H. Visser
Keyword(s):  
Peripheral Nerve ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumor ◽  
Cross Sectional ◽  
Plexiform Neurofibromas ◽  
Nerve Involvement ◽  
Peripheral Nerve Involvement ◽  
Tumor Load ◽  
High Resolution Ultrasonography

Objective:To determine ultrasonographic peripheral nerve involvement in patients with asymptomatic neurofibromatosis type 1 (NF1).Methods:Thirteen asymptomatic and 4 minimally symptomatic patients with NF1 were included in this cross-sectional pilot study to detect asymptomatic abnormalities of the brachial plexus and upper and lower extremity nerves. Patients underwent clinical examination, nerve conduction studies (NCS), and high-resolution ultrasonography (HRUS).Results:HRUS showed abnormalities in 16 patients (94.1%). Neurofibromas were identified in 10 patients (58.8%): localized neurofibromas were found in 3 patients (17.6%), plexiform neurofibromas in 3 (17.6%), and both in 4 (23.5%). In 6 patients (35.3%), only nerve enlargement without an abnormal fascicular pattern was observed. Severe involvement of the peripheral nervous system with multiple plexiform neurofibromas was observed in 7 patients (41.2%), while 4 patients (23.5%) had no or only minor involvement. Both NCS and HRUS were performed on 73 individual nerve segments. In 5.5%, abnormalities were found with both tests; in 50.7%, only with HRUS; and in 1.4%, only with NCS.Conclusions:HRUS frequently showed subclinical involvement of the peripheral nerves in NF1, also when NCS were normal. HRUS findings ranged from normal to widespread peripheral nerve involvement. Because the presence of plexiform neurofibromas and the benign tumor load are risk factors for the development of a malignant peripheral nerve sheath tumor, HRUS may be a useful tool to identify a subgroup of patients who could benefit from regular follow-up.

Peripheral nervous system alterations in infant and adult neurofibromatosis type 2

Neurology ◽  
2019 ◽  
Vol 93 (6) ◽  
pp. e590-e598 ◽  
Author(s):  
Tim Godel ◽  
Philipp Bäumer ◽  
Said Farschtschi ◽  
Isabel Gugel ◽  
Moritz Kronlage ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Dorsal Root Ganglia ◽  
Dorsal Root ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Healthy Controls ◽  
Magnetic Resonance Neurography ◽  
Nerve Lesions ◽  
Peripheral Nerve Lesions

ObjectiveTo 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.MethodsIn 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.ResultsCompared 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).ConclusionsAlterations 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.

Sign in / Sign up

Export Citation Format

Share Document