This will be an explorative study to examine the value of high-resolution ultrasound (HRUS) of the peripheral nerves in NF1 patients. The primary objective of this study is to determine HRUS abnormalities in a large group of NF1 patients, and to…
ID
Source
Brief title
Condition
- Neurological disorders congenital
- Nervous system neoplasms benign
- Nervous system neoplasms benign
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
The main study parameters are the number of HRUS abnormalities and their
characteristics (cross-sectional area, presence of vascularization) and the
presence or absence of neurological deficits.
Secondary outcome
Secondary study parameters are:
- the number of newly detected nerve abnormalities with HRUS after two years of
follow-up;
- the increase in CSA and/or vascularization of nerve abnormalities detected
with HRUS after two years of follow-up.
Background summary
Individuals with neurofibromatosis type 1 (NF1) are prone to develop benign and
malignant tumours of the central and peripheral nervous system. Plexiform
neurofibromas arise from multiple nerve fascicles, can grow along the length of
a nerve and can also extend into surrounding structures, causing pain and
neurological deficits. The relation between morphologic changes of the nerve
and pain or neurological deficits is not known. It is not yet possible to
predict pain or neurological deficits based upon (early) morphological changes.
In areas of an existing plexiform neurofibroma, there is a high risk of
developing a malignant peripheral nerve sheath tumour, a tumour with a poor
outcome. No quick and cost-effective technique exists so far for examining the
peripheral nerves of NF1 patients and screening these patients for peripheral
nerve tumours.
Study objective
This will be an explorative study to examine the value of high-resolution
ultrasound (HRUS) of the peripheral nerves in NF1 patients. The primary
objective of this study is to determine HRUS abnormalities in a large group of
NF1 patients, and to explore the differences in HRUS abnormalities between
patients with or without deficits. A secondary objective is to evaluate the
stability and growth of plexiform neurofibromas and the incidence of newly
detected nerve abnormalities during two years of follow-up.
Study design
Cross-sectional (observational) study and longitudinal study.
Study burden and risks
Participation in the study means patients will undergo a standardized
neurological examination, nerve conduction studies (NCS) and high-resolution
ultrasound (HRUS) twice: as soon as possible after inclusion and two years
later. In the case of neurological deficits, electromyography will also be
performed directly after NCS. For patients this will mean two extra visits to
the outpatient clinic, in addition to the regular visits to their neurologist.
The neurological examination will be performed during regular visits. NCS and
HRUS will be combined in one extra visit at inclusion and one after two years.
If patients want to be informed of the results they will be contacted by phone.
Clinical examination and HRUS are painless, NCS and electromyography might
cause some physical discomfort. Only electromyography may cause mild adverse
events (hematoma, haemorrhage, infection).
Dr. Molewaterplein 40
Rotterdam 3015 GD
NL
Dr. Molewaterplein 40
Rotterdam 3015 GD
NL
Listed location countries
Age
Inclusion criteria
- Age >=18 years
- Diagnosed with neurofibromatosis type 1
- Treatment at the outpatient clinic of the Erasmus MC Cancer Institute (Rotterdam)
Exclusion criteria
- comorbidity associated with (poly)neuropathy (e.g. alcoholism, diabetes mellitus)
- comorbidity mimicking neuropathic complaints (e.g. myelopathy)
- inability to give informed consent or to undergo HRUS
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL68748.078.19 |