Neuromyotonia is continuous peripheral nerve hyper-excitability manifesting in muscle twitching at rest (myokymia), inducible cramps and impaired muscle relaxation, and characterized by EMG findings of spontaneous single motor unit discharges (with doublet, triplet, or multiplet morphology). The disorder may be genetic, acquired, and often in the acquired cases autoimmune. This chapter focuses on autoimmune acquired causes. Autoimmune associations include mainly contactin-associated protein-like 2 (CASPR2) antibody-associated disease (previously termed as VGKC or voltage-gated potassium channel antibody-associated neuromyotonia) (van Sonderen et al., 2016, p. 2), leucine-rich glioma-inactivated 1 (LGI1) antibody disease, the Guillain-Barré syndrome, NMDAR encephalitis (Varley et al., 2019), and IgLON5 (Gaig et al., 2021) disease. Nonimmune associations include radiation-induced plexopathy. An association with myasthenia gravis and other autoimmune disorders, response to plasma exchange (Newsom-Davis and Mills, 1993) and physiologically induced changes in mice injected with patient-derived immunoglobulins led to the discovery of autoantibodies to juxtaparanodal proteins complexed with potassium channels (Shillito et al., 1995). The target of the antibodies is most commonly the CASPR2 protein. The disorder may be paraneoplastic, and a search for and treatment of an underlying tumor is a necessary step. In cases in which there is evidence for an immune cause, then immune suppression, with an emerging role for B cell-depleting therapies, is associated with a good clinical outcome. In parallel, sodium channel blocking drugs remain effective symptomatic therapies.

Case reports of plexopathy after prostate cancer are usually neoplastic. Radiation-induced lumbosacral plexopathy and insufficiency fractures have clinical significance due to the need to differentiate them from tumoral invasions, metastases, and spinal pathologies. Certain nuances, including clinical presentation and screening methods, help distinguish radiation-induced plexopathy from tumoral plexopathy. This case report highlights the coexistence of these two rare clinical conditions. Herein, we present a 78-year-old male with a history of radiotherapy for prostate cancer who developed right foot drop, severe lower back and right groin pain, difficulty in standing up and walking, and tingling in both legs over the past month during remission. The diagnosis of lumbosacral plexopathy and pelvic insufficiency fracture was made based on magnetic resonance imaging, positron emission tomography, and electroneuromyography. The patient received conservative symptomatic treatment and was discharged with the use of a cane for mobility. Radiation-induced lumbosacral plexopathy following prostate cancer should be kept in mind in patients with neurological disorders of the lower limbs. Pelvic insufficiency fracture should also be considered if the pain does not correspond to the clinical findings of plexopathy. These two pathologies, which can be challenging to diagnose, may require surgical or complex management approaches. However, in this patient, conservative therapies led to an improvement in quality of life and a reduction in the burden of illness.

When a patient with a prior history of malignancy and radiotherapy develops progressive weakness as a presentation of plexus involvement, the differential diagnosis usually rests between radiation-induced plexopathy and invasion from recurrent tumor. The presence of myokymic discharges is helpful in differentiating radiation-induced from neoplastic plexopathy. To present a case report of a patient with chordoma, a locally aggressive tumor, who was diagnosed with recurrent tumor accompanied by the occurrence of myokymia in needle electromyographic examination. A 55-year-old male patient with a history of chordoma and radiotherapy presented to our outpatient clinic with complaints of foot drop, and impaired walking for two months. His latest magnetic resonance imaging (MRI) which was performed three months earlier did not show recurrence. Upon electromyographic evaluation, myokymia, the pathognomic electromyography abnormal wave for radiation plexopathy was detected supporting a diagnosis of radiation plexitis rather than recurrent neoplastic invasion. One month later he presented with more severe pain and was re-evaluated by an MRI, on which a mass was detected indicating relapse. With this case report, we would like to emphasize that the behaviour of the tumor should be considered and imaging should be repeated when tumors display aggressive or recurrent behaviour.

It is difficult to contour nerve structures with the naked eye due to poor differentiation between the nerve structures with other soft tissues on CT images. Magnetic resonance neurography (MRN) has the advantage in nerve visualization. The purpose of this study is to identify one MRN sequence to better assist the delineation of the lumbosacral plexus (LSP) nerves to assess the radiation dose to the LSP using the magnetic resonance (MR)/CT deformable coregistration technique. A total of 18 cases of patients with prostate cancer and one volunteer with radiation-induced lumbosacral plexopathy (RILSP) were enrolled. The data of simulation CT images and original treatment plans were collected. Two MRN sequences (Lr_NerveVIEW sequence and Cs_NerveVIEW sequence) were optimized from a published MRN sequence (3D NerveVIEW sequence). The nerve visualization ability of the Lr_NerveVIEW sequence and the Cs_NerveVIEW sequence was evaluated via a four-point nerve visualization score (NVS) scale in the first 10 patients enrolled to determine the better MRN sequence for assisting nerve contouring. Deformable registration was applied to the selected MRN sequence and simulation CT images to get fused MR/CT images, on which the LSP was delineated. The contouring of the LSP did not alter treatment planning. The dosimetric data of the LSP nerve were collected from the dose-volume histogram in the original treatment plans. The data of the maximal dose (Dmax) and the location of the maximal radiation point received by the LSP structures were collected. The Cs_NerveVIEW sequence gained lower NVS scores than the Lr_NerveVIEW sequence (Z=-2.887, p=0.004). The LSP structures were successfully created in 18 patients and one volunteer with MRN (Lr_NerveVIEW)/CT deformable registration techniques, and the LSP structures conformed with the anatomic distribution. In the patient cohort, the percentage of the LSP receiving doses exceeding 50, 55, and 60 Gy was 68% (12/18), 33% (6/18), and 17% (3/18), respectively. For the volunteer with RILSP, the maximum irradiation dose to his LSP nerves was 69 Gy. The Lr_NerveVIEW MRN sequence performed better than the Cs_NerveVIEW sequence in nerve visualization. The dose in the LSP needs to be measured to understand the potential impact on treatment-induced neuropathy.

To investigate the diagnostic performance of diffusion-weighted (DW) MR neurography as an adjunct to conventional MRI for the assessment of brachial plexus pathology. DW MR neurography scans (short tau inversion recovery fat suppression and b-value of 800 s/mm2) of 15 consecutive patients with and 45 randomly selected patients without brachial plexus abnormalities were independently and blindly reviewed by a 5th year radiology resident, a junior neuroradiologist, and a senior neuroradiologist. Median interpretation times ranged between 20 and 30 s. Interobserver agreement was substantial (κ coefficients of 0.715-0.739). For the 5th year radiology resident, sensitivity was 53.3% (95% CI, 30.1-75.2%) and specificity was 100% (95% CI, 92.1-100%). For the junior neuroradiologist, sensitivity was 66.7% (95% CI, 41.7-84.8%) and specificity was 100% (95% CI, 92.1-100%). For the senior neuroradiologist, sensitivity was 73.3% (95% CI, 48.1-89.1%) and specificity was 95.6% (95% CI, 85.2-98.8%). Traumatic injury, metastases, radiation-induced plexopathy, schwannoma, and inflammatory process of unknown cause could be detected by the majority of readers (100% detection rate for each disease entity by at least two readers). Neuralgic amyotrophy, iatrogenic injury after first rib resection, and cervical disc herniation causing root compression were not detected by the majority of readers (0% detection rate for each disease entity by at least two readers). DW MR neurography may be a useful adjunct when assessing for brachial plexus abnormalities, because interpretation time is relatively short and the majority of abnormalities can be detected. • DW MR neurography interpretation time of the brachial plexus is relatively short (median interpretation times of 20 to 30 s). • Interobserver agreement between three readers with different levels of experience is substantial (κ coefficients of 0.715 to 0.739). • DW MR neurography can detect brachial plexus abnormalities with moderate sensitivity (53.3 to 73.3%) and high specificity (95.6 to 100%).