Severe Demyelinating Polyneuropathy and Cranial Neuropathy During Avelumab Treatment of Metastatic Merkel Cell Carcinoma.

INTRODUCTION: Avelumab is a programmed death ligand 1-blocking monoclonal antibody used for the treatment of Merkel cell carcinoma (MCC), urothelial carcinoma, and other solid tumors. It acts as an immune checkpoint inhibitor and prolongs survival of MCC patients. Immune-mediated neurological adverse effects are rare and usually respond well to specific therapy. METHODS AND RESULTS: A case of a 70-year-old man with metastatic MCC is described in this study. The patient developed diplopia after the fourth dose of avelumab, which was then discontinued. Seven months later, therapy was reinitiated and followed by a new adverse neurological event: severe demyelinating polyneuropathy combined with ophthalmoplegia refractory to a plethora of immune suppressive/modulatory treatment regimes. DISCUSSION: This report of severe demyelinating polyneuropathy and cranial neuropathy associated with an anti-programmed death ligand 1 drug refractory to immune suppressive/modulatory treatments sheds a new light to evolving spectrum of immune checkpoint inhibitor immune-related neurological adverse events.

A rare case of parasagittal meningioma causing isolated foot drop: case report and review of the literature.

Foot drop represents a very common reason for a neurologist referral and is often first seen in emergency departments or by a general practitioner. This condition is defined as weakness of ankle dorsiflexion (mainly through tibialis anterior muscle weakness). The most common causes include lower motor neuron lesion, with L4-L5 radiculopathy and peroneal neuropathy being the most frequent ones. Classical diagnostic pathway includes a thorough medical history, detailed neurological examination, radiological studies (MRI of the lumbosacral spine), EMG and nerve conduction studies, and a battery of laboratory tests. The absence of abnormal radiological and neurophysiological findings when searching for the most common causes of foot drop, should raise a red flag and broaden the diagnostic yield for central nervous system pathology (upper motor neuron, UMN) as a possible cause of foot drop. Central causes of isolated foot drop are very rare, with less than 20 cases reported in literature so far, and seven of them being a meningioma. We present a case of a 79-year-old female patient with an isolated foot drop (with no UMN signs on the initial examination) and parasagittal meningioma. Central causes of foot drop should be suspected when foot drop is associated with UMN signs on examination (hyperreflexia of the patellar or ankle jerk and extensor plantar reflex) and when standard diagnostic work-up (MRI of the lumbar spine, EMG and NCS, standard laboratory screening for most common causes of foot drop) is negative or inconclusive. Although very rare, central lesions present a far more serious cause of foot drop and require a more urgent diagnostic work up and a potential neurosurgical referral and treatment. Keeping in mind the possible central causes of foot drop would eliminate unnecessary diagnostic work up and avoid delayed diagnosis and treatment.

Lower number of plasma exchange sessions and glomerular filtration rate decline are associated with second relapses in patients with myasthenia gravis.

The aims were to determine the impact of dysphagia and glomerular filtration rate (GFR) in the prediction of myasthenia relapse and analyse whether different number of plasma exchange sessions could prolong the time before future relapse.This was a retrospective, longitudinal follow-up study with 60 enrolled patients. The patients were followed-up for a total of 50 months.Patients without relapses had significantly higher GFR and higher number of plasma exchange sessions when compared to patients with relapses. Mean time before next myasthenia relapse was significantly longer in patients with GFR ≥ 60 mL/min. Time before next and number of following myasthenia relapses were significantly higher in patients with symptoms of dysphagia.Decline in GFR levels is strongly associated with the presence of dysphagia and independently impacts the onset of myasthenia relapses. Timely initiation of plasmapheresis therapy and adequate hydration of patients with prolonged dysphagia should be one of the treatment goals for clinicians treating this disease.

Factors Contributing to the Reduction of Pain during Electromyography and Nerve Conduction Studies.

BACKGROUND: Electromyography (EMG) and nerve conduction studies (NCS) are an unpleasant and sometimes painful examinations. Pain can reduce patient's compliance and have a negative effect on the examination results. Different studies report that music affects pain perception by acting as a distractor, by inducing positive emotional valence or through the concept of convergence of different sensory modalities. The aim of this study was to explore the effect of music and different environmental and sociodemographic factors on pain perception during EMG and NCS. SUBJECTS AND METHODS: Sixty patients with suspected neuromuscular disease were randomized into music and control group. Specific questionnaire assessed sociodemographic characteristics, medical history, examination waiting time, examination extent and biometeorological forecast. The numerical rating scale was used for the evaluation of pain. The examiner evaluated patient's compliance after the examination. RESULTS: NCS was less painful for patients in the music group (p=0.03), as well as for more cooperative patients (p=0.011). For patients who previously underwent EMG/NCS, present NCS was more painful (p=0.001), regardless of the music intervention (p=0.019). EMG was more painful for older patients (p=0.041). Patients with lower level of education reported lower pain during NCS (p=0.026). Gender, financial satisfaction, biometeorological forecast, diabetes, depression or malignant disease, use and dosing of analgesics or antidepressants, symptoms, examination waiting time and the examination extent had no effect on pain perception. CONCLUSIONS: Music significantly decreased the perception of pain associated with NCS, but not the EMG portion of the examination. During EMG pain level was not significantly reduced, but the median of pain was still lower. Generally, the pain level during NCS, unlike the one during EMG, was affected by patients' compliance, level of education and painful predetermination. We propose using music during EMG/NCS because it can make the examination more comfortable for the patient and thus contribute to better quality of this examination.