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Introduction: Myasthenia gravis (MG) is an antibody mediated disease where pathogenic antibodies interact with the acetylcholine receptor or other proteins at the post-synaptic neuromuscular junction. There is growing evidence that immunoglobulin infusions are beneficial for clinical exacerbations and chronic refractory disease and may be an option for patients unresponsive to conventional immunosuppressive therapies., Areas Covered: We performed an extensive literature review, looking for evidence on the use of immunoglobulins for the treatment of MG, by conducting a search in MEDLINE (1946 to present), EMBASE (1947 to present) and Clinicaltrials.gov. We have included studies on the use of intravenous immunoglobulins (IVIG) and subcutaneous immunoglobulins (SCIG) for acute deterioration and chronic disease., Expert Opinion: The use of IVIG in MG provides an option for rapid improvement in critical deterioration, being preferred over more invasive and less available therapies such as plasmapheresis. For refractory MG, the addition of IVIG can improve a patient's status and reduce the dosage of immunosuppressive medications. The alternative of SCIG is also effective and has advantages of infusion time flexibility, fewer side-effects, and patient independence. The safety and efficacy of both interventions, patient preferences and quality of life may direct therapeutic choices in the future.

Objective: To investigate the contribution of duration and temporal dispersion (TD) of the distal compound muscle action potential (CMAP) in discriminating chronic inflammatory demyelinating polyneuropathy (CIDP) from diabetic sensorimotor polyneuropathy (DSP) and from CIDP+DSP., Methods: We performed a retrospective review of patients diagnosed with CIDP, DSP and CIDP+DSP (responsive to immunotherapy) and examined differences in CMAP duration and TD at baseline., Results: We included 59 subjects: 17 CIDP, 21 DSP and 21 CIDP+DSP. Of these, 16 (94.1%) CIDP, 18 (85.7%) CIDP+DSP and 1 (4.7%) DSP fulfilled the 2010 EFNS/PNS criteria for definite CIDP. There was no difference in CMAP duration or TD in all nerves (compound outcome) or in individual motor nerves. Patients with CIDP/CIDP+DSP had more conduction blocks, slower conduction velocities and more prolonged F wave latencies than those with DSP., Conclusion: Measures of CMAP duration and TD were not helpful in distinguishing CIDP, DSP or CIDP+DSP patients; however, parameters such as F-wave latencies, conduction blocks or the number of demyelinating parameters were useful in this separation., Significance: There are no definite nerve conduction criteria to distinguish patients with CIDP+DSP from DSP alone. Further studies focusing on measures of demyelination may provide stronger evidence to guide treatment decisions in CIDP + DSP patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Alcantara, Ngo, de la Cruz, Menon, Barnett-Tapia, Katzberg and Bril.)

Background: Myasthenia gravis (MG) is an autoimmune disorder that frequently affects young women of reproductive age. The multidirectional interplay between MG, pregnancy, and fetal health poses a complex scenario for pregnant women with MG and the healthcare team. Here, we reviewed our local experience with MG, pregnancy, and outcomes., Methods: We performed a retrospective chart review of patients with MG attending the Prosserman Family Neuromuscular Clinic from 2001 to 2019 and who were referred to a high-risk pregnancy clinic. MG status was defined as stable, better, or worse. Information was collected on the delivery route, pregnancy, and neonatal complications., Results: We identified 20 women with MG for a total of 28 pregnancies. Worsening was observed in 50% of pregnancies: 18% during pregnancy, 25% following delivery, and 7% during both. 66.7% of patients with MG duration of 2 years or less had worsening during pregnancy. Three patients who stopped immunosuppressive treatment during pregnancy worsened and one had a crisis. C-section was done in 29% of pregnancies. The rate of delivery complications was 7% and of neonatal MG was 7%., Conclusion: A high proportion of MG patients worsened during pregnancy, particularly those with disease duration less than 2 years, and those who discontinued immunosuppression during pregnancy. However, pregnancy was largely unaffected, rate of neonatal MG was low, frequencies of C-section, delivery complications, and premature births were similar to the general population. While the study has limitations due to the retrospective nature, these insights provide some guidance when counseling young myasthenic women about family planning.

Importance: Thrombosis with thrombocytopenia syndrome (TTS) has been reported after vaccination with the SARS-CoV-2 vaccines ChAdOx1 nCov-19 (Oxford-AstraZeneca) and Ad26.COV2.S (Janssen/Johnson & Johnson)., Objective: To describe the clinical characteristics and outcome of patients with cerebral venous sinus thrombosis (CVST) after SARS-CoV-2 vaccination with and without TTS., Design, Setting, and Participants: This cohort study used data from an international registry of consecutive patients with CVST within 28 days of SARS-CoV-2 vaccination included between March 29 and June 18, 2021, from 81 hospitals in 19 countries. For reference, data from patients with CVST between 2015 and 2018 were derived from an existing international registry. Clinical characteristics and mortality rate were described for adults with (1) CVST in the setting of SARS-CoV-2 vaccine-induced immune thrombotic thrombocytopenia, (2) CVST after SARS-CoV-2 vaccination not fulling criteria for TTS, and (3) CVST unrelated to SARS-CoV-2 vaccination., Exposures: Patients were classified as having TTS if they had new-onset thrombocytopenia without recent exposure to heparin, in accordance with the Brighton Collaboration interim criteria., Main Outcomes and Measures: Clinical characteristics and mortality rate., Results: Of 116 patients with postvaccination CVST, 78 (67.2%) had TTS, of whom 76 had been vaccinated with ChAdOx1 nCov-19; 38 (32.8%) had no indication of TTS. The control group included 207 patients with CVST before the COVID-19 pandemic. A total of 63 of 78 (81%), 30 of 38 (79%), and 145 of 207 (70.0%) patients, respectively, were female, and the mean (SD) age was 45 (14), 55 (20), and 42 (16) years, respectively. Concomitant thromboembolism occurred in 25 of 70 patients (36%) in the TTS group, 2 of 35 (6%) in the no TTS group, and 10 of 206 (4.9%) in the control group, and in-hospital mortality rates were 47% (36 of 76; 95% CI, 37-58), 5% (2 of 37; 95% CI, 1-18), and 3.9% (8 of 207; 95% CI, 2.0-7.4), respectively. The mortality rate was 61% (14 of 23) among patients in the TTS group diagnosed before the condition garnered attention in the scientific community and 42% (22 of 53) among patients diagnosed later., Conclusions and Relevance: In this cohort study of patients with CVST, a distinct clinical profile and high mortality rate was observed in patients meeting criteria for TTS after SARS-CoV-2 vaccination.

Introduction: There is limited evidence regarding the impact of World Health Organization (WHO) subtype of thymoma on post-thymectomy outcome of thymoma-associated myasthenia gravis (TAMG). The objective was to determine if the pathological subtypes of thymoma were associated with post-thymectomy outcomes of myasthenia gravis (MG), in patients with TAMG., Methods: We performed a retrospective study of consecutive patients with TAMG who attended the neuromuscular clinic between January 2018 and December 2019 with a minimum follow-up of 1 y after thymectomy. Outcome measures were MG Impairment Index (MGII), single-simple question (SSQ), Myasthenia Gravis Foundation of America post-intervention status (MGFA PIS) and non-responder MG status at last assessment., Results: Ninety-five patients were included; mean age at onset was 48.1 ± 12.1 y; 54(56.8%) were females. Thirteen patients developed MG post-thymectomy. The most common thymoma was WHO type B2 in 39 (41.1%). Most patients (40, 42.1%) had Masaoka stage II thymoma. There was no association of thymoma subtypes or Masaoka stage of disease with age, gender, MG phenotype, serology, post-thymectomy onset, interval from onset to thymectomy, MGII, SSQ, MGFA PIS, or non-responder status. Associations were found between positive serology and lower MGII (11.1 ± 14.2 vs 23 ± 12.9, P = .050), thymic follicular hyperplasia (TFH) and higher SSQ (89.3 ± 11.7 vs 80.1 ± 20.2, P-.043), and lack of recurrence and higher SSQ (84.1 ± 18 vs 72.5 ± 20, P = .037)., Discussion: The WHO pathological subtype of thymoma did not correlate with MG outcomes. However, positive acetylcholine antibody serology, presence of TFH, and non-recurrence of thymoma predict a favorable outcome., (© 2021 Wiley Periodicals LLC.)

Background: Lower limb muscle cramps are common and painful. They can limit exercise participation, and reduce quality of sleep, and quality of life. Many interventions are available for lower limb cramps; some are controversial or could cause harm, and often, people experience no benefit from the interventions used. This is an update of a Cochrane Review first published in 2012. We updated the review to incorporate new evidence., Objectives: To assess the effects of non-drug, non-invasive therapies for lower limb muscle cramps., Search Methods: In August 2018 and May 2020, we searched the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, Embase, the World Health Organization International Clinical Trials Registry Platform, ClinicalTrials.gov, and reference lists of included studies. We imposed no restrictions by language or publication date., Selection Criteria: We included all randomised controlled trials (RCTs) of non-drug, non-invasive interventions tested over at least four weeks, for lower limb muscle cramps in any group of people, except pregnant women. The primary outcome was cramp frequency. Secondary outcomes were cramp pain severity, cramp duration, health-related quality of life, quality of sleep, participation in activities of daily living, proportion of participants reporting lower limb muscle cramps, and adverse events., Data Collection and Analysis: Two review authors independently selected trials, assessed risk of bias, and cross-checked data extraction and analyses according to standard Cochrane procedures., Main Results: We included three trials, with 201 participants, all 50 years of age and older; none had neurological disease. All trials evaluated a form of stretching for lower limb muscle cramps. A combination of daily calf and hamstring stretching for six weeks may reduce the severity of night-time lower limb muscle cramps (measured on a 10 cm visual analogue scale (VAS) where 0 = no pain and 10 cm = worst pain imaginable) in people aged 55 years and older, compared to no intervention (mean difference (MD) -1.30, 95% confidence interval (CI) -1.74 to -0.86; 1 RCT, 80 participants; low-certainty evidence). The certainty of evidence was very low for cramp frequency (change in number of cramps per night from week zero to week six) comparing the stretching group and the no intervention group (MD -1.2, 95% CI -1.8 to -0.6; 80 participants; very low-certainty evidence). Calf stretching alone for 12 weeks may make little to no difference to the frequency of night-time lower limb muscle cramps in people aged 60 years and older (stretching group median number of cramps in the last four weeks (Md) 4, interquartile range (IQR) 8; N = 48; sham stretching group Md 3, IQR 7.63; N = 46) (U = 973.5, z = -0.995, P = 0.32, r = 0.10; 1 RCT, 94 participants; low-certainty evidence). This trial did not report cramp severity. The evidence is very uncertain about the effects of a combination of daily calf, quadriceps, and hamstring stretching on the frequency and severity of leg cramps in 50- to 60-year-old women with metabolic syndrome (N = 24). It was not possible to fully analyse the frequency data and the scale used to measure cramp severity is not validated. No study reported health-related quality of life, quality of sleep, or participation in activities of daily living. No participant in these three studies reported adverse events. The evidence for adverse events was of moderate certainty as the studies were too small to detect uncommon events. In two of the three studies, outcomes were at risk of recall bias, and tools used to measure outcomes were not validated. Due to limitations in study designs that led to risks of bias, and imprecise findings with wide CIs, we cannot be certain that findings of future studies will be similar to those presented in this review., Authors' Conclusions: A combination of daily calf and hamstring stretching for six weeks may reduce the severity of night-time lower limb muscle cramps in people aged 55 years and older, but the effect on cramp frequency is uncertain. Calf stretching alone compared to sham stretching for 12 weeks may make little or no difference to the frequency of night-time lower limb muscle cramps in people aged 60 years and older. The evidence is very uncertain about the effects of a combination of daily calf, quadriceps, and hamstring stretching on the frequency and severity of leg cramps in 50- to 60-year-old women with metabolic syndrome. Overall, use of unvalidated outcome measures and inconsistent diagnostic criteria make it difficult to compare the studies and apply findings to clinical practice. Given the prevalence and impact of lower limb muscle cramps, there is a pressing need to carefully evaluate many of the commonly recommended and emerging non-drug therapies in well-designed RCTs across all types of lower limb muscle cramps. A specific cramp outcome tool should be developed and validated for use in future research., (Copyright © 2021 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.)

The variants of chronic inflammatory demyelinating polyneuropathy (CIDP) differ not just in their clinical, pathological and electrophysiological characteristics, but often in their indifferent response to conventional immunosuppressive agents which are effective in typical CIDP. High quality evidence is lacking as far as the management of these atypical variants is concerned. In this review, we summarize the treatment approaches to each of these CIDP variants based on existing data. Distal acquired demyelinating symmetric polyneuropathy (DADS) has the phenotype of a symmetric, demyelinating sensory, length-dependent polyneuropathy and is frequently associated with paraproteinemia and anti myelin associated glycoprotein (MAG) antibodies. While the management of idiopathic DADS (DADS-I) is the same as CIDP, DADS-M responds suboptimally and has a favorable response to rituximab. Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) manifests as a chronic progressive demyelinating mononeuropathy multiplex which can evolve to a confluent pattern indistinguishable from CIDP. Evidence favors treating MADSAM with conventional immunomodulatory therapy (IMT), but this disorder responds less favorably than CIDP. Some patients present with purely sensory symptoms, known as pure sensory CIDP or chronic inflammatory sensory polyradiculoneuropathy (CISP), the latter localizing to a pre-ganglionic pathology. Both respond well to first line IMT, particularly to intravenous immunoglobulin (IVIG), but patients relapse without maintenance therapy. Pure motor CIDP resembles multifocal motor neuropathy with conduction block (MMNCB), but the previously reported worsening status after steroid treatment was not reproduced in recent studies, and IVIG remains the first-line therapy. Some focal forms of CIDP defy exact classification, but respond well to first-line IMT including IVIG. Overall, atypical CIDP responds to treatment with first-line IMT, but has a suboptimal response compared to CIDP. There is evidence for effectiveness with agents such as rituximab, especially in DADS-M, and this medication can also be used in cases refractory to conventional IMTs. Rituximab is also effective in CIDP with IgG4 antibodies which has distinct clinical features and is mostly refractory to first-line IMT., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Menon, Katzberg and Bril.)

Objectives: To estimate patient-acceptable symptom state (PASS) cut points for myasthenia gravis (MG) health scales., Methods: We conducted an electronic survey that included the Myasthenia Gravis Impairment Index (MGII), EuroQol 5-Dimension (EQ5D), and a simple PASS question. PASS-anchored thresholds were estimated for the MGII questionnaire through receiver operating characteristic curves. We used the MGII PASS cut point in a validation cohort of 257 patients to estimate PASS thresholds for other clinically relevant health scales such as the Quantitative Myasthenia Gravis Scale (QMGS), Myasthenia Gravis Activities of Daily Living (MG-ADL), Myasthenia Gravis Composite (MGC), and Myasthenia Quality of Life (MG-QoL15)., Results: One hundred twenty-four of ≈250 invited patients answered the electronic survey (49% response rate), and 80 considered their current symptom state acceptable (PASS-positive). They had lower MGII scores than PASS-negative patients (7.76 ± 9.37 vs 25.0 ± 13.7, p < 0.0001) and better EQ5D scores (0.86 ± 0.17 vs 0.69 ± 0.18, p < 0.0001). The MGII questionnaire threshold for PASS was ≤10 points. With the use of this threshold in an independent dataset of 257 patients, all patients in remission or minimal manifestation status were PASS-positive. In addition, some patients in Classes I, II, and IIIA also achieved PASS status. PASS thresholds for the QMGS, MG-ADL, MGC, and MG-QoL15 were ≤7, 2, 3, and 8 points, respectively., Conclusions: We have estimated thresholds for commonly used myasthenia health scales reflecting patient-acceptable states in patients with MG. These thresholds indicate a global state of well being, rather than a change in scores, or being better. Therefore, PASS thresholds can be used as secondary endpoints for myasthenia research., (© 2020 American Academy of Neurology.)

Background: Nerve injury from peripheral nerve block (PNB) is an uncommon but potentially serious complication. We present a retrospective cohort study to evaluate the incidence and etiology of new postoperative neurological symptoms after surgery and regional anesthesia., Methods: We performed a retrospective cohort study of all PNBs performed on elective orthopedic and plastic surgical patients over 6 years (2011-2017). We collected patient and surgical data, results of neurophysiological and imaging tests, neurology and chronic pain consultations, etiology and outcome for patients with prolonged neurological symptoms (lasting ≥10 days)., Results: A total of 26 251 PNBs were performed in 19 219 patients during the study period. Transient postoperative neurological symptoms (<10 days) were reported by 14.4% (95% CI 13.1% to 15.7%) of patients who were reached by telephone follow-up. Prolonged postoperative neurological symptoms (≥10 days) were identified and investigated in 20 cases (1:1000, 95% CI 0.6 to 1.6). Of these 20 cases, three (0.2:1000, 95% CI 0.04 to 0.5) were deemed to be block related, seven related to surgical causes, three due to musculoskeletal causes or pain syndromes, one was suspected of having an inflammatory etiology and six remained of undetermined etiology. Of those who completed follow-up, 56% had full recovery of their symptoms with the remaining having partial recovery., Conclusion: This retrospective review of 19 219 patients receiving PNBs for anesthesia or analgesia suggests that determining the etiology and causative factors of postoperative neurological symptoms is a complex, often challenging process that requires a multidisciplinary approach. We suggest a classification of cases based on the etiology. A most likely cause was identified in 70% of cases. This type of classification system can help broaden the differential diagnosis, help consider non-regional anesthesia and non-surgical causes and may be useful for clinical and research purposes., Competing Interests: Competing interests: AP has a research grant from Fisher and Paykel for an unrelated study. VC has received honoraria from Philips Healthcare., (© American Society of Regional Anesthesia & Pain Medicine 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: Multifocal motor neuropathy (MMN) is a treatable autoimmune polyneuropathy, which may prove challenging diagnostically in the setting of absent conduction blocks or advanced axonal loss. Relatively few studies have examined the role of ultrasound (US) in MMN., Methods: Retrospective, cross-sectional study of patients with MMN who underwent peripheral nerve US. Charts were reviewed to extract clinical, sonographic, and electrophysiological data., Results: Eleven patients with MMN underwent US between 2013 and 2015; of these 11 patients, 7 had ≥3 abnormal nerve segments, and 6 had ≥5 sites of increased cross-sectional area (CSA). There was moderate correlation between the degree of amplitude drop observed in the median and ulnar motor nerves, and CSA. Significant correlation between CSA and limb strength was only observed for the median nerve., Conclusions: Peripheral nerve US shows promise as a diagnostic tool in MMN and may be helpful to distinguish MMN from motor neuron disease.

Multifocal motor neuropathy (MMN), an immune neuromuscular condition causing progressive weakness, usually responds to immune-mediated treatments, including intravenous immunoglobulin (IVIG). Fifteen patients with MMN receiving IVIG were enrolled in an open-label, single-center trial and switched to 20% subcutaneous immunoglobulin (SCIG) using a smooth transition protocol (ie, changing the therapy without interruption or impact on the intended outcome of the therapy). Patients received individualized training and support based on motivation and ability to learn, follow directions, and maintain compliance. Although some patients required assistance during the training phase, most managed self-infusion and reported satisfaction in managing therapy autonomously. Educating patients with neuropathies to self-infuse high-dose SCIG at home and with flexibility in dosing schedules was successfully demonstrated in this patient group.

Purpose: Our aim in the current study was to determine the minimum number of SFEMG potential pairs required to confirm neuromuscular junction impairment and relate this number to disease severity., Methods: Ninety-four patients with myasthenia gravis (MG) attending the neuromuscular clinic from February 2013 to November 2015 were included. The SFEMG sensitivity was determined for each number of recorded pairs up to 20. In addition, we compared clinical and electrophysiologic characteristics between patients with abnormality within the first 3, 4 to 7, and ≥8 recorded pairs to determine whether this number is associated with disease severity., Results: Ninety-eight percent of patients had abnormal SFEMG, within 17 pairs in ocular MG, and within 15 pairs in generalized MG. All patients with generalized MG had at least one abnormal pair in the first five recorded pairs. Patients with three abnormal pairs apparent earlier during the test had more frequent bulbar, respiratory, and limb muscle weakness, and had higher mean jitter values and decrement values., Conclusions: In most cases, an abnormal SFEMG examination can be demonstrated in the first 15 recorded potential pairs in patients with generalized MG, and in the first 17 pairs in patients with ocular MG, thus shortening the test time and decreasing patient discomfort while preserving test sensitivity.

Purpose of Review: This article discusses the clinical features, pathophysiology, and management of toxic and endocrine myopathies., Recent Findings: Early detection and expeditious correction of metabolic disturbances in endocrinopathies such as Cushing syndrome, thyroid and parathyroid diseases, and acromegaly can minimize and prevent neurologic complications including myopathy. Recently proposed mechanisms of injury in patients with critical illness myopathy include inhibition of protein synthesis, mitochondrial dysfunction, disruption of the ubiquitin-proteasome system, oxidative stress, and disruption of intramuscular calcium homeostasis, which can cause a myosin-loss myopathy. Mechanisms underlying toxic myopathies include myosin loss; damage to cellular structures, including myofibrils and organelles such as lysosomes and mitochondria; inflammation; and necrosis. Presentations range anywhere from acute, painful, and necrotic myopathies, as can occur in statin myopathy, to more insidious presentations such as steroid myopathy., Summary: Endocrinopathies known to cause myopathy include thyroid and parathyroid diseases, disorders of the adrenal axis such as Cushing syndrome, and acromegaly. Patients in the intensive care unit are at risk for developing critical illness myopathy, also known as myosin-loss myopathy, which should be considered if intensive care unit acquired weakness develops. The most common toxic agents associated with myopathy include statins and other lipid-lowering medications, corticosteroids, colchicine, amiodarone, hydroxychloroquine, and chloroquine.

Rationale: Critical illness survivors often experience permanent functional disability due to intensive care unit (ICU)-acquired weakness. The mechanisms responsible for long-term weakness persistence versus resolution are unknown., Objectives: To delineate cellular mechanisms underlying long-term weakness persistence in ICU survivors., Methods: We conducted a nested, prospective study of critically ill patients mechanically ventilated for 7 days or longer. The patients were recruited from the RECOVER program and serially assessed over 6 months after ICU discharge. Twenty-seven of 82 patients consented to participate; 15 and 11 patients were assessed at 7 days and 6 months after ICU discharge, respectively., Measurements and Main Results: We assessed motor functional capacity, quadriceps size, strength, and voluntary contractile capacity and performed electromyography, nerve conduction studies, and vastus lateralis biopsies for histologic, cellular, and molecular analyses. Strength and quadriceps cross-sectional areas were decreased 7 days after ICU discharge. Weakness persisted to 6 months and correlated with decreased function. Quadriceps atrophy resolved in 27% patients at 6 months. Muscle mass reconstitution did not correlate with resolution of weakness, owing to persistent impaired voluntary contractile capacity. Compared with Day 7, increased ubiquitin-proteasome system-mediated muscle proteolysis, inflammation, and decreased mitochondrial content all normalized at 6 months. Autophagy markers were normal at 6 months. Patients with sustained atrophy had decreased muscle progenitor (satellite) cell content., Conclusions: Long-term weakness in ICU survivors results from heterogeneous muscle pathophysiology with variable combinations of muscle atrophy and impaired contractile capacity. These findings are not explained by ongoing muscle proteolysis, inflammation, or diminished mitochondrial content. Sustained muscle atrophy is associated with decreased satellite cell content and compromised muscle regrowth, suggesting impaired regenerative capacity.

Muscle cramps are sustained, painful contractions of muscle and are prevalent in patients with and without medical conditions. The objective of this review is to present updates on the mechanism, investigation and treatment of neurogenic muscle cramps. PubMed and Embase databases were queried between January 1980 and July 2014 for English-language human studies. The American Academy of Neurology classification of studies (classes I-IV) was used to assess levels of evidence. Mechanical disruption, ephaptic transmission, disruption of sensory afferents and persistent inward currents have been implicated in the pathogenesis of neurogenic cramps. Investigations are directed toward identifying physiological triggers or medical conditions predisposing to cramps. Although cramps can be self-limiting, disabling or sustained muscle cramps should prompt investigation for underlying medical conditions. Lifestyle modifications, treatment of underlying conditions, stretching, B-complex vitamins, diltiezam, mexiletine, carbamazepine, tetrahydrocannabinoid, leveteracitam and quinine sulfate have shown evidence for treatment.

Purpose of Review: This article discusses the clinical features, pathophysiology, and management of primary and secondary acquired immune axonal neuropathies., Recent Findings: Although there are many collagen vascular disorders associated with vasculitic neuropathy, a quarter of cases have been described to be due to nonsystemic vasculitis of the peripheral nervous system. Enhanced surveillance and aggressive treatment of conditions such as cryoglobulin-related vasculitic neuropathy with cyclophosphamide, rituximab, and alfa interferons has led to improved morbidity and mortality, however, many cases of immune axonal acquired neuropathy are still associated with poor outcomes. Acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN) are well-characterized variants of Guillain-Barré syndrome., Summary: Characterizing the clinical and electrophysiologic phenotype can help diagnose conditions such as nonsystemic vasculitic neuropathy, AMAN, AMSAN, and immune small fiber neuropathy, while careful evaluation of systemic features is key to identifying secondary immune axonal neuropathies such as vasculitic neuropathy related to collagen vascular disease. Additional research is needed to determine the exact immune pathogenesis and optimized treatment regimens for all acquired immune axonal neuropathies.