Electrophysiological patterns in patient with Guillain-Barre syndrome

Background: Guillain-Barre syndrome (GBS) is an acute, frequently severe and fulminant polyradiculoneuropathy that is autoimmune in nature. Incidence and predominant subtypes of GBS differ geographically. Electrophysiology has important role in subtyping GBS. This study aimed to evaluate the electrophysiological findings in patient of GBS. Methods: This was a hospital based cross-sectional descriptive study and conducted at the Department of Neurology in Sir Salimullah Medical College & Mitford Hospital, Dhaka and National Institute of Neurosciences and Hospital, Dhaka during July 2017 to June 2018. Clinically diagnosed 53 patients with GBS were enrolled according to prefixed selection criteria. Detail history taking, clinical examination, nerve conduction study and cerebrospinal fluid (CSF) examination was performed in all cases. Clinical findings, nerve conduction study (NCS) parameters, CSF findings and demographic profiles were evaluated. Results: Mean ± SD age of presentation was 41.64 (±14.56) years and median age was 42.0 years. There were total 33(62 %) males and 20 (38 %) females with male: female ratio of 1.7:1. Clinically two-thirds(62.3%) of patients had both upper and lower limb involvement (62.3%), facial weakness was in 32.1% and 13.2% had bulbar involvement. Acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN)were found to be 51%, 32% and 17% respectively. CSFprotein was elevated in most of the patients with a range of 16-725 mg/dl. Highest CSF protein was found in AIDP. Conclusion: Electrophysiological studies play an important role in the early detection; characterization of GBS.In this study, the commonest type of GBS was AIDP. Higher levels of CSF protein, absent H-reflex and Fresponse, sural sparing and unexcitable nerves are more frequently present in AIDP. BIRDEM Med J 2022; 12(1): 16-21

Pearls & Oy-sters: Delayed Diagnosis of Acute Motor Axonal Neuropathy With Cardiac Arrest

We present the case of a 53-year-old female who presented with right lower extremity weakness with preceding systemic symptoms including fever and chest pain. She developed rapid quadriparesis over 24 hours and had ventricular fibrillation with cardiac arrest. Examination demonstrated tetraplegia, facial diplegia with spared extra-ocular movements and areflexia. Electrodiagnostic studies including nerve conduction studies and electromyography were consistent with Acute Motor Axonal Neuropathy (AMAN). This case highlights an atypical asymmetric presentation with initially preserved reflexes, rapid progression and cardiac dysfunction that can occur independent of dysautonomia. Treatment options include intravenous immunoglobulin (IVIg) or plasmapheresis as well as supportive care and long term multidisciplinary rehabilitation and communication strategies.

COVID-19 Neuromuscular Involvement in Post-Acute Rehabilitation

Background: Coronavirus disease 2019 (COVID-19) is associated with muscle and nerve injuries as a consequence of prolonged critical illness or the infection itself. In this study, we evaluated neuromuscular involvement in patients who underwent post-acute intensive rehabilitation after COVID-19. Methods: Clinical and neurophysiological evaluations, including nerve conduction studies and electromyography, were performed on 21 consecutive patients admitted for rehabilitation after COVID-19. Results: Clinical signs suggesting muscle or nerve involvement (weakness, reduced deep tendon reflexes, impaired sensitivity, abnormal gait) were found in 19 patients. Neurophysiological examinations confirmed neuromuscular involvement in 17 patients: a likely association of critical illness myopathy (CIM) and critical illness polyneuropathy (CIP) was found in 5 patients; CIM alone was found in 4 patients; axonal sensory-motor polyneuropathy was found in 4 patients (CIP in 2 patients, metabolic polyneuropathy in 2 patients); Guillain-Barré syndrome was found in 2 patients (classical demyelinating sensory-motor polyneuropathy and acute motor axonal neuropathy, respectively); peroneal nerve injury was found in 1 patient; and pre-existing L4 radiculopathy was found in 1 patient. Conclusions: Neuromuscular involvement is a very common finding among patients admitted for rehabilitation after COVID-19, and proper investigation should be conducted when muscle or nerve injury is suspected for adequate rehabilitative strategy planning.

Protective Effect of Hyperbaric Oxygen Treatment on Axon Degeneration after Acute Motor Axonal Neuropathy

Objectives. Acute motor axonal neuropathy (AMAN) is a disease that leads to acute flaccid paralysis and may result from the binding of antibody and antigen to the spinal cord. The objective of this study is to evaluate the protective effect of hyperbaric oxygen treatment (HBOT) on axon degeneration of the spinal cord and sciatic nerve of the AMAN model rabbit. Axonal degeneration was assessed by evaluating glutathione (GSH) activity, interleukin-1β (IL-1β) expression, and clinical and histopathological features. Methods. Twenty-one New Zealand rabbits were divided into three groups. The treatment group was exposed to 100% oxygen at 2.4 ATA 90 minutes for 10 days at a decompression rate of 2.9 pounds per square inch/minute. GSH level was evaluated using an enzyme-linked immune-sorbent assay. An expression of IL-1β in the spinal cord was determined by immunohistochemistry. Clinical appearances were done by motor scale and body weight. Histological features observed neuronal swelling and inflammatory infiltration in the sagittal lumbar region and the undulation of the longitudinal sciatic nerve. Results. Rabbits exposed to HBO had high GSH activity levels ( p < 0.05 ) but unexpectedly had high IL1β expression ( p > 0.05 ). In addition, the HBO-exposed rabbits had a better degree of undulation, the size of neuronal swelling was smaller, the number of macrophages was higher, and motor function was better than the AMAN model rabbits ( p < 0.05 ). Conclusions. These findings indicate that HBO therapy can decrease axon degeneration by triggering GSH activity, increasing IL-1β level, and restoring tissues and motor status. In conclusion, HBO has a protective effect on axon degeneration of the spinal cord and sciatic nerve of the AMAN model rabbit.

NCMP-21. IMMUNE-RELATED ACUTE MOTOR AXONAL NEUROPATHY: A SMALL CASE SERIES AND REVIEW OF THE LITERATURE

BACKGROUND Immunotherapy revolutionized cancer treatment in the past decade, with a significant increased survival in patients with solid tumors. However, immune checkpoint inhibitors (ICIs) have been associated with a growing number of neurotoxicities, some of which can be fatal if not recognized and treated promptly. Some of these neurotoxicities include very uncommon syndromes like Acute Motor Axonal Neuropathy (AMAN). Herein we present four oncological cases of patients who underwent immunotherapy and developed AMAN. METHODS Four patients were diagnosed with immune-related AMAN between 2017 and 2000 at H. Lee Moffitt Cancer Center. Patients were treated with standard of care. RESULTS We describe four oncological patients who developed a motor axonal neuropathy (i.e., AMAN) confirmed on nerve conduction studies following 2 cycles of immunotherapy, including a 28 year old woman with melanoma brain metastasis and a 50 year old woman with renal cell carcinoma both treated with ipilimumab and nivolumab, a 32 year old man with Hodgkin lymphoma who was treated with nivolumab and brentuximab, and a 77 year old woman with renal urothelial cancer who was treated with pembrolizumab and cabozantinib. All four patients were promptly recognized as having immune-related neurotoxicity (irNs), were promptly treated (i.e., high dose steroids +/- IVIG +/- other immunomodulators), and significantly improved and have remained stable. CONCLUSION This is the first case series of patients with AMAN following two cycles of immunotherapy, who were successfully treated. It is crucial to develop a better understanding of irNs, including those rare conditions that are difficult to diagnose and treat, as the utilization of these immunomodulating therapies continues to expand to include other solid malignancies. Neurologists should be involved early on in any case of suspected irN to assist in the management of these complicated patients and a swift work up should be initiated for timely diagnosis and treatment.