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Severe acute respiratory syndrome coronavirus 2, or SARS-CoV-2, causes acute respiratory disease (coronavirus disease 2019; COVID-19). However, the involvement of other mechanisms is also possible, and neurological complications are being diagnosed more frequently. Here, we would like to present a case of a Polish patient with Guillain–Barré syndrome (GBS), after a documented history of COVID-19: A 50-year-old man, 18 days after the onset of COVID-19 symptoms, had progressive quadriparesis preceded by 1-day sensory disturbances. Based on the clinical picture, the results of diagnostic work-up including a nerve conduction study (ENG) that revealed a demyelinating and axonal sensorimotor polyneuropathy, and cerebrospinal fluid (CSF) analysis that showed albumin–cytological dissociation, an acute inflammatory demyelinating polyneuropathy was confirmed, consistent with GBS. Upon a therapeutic plasma exchange (TPE), the patient’s condition improved. The presented case of GBS in a patient after mild COVID-19 is the first case in Poland that has supplemented those already described in the global literature. Attention should be drawn to the possibility of GBS occurring after SARS-CoV-2 infection, even when it has a mild course.

Guselkumab is an IL-23 inhibitor that binds to the p19 subunit of IL-23 that is highly efficacious and well tolerated for the treatment of moderate-to-severe plaque psoriasis. We report a 20-year-old male who developed sensorimotor axonal polyneuropathy starting treatment with guselkumab, confirmed by neurological examination and serial neurophysiologic studies. His symptoms improved within 5 months of stopping the treatment. The neurophysiologic studies also showed improvement but with continued neuropathy and re-innervation changes on electromyography after about 10 months of stopping treatment. The time line of symptoms and a positive de-challenge are strong but not definitive evidence of guselkumab as a cause.

Nitrous oxide, often used as an anesthetic agent, is also increasingly a drug of abuse due to its euphoric and anxiolytic effects. Frequent exposure to nitrous oxide can lead to neurologic complications, including B12 deficiency and resultant subacute myeloneuropathy, as well as direct neurotoxicity. A clinical presentation of acute sensorimotor polyneuropathy mimicking Guillain-Barré syndrome after chronic nitrous oxide abuse has been reported only rarely. Here we present a 17-year-old previously healthy girl presented with 10 days of progressive ascending sensory loss and weakness in the legs. She admitted to heavy nitrous oxide abuse over a period of a year or more. Laboratory evaluation was significant for normal vitamin B12 level with elevated homocysteine. A magnetic resonance imaging (MRI) of her spine showed abnormal signal involving the bilateral dorsal columns. Nerve conduction studies were suggestive of severe axonal sensorimotor polyneuropathy. This patient demonstrates concurrent multifactorial neurologic injury as a result of nitrous oxide abuse. She had a functional vitamin B12 deficiency as indicated by the elevated homocysteine, leading to a subacute combined degeneration that was evident on the MRI. In addition, she had evidence of direct neurotoxicity leading to axonal injury and sensorimotor polyneuropathy reminiscent of Guillain-Barré syndrome. This clinical picture is a serious but seldom reported possible complication if nitrous oxide abuse and should be considered in patients presenting with a clinical picture suspicious for Guillain-Barré syndrome or its variants.

Clinical and pathologic studies on adults with uremic neuropathy are numerous, but less is known about this disorder in children and adolescents. We report the clinical, electrophysiologic, and pathologic findings in an adolescent female with uremic neuropathy. Electrophysiologic findings were consistent with a primarily axonal sensorimotor polyneuropathy. Sural nerve biopsy revealed areas of focal depletion in myelin sheaths and loss of axons. Axonal degeneration with secondary myelin changes appears to be the characteristic pathology in this case, one of the youngest to our knowledge for which nerve biopsy data are available. Our patient experienced dramatic recovery after renal transplantation, similar to reports of older patients.

Introduction. Hereditary Spastic Paraplegia (HSP) is a rare hereditary disorder that primarily involves progressive spasticity of the legs (hamstrings, quadriceps, and calves).Methods. A 27-year-old gentleman was a fast runner and able to play soccer until age 9 when he developed slowly progressive weakness. He was wheelchair-bound by age 25. He was evaluated by laboratory testing, imaging, electrodiagnostics, and molecular genetics.Results. Electrodiagnostic testing revealed an axonal sensorimotor polyneuropathy. Genetic testing for HSP in 2003 was negative; repeat testing in 2013 revealed a mutation in KIF5A (c.611G>A;p.Arg204Gln).Conclusions. A recent advance in neurogenetics has allowed for more genes and mutations to be identified; over 76 different genetic loci for HSP and 59 gene products are currently known. Even though our patient had a sensorimotor polyneuropathy on electrodiagnostic testing and a 2003 HSP genetic panel that was negative, a repeat HSP genetic panel was performed in 2013 due to the advancement in neurogenetics. This revealed a mutation inKIF5A.

A case of a 22-year-old woman with rare neurologic complications including encephalopathy and acute axonal sensorimotor polyneuropathy in the course of acute pancreatitis is reported. The encephalopathy emerged 3 weeks after the onset of the illness with complete remission being noted 1 week later. The polyneuropathy presented as quadriplegia and respiratory failure that required intubation and partially remitted gradually. There was no pancreatic lesion, no major pancreatic surgery, no sepsis, and no multiple organ failure, all of which had been proposed as the predisposing factors. Severe inflammatory response syndrome (SIRS) that developed during the clinical course of this patient might have induced these neurologic complications.

Introduction Neurological complications leading to morbidity and mortality are not frequent in renal transplant recipients. Here, we report a renal transplant recipient who presented with diminished strength in his limbs probably due to multiple etiologies of axonal sensorimotor polyneuropathy, which resolved with intravenous immunoglobulin. Case presentation A 49-year-old Iranian male renal transplant recipient with previous history of autosomal dominant polycystic kidney disease presented with diminished strength in his limbs one month after surgery. Our patient was on cyclosporine A, mycophenolate mofetil and prednisone. Although a detected hypophosphatemia was corrected with supplemental phosphate, the loss of strength was still slowly progressive and diffuse muscular atrophy was remarkable in his trunk, upper limb and pelvic girdle. Meanwhile, his cranial nerves were intact. Post-transplant diabetes mellitus was diagnosed and insulin therapy was initiated. In addition, as a high serum cyclosporine level was detected, the dose of cyclosporine was reduced. Our patient was also put on intravenous ganciclovir due to positive serum cytomegalovirus immunoglobulin M antibody. Despite the reduction of oral cyclosporine dose along with medical therapy for the cytomegalovirus infection and diabetes mellitus, his muscular weakness and atrophy did not improve. One week after administration of intravenous immunoglobulin, a significant improvement was noted in his muscular weakness. Conclusion A remarkable response to intravenous immunoglobulin is compatible with an immunological basis for the present condition (post-transplant polyneuropathy). In cases of post-transplant polyneuropathy with a high clinical suspicion of immunological origin, administration of intravenous immunoglobulin may be recommended.

We report a patient with common variable immunodeficiency (CVID) who developed an axonal sensorimotor polyneuropathy, a hitherto unreported association to our knowledge. These conditions may be linked at the pathogenetic level, since some CVID patients are prone to the development of autoimmune disease.

Myofibrillar myopathies (MFM) are a group of phenotypically and genetically heterogenous neuromuscular disorders, which are characterized by protein aggregations in muscle fibres and can be associated with respiratory and/or extraskeletal involvement. We screened a large cohort of 38 index patients with MFM for mutations in the nine thus far known causative genes using Sanger and next generation sequencing (NGS). We studied the clinical and histopathological characteristics in 38 index patients and five additional relatives ( n =43) and particularly focused on the associated extraskeletal symptoms. We identified 14 heterozygous mutations (diagnostic yield of 37%), among them the novel p.Pro209Gln mutation in the BAG3 gene, which was associated with a mild phenotype and an axonal sensorimotor polyneuropathy. We detected a polyneuropathy in 28% of the MFM patients, including a BAG3 and a MYOT case, hearing loss in 13%, including one patient with a FLNC mutation and two with mutations in the DES gene, dysphonia with a DES mutation and the first patient with a FLNC mutation presenting respiratory insufficiency as the initial symptom. Moreover, we described for the first time respiratory insufficiency occurring in a patient with the p.Gly154Ser mutation in CRYAB. In four index patients with a mutation in one of the MFM genes, typical histological findings were only identified at the ultrastructural level (29%). We conclude that extraskeletal symptoms frequently occur in MFM, particularly polyneuropathy, deafness, cardiac and respiratory involvement. In case of muscle weakness and extraskeletal symptoms the presence of a MFM should be considered. We identified a genetic defect in one of the known genes in less than half of the MFM patients, indicating that more causative genes are still to be found. Next generation sequencing techniques should be helpful in achieving this aim.

The amyloidoses are heterogeneous disorders characterized by interstitial deposition of proteinaceous fibrils, arranged in cross-beta pleated sheets. Many diverse proteins are capable of undergoing conformational changes and polymerization to produce these fibrils; the fibrils are insoluble, resistant to proteolysis, and often cytotoxic.1 Monoclonal immunoglobulin (Ig) light chains produce the fibrils in primary amyloidosis, which comprises some 75% of amyloidosis cases seen at tertiary referral centers.1 Neuropathy occurs in approximately 15% of primary amyloidosis cases, typically manifesting as an axonal sensorimotor polyneuropathy, carpal tunnel syndrome, or both.1,2⇓ Certain familial amyloidoses, particularly type IV, commonly produce cranial neuropathies,1 but multiple cranial neuropathies are uncommon in primary amyloidosis.1-3⇓⇓ We report a patient in whom primary amyloidosis presented with intractable epistaxis and multiple cranial neuropathies. A 59-year-old right-handed woman had first developed recurrent, unexplained epistaxis about 7 years previously. Over the next 2 years, she additionally experienced persistent …