immunoglobulin (Bivigam)

Guillain-Barré Syndrome Revealing Coexisting Cervical Spondylotic Myelopathy: A Diagnostic Pitfall.

Boubekri Hatim H, Salah Anass A, Mankar Bennis Najoua N, Khalfaoui Saloua S et al.

Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy characterized by rapidly progressive weakness and areflexia. Although the diagnosis is usually straightforward, atypical clinical evolution should prompt investigation for concomitant central nervous system pathology. We report the case of a 64-year-old man initially diagnosed with severe GBS based on clinical presentation, cerebrospinal fluid (CSF) analysis, and electroneuromyography (EMNG) findings. Despite partial neurological improvement after intravenous immunoglobulin therapy, the patient later developed cervical pain, brisk reflexes, and a positive Babinski sign. Cervical magnetic resonance imaging (MRI) revealed severe multilevel cervical spondylotic myelopathy (CSM) with spinal cord compression and intramedullary T2 hyperintensity. Surgical decompression was subsequently indicated. This case highlights the importance of reassessing patients with GBS who develop pyramidal signs or atypical neurological findings, as concomitant cervical myelopathy may be overlooked and delay appropriate management.

Type 2-interferon imbalance in allergic barrier disease: An asthma-centered, cross-disease perspective.

Rao Shenghong S, Li Shumei S, Shen Haoyue H, Jin Tengchuan T

Allergic diseases are typically regarded as type 2 inflammatory disorders driven by interleukin-4, interleukin-5, and interleukin-13, which mediate immunoglobulin E class switching, eosinophilic inflammation, mucus hypersecretion, pruritus, tissue remodeling, and epithelial barrier dysfunction. However, type 2 cytokine activity alone does not fully account for variations in exacerbation risk, susceptibility to infections, comorbidities, or responses to biologic therapy. This narrative review proposes an asthma-centered type 2-interferon imbalance framework and discusses its cautious, disease-specific extension to atopic dermatitis, chronic rhinosinusitis with nasal polyps, eosinophilic esophagitis, and food allergy. The model emphasizes that, particularly in asthma, allergic barrier inflammation arises and persists in injured tissues where excessive type 2 inflammation may coexist with impaired interferon-mediated host defense. Evidence is strongest in asthma, where deficiencies in type I and type III interferon responses are linked to rhinovirus susceptibility, delayed viral clearance, and recurrent exacerbations. In other allergic diseases, interferon dysfunction appears more variable, reflecting differences in tissue context, disease stage, and environmental exposure. In asthma, and potentially in selected allergic barrier diseases, persistent inflammation may result from a cycle of epithelial injury, alarmin release, cytokine amplification, impaired antiviral defense, ongoing exposure, and incomplete tissue repair. These mechanisms provide a rationale for tiered intervention, including blockade of upstream epithelial alarmins, inhibition of downstream type 2 effector pathways, and selected investigational approaches aimed at restoring mucosal host defense.

Pan-Neurofascin Antibody-Associated Nodopathy: A Critical Guillain-Barré Syndrome Mimic in the Differential Diagnosis-Report of Two Cases.

Nemethova Andrea A, De Ridder Willem W, Baar Ingrid I, Alonso-Jimenez Alicia A

Guillain-Barré syndrome (GBS) is an acute autoimmune polyradiculoneuropathy typically characterized by an ascending sensorimotor deficit with potential involvement of bulbar and respiratory muscles that may necessitate intensive care admission and mechanical ventilation. Standard treatment includes supportive care, management of complications such as weakness, immobility, respiratory failure, autonomic dysfunction and pain, along with early initiation of immunotherapy-either intravenous immunoglobulin (IVIg) or plasma exchange (PE). However, lack of significant clinical improvement following immunotherapy should prompt consideration of alternative diagnoses, including pan-neurofascin antibody-positive autoimmune nodopathy (panNF + AN). In this report, we present two patients presenting with a severe GBS-like neuropathy. Initial treatment with IVIg resulted in either no response or only transient, mild improvement, followed by rapid clinical deterioration to near-complete tetraplegia, respiratory failure, and autonomic and cranial nerve involvement. Both patients were unresponsive to further treatment with a second course of IVIg, PE and corticosteroids. Subsequent diagnostic evaluation revealed the presence of pan-neurofascin antibodies, confirming panNF + AN. This prompted initiation of treatment with rituximab, which resulted in sustained and complete clinical recovery in both cases. A transient or mild clinical response-or an initial lack of response-to standard GBS-treatment followed by rapid and severe deterioration in cases initially presenting as GBS should be considered a red flag warranting evaluation for AN-associated antibodies. Recognition of this important GBS mimic is critical, as it requires an alternative treatment approach with rituximab, which has been associated excellent clinical outcomes.

Clinical table of immunoglobulin A deficiency.

Özdemir Öner Ö