Arnau Llauradó

Objective: Immune‐related polyradiculoneuropathy (irPRN) is a rare but potentially severe neurological adverse event secondary to immune checkpoint inhibitors (ICIs), closely resembling Guillain–Barré syndrome. This study aims to characterize the clinical presentation, neurophysiological findings, therapeutic strategies, and clinical outcomes of patients diagnosed with irPRN. Methods: We conducted a retrospective single‐center study including patients diagnosed with irPRN following ICI therapy between May 2018 and July 2024. Clinical, electrophysiological, and cerebrospinal fluid (CSF) data were analyzed. Results: Nine patients (mean age 67.9 years; 66.7% male) developed immune‐related polyradiculoneuropathy (irPRN), with a median onset of 6 weeks following initiation of immune checkpoint inhibitors (ICIs). Most cases (66.7%) occurred in patients receiving anti‐PD‐1 monotherapy. The clinical presentation was characterized by symmetric limb weakness (100%) and sensory disturbances (88.9%). Electrodiagnostic studies demonstrated a demyelinating polyradiculoneuropathy in 8 of 9 patients; only one exhibited an axonal pattern. CSF analysis revealed elevated protein levels in all tested patients, with mild pleocytosis in 62.5%. At nadir, the modified Rankin Scale (mRS) ranged from 3 to 5. Immunomodulatory therapy was administered in 8 of 9 patients (88.9%), leading to notable functional improvement (mRS 1–2) in five cases. ICI rechallenge was attempted in two patients, neither of whom experienced recurrence. No deaths were directly attributed to irPRN. Conclusion: IrPRN is a rare but distinct complication of ICI therapy, typically presenting with a demyelinating neurophysiological pattern and favorable response to immunotherapy. Early recognition and treatment are critical to improving functional outcomes. Rechallenge may be feasible in selected cases without recurrence.

Aim: This study aimed to describe neurological manifestations secondary to hepatitis E virus (HEV) through the description of two clinical cases.Methods: Two different cases of neuralgic amyotrophy and meningoradiculitis are evaluated in the emergency department of a tertiary referral hospital in 2024.Results: Case 1: A 43‐year‐old male presented to the emergency department with proximal weakness and pain in the right upper extremity associated with acute onset of orthopnea. Laboratory tests revealed elevated AST/ALT levels (184/1164 IU/L) and positive HEV IgM and IgG, with detectable serum HEV viral load. Cerebrospinal fluid (CSF) was negative for HEV RNA. A significant decrease in forced vital capacity was observed on transition from the upright to the supine position. Electromyography showed severe bilateral phrenic nerve involvement. The diagnosis of neuralgic amyotrophy with diaphragmatic paralysis secondary to HEV was made. The patient was treated with intravenous immunoglobulins and noninvasive ventilation with partial improvement. Case 2: A 37‐year‐old male presented to the emergency department with paresthesias and weakness, initially affecting the distal upper and lower extremities and progressing proximally. Laboratory tests showed elevated AST/ALT levels (238/626 IU/L), positive HEV IgM and IgG, and a detectable HEV viral load in serum. HEV RNA was also detected in the CSF. Neurophysiological findings were normal. The patient was diagnosed with acute meningoradiculitis secondary to HEV. Treatment with intravenous immunoglobulins led to complete resolution of symptoms.Conclusions: In cases of acute neurological symptoms and liver dysfunction, HEV should be considered as a potential causative agent.

The optimal immunosuppressive treatment for autoimmune chronic intestinal pseudo-obstruction (CIPO) is unknown due to lack of clinical trials. Even less data exist on treatment recommendations for patients who do not respond to first-line immunotherapy. We describe 4 patients with autoimmune CIPO treated with vedolizumab (3/4), a monoclonal antibody that interferes the lymphocyte trafficking to the gastrointestinal tract, or rituximab (1/4) who did not respond to steroids or IV immunoglobulins. We made a systematic review of previously published cases of CIPO treated with these biological agents. Vedolizumab was effective in 2 of 3 patients but failed in a child with nonparaneoplastic anti-Hu-associated CIPO, who had generalized dysautonomia. The 2 patients who responded to vedolizumab had an isolated CIPO, and they did not present neuronal antibodies. Rituximab was prescribed in a case of anti-Hu-associated, nonparaneoplastic CIPO, who showed a complete clinical response after this treatment. Our review of the literature retrieved 4 previous cases of autoimmune CIPO treated with rituximab but none treated with vedolizumab. All patients treated with rituximab had Hu antibodies. Two patients showed a clinical response to the treatment with rituximab. Our findings underscore the potential efficacy of rituximab and vedolizumab in the management of autoimmune CIPO refractory to first-line treatments.

Introduction: We present a rare case of acute immune-mediated polyradiculoneuritis, a Guillain-Barré Syndrome (GBS) variant, manifesting as ophthalmoparesis-ataxia, facial diplegia, and acute bulbar palsy, accompanied by a unique autoimmune profile. Clinical Case: A 75-year-old female developed rapidly progressive symptoms, including bilateral non-reactive mydriasis, ptosis, complete ophthalmoplegia, bilateral facial weakness, tongue immobility, palatal paralysis, limb dysmetria, ataxia, and brisk generalized tendon reflexes, all while maintaining a preserved mental state. Symptoms emerged 10 days after a probable gastrointestinal infection. Severe bulbar dysfunction necessitated orotracheal intubation and a tracheotomy. Extensive cranial nerve involvement initially suggested a brainstem lesion, with oculomotor and acute bulbar palsy as prominent signs. However, brainstem and spinal magnetic resonance imaging along with cerebrospinal fluid analysis yielded negative results. Electromyography reveled a sensorimotor demyelinating polyradiculoneuropathy, and serum testing identified IgG antibodies targeting multiple gangliosides, including the disialosyl group and terminal NeuNAc(α2-3)Gal. Treatment with intravenous immunoglobulin (IVIG) led to gradual clinical improvement. Conclusions: This case highlights a rare and severe GBS phenotype characterized by reactivity to multiple gangliosides. It highlights the role of shared ganglioside epitopes in antibody-mediated neurological damage and expands the clinical spectrum of GBS variants.

Introduction/Aims: Somatosensory evoked potentials (SSEPs) are described as a supportive tool to diagnose chronic inflammatory demyelinating polyradiculoneuropathy (CIDP); however, there is a lack of studies determining the effectiveness of SSEPs in monitoring the clinical course of individuals with this condition. The aims of this study are to evaluate the utility of SSEPs in monitoring patients with CIDP and to assess their association with clinical outcomes following immunomodulatory therapy. Methods: This was a single‐center retrospective observational study that included patients who met European Federation of Neurological Societies and Peripheral Nerve Society criteria for CIDP between 2018 and 2023. SSEPs were performed at diagnosis and during follow‐up after the start of immunomodulatory treatment. Fisher's exact test was employed to assess the association between clinical improvement and SSEP improvement. Results: Eighteen patients were included in the study. Ten patients had a typical CIDP pattern and 11 were male. In 17, SSEPs were abnormal prior to the start of immunomodulatory treatment. In patients who showed clinical improvement with immunomodulatory therapy, we observed that 15/17 had partial or complete improvement in SSEPs. Patients who showed no clinical improvement with first‐line treatment exhibited worsening SSEPs. There was a significant association between clinical and SSEPs improvement ( p = 0.009). Discussion: We observed a positive association between improvement in SSEPs and clinical improvement in patients with CIDP. Our data suggest that SSEPs may be useful for monitoring the clinical course of patients with CIDP, but additional, larger studies are needed.

Background and Aims . Gait impairment is a common manifestation of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). However, clinicians lack an effective monitoring tool, as no gait test has been validated for CIDP. The aim of this study was to determine the usefulness of three tests in monitoring the clinical course of patients with CIDP: Timed Up and Go (TUG), 10‐Meter Walk Test (10MWT), and 30‐Second Chair Stand (30SCS). Methods . This is a prospective, single‐center observational study. We included newly diagnosed CIDP patients starting treatment or relapsed CIDP patients requiring new treatment. We monitored the clinical course using CIDP‐validated clinical scales and correlated changes in clinical status with the results of the gait tests. A ROC curve was developed, and we chose the cut‐off point on each scale with the best specificity and sensitivity to detect change in clinical status. Results . A total of 20 patients have been recruited. The 3 tests show a statistical correlation with objective clinical improvement. In patients who have showed clinical improvement during the follow‐up examination, a mean reduction of 4.8 seconds in TUG and 2.6 in 10MWT and a gain of 3 repetitions in 30SCS have been observed. The optimal cut‐off points for each test were TUG ≤ 1 seconds, 10MWT ≤ 1 seconds, and 30SCS ≥ 1 repetition. The TUG test has the highest sensitivity (82.6%), and the 30SCS test has the highest specificity (100%) for detecting clinical improvement. Conclusions . The study found that the TUG and 30SCS tests could become effective tools for monitoring treatment response in CIDP patients.

We present a case of an 82-year-old female with subacute altered mental status, oculomotor disturbances, and ataxia. On examination, she exhibited bilateral ptosis, complete horizontal ophthalmoplegia and limited vertical eye movements during upgaze associated with prominent truncal ataxia. Cerebral MRI showed a mild hyperintensity on T2 and fluid attenuated inversion recovery sequences in the posterior brainstem extending to the upper cervical cord, without gadolinium enhancement. Clinical and radiological features suggested an encephalomyelitis with prominent brainstem involvement. We summarize the comprehensive differential diagnosis in patients with subacute brainstem encephalitis, that includes infectious, paraneoplastic syndromes and inflammatory disorders. This case highlights the relevance of performing a wide, methodical screening for malignancy in case of negative initial work-up.