Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystem paraneoplastic disorder. The syndrome presents unique perioperative challenges, including sclerotic bone changes that may preclude conventional neuraxial techniques. The ultrasound-guided sacral erector spinae plane (SESP) block represents a novel interfacial plane block that offers an alternative approach for sacral region analgesia. We describe a 58-year-old man with POEMS syndrome who presented with an acute perianal abscess requiring surgical drainage. The patient exhibited multiple high-risk features: sacrococcygeal sclerosis preventing identification of the sacral hiatus, severe hyperkalemia (6.54 mmol/L), stage 3 chronic kidney disease, pericardial effusion, and previous ischemic stroke. These comorbidities rendered traditional caudal epidural block contraindicated and general anesthesia high risk. Under ultrasound guidance, a single-shot SESP block was performed at the S3-S4 level using 25 mL of 0.2% ropivacaine with 25 µg dexmedetomidine. The block provided complete intraoperative analgesia (visual analog scale [VAS] score of 0) and effective postoperative pain control (VAS score <2 for 24 hours), facilitating successful surgery and discharge on postoperative day 1. This case demonstrates that the SESP block is a feasible and effective alternative for patients with complex conditions such as POEMS syndrome, where neuraxial techniques are anatomically precluded or clinically contraindicated. Based on this experience and supporting literature, the SESP block warrants consideration as a valuable component of multimodal analgesic strategies for anorectal and pelvic floor surgery, particularly in high-risk patients with challenging anatomy or significant comorbidities.

Charcot-Marie-Tooth (CMT) disease is the most common hereditary motor and sensory neuropathy. While diagnosis is typically established through clinical findings, nerve conduction studies (NCS), and genetic testing, imaging manifestations such as nerve root hypertrophy are increasingly recognized. This case report presents a 43-year-old man with lifelong progressive distal weakness who presented with severe lower extremity atrophy, pes cavus deformities, and wheelchair dependence. Neurologic examination revealed marked distal weakness and hyporeflexia, while nerve conduction studies demonstrated severely reduced sural nerve conduction velocity. MRI of the lumbar spine revealed diffuse, symmetric hypertrophy of the non-enhancing lumbosacral nerve roots. Corresponding filling defects were seen on myelography, consistent with hypertrophic demyelinating neuropathy. This case highlights advanced CMT with pronounced nerve root enlargement, a feature that can support diagnosis when genetic testing is unavailable. Recognition of imaging findings helps differentiate CMT from other causes of polyneuropathy.

Guillain-Barré syndrome (GBS) is an immune-mediated acute inflammatory peripheral neuropathy. While it is well-established that its typical manifestations involve the peripheral nerves, it is noteworthy that involvement of the central nervous system and the reproductive system is exceedingly rare. Here we report the case of a 67-year-old man with GBS in whom the [18]F-FDG PET/CT demonstrated systemic inflammatory hypermetabolism. The imaging revealed multifocal, heterogeneous high-activity along the nerve roots, cerebral cortex, hypothalamus, and medulla oblongata. Crucially, it also identified significant involvement of the right testis and the transition zone of the right prostate, suggesting direct inflammatory engagement of the reproductive system. This case underscores the potential of [18]F-FDG PET/CT as a comprehensive imaging tool for assessing the extent of systemic disease and inflammatory activity in complex GBS presentations.

A 10-year-old girl diagnosed with hyper-IgE syndrome for more than six years developed dizziness and symmetrical progressive limb weakness one week prior to hospital admission, occurring 4 months after allogeneic hematopoietic stem cell transplantation. On post-transplant day 121, dizziness and progressive, symmetric limb weakness appeared. Cerebrospinal fluid analysis revealed albuminocytologic dissociation. Electromyography confirmed multiple peripheral nerve lesions, consistent with the diagnosis of Guillain-Barré syndrome. Intravenous immunoglobulin therapy showed limited efficacy, whereas corticosteroid therapy produced significant clinical improvement. Neurological symptoms fully resolved after treatment; however, severe chronic graft-versus-host disease occurred subsequently. Based on this case, related literature was reviewed and summarized to provide references for early diagnosis, mechanistic investigation, and treatment options.

Charcot-Marie-Tooth disease type 2A (CMT2A) is the most common axonal CMT and is associated with an early onset and severe motor neuropathy. CMT2A is mainly caused by dominant mutations in the MFN2 gene, encoding mitofusin-2, a GTPase located in the outer membrane of the mitochondria and endoplasmic reticulum (ER). Mutations in MFN2 affect mitochondrial dynamics. We previously demonstrated that mutated MFN2 further disrupts contacts between the ER and the mitochondria, leading to axonal degeneration. There are no treatments for CMT2A, and those currently under development primarily focus on restoring mitochondrial function. Here, we provide proof of concept that neuronal overexpression of wild-type MFN2 (MFN2[WT]) provides therapeutic benefit in transgenic CMT2A mice as well as in CMT2A-motor neurons derived from induced pluripotent stem cells. Intrathecal delivery of an AAV9 vector expressing MFN2[WT] effectively targets motor and sensory neurons, restoring ER-mitochondria contacts and mitochondrial morphology, thereby preserving both neuromuscular junction integrity and motor function. Strikingly, therapeutic efficacy is also achieved by administering the vector after the onset of symptoms. Importantly, AAV administration was well tolerated, with no evidence of hepatotoxicity or dorsal root ganglion inflammation. We further show that CMT2A pathology can be corrected in vitro and in vivo using an ER-targeting MFN1 isoform that selectively enhances ER-mitochondria contacts. These results establish that restoring contacts between the ER and mitochondria using gene therapy is a promising therapeutic avenue for CMT2A.

This ecological study was based on secondary, population-level data from the Global Burden of Disease 2021 study. We systematically assessed the burden of Guillain-Barré syndrome (GBS) from 1990 to 2021 at global, regional, and national levels, and projected trends through 2041. Temporal trends were evaluated using joinpoint regression to calculate the average annual percentage change. Future trends up to 2041 were projected using autoregressive integrated moving average models. In 2021, the global number of prevalent GBS cases reached 471,850, with an age-standardized prevalence rate of 5.91 per 100,000, a 229% increase since 1990 (average annual percentage change: 3.34). Substantial regional disparities were observed, and frontier analysis indicated an inverse correlation between the sociodemographic index (SDI) and age-standardized rates. The disease burden was higher in males than in females, with peak prevalence occurring among children aged 5 to 9 years. The coronavirus disease 2019 pandemic was temporally associated with a marked increase in GBS burden in 2021, coinciding with a shift in the burden toward low-SDI regions. Projections suggest that the age-standardized prevalence rate will continue to rise through 2041, disproportionately affecting resource-limited areas. The global burden of GBS increased markedly from 1990 to 2021, with a notable rise after 2019 that was temporally associated with the coronavirus disease 2019 pandemic. Addressing this growing burden will warrant targeted interventions, strengthened surveillance, and equitable allocation of resources, particularly in low-SDI populations.

This is the case of a 67-year-old immunocompetent Hispanic male residing in Mexico who presented with acute right lower back and right lateral thigh pain. Initial imaging was unremarkable. He later developed painful vesicular lesions on the right foot and buttock. He was diagnosed with herpes zoster and initiated acyclovir and gabapentin. Ten days later, he developed foot drop, leg weakness, and urinary incontinence. Magnetic resonance imaging (MRI) showed enhancement of the cauda equina, and electrodiagnostic studies demonstrated right lumbar polyradiculopathy. The patient improved significantly with pain medications and physical therapy. After 8 months of physical therapy, he demonstrated significant clinical improvement. This case highlights the uncommon presentation of motor involvement in herpes zoster and emphasizes the role of imaging and electrophysiology in guiding diagnosis and management.

Therapeutic apheresis (TA) is effective in autoimmune neurological disorders and is increasingly used due to intravenous immunoglobulin (IVIg) shortages. Although three modalities exist-membrane-based therapeutic plasma exchange (mTPE), centrifugal therapeutic plasma exchange (cTPE), and double-filtration plasmapheresis (DFPP)-comparative real-world data, especially within the same patients, remain scarce. We conducted a retrospective, intraindividual comparison of mTPE, cTPE, and DFPP in 72 sessions from five clinically stable chronic inflammatory demyelinating polyneuropathy patients. Outcomes included session duration, plasma removal efficiency (PRE), technical aspects, tolerance, clotting, and per-session costs. All sessions used peripheral venous access. cTPE achieved the highest PRE but required longer sessions. DFPP and mTPE enabled faster exchanges, but required more nursing input. Clotting occurred only with filtration modalities, mostly linked to intravenous calcium under citrate. All approaches were well tolerated, with no bleeding or relapses. DFPP was feasible with saline-only replacement, without causing hypotension. Session costs were lowest with cTPE, although the cost per liter was similar to that of DFPP. This is the first real-world study comparing all three modalities within the same patients. Each has distinct advantages and limitations. DFPP emerges as an efficient, cost-saving alternative, especially in settings with limited resources. These results support a tailored approach to TA, particularly as plasma exchange gains importance amid IVIg shortages.