Simone Thomas, Remi Ben-Davies, Aysel Cetinkaya-Fisgin, Xindan Hu, Xiaoling Li, Sarah Stewart, Baohan Pan, Jeffery L. Twiss, Rajiv R. Ratan, Dianna Willis, Michael Polydefkis, Ahmet Höke
Background and Aims
Recent preclinical studies have shown that nicotinamide adenine dinucleotide (NAD+) plays a critical role in molecular mechanisms of axon degeneration, and reductions in NAD+ levels are associated with axonal degeneration. Nicotinamide riboside (NR) is a safe and widely available pyridine-nucleoside form of vitamin B3 and is an NAD+ precursor.
Methods
To investigate if oral supplementation of synthetic NR can act as a therapeutic agent to prevent degeneration of small somatic sensory axons innervating the skin or promote regeneration of these same fibers in humans, we utilized a validated experimental model of cutaneous nerve degeneration and regeneration and conducted a placebo-controlled, double-blinded Phase 2 study.
Results
NR supplementation did not result in elevation of plasma NAD+ levels but resulted in a small increase in NAD+ in the skin samples. NR supplementation did not prevent capsaicin-induced degeneration of the epidermal sensory nerve fibers, and there was no difference in the amount of epidermal reinnervation at the 90-day visit. Although there was a small but statistically significant increase in the number of epidermal sensory nerve fibers at the 60-day visit, these results are preliminary and will need to be validated in larger studies.
Interpretation
At present oral NR supplementation, at the doses used in this study, cannot be recommended to prevent neuropathy or to improve nerve regeneration.
{"title":"Evaluation of Nicotinamide Riboside in Prevention of Small Nerve Fiber Axon Degeneration and Promotion of Nerve Regeneration","authors":"Simone Thomas, Remi Ben-Davies, Aysel Cetinkaya-Fisgin, Xindan Hu, Xiaoling Li, Sarah Stewart, Baohan Pan, Jeffery L. Twiss, Rajiv R. Ratan, Dianna Willis, Michael Polydefkis, Ahmet Höke","doi":"10.1111/jns.70101","DOIUrl":"10.1111/jns.70101","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Recent preclinical studies have shown that nicotinamide adenine dinucleotide (NAD<sup>+</sup>) plays a critical role in molecular mechanisms of axon degeneration, and reductions in NAD<sup>+</sup> levels are associated with axonal degeneration. Nicotinamide riboside (NR) is a safe and widely available pyridine-nucleoside form of vitamin B3 and is an NAD<sup>+</sup> precursor.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>To investigate if oral supplementation of synthetic NR can act as a therapeutic agent to prevent degeneration of small somatic sensory axons innervating the skin or promote regeneration of these same fibers in humans, we utilized a validated experimental model of cutaneous nerve degeneration and regeneration and conducted a placebo-controlled, double-blinded Phase 2 study.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>NR supplementation did not result in elevation of plasma NAD<sup>+</sup> levels but resulted in a small increase in NAD<sup>+</sup> in the skin samples. NR supplementation did not prevent capsaicin-induced degeneration of the epidermal sensory nerve fibers, and there was no difference in the amount of epidermal reinnervation at the 90-day visit. Although there was a small but statistically significant increase in the number of epidermal sensory nerve fibers at the 60-day visit, these results are preliminary and will need to be validated in larger studies.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>At present oral NR supplementation, at the doses used in this study, cannot be recommended to prevent neuropathy or to improve nerve regeneration.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 1","pages":""},"PeriodicalIF":3.2,"publicationDate":"2026-01-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145916973","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
The modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) has been proposed to be a useful tool for predicting the risk of mechanical ventilation (MV) in Guillain–Barré syndrome (GBS), whereas most of the patients included in previous studies had classical demyelinating GBS. This study validated the utility of the mEGRIS in axonal, as well as demyelinating GBS, defined by electrophysiologic criteria in Japan.
Methods
Data from 214 consecutive patients diagnosed with GBS at our institution within 28 days from disease onset between 1998 and 2023 were reviewed and 200 patients with adequate data were analyzed. Acute motor axonal neuropathy (AMAN) and acute inflammatory demyelinating polyneuropathy (AIDP) were diagnosed by sequential nerve conduction studies and mEGRIS was applied to each group.
Results
A total of 200 GBS patients were classified as AMAN (n = 73 [37%]), AIDP (n = 72 [36%]), or unclassified (n = 55 [28%]) and 27 (14%) patients required MV (18% of AMAN, 15% of AIDP). Patients with MV had a significantly higher mEGRIS than those without MV (17 [median range: 5–29] vs. 6 [0–22]). Approximately 81% of the patients in the moderate- or high-risk group (mEGRIS ≧ 18) required MV. Area under the curves (AUCs) of the mEGRIS prediction formulas was 0.89 (95% CI, 0.82–0.96) for total GBS group and 0.92 (95% CI, 0.85–1.00) for the AMAN group.
Interpretation
The mEGRIS is useful for predicting MV risk in patients with AMAN, as well as AIDP. Close monitoring was required for patients who were classified as moderate or high-risk by mEGRIS, irrespective of GBS subtypes.
{"title":"Utility of the “Modified Erasmus GBS Respiratory Insufficiency Score” in Axonal and Demyelinating Guillain–Barré Syndrome","authors":"Yukari Sekiguchi, Sonoko Misawa, Marie Morooka, Tomoki Suichi, Yuki Shiko, Kohei Takahashi, Satoshi Kuwabara","doi":"10.1111/jns.70096","DOIUrl":"10.1111/jns.70096","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) has been proposed to be a useful tool for predicting the risk of mechanical ventilation (MV) in Guillain–Barré syndrome (GBS), whereas most of the patients included in previous studies had classical demyelinating GBS. This study validated the utility of the mEGRIS in axonal, as well as demyelinating GBS, defined by electrophysiologic criteria in Japan.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Data from 214 consecutive patients diagnosed with GBS at our institution within 28 days from disease onset between 1998 and 2023 were reviewed and 200 patients with adequate data were analyzed. Acute motor axonal neuropathy (AMAN) and acute inflammatory demyelinating polyneuropathy (AIDP) were diagnosed by sequential nerve conduction studies and mEGRIS was applied to each group.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 200 GBS patients were classified as AMAN (<i>n</i> = 73 [37%]), AIDP (<i>n</i> = 72 [36%]), or unclassified (<i>n</i> = 55 [28%]) and 27 (14%) patients required MV (18% of AMAN, 15% of AIDP). Patients with MV had a significantly higher mEGRIS than those without MV (17 [median range: 5–29] vs. 6 [0–22]). Approximately 81% of the patients in the moderate- or high-risk group (mEGRIS ≧ 18) required MV. Area under the curves (AUCs) of the mEGRIS prediction formulas was 0.89 (95% CI, 0.82–0.96) for total GBS group and 0.92 (95% CI, 0.85–1.00) for the AMAN group.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>The mEGRIS is useful for predicting MV risk in patients with AMAN, as well as AIDP. Close monitoring was required for patients who were classified as moderate or high-risk by mEGRIS, irrespective of GBS subtypes.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 1","pages":""},"PeriodicalIF":3.2,"publicationDate":"2026-01-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145906216","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Brad Wright, Samantha R. Eiffert, Abagail Cirincione, Joshua Nardin, James F. Howard Jr, Rebecca E. Traub
Background and Aims
Understanding of population-level outcomes for patients with Guillain–Barré syndrome (GBS) remains limited. We identified which GBS patients are most likely to experience worse outcomes using the largest and most current GBS cohort in the United States.
Methods
This retrospective cohort study used 2005–2020 fee-for-service Medicare claims to identify individuals newly diagnosed with GBS (N = 16 280). We used three person-level modified Poisson regressions to estimate hospital and intensive care unit (ICU) lengths of stay and GBS episode length as a function of sociodemographic characteristics, comorbid conditions, and year of diagnosis. We also documented hospital-acquired pressure ulcers and deep vein thromboses as quality indicators.
Results
Median hospital length of stay was 9 days [IQR: 6–18 days] and median GBS episode length was 26 days [IQR: 10–60 days]. Approximately 43% of patients spent time in the ICU, with most of those stays (57%) lasting ≤ 1 week. On average, stays and episodes were significantly longer for men, those with a disability, and those diagnosed with an impulse control disorder, other nervous system diagnoses, or certain cancers, but significantly shorter for Black individuals and those dually eligible for Medicare and Medicaid. Most patients avoided developing pressure ulcers (96%) or deep vein thromboses (91%).
Interpretation
The GBS disease course varies significantly among Medicare enrollees. We identified some factors associated with worse GBS outcomes and others suggesting structural barriers to care. Our findings can improve care delivery by helping clinicians identify high-risk patients, facilitate early interventions, and reduce morbidity and mortality.
{"title":"Patient Outcomes Among Medicare Beneficiaries With Guillain–Barré Syndrome","authors":"Brad Wright, Samantha R. Eiffert, Abagail Cirincione, Joshua Nardin, James F. Howard Jr, Rebecca E. Traub","doi":"10.1111/jns.70094","DOIUrl":"10.1111/jns.70094","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Understanding of population-level outcomes for patients with Guillain–Barré syndrome (GBS) remains limited. We identified which GBS patients are most likely to experience worse outcomes using the largest and most current GBS cohort in the United States.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This retrospective cohort study used 2005–2020 fee-for-service Medicare claims to identify individuals newly diagnosed with GBS (<i>N</i> = 16 280). We used three person-level modified Poisson regressions to estimate hospital and intensive care unit (ICU) lengths of stay and GBS episode length as a function of sociodemographic characteristics, comorbid conditions, and year of diagnosis. We also documented hospital-acquired pressure ulcers and deep vein thromboses as quality indicators.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Median hospital length of stay was 9 days [IQR: 6–18 days] and median GBS episode length was 26 days [IQR: 10–60 days]. Approximately 43% of patients spent time in the ICU, with most of those stays (57%) lasting ≤ 1 week. On average, stays and episodes were significantly longer for men, those with a disability, and those diagnosed with an impulse control disorder, other nervous system diagnoses, or certain cancers, but significantly shorter for Black individuals and those dually eligible for Medicare and Medicaid. Most patients avoided developing pressure ulcers (96%) or deep vein thromboses (91%).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>The GBS disease course varies significantly among Medicare enrollees. We identified some factors associated with worse GBS outcomes and others suggesting structural barriers to care. Our findings can improve care delivery by helping clinicians identify high-risk patients, facilitate early interventions, and reduce morbidity and mortality.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"31 1","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12748927/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145856773","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Michael P. Skolka, Grace Swart, Shahar Shelly, Thapat Wannarong, Stefan Stålberg, Benjamin Stålberg, Abe Gardner, Jacob Price, Ruple S. Laughlin, Divyanshu Dubey, John R. Mills, Jay Mandrekar, Christopher J. Klein
Background and Aims
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a common, treatable, autoimmune, neuropathy frequently misdiagnosed. The complex electrodiagnostic (EDX) criteria required for diagnosis are challenging to apply in routine practice. This study evaluates the effectiveness of a machine-assisted EDX tool in real-time identification of demyelinating nerve conduction studies (NCS) stipulated by 2021-EAN/PNS CIDP criteria in conjunction with an online CIDP clinical probability calculator.
Methods
We prospectively evaluated 100 consecutive patients with referral or EDX diagnosis of polyradiculoneuropathy beginning January 1, 2024. Fifty patients were included, each from Jacksonville and Rochester Mayo Clinics. Routine electromyography (EMG) reports were compared to a machine-assisted support tool applying 2021-EAN/PNS CIDP EDX criteria to NCS assessing demyelination. The EDX-assistant performance was compared to the final clinical diagnosis and our previously reported online CIDP clinical prediction tool (https://news.mayocliniclabs.com/cidp-calculator/).
Results
Among 100 patients (60% male; median age 64), 30 had a final clinical CIDP diagnosis with 29 having intravenous immunoglobulin follow-up; 27 (93%) improved and 2 (7%) stabilized. Routine EMGs reported demyelination in 14 of 30 (47%), with 46% sensitivity and 79% specificity. The EDX-assistant identified CIDP demyelination in 28 of 30 (93%), with 93% sensitivity and 57% specificity. The clinical calculator detected CIDP in 30 of 30 (100%) with 86% specificity, increasing to 94% after excluding false positives lacking demyelinating features on the EDX tool.
Interpretation
A real-time machine-assisted EDX support tool, used with a clinical web-based calculator, streamlines CIDP assessment by improving standardized identification of 2021-EAN/PNS CIDP demyelinating NCS criteria and aiding clinical decisions.
{"title":"Prospective Evaluation of Machine-Assisted Electrophysiologic and Online Clinical Diagnostic Support Tools in Chronic Inflammatory Demyelinating Polyradiculoneuropathy","authors":"Michael P. Skolka, Grace Swart, Shahar Shelly, Thapat Wannarong, Stefan Stålberg, Benjamin Stålberg, Abe Gardner, Jacob Price, Ruple S. Laughlin, Divyanshu Dubey, John R. Mills, Jay Mandrekar, Christopher J. Klein","doi":"10.1111/jns.70084","DOIUrl":"10.1111/jns.70084","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a common, treatable, autoimmune, neuropathy frequently misdiagnosed. The complex electrodiagnostic (EDX) criteria required for diagnosis are challenging to apply in routine practice. This study evaluates the effectiveness of a machine-assisted EDX tool in real-time identification of demyelinating nerve conduction studies (NCS) stipulated by 2021-EAN/PNS CIDP criteria in conjunction with an online CIDP clinical probability calculator.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We prospectively evaluated 100 consecutive patients with referral or EDX diagnosis of polyradiculoneuropathy beginning January 1, 2024. Fifty patients were included, each from Jacksonville and Rochester Mayo Clinics. Routine electromyography (EMG) reports were compared to a machine-assisted support tool applying 2021-EAN/PNS CIDP EDX criteria to NCS assessing demyelination. The EDX-assistant performance was compared to the final clinical diagnosis and our previously reported online CIDP clinical prediction tool (https://news.mayocliniclabs.com/cidp-calculator/).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among 100 patients (60% male; median age 64), 30 had a final clinical CIDP diagnosis with 29 having intravenous immunoglobulin follow-up; 27 (93%) improved and 2 (7%) stabilized. Routine EMGs reported demyelination in 14 of 30 (47%), with 46% sensitivity and 79% specificity. The EDX-assistant identified CIDP demyelination in 28 of 30 (93%), with 93% sensitivity and 57% specificity. The clinical calculator detected CIDP in 30 of 30 (100%) with 86% specificity, increasing to 94% after excluding false positives lacking demyelinating features on the EDX tool.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>A real-time machine-assisted EDX support tool, used with a clinical web-based calculator, streamlines CIDP assessment by improving standardized identification of 2021-EAN/PNS CIDP demyelinating NCS criteria and aiding clinical decisions.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 4","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145781638","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ivo Bozovic, Kelly Gwathmey, Stojan Peric, Aleksandra Kacar, Jelena Lazovic, Reza Sadjadi, Ivana Basta
Background and Aims
In most studies to date, generic health-related quality of life (QoL) questionnaires have been used in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Unfortunately, these tools are often considered insufficient for capturing the unique characteristics of this specific, rare disease. The Chronic Acquired Polyneuropathy Patient-Reported Index (CAP-PRI) is a measure which specifically targets patients with chronic immune-mediated polyneuropathies. This is the first study which longitudinally assessed QoL and functionality by CAP-PRI in patients with active CIDP.
Methods
CAP-PRI testing was conducted at three time points (baseline, year one and year four) in 57 CIDP patients with active disease at baseline. In addition, the Medical Research Council Sum Score (MRC-SS), Inflammatory Neuropathy Cause and Treatment (INCAT) disability score, Inflammatory Rasch-built Overall Disability Scale (I-RODS), and the generic QOL instrument, the Short Form 36 (SF-36) were used in all tested patients at all three time points.
Results
At the baseline and two other time points, CAP-PRI showed strong correlations with impairment (MRC-SS), functionality (INCAT, I-RODS), and the SF-36. At year one and year four, CAP-PRI was able to make a differentiation between patients whose impairment/disability improved compared to patients who deteriorated, and it performed better than SF-36. One- and four-year changes in CAP-PRI correlated with a change in MRC-SS, INCAT, and I-RODS.
Interpretation
CAP-PRI is a highly reliable and sensitive measure for assessing QoL in patients with active CIDP. Its consistent performance over time supports its utility in monitoring CIDP patients with active disease in clinical and research settings.
{"title":"Longitudinal Assessment of Quality of Life and Functionality by CAP-PRI in Patients With Active Chronic Inflammatory Demyelinating Polyradiculoneuropathy","authors":"Ivo Bozovic, Kelly Gwathmey, Stojan Peric, Aleksandra Kacar, Jelena Lazovic, Reza Sadjadi, Ivana Basta","doi":"10.1111/jns.70091","DOIUrl":"10.1111/jns.70091","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>In most studies to date, generic health-related quality of life (QoL) questionnaires have been used in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Unfortunately, these tools are often considered insufficient for capturing the unique characteristics of this specific, rare disease. The Chronic Acquired Polyneuropathy Patient-Reported Index (CAP-PRI) is a measure which specifically targets patients with chronic immune-mediated polyneuropathies. This is the first study which longitudinally assessed QoL and functionality by CAP-PRI in patients with active CIDP.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>CAP-PRI testing was conducted at three time points (baseline, year one and year four) in 57 CIDP patients with active disease at baseline. In addition, the Medical Research Council Sum Score (MRC-SS), Inflammatory Neuropathy Cause and Treatment (INCAT) disability score, Inflammatory Rasch-built Overall Disability Scale (I-RODS), and the generic QOL instrument, the Short Form 36 (SF-36) were used in all tested patients at all three time points.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>At the baseline and two other time points, CAP-PRI showed strong correlations with impairment (MRC-SS), functionality (INCAT, I-RODS), and the SF-36. At year one and year four, CAP-PRI was able to make a differentiation between patients whose impairment/disability improved compared to patients who deteriorated, and it performed better than SF-36. One- and four-year changes in CAP-PRI correlated with a change in MRC-SS, INCAT, and I-RODS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>CAP-PRI is a highly reliable and sensitive measure for assessing QoL in patients with active CIDP. Its consistent performance over time supports its utility in monitoring CIDP patients with active disease in clinical and research settings.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 4","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145774840","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Victor Jia Wei Zhang, Laura Mantoan Ritter, Chandrashekar Hoskote, Ata Siddiqui, Michael Lunn, Julian Gillmore, Mary M. Reilly
Background
Treatment options for central nervous system (CNS) manifestations of ATTRv amyloidosis remain limited, with no disease-modifying therapy. However, previous case series have indicated that tafamidis (a transthyretin stabiliser) crosses the blood–brain barrier in small quantities. We present long-term follow-up data on two patients with CNS manifestations of ATTRv who received high-dose (61 mg) oral tafamidis.
Methods
Patient one is a 48-year-old man with p.Gly73Ala ATTRv peripheral neuropathy and commenced on gene-silencing therapy. He was subsequently diagnosed with early ATTRv CNS involvement, prompting the addition of tafamidis since age 45. Patient two is a 27-year-old woman with p.Gly67Arg ATTRv with significant CNS involvement resulting in epilepsy and encephalopathy. Since age 21, she has been on gene-silencing therapy and tafamidis.
Results
Following 3 years of therapy, patient one has remained clinically asymptomatic. However, brain magnetic resonance imaging (MRI) and cerebrospinal fluid assessment have demonstrated progression of his CNS ATTRv. Followed 6 years of therapy, patient two has continued to have seizures requiring escalation of anti-seizure medications and MRI brain has shown progression of CNS disease.
Conclusions
In our two patients, progression of CNS disease occurred whilst on high-dose tafamidis, highlighting the importance of further studies into the monitoring and treatment of CNS manifestations of ATTRv.
{"title":"Impact of High-Dose Tafamidis on Hereditary ATTR (ATTRv) Amyloidosis With Central Nervous System Involvement: Two Case Reports With Clinical, Radiological and Cerebrospinal Fluid Follow Up","authors":"Victor Jia Wei Zhang, Laura Mantoan Ritter, Chandrashekar Hoskote, Ata Siddiqui, Michael Lunn, Julian Gillmore, Mary M. Reilly","doi":"10.1111/jns.70088","DOIUrl":"https://doi.org/10.1111/jns.70088","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Treatment options for central nervous system (CNS) manifestations of ATTRv amyloidosis remain limited, with no disease-modifying therapy. However, previous case series have indicated that tafamidis (a transthyretin stabiliser) crosses the blood–brain barrier in small quantities. We present long-term follow-up data on two patients with CNS manifestations of ATTRv who received high-dose (61 mg) oral tafamidis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Patient one is a 48-year-old man with p.Gly73Ala ATTRv peripheral neuropathy and commenced on gene-silencing therapy. He was subsequently diagnosed with early ATTRv CNS involvement, prompting the addition of tafamidis since age 45. Patient two is a 27-year-old woman with p.Gly67Arg ATTRv with significant CNS involvement resulting in epilepsy and encephalopathy. Since age 21, she has been on gene-silencing therapy and tafamidis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Following 3 years of therapy, patient one has remained clinically asymptomatic. However, brain magnetic resonance imaging (MRI) and cerebrospinal fluid assessment have demonstrated progression of his CNS ATTRv. Followed 6 years of therapy, patient two has continued to have seizures requiring escalation of anti-seizure medications and MRI brain has shown progression of CNS disease.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>In our two patients, progression of CNS disease occurred whilst on high-dose tafamidis, highlighting the importance of further studies into the monitoring and treatment of CNS manifestations of ATTRv.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 4","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145739641","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Dysfunctional sphingolipid metabolism leads to nerve fiber degeneration, particularly of small caliber Aδ and C fibers, in hereditary sensory and autonomic neuropathies. We aimed to investigate the association of blood 1-deoxysphingolipid (1-deoxySL) profiles and skin denervation in idiopathic small fiber neuropathy (SFN).
Methods
Seventy-five patients with idiopathic SFN were recruited prospectively. Patients underwent a skin punch biopsy at the lower leg and upper thigh for intraepidermal nerve fiber density (IENFD) quantification. IENFD was correlated with individual blood 1-deoxySL levels, amino acid profiles, and determinants of glucose and lipoprotein metabolism.
Results
Median distal IENFD in SFN patients was 5.3 fibers/mm. In 38/75 (51%) patients, IENFD was ≤ 5.3 fibers/mm (i.e., “low fiber density,” LFD), while in 37/75 (49%) patients, IENFD was > 5.3 fibers/mm (i.e., “high fiber density,” HFD). 1-deoxySL was higher in patients with LFD compared to HFD (p < 0.05). Fasting plasma glucose was also higher in patients with LFD than in patients with HFD (p < 0.01), while HDL was lower in patients with LFD compared to HFD (p < 0.001).
Interpretation
1-deoxySL, lipoprotein metabolism, and glucose levels are associated with the extent of epidermal denervation in SFN, supporting the role of this metabolic axis in small nerve fiber pathology.
{"title":"Blood 1-Deoxysphingolipid Levels Are Associated With Epidermal Denervation in Small Fiber Neuropathy","authors":"Luisa Kreß, Caren Meyer zu Altenschildesche, Nadine Egenolf, Claudia Sommer, Thorsten Hornemann, Nurcan Üçeyler","doi":"10.1111/jns.70089","DOIUrl":"10.1111/jns.70089","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Dysfunctional sphingolipid metabolism leads to nerve fiber degeneration, particularly of small caliber Aδ and C fibers, in hereditary sensory and autonomic neuropathies. We aimed to investigate the association of blood 1-deoxysphingolipid (1-deoxySL) profiles and skin denervation in idiopathic small fiber neuropathy (SFN).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Seventy-five patients with idiopathic SFN were recruited prospectively. Patients underwent a skin punch biopsy at the lower leg and upper thigh for intraepidermal nerve fiber density (IENFD) quantification. IENFD was correlated with individual blood 1-deoxySL levels, amino acid profiles, and determinants of glucose and lipoprotein metabolism.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Median distal IENFD in SFN patients was 5.3 fibers/mm. In 38/75 (51%) patients, IENFD was ≤ 5.3 fibers/mm (i.e., “low fiber density,” LFD), while in 37/75 (49%) patients, IENFD was > 5.3 fibers/mm (i.e., “high fiber density,” HFD). 1-deoxySL was higher in patients with LFD compared to HFD (<i>p</i> < 0.05). Fasting plasma glucose was also higher in patients with LFD than in patients with HFD (<i>p</i> < 0.01), while HDL was lower in patients with LFD compared to HFD (<i>p</i> < 0.001).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>1-deoxySL, lipoprotein metabolism, and glucose levels are associated with the extent of epidermal denervation in SFN, supporting the role of this metabolic axis in small nerve fiber pathology.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 4","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jns.70089","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145723998","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Victor Evangelista Rodrigues Pereira, Roberto Pereira Santos, Marcos Raimundo Gomes de Freitas, Manuella Lima Gomes Ochtrop, Ana Caroline Siquara-De-Souza, Salim L. Balassiano, Marcia Rodrigues Jardim
Background and Aims
Vasculitides are a heterogeneous group of immune-mediated inflammatory disorders that compromise the vascuar wall, leading to luminal narrowing and tissue ischemia. When inflammation selectively affects the vasa nervorum without systemic involvement, it results in nonsystemic vasculitic neuropathy (NSVN), an underrecognized condition. NSVN presents diagnostic challenges due to its variable clinical manifestations and reliance on nerve biopsy for definitive diagnosis. This study aimed to characterize the clinical, neurophysiological, and histopathological features of NSVN in a Brazilian cohort.
Methods
We conducted a cross-sectional, ambispective cohort study combining retrospective chart review and prospective patient assessments. Inclusion required histopathological confirmation of isolated peripheral nerve vasculitis; cases with systemic or secondary vasculitis were excluded. Data collection included clinical evaluation, neurophysiology, and nerve biopsy.
Results
A total of 14 patients were included (9 female, 64%; mean age: 61). Most (n = 8, 57%) had subacute onset of painful sensory or sensorimotor deficits. Multiple mononeuropathies predominated (n = 11, 78%), but a subset exhibited chronic progression (n = 5, 35%) and axonal polyneuropathy (n = 3, 21%). Electrophysiological studies revealed a consistent axonal pattern. Biopsies confirmed possible vasculitis in six (43%), and probable vasculitis in six (42%), with only two (14%) fulfilling criteria for definite vasculitis. Serologies were nonspecific. Treatment involved corticosteroid pulse therapy, with immunosuppression in refractory cases.
Interpretation
These findings highlight that NSVN often presents with painful sensorimotor symptoms and may clinically mimic progressive axonal polyneuropathies. Given its potential for significant morbidity if left untreated, early recognition and consideration of nerve biopsy remain critical. The diagnostic complexity and variability in presentation suggest that NSVN may be underrecognized. We hope this cohort contributes to a broader understanding of its clinical spectrum and informs future diagnostic strategies.
{"title":"Nonsystemic Vasculitic Neuropathy—A Brazilian Case Series","authors":"Victor Evangelista Rodrigues Pereira, Roberto Pereira Santos, Marcos Raimundo Gomes de Freitas, Manuella Lima Gomes Ochtrop, Ana Caroline Siquara-De-Souza, Salim L. Balassiano, Marcia Rodrigues Jardim","doi":"10.1111/jns.70086","DOIUrl":"10.1111/jns.70086","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Vasculitides are a heterogeneous group of immune-mediated inflammatory disorders that compromise the vascuar wall, leading to luminal narrowing and tissue ischemia. When inflammation selectively affects the vasa nervorum without systemic involvement, it results in nonsystemic vasculitic neuropathy (NSVN), an underrecognized condition. NSVN presents diagnostic challenges due to its variable clinical manifestations and reliance on nerve biopsy for definitive diagnosis. This study aimed to characterize the clinical, neurophysiological, and histopathological features of NSVN in a Brazilian cohort.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We conducted a cross-sectional, ambispective cohort study combining retrospective chart review and prospective patient assessments. Inclusion required histopathological confirmation of isolated peripheral nerve vasculitis; cases with systemic or secondary vasculitis were excluded. Data collection included clinical evaluation, neurophysiology, and nerve biopsy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 14 patients were included (9 female, 64%; mean age: 61). Most (<i>n</i> = 8, 57%) had subacute onset of painful sensory or sensorimotor deficits. Multiple mononeuropathies predominated (<i>n</i> = 11, 78%), but a subset exhibited chronic progression (<i>n</i> = 5, 35%) and axonal polyneuropathy (<i>n</i> = 3, 21%). Electrophysiological studies revealed a consistent axonal pattern. Biopsies confirmed possible vasculitis in six (43%), and probable vasculitis in six (42%), with only two (14%) fulfilling criteria for definite vasculitis. Serologies were nonspecific. Treatment involved corticosteroid pulse therapy, with immunosuppression in refractory cases.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>These findings highlight that NSVN often presents with painful sensorimotor symptoms and may clinically mimic progressive axonal polyneuropathies. Given its potential for significant morbidity if left untreated, early recognition and consideration of nerve biopsy remain critical. The diagnostic complexity and variability in presentation suggest that NSVN may be underrecognized. We hope this cohort contributes to a broader understanding of its clinical spectrum and informs future diagnostic strategies.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 4","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145708469","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Anthony Maino, Florence Hazane-Puch, Philippe Petiot, Nathalie Roux-Buisson, John Rendu, Julien Fauré, Gaëlle Hardy
Background and Aims
Variants in the myelin protein zero coding MPZ gene are responsible for a broad spectrum of peripheral demyelinating and axonal neuropathies, including different types of Charcot–Marie–Tooth diseases, challenging for genotype–phenotype correlation.
Methods
Minigene splicing reporter assay was used to unveil the pathogenic mechanism of a novel MPZ(NM_000530.8) c.234 + 1G>C p.(?) heterozygous splice variant.
Results
The variant was identified in a 47-year-old female patient presenting with atypical clinical features, including balance disturbance with positive Romberg, absent Achilles tendon reflexes, distal hypoesthesia and bulbar involvement, including dysarthria and dysphagia. Electromyography revealed a sensory-motor neuropathy with moderately reduced nerve conduction velocities. In silico analysis predicted this variant to disrupt the consensual donor splice site located in intron 2 of MPZ. Minigene construction confirmed the functional impact of this variant, revealing exon 2 skipping and the apparition of a premature termination codon.
Interpretation
This case expends the genotype–phenotype correlations of MPZ-related Charcot–Marie–Tooth diseases, associating atypical mild phenotype with a rare splice dominant variant, and provides new insights into MPZ haploinsufficiency and pathogenic mechanisms.
背景和目的：髓鞘蛋白零编码MPZ基因的变异是广泛的外周脱髓鞘和轴突神经病变的原因，包括不同类型的沙科-玛丽-牙病，对基因型-表型相关性具有挑战性。方法：采用Minigene剪接报告基因试验，揭示新型MPZ(NM_000530.8) C .234 + 1G>C . p.（?）杂合剪接变异的致病机制。结果：该变异在一名47岁女性患者中被发现，其临床特征不典型，包括平衡障碍，Romberg阳性，跟腱反射缺失，远端感觉减退和球受累，包括构音障碍和吞咽困难。肌电图显示感觉-运动神经病变，神经传导速度中度降低。计算机分析预测这种变异会破坏位于MPZ内含子2上的供体剪接位点。miniigene的构建证实了该变异的功能影响，揭示了外显子2的跳跃和一个过早终止密码子的出现。解释：本病例扩展了MPZ相关沙克-玛丽-图斯病的基因型-表型相关性，将非典型轻度表型与罕见的剪接显性变异联系起来，并为MPZ单倍性不足和致病机制提供了新的见解。
{"title":"Novel Dominant Splicing Variant in MPZ Associated With Unusual Charcot–Marie–Tooth Disease","authors":"Anthony Maino, Florence Hazane-Puch, Philippe Petiot, Nathalie Roux-Buisson, John Rendu, Julien Fauré, Gaëlle Hardy","doi":"10.1111/jns.70085","DOIUrl":"10.1111/jns.70085","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background and Aims</h3>\u0000 \u0000 <p>Variants in the myelin protein zero coding <i>MPZ</i> gene are responsible for a broad spectrum of peripheral demyelinating and axonal neuropathies, including different types of Charcot–Marie–Tooth diseases, challenging for genotype–phenotype correlation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Minigene splicing reporter assay was used to unveil the pathogenic mechanism of a novel <i>MPZ</i>(NM_000530.8) c.234 + 1G>C p.(?) heterozygous splice variant.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The variant was identified in a 47-year-old female patient presenting with atypical clinical features, including balance disturbance with positive Romberg, absent Achilles tendon reflexes, distal hypoesthesia and bulbar involvement, including dysarthria and dysphagia. Electromyography revealed a sensory-motor neuropathy with moderately reduced nerve conduction velocities. In silico analysis predicted this variant to disrupt the consensual donor splice site located in intron 2 of <i>MPZ</i>. Minigene construction confirmed the functional impact of this variant, revealing exon 2 skipping and the apparition of a premature termination codon.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>This case expends the genotype–phenotype correlations of <i>MPZ</i>-related Charcot–Marie–Tooth diseases, associating atypical mild phenotype with a rare splice dominant variant, and provides new insights into <i>MPZ</i> haploinsufficiency and pathogenic mechanisms.</p>\u0000 </section>\u0000 </div>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 4","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12687107/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145708377","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Naomi Behanan, Sarab Mohamed, Rhona Chen, Gloria Mak, Jian-Qiang Lu
Human cytomegalovirus (HCMV) is a neurotropic, double-stranded DNA virus from the Herpesviridae family. It has a large genome, infects the majority of populations, and typically causes asymptomatic infections in healthy individuals. After the initial infection with established lifelong latency, HCMV can reactivate and cause disorders including neuropathies. Besides the infections typically in immunocompromised patients, HCMV may also trigger autoimmunity leading to tissue injury and associated pathologies. HCMV-associated neuropathies are a pathogenically heterogeneous group of peripheral nervous system (PNS) disorders that include direct HCMV infection of the nerve(s), as well as non-infectious associated neuropathies such as axonal or degenerative, vasculitic/ischemic or necrotizing, inflammatory demyelinating, and immune-mediated forms. Congenital HCMV-associated neuropathies primarily involve the cochlear/auditory and optic nerves with somewhat distinct pathogenic mechanisms compared with their postnatal counterparts, largely due to the immaturity of the fetal immune system. This article reviews the pathophysiology of PNS involvement in HCMV infection, followed by congenital HCMV-associated neuropathies with a case demonstration, and various postnatal HCMV-associated neuropathies, including a detailed review of HCMV-associated optic neuropathies, from pathogenic mechanisms to clinical and therapeutic implications. As the PNS has a few immune protective mechanisms against pathogens, direct HCMV infection of nerves is rare and occurs only in immunocompromised patients; most HCMV-associated neuropathies are secondary with multiple pathogenic mechanisms including varying degrees of autoimmunity. While the clinical manifestations of HCMV-associated neuropathies are variable, their treatment is typically empirical and case-based, focusing on antiviral therapy often combined with immunomodulatory approaches. Prompt and appropriate management can improve outcomes of HCMV-associated neuropathies.
{"title":"Human Cytomegalovirus Associated Neuropathies: A Comprehensive Review From Pathophysiology to Clinical and Therapeutic Considerations","authors":"Naomi Behanan, Sarab Mohamed, Rhona Chen, Gloria Mak, Jian-Qiang Lu","doi":"10.1111/jns.70087","DOIUrl":"10.1111/jns.70087","url":null,"abstract":"<p>Human cytomegalovirus (HCMV) is a neurotropic, double-stranded DNA virus from the Herpesviridae family. It has a large genome, infects the majority of populations, and typically causes asymptomatic infections in healthy individuals. After the initial infection with established lifelong latency, HCMV can reactivate and cause disorders including neuropathies. Besides the infections typically in immunocompromised patients, HCMV may also trigger autoimmunity leading to tissue injury and associated pathologies. HCMV-associated neuropathies are a pathogenically heterogeneous group of peripheral nervous system (PNS) disorders that include direct HCMV infection of the nerve(s), as well as non-infectious associated neuropathies such as axonal or degenerative, vasculitic/ischemic or necrotizing, inflammatory demyelinating, and immune-mediated forms. Congenital HCMV-associated neuropathies primarily involve the cochlear/auditory and optic nerves with somewhat distinct pathogenic mechanisms compared with their postnatal counterparts, largely due to the immaturity of the fetal immune system. This article reviews the pathophysiology of PNS involvement in HCMV infection, followed by congenital HCMV-associated neuropathies with a case demonstration, and various postnatal HCMV-associated neuropathies, including a detailed review of HCMV-associated optic neuropathies, from pathogenic mechanisms to clinical and therapeutic implications. As the PNS has a few immune protective mechanisms against pathogens, direct HCMV infection of nerves is rare and occurs only in immunocompromised patients; most HCMV-associated neuropathies are secondary with multiple pathogenic mechanisms including varying degrees of autoimmunity. While the clinical manifestations of HCMV-associated neuropathies are variable, their treatment is typically empirical and case-based, focusing on antiviral therapy often combined with immunomodulatory approaches. Prompt and appropriate management can improve outcomes of HCMV-associated neuropathies.</p>","PeriodicalId":17451,"journal":{"name":"Journal of the Peripheral Nervous System","volume":"30 4","pages":""},"PeriodicalIF":3.2,"publicationDate":"2025-12-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12686851/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145708398","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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