Guillain-Barré Syndrome in a Patient Receiving Anti-Tumor Necrosis Factor for Crohn Disease: Coincidence or Consequence?

BACKGROUND Antibodies against tumor necrosis factor alpha (anti-TNF-alpha) are currently widely used in the treatment of inflammatory bowel diseases (IBD), despite a number of reported adverse effects. Diverse neurologic syndromes, including the Guillain-Barre syndrome (GBS), an immune-mediated disease characterized by evolving ascending limb weakness, sensory loss, and areflexia, have been described in association with anti-TNF-alpha therapy. CASE REPORT A 45-year-old White woman was in follow-up with fistulizing ileocolonic Crohn disease using combination therapy (infliximab plus azathioprine) as CD maintenance therapy. After 3 years of this immunosuppressive therapy, she presented with symmetrical and ascending paresis in the lower limbs, and later in the upper limbs, in addition to reduced reflexes in the knees, 1 day after an infliximab infusion. The patient was hospitalized and treatment for CD was suspended. Neurophysiology studies demonstrated a pattern compatible with acute inflammatory demyelinating polyradiculopathy, with predominantly motor involvement, consistent with Guillain-Barre syndrome (GBS). Clinical, laboratory, and imaging exams were unremarkable. She was treated with intravenous immunoglobulins, with a progressive and complete resolution of neurological symptoms. After 1-year follow-up, she presented with active Crohn disease, and we opted for treating her with vedolizumab, with which she achieved clinical and endoscopic remission. CONCLUSIONS Patients receiving biological therapy with anti-TNF-alpha agents should be monitored for central or peripheral neurological signs and symptoms. The development of GBS can be secondary to anti-TNF-alpha treatment. The positive temporal relationship with TNF-alpha therapy and onset of neurological symptoms reinforces this possibility.

A case of variant of GBS with positive serum ganglioside GD3 IgG antibody.

BACKGROUND: Acute bulbar palsy-plus (ABPp) syndrome is an unusual variant of Guillain-Barré syndrome (GBS). Anti-GT1a and anti-GQ1b antibodies have been reported in patients with ABPp, but without reports related to GD3 antibodies. METHODS: Clinical data of a patient diagnosed as ABPp syndrome were reviewed clinically. And we summarized the GBS patients with ABP and facial paralysis reported in the literature. RESULTS: We reported a 13-year-old girl presented with asymmetric bifacial weakness, bulbar palsy and transient limb numbness, and had positive serum IgG anti-GD3 antibody. Through reviewing the GBS patients with ABP and facial paralysis reported previously, we found that facial palsy could be unilateral or bilateral. The bilateral facial palsy could present successively or simultaneously, and could be symmetrical or asymmetrical. Other common symptoms included ophthalmoplegia, sensory abnormality and ataxia. IgG anti-GT1a and IgG anti-GQ1b antibodies were the most frequent. Most of the patients had full recovery within two weeks to one year of follow-up. CONCLUSIONS: We reported a patient with asymmetric bifacial palsy and bulbar palsy, which seemed to fit the diagnosis of ABPp syndrome. This was the first report of ABPp variant of GBS with positive serum ganglioside GD3 IgG antibody.

[Fisher Syndrome].

Fisher syndrome is recognized as a variant of Guillain-Barré syndrome, encompassing acute onset immune-mediated neuropathies marked by the classical triad of ataxia, areflexia, and ophthalmoplegia. Generally, Fisher syndrome follows a self-limited course with a good prognosis. Ophthalmoplegia, typically bilateral, progresses to complete external ophthalmoplegia within 1-2 weeks. Ataxia, often very severe, may cause an inability to walk without support despite normal strength. Fisher syndrome is also frequently concomitant with additional clinical features, including ptosis, internal ophthalmoplegia, facial nerve palsy, sensory deficits, and bulbar palsy. The confirmation of an antecedent infection is often established. Among the ganglioside antibodies, anti-GQ1b antibodies exhibit positivity in over 80% of patients. The syndrome manifests in three distinct types: a partial subtype exhibiting only a subset of the triad symptoms, Bickerstaff's brainstem encephalitis marked by impaired consciousness and pyramidal tract signs, and an overlapping subtype with Guillain-Barré syndrome, characterized by weakness in the extremities.

[Guillain-Barré syndrome].

Guillain-Barré syndrome (GBS), an acute immune-mediated neuropathy, occurs following immunological stimulation, such as infection, with complement-mediated neuropathy implicated in the pathophysiology of this condition. Glycolipid antibodies produced by molecular mimicry are detected in approximately 60% of cases. Recent studies have suggested the role of cell-mediated immunity in the pathogenesis of GBS. Intravenous immunoglobulin and plasma exchange are established immunotherapies. In this article, based on the latest knowledge, we describe the pathophysiology, diagnosis, treatment, prognosis, and prognostic prediction of GBS. Furthermore, we discuss some GBS guidelines published by the European Academy of Neurology/Peripheral Nerve Society.

Assessment of potential adverse events following the 2022-2023 seasonal influenza vaccines among U.S. adults aged 65 years and older.

BACKGROUND: While safety of influenza vaccines is well-established, some studies have suggested potential associations between influenza vaccines and certain adverse events (AEs). This study examined the safety of the 2022-2023 influenza vaccines among U.S. adults ≥ 65 years. METHODS: A self-controlled case series compared incidence rates of anaphylaxis, encephalitis/encephalomyelitis, Guillain-Barré Syndrome (GBS), and transverse myelitis following 2022-2023 seasonal influenza vaccinations (i.e., any, high-dose or adjuvanted) in risk and control intervals among Medicare beneficiaries ≥ 65 years. We used conditional Poisson regression to estimate incidence rate ratios (IRRs) and 95 % confidence intervals (CIs) adjusted for event-dependent observation time and seasonality. Analyses also accounted for uncertainty from outcome misclassification where feasible. For AEs with any statistically significant associations, we stratified results by concomitant vaccination status. RESULTS: Among 12.7 million vaccine recipients, we observed 76 anaphylaxis, 276 encephalitis/encephalomyelitis, 134 GBS and 75 transverse myelitis cases. Only rates of anaphylaxis were elevated in risk compared to control intervals. With all adjustments, an elevated, but non-statistically significant, anaphylaxis rate was observed following any (IRR: 2.40, 95% CI: 0.96-6.03), high-dose (IRR: 2.31, 95% CI: 0.67-7.91), and adjuvanted (IRR: 3.28, 95% CI: 0.71-15.08) influenza vaccination; anaphylaxis IRRs were 2.54 (95% CI: 0.49-13.05) and 1.64 (95% CI: 0.38-7.05) for persons with and without concomitant vaccination, respectively. CONCLUSIONS: Rates of encephalitis/encephalomyelitis, GBS, or transverse myelitis were not elevated following 2022-2023 seasonal influenza vaccinations among U.S. adults ≥ 65 years. There was an increased rate of anaphylaxis following influenza vaccination that may have been influenced by concomitant vaccination.

Mimics of Guillain-Barré Syndrome in Pediatric Patients Admitted to the Neuro-Intensive Care Unit of a Tertiary Care Hospital-A Case Series.

Guillain-Barré syndrome is the most common cause of acute flaccid paralysis in children, but several diseases mimic GBS. We aimed to identify and report the clinical pointers and battery of tests required to differentiate Guillain-Barré syndrome from its observed mimics in the pediatric population admitted to our neuro-critical care unit. We conducted a retrospective record analysis of all pediatric patients admitted over ten years from 2008-2018, whose initial presentation was compatible with a clinical diagnosis of GBS. Eighty-three patients were at first treated as GBS, of which seven (8.4%) were found to have an alternate diagnosis-three cases of paralytic rabies, one case each of acute disseminated encephalomyelitis, cervical myeloradiculopathy, neuromyelitis optica, and a case of community-acquired Staphylococcus aureus pneumonia associated sepsis. Neurophysiological and neuro-virological testing, central nervous system imaging, and sepsis screening helped to confirm the alternate diagnosis. Our case series provides knowledge of subtle clinical differences along with the mindful use of diagnostic testing to facilitate the accurate diagnosis of GBS mimics.

Concurrent transverse myelitis and acute inflammatory demyelinating polyneuropathy.

A woman in her 40s presented with thoracic banding dysaesthesia and lower motor neuron weakness. Spinal imaging revealed a short segment of transverse myelitis and neurophysiology was suggestive of concurrent acute inflammatory demyelinating polyneuropathy. The patient improved with consecutive intravenous immunoglobulin and methylprednisolone treatment. Acute inflammatory demyelinating polyneuropathy is a progressive immune-mediated peripheral neuropathy which responds to intravenous immunoglobulin or plasmapheresis, whereas transverse myelitis is a central inflammatory syndrome usually treated with corticosteroid. We highlight differentiating features of the clinical presentation and the utility of investigations such as neurophysiology and MRI along with a review of treatment and the role for corticosteroid therapy.

Genetic association between gut microbiota and the risk of Guillain-Barré syndrome.

BACKGROUND: Guillain-Barré Syndrome (GBS) is an autoimmune disease that typically develops after a previous gastrointestinal (GI) infection. However, the exact association between Gut Microbiota (GM) and GBS still remains unknown due to various challenges. This study aimed to investigate the potential causal association between GM and GBS by using a two-sample Mendelian Randomization (TSMR) analysis. METHODS: Utilizing the largest available genome-wide association study (GWAS) meta-analysis from the MiBioGen consortium (n = 13,266) as a foundation, we conducted a TSMR to decipher the causal relationship between GM and GBS. Various analytical methods were employed, including the inverse variance weighted (IVW), MR-PRESSO, MR-Egger, and weighted median. The heterogeneity of instrumental variables (IVs) was assessed using Cochran's Q statistics. RESULTS: The analysis identified three microbial taxa with a significantly increased risk association for GBS, including Ruminococcus gnavus group (OR = 1.40, 95 % CI: 1.07-1.83), Ruminococcus gauvreauii group (OR = 1.51, 95 % CI: 1.02-2.25), and Ruminococcaceae UCG009 (OR = 1.42, 95 % CI: 1.02-1.97), while Eubacterium brachy group (OR = 1.44, 95 % CI: 1.10-1.87) and Romboutsia (OR = 1.67, 95 % CI: 1.12-2.47) showed a suggestively causal association. On the other hand, Ruminococcaceae UCG004 (OR = 0.61, 95 % CI: 0.41-0.91) had a protective effect on GBS, while Bacilli (OR = 0.60, 95 % CI: 0.38-0.96), Gamma proteobacteria (OR = 0.63, 95 % CI: 0.41-0.98) and Lachnospiraceae UCG001 (OR = 0.69, 95 % CI: 0.49-0.96) showed a suggestively protective association for GBS. CONCLUSION: The MR analysis suggests a potential causal relationship between specific GM taxa and the risk of GBS. However, further extensive research involving diversified populations is imperative to validate these findings.

Triple Trouble.

We present a case of a 24-year-old female recently diagnosed with acute leukemia who came with complaints of fever for 14 days, progressive lower limb weakness, and multiple episodes of vomiting in the last 1 day. In nerve conduction studies, a diagnosis of Guillain-Barré syndrome (GBS) was established. Fever with thrombocytopenia workup revealed a positive dengue nonstructural protein 1 (NS1) and immunoglobulin M (IgM) report. Immunophenotyping confirmed pre-B acute lymphoblastic leukemia (ALL). As leukemia is an immunocompromised state, the peripheral nervous system vulnerability is increased, or infection could precipitate an immune neuropathy. About 10% of adult ALL presents with central nervous system (CNS) leukemias; a higher incidence is seen in mature B ALL. There is some evidence to suggest immunosuppression secondary to intensive chemotherapy (vincristine-induced dying back neuropathy), which was not started in our case. This rare combination in a short period of time with a worsening situation paralyzed the line of management. Few reports described GBS in patients with dengue in adults. The association of Guillan-Barre syndrome and ALL could be coincidental or has a pathophysiological basis and is under basic investigation.

Superacute onset of Guillain-Barré syndrome after elective spinal surgery: A case report and literature review.

RATIONALE: Guillain-Barré syndrome (GBS) epitomizes an acute peripheral neuropathy hallmarked by an autoimmune retort directed at the myelin sheath enwrapping peripheral nerves. While it is widely acknowledged that a majority of GBS patients boast a history of antecedent infections, the documentation of postoperative GBS occurrences is progressively mounting. Drawing upon an exhaustive compendium of recent case reports, the disease's inception spans a gamut from within 1 hour to 1.2 years. PATIENT CONCERNS: At this juncture, we proffer a singular case: an instance involving a 51-year-old gentleman who underwent lumbar spine surgery, only to encounter immediate debilitation of limb and respiratory musculature. DIAGNOSES: Post elimination of variables linked to anesthetic agents, encephalon, and spinal cord pathologies, a potent suspicion of superacute GBS onset emerged. INTERVENTIONS: Subsequent to immunoglobulin therapy, plasmapheresis, and adjunctive support, the patient's ultimate demise became manifest. OUTCOMES: No progress was found to date. LESSONS: Given GBS's potential to instigate paralysis, respiratory collapse, and autonomic nervous system aberrations, alongside other pernicious sequelae, coupled with the exceptional rarity of the temporal onset in this particular instance, it undeniably proffers an imposing conundrum for anesthetists in the realm of differential diagnosis and therapeutic conduct. During the postoperative convalescence phase under anesthesia, should the patient evince deviant limb musculature vigor and compromised respiratory sinews, the prospect of GBS must not be consigned to oblivion. Precision in diagnosis conjoined with apt therapeutic measures could well be the harbinger of a divergent denouement for the afflicted patient.

Relative frequencies and clinical features of Guillain-Barré Syndrome before and during the COVID-19 pandemic in North China.

OBJECTIVE: Most studies investigated the relationship between COVID-19 and Guillain-Barré syndrome (GBS) by comparing the incidence of GBS before and during the pandemic of COVID-19. However, the findings were inconsistent, probably owing to varying degrees of the lockdown policy. The quarantine requirements and travel restrictions in China were lifted around December 7, 2022. This study aimed to explore whether the relative frequency of GBS increased during the major outbreak in the absence of COVID-19-mandated social restrictions in China. METHODS: GBS patients admitted to the First Hospital, Shanxi Medical University, from December 7, 2022 to February 20, 2023, and from June, 2017 to August, 2019 were included. The relative frequencies of GBS in hospitalized patients during different periods were compared. The patients with and without SARS-CoV-2 infection within six weeks prior to GBS onset formed the COVID-GBS group and non-COVID-GBS group, respectively. RESULTS: The relative frequency of GBS among hospitalized patients during the major outbreak of COVID-19 (13/14,408) was significantly higher than that before the COVID-19 epidemic (29/160,669, P < 0.001). More COVID-GBS patients (11/13) presented AIDP subtype than non-COVID-GBS cases (10/27, P = 0.003). The mean interval between onset of infective symptoms and GBS was longer in COVID-GBS (21.54 ± 11.56 days) than in non-COVID-GBS (5.76 ± 3.18 days, P < 0.001). CONCLUSIONS: COVID-19 significantly increased the incidence of GBS. Most COVID-GBS patients fell into the category of AIDP, responded well to IVIg, and had a favorable prognosis.

Effect of intravenous immunoglobulin and plasmapheresis on nerve conduction parameters compared to the natural course of Guillain-Barré syndrome.

BACKGROUND: Intravenous immunoglobulin (IVIg) and plasmapheresis (PLEX) are recommended in moderate to severe Guillain-Barré Syndrome (GBS), but there is paucity of studies evaluating its effect on nerve conduction studies (NCS). We report the effect of IVIg and PLEX on the NCS parameters and clinical outcomes compared to natural course (NC) of GBS patients. METHOD: Moderate to severe GBS patients were included based on clinical, cerebrospinal fluid, and NCS finding. Six motor and sensory nerves were evaluated at admission, one month and 3 months, and NCS subtyping was done. Axonal and demyelination burden in motor nerves and early reversible conduction block (ERCB) were noted. Patients receiving IVIg, PLEX or on NC were noted. Outcome was defined at 3 months into complete, partial and poor using a 0-6 GBS Disability Scale (GBSDS). RESULT: Seventy-two patients were included, whose median age was 36 years and 22(30.6 %) were females. 44 patients received IVIg, 9 PLEX and 19 were in NC, and they had comparable peak disability. AIDP was the dominant subtype at admission (58.3 %), which remained so at 3 months (50 %). The shift of subtypes was the highest from the equivocal group followed by AMAN and the least from AIDP. IVIg and PLEX group had more reduction in axonal burden and had ERCB compared to NC. 33(44 %) patients had complete recovery, and 40(55.5 %) patients had concordance in clinical and neurophysiological outcome. CONCLUSION: Transition of GBS subtype may occur at follow-up from all the subtypes, the highest from the equivocal and the lowest from the AIDP group. IVIg/PLEX treatment may help in reducing conduction block and axonal burden.

Implications of anti-ganglioside antibodies in isolated dysphagia following COVID-19 infection: Case series.

BACKGROUND: There have been multiple reports about the occurrence of dysphagia after the contraction of coronavirus disease 2019 (COVID-19). However, a detailed pathology and epidemiologic relation between COVID-19 infection and dysphagia have yet to be established. Here, we report three cases of unexplained dysphagia after COVID-19 diagnosis, with atypical clinical presentations. CASE REPORT: All patients showed severe isolated lower cranial nerve involvement with dysphagia and aspiration, which required full tube feeding but showed no evidence of limb weakness or sensory symptoms. All tested positive for anti-ganglioside antibody tests, which all commonly (GD1b, GM1, and GQ1b) are known to have terminal NeuNAc(α2-3)Gal epitope. DISCUSSION: We report a series of cases featuring severe, isolated dysphagia post-COVID-19 infection, concomitant with positive anti-ganglioside antibodies. One potential etiology is a variant of Guillain-Barré syndrome. Because only isolated dysphagia with sparing of the facial and extraocular muscles was evident in these cases, we explore the association between anti-ganglioside antibodies specific to NeuNAc(α2-3)Gal, which has been frequently associated with the development of bulbar dysfunction. Given that NeuNAc(α2-3)Gal exhibits an affinity for the spike glycoprotein of SARS-CoV-2, a cross-reaction against NeuNAc(α2-3)Gal may possibly contribute to isolated dysphagia following COVID-19 infection.

A Study on the Epidemiology of COVID-19-Related Guillain-Barré Syndrome in the United States.

INTRODUCTION: Several neurological complications have been reported with COVID-19, including Guillain-Barré syndrome (GBS). We looked at incidence, baseline characteristics, and in-hospital outcomes of COVID-19-associated GBS in the United States. STUDY DESIGN AND METHODS: We conducted a retrospective analysis using the US National Inpatient Sample database to identify hospitalizations for COVID-19 and GBS, using International Classification of Disease, 10th Revision, codes G610 and G650 for GBS and U071 for COVID-19. The codes used in this study are listed in Supplemental Digital Content 1 (see e Appendix, http://links.lww.com/JCND/A69). RESULTS: In total, 13,705 GBS admissions were recorded nationwide in 2020; of these, 1155 (8.43%) were associated with COVID-19. The frequency of GBS in COVID-19 admissions was 0.07%, compared with 0.08% in non-COVID-19 admissions (P = 0.8166). COVID-19 cohort with GBS had higher utilization of invasive mechanical ventilation (20.8% vs. 11.8%, P < 0.001) in comparison with COVID-19 cohort without GBS. GBS admissions with COVID-19 exhibited significantly higher inpatient mortality (12.2% vs. 3%, P < 0.001) compared with GBS admissions without COVID-19. INTERPRETATION: Our findings underscore GBS as a rare yet severe complication of COVID-19, highlighting a significant difference in mortality when compared with GBS not associated with COVID-19.

Coletanêa de Respostas Rápidas Turma 1 / Collection of Rapid Review Class 1

Coletânea dedicada aos estudos das respostas rápidas do Programa Educacional em Vigilância em Saúde no enfrentamento da COVID-19 e outras Doenças Virais (VigiEpidemia). Esse tema é de extrema relevância e atualidade em nosso contexto da saúde global e na resposta as emergências em saúde pública (ESP) de forma geral. As ESP, que englobam surtos e epidemias, desastres e desassistência à população, representam desafios complexos que exigem respostas ágeis e eficazes por parte das autoridades sanitárias, profissionais da saúde e comunidades como um todo. Até o momento, a pandemia de COVID-19 foi a maior ESP do Século XXI. Ela serviu como um lembrete doloroso da vulnerabilidade da humanidade diante da ameaça de doenças virais. Esta ESP, que teve resposta catastrófica em diversos momentos, evidenciou a importância do investimento em preparação, vigilância e resposta, destacando a necessidade de sistemas de vigilância robustos, colaboração internacional, Inteligência epidemiológica e comunicação transparente para mitigar o impacto devastador das doenças infecciosas na sociedade. As lições aprendidas com a pandemia de COVID-19 são vastas e multifacetadas. A importância da pesquisa, da educação em saúde e do desenvolvimento de vacinas foi evidenciada como uma prioridade crucial na proteção da saúde pública mundial. O investimento em pesquisas e em cursos para formação de profissionais que possam estar atentos as mudanças nos padrões e comportamentos das doenças infecciosas, além de atuar na resposta rápida quando necessário, é fundamental para estarmos preparados para as futuras pandemias. A vacinação, por exemplo, sempre foi uma das ferramentas mais poderosas para evitar surtos e epidemias e, durante a pandemia de COVID-19, ajudou a controlar os óbitos pela doença e possibilitou que voltássemos a ter uma vida normal. Além da vacina contra COVID-19, as vacinas de influenza e dengue também são exemplos notáveis de avanços científicos que desempenham um papel fundamental na prevenção de futuras ESP. Ao explorar os diversos aspectos da resposta, monitoramento e controle de surtos, epidemias e pandemias, esta coletânea visa fornecer uma compreensão abrangente dos desafios enfrentados, das melhores práticas e das estratégias eficazes para mitigar os impactos adversos desses eventos. Espera-se que este trabalho não apenas informe e eduque, mas também inspire ações concretas para fortalecer a recuperação e resiliência dos sistemas de saúde e proteger o bem-estar das comunidades mais vulneráveis do nosso pais.

A collection dedicated to the study of rapid responses by the Educational Program in Health Surveillance in addressing COVID-19 and other Viral Diseases (VigiEpidemia). This theme is of utmost relevance and timeliness in our context of global health and in responding to public health emergencies (PHE) in general. PHEs, which encompass outbreaks and epidemics, disasters, and neglect of the population, represent complex challenges that require swift and effective responses from health authorities, healthcare professionals, and communities as a whole. To date, the COVID-19 pandemic has been the largest PHE of the 21st century. It served as a painful reminder of humanity's vulnerability in the face of viral disease threats. This PHE, which had catastrophic responses at various times, highlighted the importance of investing in preparedness, surveillance, and response, underscoring the need for robust surveillance systems, international collaboration, epidemiological intelligence, and transparent communication to mitigate the devastating impact of infectious diseases on society. The lessons learned from the COVID-19 pandemic are vast and multifaceted. The importance of research, health education, and vaccine development was highlighted as a crucial priority in protecting global public health. Investing in research and training courses to prepare professionals who can be attentive to changes in the patterns and behaviors of infectious diseases and act quickly when needed is essential to be prepared for future pandemics. Vaccination, for example, has always been one of the most powerful tools to prevent outbreaks and epidemics, and during the COVID-19 pandemic, it helped control disease-related deaths and allowed us to return to a normal life. In addition to the COVID-19 vaccine, influenza and dengue vaccines are also notable examples of scientific advancements that play a key role in preventing future PHEs. By exploring the various aspects of response, monitoring, and control of outbreaks, epidemics, and pandemics, this collection aims to provide a comprehensive understanding of the challenges faced, best practices, and effective strategies to mitigate the adverse impacts of these events. It is hoped that this work will not only inform and educate but also inspire concrete actions to strengthen the recovery and resilience of health systems and protect the well-being of the most vulnerable communities in our country.

Association of CSF Total Protein with Clinical Heterogeneity, Disease Severity and Electrophysiological Pattern in GBS Patients.

Guillain-Barre Syndrome (GBS) is considered as an immune mediated inflammatory disease of peripheral nerves and nerve roots. The significance of CSF total protein (CSF-TP) in subtypes of Guillain-Barre syndrome has not been well established. This observational, cross sectional study's aim was to identify association of CSF total protein with clinical heterogeneity, disease severity and electrophysiological subtypes in GBS patients. This study was carried out in the Department of Neurology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Bangladesh from September 2017 to February 2019 on 50 (fifty) admitted GBS patients as per inclusion and exclusion criteria. About 3-5 ml of CSF was taken around 10±2 days from disease onset for detection CSF cell count and protein. Pattern of clinical presentation, disability status by Hughes scale and NCS findings of these patients was documented. Mean CSF-TP were substantially higher for Sensori-motor GBS (195.42 mg/dl) and GBS with cranial involvement (226.12 mg/dl) than that of GBS with motor (134.00 mg/dl) and autonomic involvement (155.21 mg/dl). Mean CSF total protein (CSF-MTP) in severely ill GBS patients (Grade-IV) was 217.04 mg/dl and very severely ill GBS patients (Grade-V) was 138.00mg/dl which was significantly higher than mean CSF total protein in mild GBS patients (CSF-MTP: 99.86mg/dl) and moderately ill GBS patients (CSF-MTP: 172.00 mg/dl). Mean CSF total protein is 245.00mg/dl in AIDP which is also higher than mean CSF total protein of AMAN (153.36 mg/dl) and AMSAN (165.17mg/dl). CSF-TP is thought to be a sensitive test for GBS in the second week after onset, but it may be a reliable predictor of clinical severity. There is a significant association of CSF-TP elevation with demyelinating electrophysiologic pattern.

The sural-sparing pattern in clinical variants and electrophysiological subtypes of Guillain-Barré syndrome.

BACKGROUND: Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide and can be classified into electrophysiological subtypes and clinical variants. OBJECTIVE: This study aimed to compare the frequency of the sural-sparing pattern (SSP) in subtypes and variants of GBS. METHODS: This retrospective cohort study analyzed clinical and electrophysiological data of 171 patients with GBS hospitalized in public and private hospitals of Natal, Rio Grande do Norte, Brazil, between 1994 and 2018; all cases were followed up by the same neurologist in a reference neurology center. Patients were classified according to electrophysiological subtypes and clinical variants, and the SSP frequency was compared in both categories. The exact Fisher test and Bonferroni correction were used for statistical analysis. RESULTS: The SSP was present in 53% (57 of 107) of the patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 8% (4 of 48) of the patients with axonal subtypes, and 31% (5 of 16) of the equivocal cases. The SSP frequency in the AIDP was significantly higher than in the axonal subtypes (p < 0.0001); the value was kept high after serial electrophysiological examinations. Only the paraparetic subtype did not present SSP. CONCLUSION: The SSP may be present in AIDP and axonal subtypes, including acute motor axonal neuropathy, but it is significantly more present in AIDP. Moreover, the clinical variants reflect a specific pathological process and are correlated to its typical electrophysiological subtype, affecting the SSP frequency.

ANTECEDENTES: A síndrome de Guillain-Barré (GBS) é a causa mais comum de paralisia flácida aguda em todo o mundo e pode ser classificada em subtipos eletrofisiológicos e variantes clínicas. OBJETIVO: Este estudo teve como objetivo comparar a frequência do padrão de preservação do sural (SSP) em subtipos e variantes de GBS. MéTODOS: É um estudo de coorte retrospectivo que analisou dados clínicos e eletrofisiológicos de 171 pacientes com GBS internados em hospitais públicos e privados de Natal, Rio Grande do Norte, Brasil, entre 1994 e 2018. Todos os casos foram acompanhados pelo mesmo neurologista em centro de referência em neurologia. Os pacientes foram classificados de acordo com os subtipos eletrofisiológicos e variantes clínicas e a frequência do SSP foi comparada em ambas as categorias. O teste exato de Fisher e a correção de Bonferroni foram utilizados para análise estatística. RESULTADOS: O SSP esteve presente em 53% (57 de 107) dos pacientes com polirradiculoneuropatia desmielinizante inflamatória aguda (PDIA), em 8% (4 de 48) dos pacientes com subtipos axonais e em 31% (5 de 16) dos casos não definidos. A frequência do SSP no AIDP foi significativamente maior do que nos subtipos axonais (p < 0,0001); o valor manteve-se elevado após exames eletrofisiológicos seriados. Apenas o subtipo paraparético não apresentou SSP. CONCLUSãO: O SSP pode estar presente na PDIA e nos subtipos axonais, incluindo a neuropatia axonal motora aguda, mas está significativamente mais presente na PDIA. Além disso, as variantes clínicas refletem um processo patológico específico e estão correlacionadas ao seu subtipo eletrofisiológico típico, afetando a frequência do SSP.