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A 20's primiparous woman, following spontaneous expulsion of intrauterine death of the fetus at 30 weeks of gestation, presented on post-partum day 8 with acute onset flaccid quadriparesis and breathing difficulty, which had rapidly progressed to involve the legs on day 3 up to her upper limbs on post-partum day 5. Following examination, Guillain Barre Syndrome (GBS) with ascending diaphragmatic involvement was diagnosed, and plasma exchange was initiated. She developed raised blood pressure, headache, sudden onset visual loss with 2 episodes of generalized seizures on post-partum day 14. Brain MRI and clinical suspicion helped diagnose Posterior Reversible Encephalopathy Syndrome (PRES). The patient was treated with anticonvulsants and antihypertensive agents. She regained her vision over the next two days, completed the treatment for GBS, and made a good recovery with independence for advanced activities of daily living on follow-up.

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Background: Guillain-Barre Syndrome (GBS) is the most prevalent acute peripheral polyneuropathy disorder. The disparities between populations and variations in the major risk factors highlight the importance of country-specific studies. This study aimed to report clinical characteristics and outcomes of ICU-admitted patients with GBS in an academic medical center in Iran. Methods: The data were collected retrospectively from all patients with GBS admitted to Namazi Hospital, affiliated with Shiraz University of Medical Sciences, (Shiraz, Iran), between March 2016 to March 2021. Specialized neurological information and the Acute Physiology and Chronic Health Evaluation (APACHE II) score were recorded. The SPSS software was used to analyze the data. The analyzed data were reported as numbers and percentages, or mean±SD, or median(Interquartile). Results: The study included 132 GBS patients, with an average age of 47.87±15.4 years and a male-to-female ratio of 1.69:1. More than half of the patients (58.5%) were classified as having an axonal disease. In patients with axonal illness, 51.4% of patients had lower limb powers<3, while only 36% of those had the demyelinating disease. This group also required mechanical ventilation more frequently (54% vs. 46%) and for a longer duration (26 [9-37] vs. 10 [1-61]) days. Pneumonia and sepsis were each observed in 16% of patients, and 12% developed a urinary tract infection. The most common type of GBS was acute inflammatory demyelinating polyneuropathy (AIDP). Only 6 (3.8%) patients died. Conclusion: The axonal type of GBS was more frequent, and these patients required mechanical ventilation more frequently and for a longer duration than those in other electrophysiological categories. A preprint version of the manuscript is available at DOI: https://doi.org/10.21203/rs.3.rs-2181605/v1.

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We report the case of a 13-year-old child presenting an unusual case of Guillian-Barre Syndrome (SGB). Its presentation is usually a progression of symmetrical muscle weakness that ascends from the lower extremities, moves toward a more proximal pathway, and is accompanied by absent or depressed tendon reflexes. Here, the patient presented with a rare presentation of Pharyngeal-Cervical-Brachial (PCB) variant of Guillain-Barré syndrome, where the symptomatology began with dysphagia and dyspnea, and the weakness was descending paralysis. The objective of this clinical case report is to highlight this extremely rare presentation of PCB variant of Guillain-Barré syndrome.

RésuméNous décrivons le cas d'un enfant âgé de 13 ans présentant une manifestation inhabituelle du syndrome de Guillain-Barré (SGB). Habituellement, le SGB se caractérise par une faiblesse musculaire symétrique ascendante des deux membres inférieurs, accompagnée d'une perte des réflexes ostéotendineux. Dans ce cas, le patient présentait une variante rare du SGB, appelée la variante Pharyngo-Cervico-Brachiale, où les symptômes ont débuté par une dysphagie et une dyspnée, et la faiblesse musculaire était descendante. L'objectif de cet article est de documenter cette présentation extrêmement rare de la variante Pharyngo-Cervico-Brachiale du syndrome de Guillain-Barré.

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CASE: Spinal deformity associated with Guillain-Barre syndrome (GBS) is not commonly reported. We present a 6-year-old girl who developed kyphoscoliosis after being diagnosed GBS. She had extensive motor deficits requiring 2 hospitalizations and treatment with IV immunoglobulin therapy. Five months after diagnosis, she presented to our clinic with a 15° coronal scoliosis and a 65° thoracic kyphosis. At 6-month follow-up, kyphosis progressed to 77° with no significant change in the coronal curve. At 1 year, sagittal alignment was within normal limits and the coronal curve had completely resolved. CONCLUSION: Spinal deformity in GBS can resolve spontaneously.

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We present a case of a 75-year-old Asian woman with Guillain-Barré syndrome (GBS) who underwent a 1-month comprehensive rehabilitation training program supplemented by robot-assisted gait training (RAGT). GBS can lead to fatigue and prolonged bed rest, thereby further debilitating older patients. Although exercise intervention is recommended for GBS, a consensus regarding the appropriate intensity has yet to be established. Individualized strategies are required because older patients experience varying levels of fatigue and frailty. We used a technological adjunct to support comprehensive rehabilitation for GBS reconditioning in an older patient. To the best of our knowledge, research involving the use of an exoskeleton robotic device in the geriatric population with GBS is limited. Our case demonstrates the feasibility and safety of RAGT for improving lower limb muscle power and scores on the Barthel Index, Clinical Frailty Scale, and Instrumental Activities of Daily Living Scale at discharge from a geriatric ward.

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The objective of the present study was to evaluate the risk factors and outcomes associated with hyponatremia in patients with Guillain-Barré syndrome (GBS). We retrospectively studied 80 consecutive patients with GBS who visited our hospital and compared clinical, laboratory, and electrophysiological findings of patients with and without hyponatremia. Disability was evaluated using the Hughes grading system. Of the 80 patients, 18 (23%) had hyponatremia. Hyponatremia was significantly associated with older age (P = 0.003), urinary retention (P < 0.0001), Hughes grade ≥ 4 at admission and nadir (P = 0.003 and P < 0.001, respectively), acute inflammatory demyelinating polyneuropathy subtype (P = 0.017), sepsis (P = 0.001), mechanical ventilator support (P = 0.013), longer hospitalization length of stay (P < 0.0001), and inability to walk independently at 6 months (P < 0.001). Multivariate analysis performed to assess the risk factors of hyponatremia revealed that urinary retention (odds ratio [OR] 30.7, 95% confidence interval [CI] 3.6-264.4; P = 0.002) and mechanical ventilator support (OR 13.8, 95% CI 1.6-118.0; P = 0.017) were significant independent risk factors of hyponatremia. In assessing the outcomes of patients with hyponatremia, multivariate analysis showed that hyponatremia was independently associated with hospitalization length of stay ≥ 60 days and inability to walk independently at 6 month, with the former showing statistical significance but the latter not (OR 9.3, 95% CI 1.8-47.7; P = 0.007 and OR 4.9, 95% CI 0.9-26.3; P = 0.066, respectively). Therefore, we demonstrate that, along with mechanical ventilator support, urinary retention-possibly indicating autonomic dysfunction-is a risk factor of hyponatremia in GBS. Moreover, we confirm that hyponatremia is associated with poor outcome in GBS.

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Thyroid storm is an endocrine emergency, and treatment must ensure primary goals, including reducing the production and release of thyroid hormones, mitigating the effects of thyroid hormones, increasing the elimination of thyroid hormones, treating systemic disturbances, and managing triggering factors. However, in a few cases where thyroid storm does not respond to initial treatment, therapeutic plasma exchange (TPE) should be considered. A 50-year-old male patient was admitted to the University Medical Center Ho Chi Minh City due to hypotonia and sensory disturbances gradually spreading from the lower extremities to the entire body. The patient was diagnosed with Guillain-Barré syndrome (GBS) and newly discovered hyperthyroidism. During the treatment course, the patient developed hospital-acquired pneumonia, acting as a trigger factor for a thyroid storm. Despite aggressive treatment for thyroid storm, the patient's condition worsened, leading to the decision to perform TPE. The replacement fluid was a combination of fresh frozen plasma (FFP) and albumin 5%. Subsequently, the patient returned to a euthyroid state and was discharged. Combining FFP and albumin 5% in TPE advantages FFP's high thyroid hormones-binding capacity and albumin's cost-effectiveness, safety, and efficiency. This reduces the drawbacks associated with high volumes of FFP and offers a balanced and effective approach to managing thyroid storms. Moreover, the concurrent presence of GBS and thyroid storm is extremely rare. Through this case, we aim to discuss the role of TPE in the treatment of thyroid storms and the effectiveness of the combination of FFP and albumin 5% as the replacement fluid.

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Guillain-Barré syndrome/Miller-Fisher syndrome (GBS/MFS) overlap syndrome is an extremely rare variant of Guillain-Barré syndrome (GBS) in which Miller-Fisher syndrome (MFS) coexists with other characteristics of GBS, such as limb weakness, paresthesia, and facial paralysis. We report the clinical case of a 12-year-old patient, with no pathological history, who acutely presents with ophthalmoplegia, areflexia, facial diplegia, and swallowing and phonation disorders, followed by progressive, descending, and symmetrical paresis affecting first the upper limbs and then the lower limbs. An albuminocytological dissociation was found in the cerebrospinal fluid study. Magnetic resonance imaging of the spinal cord showed enhancement and thickening of the cauda equina roots. The patient was treated with immunoglobulins with a favorable clinical outcome.

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A primigravida in mid 30s presented to hospital at 30+2 weeks gestation, due to progressive neurological symptoms including ascending limb weakness and paraesthesia bilaterally as well as dysphagia, facial weakness and dysphasia.The patient was diagnosed with Guillain-Barré syndrome after physical examination and electromyography, which showed a patchy demyelinating sensorimotor polyneuropathy. The patient underwent a 5-day course of intravenous immunoglobulin, beginning the day after admission. Markers of severity including forced vital capacity improved thereafter until delivery.With limited evidence favouring one particular anaesthetic technique in parturients with Guillain-Barré syndrome, combined spinal epidural anaesthesia was preferred over general anaesthesia in order to avoid the potential for prolonged intubation postoperatively and to allow careful titration of neuraxial blockade. Delivery by caesarean section at 34+1 weeks due to pre-eclampsia was uncomplicated. Thereafter the patient's condition deteriorated, requiring a further 5-day course of intravenous immunoglobulin with symptoms gradually improving over a 6-month admission.

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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.

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BACKGROUND: Guillain-Barré syndrome (GBS) is an autoimmune disorder characterized by demyelination of peripheral nerves. GBS-associated posterior reversible encephalopathy syndrome (PRES) is a rare and potentially life-threatening complication in the pediatric population. We aimed to report and analyze the clinical features, management, and outcomes of three cases of GBS-associated PRES in our setting in the light of the existing literature. METHODS: Medical records of 75 pediatric patients with GBS were reviewed for autonomic changes and GBS-associated PRES. Thirty-one developed dysautonomia while three were identified to have PRES. Clinical, radiological, laboratory, and treatment data were collected and analyzed. RESULTS: All three patients were male and presented with symptoms of acute flaccid paralysis and respiratory distress requiring mechanical ventilation. All three patients experienced various complications, including hypertension, seizures, and hyponatremia, and were subsequently diagnosed with PRES. Multimodal intensive care resulted in patient improvement and discharge in an ambulatory state after an average of 104 days of care. CONCLUSIONS: GBS-associated PRES is a rare and potentially life-threatening complication that can occur in pediatric patients with GBS. Our findings suggest that early recognition, prompt intervention, and multimodal intensive care can improve patient outcomes. Further studies are needed to determine optimal treatment strategies for GBS-associated PRES.

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BACKGROUND AND PURPOSE: Takotsubo cardiomyopathy (TCM) is a serious autonomic complication of Guillain-Barré syndrome (GBS). However, the association between TCM and GBS has not been investigated in detail. We investigated the characteristics of GBS patients with TCM (GBS-TCM). METHODS: Clinical features and anti-ganglioside antibody between the GBS-TCM patients and 62 classical GBS patients without TCM as control patients were compared. RESULTS: Eight GBS-TCM patients were identified, in whom TCM was diagnosed at a mean of 6.5 [range 3-42] days after the onset of GBS. The age at onset of GBS was elder in the GBS-TCM patients than in the control GBS patients (76.5 [56-87] vs. 52 [20-88] years, p < 0.01). Notably, cranial nerve deficits, particularly in the lower cranial nerves, were observed in all GBS-TCM patients (100% vs. 41.9%, p < 0.01). Additionally, the GBS-TCM patients showed a higher GBS disability score at nadir (5 [4-5] vs. 4 [1-5], p < 0.01), and lower Medical Research Council sum scores at admission and nadir (37 [30-44] vs. 48 [12-60] at admission, p < 0.05, and 20 [12-44] vs. 40 [0-60] at nadir, p < 0.05, respectively). Mechanical ventilation was more frequently required in the GBS-TCM patients (62.5% vs. 11.3%, p < 0.01). Three GBS-TCM patients were positive for anti-ganglioside antibodies. CONCLUSIONS: TCM occurred at a relatively early phase of GBS. The characteristics of GBS-TCM were the elder, lower cranial nerve involvements, severe limb weakness, and respiratory failure.

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We discuss a patient who presented with bilateral VI and VII cranial nerve palsies, symmetric upper and lower limb weakness and areflexia, 2 weeks following an flu-like illness. At presentation, there was no papilloedema, and her visual function was normal. Cerebrospinal fluid analysis and electrophysiology supported the diagnosis of Guillain-Barré Syndrome (GBS). She received intravenous immunoglobulins. She subsequently developed headaches and vision loss. Funduscopy demonstrated severe papilloedema with visual acuity of 6/18 right eye, 6/12 left eye with bitemporal visual field depression. Lumbar puncture revealed elevated opening pressure with high protein and normal cell count. She received acetazolamide. There was resolution of papilloedema and normal visual function at 3 months. Of note, the patient's body mass index was 17 kg/m2Our case highlights the rare occurrence of papilloedema in GBS, reiterating the importance of performing funduscopy on patients with any neurological diagnosis. Early detection and prompt management of papilloedema can prevent permanent vision loss.

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Guillain Barre syndrome (GBS) following Varicella zoster is a rare presentation and has only been reported in a few cases around the world. Of the reported cases, the type of GBS is not specified in the majority, and where specified is of the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) type. We report a case of acute motor axonal neuropathy (AMAN) type GBS following herpes zoster in a 27-year-old male who presented with bilateral lower limb weakness and left sided lower motor neuron type facial nerve palsy a week after herpes zoster infection.

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To investigate the clinical characteristics of Guillain-Barré syndrome (GBS) in patients with primary Sjögren's syndrome (SS). Records of patients with positive anti-SSA antibodies hospitalized in the Beijing Tiantan Hospital between December 2011 and May 2020 were retrieved. Patients who fulfilled the criteria for diagnosis of GBS and primary SS were included, and their clinical data were analyzed. Among the 785 patients with positive anti-SSA, 52 patients were identified in this study. They were 27 males and 25 females with median age of 59 years old. Besides anti-SSA antibodies, multiple autoantibodies were detected in these patients including antinuclear antibody, anti-Ro52, anti-mitochondrial M2, anti-thyroid peroxidase and anti-thyroglobulin autoantibodies. Preceding infection was reported in 42 patients. Hyporeflexia/areflexia and limbs weakness were the most common manifestation and 35 patients presented cranial nerve injuries. GBS disability score of 3, 4 and 5 was scaled in 28 (53.8%), 15 (28.8%) and 3 (5.8%) patients respectively. Forty-six patients received intravenous immunoglobulin (IVIG) monotherapy, 5 patients were treated by IVIG plus glucocorticoids, and 51 patients improved during hospitalization. The frequency of male gender among the patients with both GBS and primary SS suggests an independent onset of GBS and the co-existence of these autoimmune diseases in patients with multiple autoantibodies. Majority of patients with GBS and primary SS experience benign disease course.

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Acute generalised muscle weakness in children is a paediatric emergency with a broad differential diagnosis. A careful history and neurologic examination guides timely investigation and management. We review some of the more common causes of acute generalised muscle weakness in children, highlighting key history and examination findings, along with an approach to lesion localisation to guide differential diagnosis and further investigation.

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Background Guillain–Barré syndrome (GBS) is an acute inflammatory polyneuropathy that can lead to respiratory failure. In this study, we evaluate early clinical risk factors for respiratory failure at the time of hospital admission.Methods We studied a retrospective cohort of patients with GBS admitted to a tertiary care center. The potential risk factors studied were sociodemographic characteristics, GBS symptoms, overall and cervical muscle weakness (Medical Research Council [MRC] scores), electromyography findings, and cerebrospinal fluid analysis findings. Unadjusted odds ratios (OR) were calculated and exact logistic regression analysis (adjusted OR) performed to assess the association between baseline risk factors and respiratory failure. Results Overall, 13 of 113 (12%) patients included in the study developed respiratory failure. Unadjusted analyses showed that involvement of any cranial nerve (OR: 14.7; 95% CI, 1.8–117.1), facial palsy (OR: 17.3; 95% CI, 2.2–138.0), and bulbar weakness (OR: 10.7; 95% CI, 2.3–50.0) were associated with increased risk of respiratory failure. Lower MRC sum scores (for scores <30, OR: 14.0; 95% CI, 1.54–127.2) and neck MRC scores (for scores ≤3, OR: 21.0; 95% CI, 3.5–125.2) were associated with higher likelihood of respiratory failure. Adjusted analyses showed that presence of bulbar weakness (OR: 7.6; 95% CI, 1.3–43.0) and low neck MRC scores (scores ≤3, OR: 9.2; 95% CI, 3.5–125.2, vs scores >3) were independently associated with respiratory failure. Conclusions Bulbar and neck muscle weakness at admission are clinical predictors of increased risk of respiratory failure in patients with GBS. These findings could guide the adequate management of high-risk patients. (AU)

Introducción El síndrome de Guillain-Barré es una polineuropatía inflamatoria aguda que puede causar insuficiencia respiratoria. Evaluamos los factores de riesgo clínicos en el momento de la hospitalización. Métodos Realizamos un estudio de una cohorte retrospectiva de pacientes con síndrome de Guillain-Barré hospitalizados en un centro de tercer nivel. Analizamos las características sociodemográficas, síntomas de la enfermedad, fuerza muscular general y cervical (escala del Medical Research Council [MRC]), hallazgos electromiográficos, y resultados del análisis del líquido cefalorraquídeo. Calculamos el odds ratio (OR) sin ajustar y realizamos una regresión logística exacta (OR ajustada) para evaluar la asociación entre los factores de riesgo y la insuficiencia respiratoria. Resultados Trece de los 113 pacientes incluidos (12%) presentó insuficiencia respiratoria. Los análisis no ajustados mostraron una asociación entre mayor riesgo de insuficiencia respiratoria y la afectación de cualquier par craneal (OR: 14,7; IC 95%, 1,8-117,1), parálisis facial (OR: 17,3; IC 95%, 2,2-138,0) y debilidad bulbar (OR: 10,7; IC 95%, 2,3-50,0). Unas puntuaciones más bajas en la MRC-total (puntuaciones <30, OR: 14,0; IC 95%, 1,54-127,2) y en la MRC-cervical (puntuaciones <3, OR: 21,0; IC 95%, 3,5-125,2) se asociaron con una mayor probabilidad de presentar insuficiencia respiratoria. En los análisis ajustados, la presencia de debilidad bulbar (OR: 7,6; IC 95%, 1,3-43,0) y una puntuación baja en la MRC-cervical (puntuaciones ≤3, OR: 9,2; IC 95%, 3,5-125,2, frente a puntuaciones >3) se asociaron de forma independiente con la insuficiencia respiratoria. Conclusiones La presencia de debilidad bulbar y cervical en el momento de la hospitalización es un factor de riesgo de insuficiencia respiratoria en pacientes con síndrome de Guillain-Barré. Estos hallazgos pueden servir de guía para el manejo de los pacientes con mayor riesgo de presentar dicha complicación. (AU)

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Background Guillain–Barré syndrome (GBS) is an acute inflammatory polyneuropathy that can lead to respiratory failure. In this study, we evaluate early clinical risk factors for respiratory failure at the time of hospital admission.Methods We studied a retrospective cohort of patients with GBS admitted to a tertiary care center. The potential risk factors studied were sociodemographic characteristics, GBS symptoms, overall and cervical muscle weakness (Medical Research Council [MRC] scores), electromyography findings, and cerebrospinal fluid analysis findings. Unadjusted odds ratios (OR) were calculated and exact logistic regression analysis (adjusted OR) performed to assess the association between baseline risk factors and respiratory failure. Results Overall, 13 of 113 (12%) patients included in the study developed respiratory failure. Unadjusted analyses showed that involvement of any cranial nerve (OR: 14.7; 95% CI, 1.8–117.1), facial palsy (OR: 17.3; 95% CI, 2.2–138.0), and bulbar weakness (OR: 10.7; 95% CI, 2.3–50.0) were associated with increased risk of respiratory failure. Lower MRC sum scores (for scores <30, OR: 14.0; 95% CI, 1.54–127.2) and neck MRC scores (for scores ≤3, OR: 21.0; 95% CI, 3.5–125.2) were associated with higher likelihood of respiratory failure. Adjusted analyses showed that presence of bulbar weakness (OR: 7.6; 95% CI, 1.3–43.0) and low neck MRC scores (scores ≤3, OR: 9.2; 95% CI, 3.5–125.2, vs scores >3) were independently associated with respiratory failure. Conclusions Bulbar and neck muscle weakness at admission are clinical predictors of increased risk of respiratory failure in patients with GBS. These findings could guide the adequate management of high-risk patients. (AU)

Introducción El síndrome de Guillain-Barré es una polineuropatía inflamatoria aguda que puede causar insuficiencia respiratoria. Evaluamos los factores de riesgo clínicos en el momento de la hospitalización. Métodos Realizamos un estudio de una cohorte retrospectiva de pacientes con síndrome de Guillain-Barré hospitalizados en un centro de tercer nivel. Analizamos las características sociodemográficas, síntomas de la enfermedad, fuerza muscular general y cervical (escala del Medical Research Council [MRC]), hallazgos electromiográficos, y resultados del análisis del líquido cefalorraquídeo. Calculamos el odds ratio (OR) sin ajustar y realizamos una regresión logística exacta (OR ajustada) para evaluar la asociación entre los factores de riesgo y la insuficiencia respiratoria. Resultados Trece de los 113 pacientes incluidos (12%) presentó insuficiencia respiratoria. Los análisis no ajustados mostraron una asociación entre mayor riesgo de insuficiencia respiratoria y la afectación de cualquier par craneal (OR: 14,7; IC 95%, 1,8-117,1), parálisis facial (OR: 17,3; IC 95%, 2,2-138,0) y debilidad bulbar (OR: 10,7; IC 95%, 2,3-50,0). Unas puntuaciones más bajas en la MRC-total (puntuaciones <30, OR: 14,0; IC 95%, 1,54-127,2) y en la MRC-cervical (puntuaciones <3, OR: 21,0; IC 95%, 3,5-125,2) se asociaron con una mayor probabilidad de presentar insuficiencia respiratoria. En los análisis ajustados, la presencia de debilidad bulbar (OR: 7,6; IC 95%, 1,3-43,0) y una puntuación baja en la MRC-cervical (puntuaciones ≤3, OR: 9,2; IC 95%, 3,5-125,2, frente a puntuaciones >3) se asociaron de forma independiente con la insuficiencia respiratoria. Conclusiones La presencia de debilidad bulbar y cervical en el momento de la hospitalización es un factor de riesgo de insuficiencia respiratoria en pacientes con síndrome de Guillain-Barré. Estos hallazgos pueden servir de guía para el manejo de los pacientes con mayor riesgo de presentar dicha complicación. (AU)

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