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.

Severe papilloedema with vision loss secondary to Guillain-Barré syndrome.

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.

Guillain-Barre syndrome of acute motor axonal neuropathy (AMAN) type associated with herpes zoster: a case report.

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.

Practical approach to the child presenting with acute generalised weakness.

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.

Clinical characteristics of Guillain-Barré syndrome in patients with primary Sjögren's syndrome.

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.

Risk factors for respiratory failure among hospitalized patients with Guillain–Barré syndrome / Factores de riesgo de insuficiencia respiratoria en pacientes hospitalizados con síndrome de Guillain-Barré

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)

Risk factors for respiratory failure among hospitalized patients with Guillain–Barré syndrome / Factores de riesgo de insuficiencia respiratoria en pacientes hospitalizados con síndrome de Guillain-Barré

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)

The pathophysiological role of endoneurial inflammatory edema in early classical Guillain-Barré syndrome.

The objective of this review was to analyze the pathophysiological role of endoneurial inflammatory edema in initial stages of classic Guillain-Barré syndrome (GBS), arbitrarily divided into very early GBS (≤ 4 days after symptom onset) and early GBS (≤ 10 days). Classic GBS, with variable degree of flaccid and areflexic tetraparesis, encompasses demyelinating and axonal forms. Initial autopsy studies in early GBS have demonstrated that endoneurial inflammatory edema of proximal nerve trunks, particularly spinal nerves, is the outstanding lesion. Variable permeability of the blood-nerve barrier dictates such lesion topography. In proximal nerve trunks possessing epi-perineurium, edema may increase the endoneurial fluid pressure causing ischemic changes. Critical analysis the first pathological description of the axonal form GBS shows a combination of axonal degeneration and demyelination in spinal roots, and pure Wallerian-like degeneration in peripheral nerve trunks. This case might be reclassified as demyelinating GBS with secondary axonal degeneration. Both in acute motor axonal neuropathy and acute motor-sensory axonal neuropathy, Wallerian-like degeneration of motor fibers predominates in the distal part of ventral spinal roots abutting the dura mater, another feature re-emphasizing the pathogenic relevance of this area. Electrophysiological and imaging studies also point to a predominant alteration at the spinal nerve level, which is a hotspot in any early GBS subtype. Serum biomarkers of axonal damage, including neurofilament light chain and peripherin, are increased in the great majority of patients with any early GBS subtype; endoneurial ischemia of proximal nerve trunks could contribute to such axonal damage. It is concluded that inflammatory edema of proximal nerve trunks is an essential pathogenic event in early GBS, which has a tangible impact for accurate approach to the disease.

Factors Associated with Respiratory Insufficiency in Children with Guillain-Barré Syndrome.

OBJECTIVE: The risk factors for respiratory insufficiency in children with Guillain-Barré syndrome (GBS) are poorly known. This study aimed to investigate the factors associated with respiratory insufficiency in children with GBS. METHODS: This retrospective study included children diagnosed with GBS by pediatric neurologists and admitted at the Wuhan Children's Hospital and other hospitals from January 2013 to October 2022. The patients were divided into the respiratory insufficiency and nonrespiratory insufficiency groups according to whether they received assist breathing during treatment. RESULTS: The median (interquartile range) age of onset of 103 patients were 5 (3.1-8.5) years, 69 (67%) were male, and 64 (62.1%) had a history of precursor infection. Compared with the nonrespiratory insufficiency group, the respiratory insufficiency group showed more facial and/or bulbar weakness (p = 0.002), a higher Hughes Functional Grading Scale (HFGS) at admission (p < 0.001), and a shorter onset-to-admission interval (p = 0.017). Compared with the acute motor axonal neuropathy (AMAN) subtype, the acute inflammatory demyelinating polyneuropathy (AIDP) subtype showed longer days from onset to lumbar (p = 0.000), lower HFGS at admission (p = 0.04), longer onset-to-admission interval (p = 0.001), and more cranial nerve involvement (p = 0.04). The incidence of respiratory insufficiency between AIDP and AMAN showed no statistical difference (p > 0.05). CONCLUSION: In conclusion, facial and/or bulbar weakness, HFGS at admission, and onset-to-admission interval were associated with respiratory insufficiency and might be useful prognostic markers in children with GBS.

Reversible splenial lesion syndrome in patient with acute motor and sensory axonal neuropathy.

Even though the classical clinical concept supports the clear difference between diseases affecting the central and peripheral nervous systems, this difference is becoming less rigid. Here, we report the case of a 50-year-old male patient who presented with acroparaesthesia, headache, and flaccid tetraparesis after febrile diarrhea. Nerve conduction studies indicated action potentials with low amplitudes, which are typical in acute motor and sensory axonal neuropathy. Magnetic resonance revealed a round lesion in the splenium consistent with a diagnosis of reversible splenial lesion syndrome. A polyclonal antiganglioside antibody response was detected. The patient was successfully treated with intravenous immunoglobulins. The coexistence of reversible splenial lesion syndrome and acute motor and sensory axonal neuropathy has not been described in the literature so far. We discuss our diagnostic dilemmas and the possible role of antiganglioside antibodies in the occurrence of simultaneous lesions of the central and peripheral nervous systems.

Cervical Myelopathy with Concomitant Guillain-Barré Syndrome: A Case Report.

CASE: We present a case of a 67-year-old female patient with concomitant cervical spondylotic myelopathy (CSM) and Guillain-Barré syndrome (GBS). Surgical intervention, including cervical decompression and arthrodesis, was performed to address cervical myelopathy symptoms. Despite initial improvement, the patient's motor function deteriorated, leading to the diagnosis of GBS. The patient's hospital course was protracted with a complicated recovery. CONCLUSION: This case emphasizes the clinical details of coexisting CSM and GBS, highlighting the importance of diagnosing and considering demyelinating diseases when determining the optimal timeline for surgical intervention. These findings inform decision-making for clinicians encountering similar patient presentations.

Clinical and laboratory findings in scrub typhus associated Guillain-Barré syndrome in South Korea.

BACKGROUND AND AIMS: Scrub typhus is an endemic disease in the fall season that occurs in a limited number of places known as the Tsutsugamushi Triangle. Peripheral neuropathy is a common complication of scrub typhus. Herein, we encountered several patients with ascending paralysis after scrub typhus infection, who were diagnosed with Guillain-Barré syndrome (GBS). We aimed to investigate the clinical and laboratory characteristics of patients who developed GBS after scrub typhus. METHODS: Patients were retrospectively recruited from six nationwide tertiary centers in South Korea from January 2017 to December 2021. Patients who had been clinically diagnosed with GBS and confirmed to have scrub typhus via laboratory examination and/or the presence of an eschar before the onset of acute limb paralysis were included. The GBS-associated clinical and electrophysiological characteristics, outcomes, and scrub typhus-associated features were collected. RESULTS: Of the seven enrolled patients, six were female and one was male. The median time from scrub typhus infection to the onset of limb weakness was 6 (range: 2-14) days. All patients had eschar on their bodies. Four patients (57.1%) were admitted to the intensive care unit and received artificial ventilation for respiratory distress. At 6 months, the median GBS disability score was 2 (range, 1-4) points. INTERPRETATION: Patients with scrub typhus-associated GBS have a severe clinical presentation and require intensive treatment with additional immunotherapies. Therefore, GBS should be included in the differential diagnosis when peripheral neuropathies develop during scrub typhus treatment. Notably, scrub typhus is associated to GBS.

COVID-19-associated serum and cerebrospinal fluid cytokines in post- versus para-infectious SARS-CoV-2-related Guillain-Barré syndrome.

INTRODUCTION: Guillain-Barré syndrome associated with Coronavirus-2-related severe acute respiratory syndrome (COV-GBS) occurs as para- or post-infectious forms, depending on the timing of disease onset. In these two forms, we aimed to compare the cerebrospinal fluid (CSF) and serum proinflammatory cytokine profiles to evaluate differences that could possibly have co-pathogenic relevance. MATERIALS AND METHODS: We studied a retrospective cohort of 26 patients with either post-COV-GBS (n = 15), with disease onset occurring > 7 days after SARS-CoV-2 infection, or para-COV-GBS (n = 11), with disease onset 7 days or less. TNF-α, IL-6, and IL-8 were measured in the serum with SimplePlex™ Ella™ immunoassay. In addition to the para-/post-COV-GBS patients, serum levels of these cytokines were determined in those with non-COVID-associated-GBS (NC-GBS; n = 43), paucisymptomatic SARS-CoV-2 infection without GBS (COVID, n = 20), and in healthy volunteers (HV; n = 12). CSF cytokine levels were measured in patients with para-/post-COV-GBS, in those with NC-GBS (n = 29), or with Alzheimer's disease (AD; n = 24). RESULTS: Serum/CSF cytokine levels did not differ in para- vs post-COV-GBS. We found that SARS-CoV-2 infection raises the serum levels of TNF-α, IL-6, and IL-8, as well as an increase of IL-6 (in serum and CSF) and IL-8 (in CSF) in either NC-GBS or COV-GBS than controls. CSF and serum cytokine levels resulted independent one with another. CONCLUSIONS: The change of cytokines linked to SARS-CoV-2 in COV-GBS appears to be driven by viral infection, although it has unique characteristics in GBS as such and does not account for cases with para- or post-infectious onset.

Factors Predicting Prolonged Mechanical Ventilation in Guillain-Barré Syndrome.

BACKGROUND: Up to 30% of patients with Guillain-Barré syndrome require mechanical ventilation and 5% die due to acute complications of mechanical ventilation. There is a considerable group of patients that will need prolonged mechanical ventilation (considered as >14 days) and should be considered for early tracheostomy. The objective of this study is to identify risk factors for prolonged mechanical ventilation. METHODS: We prospectively analyzed patients with Guillain-Barré diagnosis with versus without prolonged mechanical ventilation. We considered clinical and electrophysiological characteristics and analyzed factors associated with prolonged mechanical ventilation. RESULTS: Three hundred and three patients were included; 29% required mechanical ventilation. When comparing the groups, patients with prolonged invasive mechanical ventilation (IMV) have a lower score on the Medical Research Council score (19.5 ± 16.2 vs 27.4 ± 17.5, p = 0.03) and a higher frequency of dysautonomia (42.3% vs 19.4%, p = 0.037), as well as lower amplitudes of the distal compound muscle action potential (CMAP) of the median nerve [0.37 (RIQ 0.07-2.25) vs. 3.9 (RIQ1.2-6.4), p = <0.001] and ulnar nerve [0.37 (RIQ0.0-3.72) vs 1.5 (RIQ0.3-6.6), p = <0.001], and higher frequency of severe axonal damage in these nerves (distal CMAP ≤ 1.0 mV). Through binary logistic regression, severe axonal degeneration of the median nerve is an independent risk factor for prolonged IMV OR 4.9 (95%CI 1.1-21.5) p = 0.03, AUC of 0.774, (95%CI 0.66-0.88), p = < 0.001. CONCLUSIONS: Severe median nerve damage is an independent risk factor for prolonged mechanical ventilation.

Long-term outcomes of paediatric Guillain-Barré syndrome.

AIM: To study long-term sequelae in children with Guillain-Barré syndrome (GBS). METHOD: This was a prospective observational study with children from two French tertiary centres. Data were from clinical and several standardized scales or questionnaires. RESULTS: Fifty-one patients were included with a median follow-up of 6 years 4 months (range 3-20 years) after the acute phase. The sequelae rate was 67% (95% confidence interval [CI] 53-78) and did not vary with time. Most children had minor sequelae (Guillain-Barré Syndrome Disability Score [GBSDS] = 1); only one was unable to run (GBSDS = 2). The most frequent complaints were paraesthesia (43%), pain (35%), and fatigue (31%). The neurological examination was abnormal in 18% of children, autonomy was compromised in 14%, and symptoms of depression occurred in 34%. The factors associated with late-onset sequelae were correlated with severity during the initial phase (i.e. initial GBSDS >4, odds ratio 6.6, 95% CI 1.8-33; p = 0.009). The predictive factors of more severe late-onset conditions were initial severity (p = 0.002) and sex (female patients; p = 0.01). INTERPRETATION: Two-thirds of children with GBS had late-onset sequelae following an episode, often minor, but sometimes with continuing effects on their everyday lives. Particularly affected were those who had severe GBS during the acute phase and who lost the ability to walk. WHAT THIS PAPER ADDS: Two-thirds of children with Guillain-Barré syndrome (GBS) had persistent sequelae. Sequelae were often minor, but daily repercussions of them were sometimes serious. Sequelae were significantly associated with severe GBS during the acute phase.

Retrospective analysis of COVID-19 patients with Guillain-Barre, Miller-Fisher, and opsoclonus-myoclonus-ataxia syndromes-a case series.

BACKGROUND: In accordance with the rising number of SARS-CoV­2 infections, reports of neurological complications have also increased. They include cerebrovascular diseases but also immunological diseases such as Guillain-Barre syndrome (GBS), Miller-Fisher syndrome (MFS), and opsoclonus-myoclonus-ataxia syndrome (OMAS). While GBS and MFS are typical postinfectious complications, OMAS has only recently been described in the context of COVID-19. GBS, MFS, and OMAS can occur as para- and postinfectious, with different underlying pathomechanisms depending on the time of neurological symptom onset. The study aimed to describe clinical features, time between infection and onset of neurological symptoms, and outcome for these diseases. METHODS: All COVID-19 patients treated in the neurological ward between January 2020 and December 2022 were screened for GBS, MFS, and OMAS. The clinical features of all patients, with a particular focus on the time of onset of neurological symptoms, were analyzed. RESULTS: This case series included 12 patients (7 GBS, 2 MFS, 3 OMAS). All GBS and one MFS patient received immunomodulatory treatment. Three patients (2 GBS, 1 OMAS) had a severe COVID-19 infection and received mechanical ventilation. In patients with OMAS, only one patient received treatment with intravenous immunoglobulin and cortisone. The remaining two patients, both with disease onset concurrent with SARS-COV­2 infection, recovered swiftly without treatment. In all subgroups, patients with concurrent onset of neurological symptoms and COVID-19 infection showed a trend toward shorter disease duration. CONCLUSION: All patient groups displayed a shorter disease duration if the onset of neurological symptoms occurred shortly after the COVID-19 diagnosis. In particular, both the OMAS patients with symptom onset concurrent with COVID-19 showed only abortive symptoms followed by a swift recovery. This observation would suggest different pathomechanisms for immune-mediated diseases depending on the time of onset after an infection.

Reversible cerebral vasoconstriction syndrome in Guillain-Barre syndrome: a case report and literature review.

BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by transient constriction of cerebral arteries, leading to severe headache and potential complications. The association between RCVS and Guillain-Barre syndrome (GBS) is rare and poorly understood and warrants further investigation. METHODS: A detailed case of RCVS in a patient with GBS was presented, followed by a comprehensive literature review. PubMed, Embase, and Google Scholar were searched for relevant cases and studies. RESULTS: The case involved a 62-year-old woman with GBS who developed RCVS. The literature review identified three additional reported cases. RCVS in GBS primarily affected middle-aged women and presented with a variety of neurological symptoms. Neuroimaging showed reversible vasoconstriction in the cerebral arteries, along with other complications such as posterior reversible encephalopathy syndrome, subarachnoid hemorrhage, and infarcts. While the treatment for GBS consisted mainly of intravenous immunoglobulin, specific treatments for RCVS remain unclear. CONCLUSIONS: The coexistence of RCVS and GBS is a rare occurrence. RCVS in GBS may result from the disruption of cerebral vascular tone regulation, possibly influenced by GBS-related dysautonomia and consequent high blood pressure. Recognizing RCVS in GBS patients is critical for appropriate management.

Diagnostic odyssey of Guillain-Barré syndrome in children.

BACKGROUND AND OBJECTIVES: A gap exists between difficulty in diagnosis and importance of early recognition and intervention in pediatric Guillain-Barré syndrome (GBS). Therefore, this study aimed to establish a diagnostic odyssey plot that allows "at-a-glance" overview of the diagnostic odyssey of GBS in children, including overall diagnostic delay, physician-related and patient-related diagnostic delays, and length and frequency of diagnostic errors. METHODS: In this single-center retrospective cohort study, standardized data were obtained from children with GBS from 2003 to 2020. Overall diagnostic delay (time between symptom onset and diagnosis), physician-related diagnostic delay (time between the first medical visit and diagnosis), and patient-related diagnostic delay (time between symptom onset and the first medical visit) were analyzed. RESULTS: The study examined a total of 21 patients (11 men, median age 4.5 years). Overall, there were 40 misdiagnoses among 17 patients, while four were diagnosed correctly at the first visit. The overall diagnostic delay was 9 days [interquartile range (IQR), 6-17 days]. Physician-related diagnostic delay, but not patient-related diagnostic delay, was correlated with the overall diagnostic delay. Patients in the late-diagnosed group were more frequently misdiagnosed during their diagnostic odyssey than patients in the other groups. Risk factors associated with diagnostic delay included delayed onset of weakness and sensory deficits, absence of swallowing problems, and misdiagnosis as orthopedic disorders or viral infections. DISCUSSION: A unique diagnostic odyssey exists in pedaitric GBS. Several clinical risk factors were associated with the diagnostic delay.