Recurrent simultaneous central nervous system demyelination with possible peripheral demyelination / nodopathy in a seronegative patient.

Combined central and peripheral demyelination (CCPD) is a rare disease entity. Onset with the simultaneous central nervous system (CNS) and peripheral nervous system (PNS) involvement and its recurrence are exceptional. Anti-neurofascin antibodies have been shown to be present in up to 70% of cases, yet seronegative patients also exist. We present a case of seronegative recurrent CCPD. The PNS involvement was compatible with two episodes of recurrent Guillain-Barre syndrome (GBS), whereas the CNS involvement pattern was not typical for either multiple sclerosis (MS) or acute disseminated encephalomyelitis. The prognosis was excellent with pulse methylprednisolone, intravenous immunoglobulin, and plasmapheresis. This case highlights the varied clinical presentations of CCPD, extending beyond the realms of MS and chronic inflammatory demyelinating polyneuropathy, and underscores the potential for relapse. Importantly, to the best of our knowledge, this represents the inaugural instance of CCPD featuring PNS involvement in the form of recurrent GBS.

Case report: Rare Guillain-Barré syndrome variants and mild encephalitis/encephalopathy with a reversible splenial lesion as the para-infectious manifestations of SARS-CoV-2 infection.

Background: The Coronavirus disease 19 (COVID-19), caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is a threat to human health. Although the COVID-19 pandemic is finished, some peoples are still suffering from this disease. Herein, we report the first case of SARS-CoV-2-associated Guillain-Barré syndrome (GBS) presenting as polyneuritis cranialis (PNC) and acute panautonomic neuropathy (APN) variants, accompanied by mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) and hyponatremia. Case presentation: A 32-year-old female patient with symptoms indicating multiple cranial nerve involvement, as well as sympathetic and parasympathetic nervous system dysfunction, was diagnosed as SARS-CoV-2-associated GBS presenting as PNC and APN variants, accompanied by MERS and hyponatremia. Following treatment with immunoglobulin, methylprednisolone, and symptomatic care, the patient's inflammatory cytokines and serum sodium became normal. However, some residual symptoms such as postural hypotension, fatigue, and mild dysarthria persisted at the 9-month follow-up. Conclusion: This case highlights the unique presentation of SARS-CoV-2 infection. The involvement of both the central nervous system (CNS) and the peripheral nervous system (PNS) in this case underscores the complex neurological manifestations of COVID-19. Although the exact underlying pathogenesis of this case is unclear, inflammatory cytokines, particularly IL-6, may be implicated. Further research is needed to better understand the mechanisms underlying these complications and to optimize treatment strategies for affected patients.

Guillain-Barré syndrome with overlap between the finger drop variant and acute bulbar palsy: a case report.

BACKGROUND: Guillain-Barré syndrome (GBS) is a clinically heterogenous disease and encompasses several distinct clinical variants. Overlap between these variants can pose a diagnostic challenge. We report a case of finger drop variant and acute bulbar palsy overlap as an unusual manifestation of GBS. CASE PRESENTATION: An 81-year-old man presented with dysarthria, dysphagia, and upper limb weakness. Neurological examination revealed impaired tongue protrusion, the finger drop sign, and diminished brachioradial and triceps muscle reflexes. Nerve conduction studies showed reduced amplitudes and decreased velocities in the median and ulnar nerves. Cerebrospinal fluid analysis revealed albuminocytological dissociation and an anti-ganglioside antibody study revealed positivity for GM1, asialo-GM1, GT1a, GD1b, and GQ1b. As GBS was suspected, we initiated intravenous immunoglobulin treatment, resulting in gradual improvement within the next 3 weeks. CONCLUSION: To the best of our knowledge, this is the first reported case of an overlap between the finger drop variant and acute bulbar palsy in GBS, highlighting the importance of considering GBS when patients present with a combination of atypical symptoms. Anti-ganglioside antibodies can be helpful and add diagnostic value in these complex cases.

Clinical features of Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuritis associated with SLE.

OBJECTIVE: We report on the clinical characteristics, treatments and outcomes of Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuritis (CIDP) associated with SLE. METHODS: Patients treated at Peking Union Medical College Hospital between January 2004 and November 2021 who fulfilled the diagnostic criteria for SLE and GBS/CIDP (n=9) were included. Clinical presentations, lab results, treatment regimens and prognoses were retrieved and analysed. RESULTS: Six patients were diagnosed with SLE and GBS, while three were diagnosed with SLE and CIDP, with the average age at diagnosis of 38.6±18.2 years. SLE disease duration ranges from 1 week to 36 years, and the courses of GBS and CIDP range from 1 week to 2 months and from 2 months to 15 months, respectively. All patients exhibited either or both limb paresthesia and weakness, other neurological symptoms include dysphagia, peripheral facial nerve palsy and respiratory and cardiac arrest. The median cerebral spinal fluid white blood cell count and protein level were 0.002×109/L (0-0.006×109/L) and 0.79 g/L (0.57-7.09 g/L), respectively. All patients received glucocorticoid and immunoglobulin therapy. Seven patients received cyclophosphamide, and seven patients received intrathecal injections of methotrexate and dexamethasone. Two patients had complete resolution, five experienced marked improvements and two failed to improve with treatments. CONCLUSION: SLE-associated GBS/CIDP may manifest regardless of disease systemic activity. Clinical features may differ from that of pure GBS/CIDP, and treatment often requires immunosuppressants, making differential diagnosis crucial, especially for patients with GBS/CIDP presenting as the first manifestation of SLE.

Claw Hand in Acute Motor Axonal Neuropathy Variant.

BACKGROUND: Guillain-Barré syndrome (GBS) is an acute autoimmune polyradiculoneuropathy with various subtypes, including the acute motor axonal neuropathy (AMAN) variant. Distal muscle weakness is typically rare in AMAN. Myositis, an inflammatory muscle condition, is infrequently documented in GBS. This case report presents an unusual presentation of GBS with unilateral claw hand and myositis. CASE DESCRIPTION: A 55-year-old male presented with bilateral limb pain and weakness, progressing to significant motor impairment over 5 days. Symptoms began after a brief febrile illness with gastrointestinal distress. Upon examination, the patient exhibited decreased muscle strength in all limbs, dysphagia, and partial clawing of the left hand. Neurological assessment showed cranial nerve involvement and dysautonomia. Blood tests revealed elevated creatine phosphokinase (CPK) levels, and cerebrospinal fluid (CSF) analysis showed high protein without cellular abnormalities. Diagnosed with the AMAN variant of GBS, the patient was treated with intravenous immunoglobulin (IVIG) and antibiotics. Physiotherapy for speech, limbs, chest, and swallowing was initiated. Gradual improvement was observed, with increased limb power by the third week, although swallowing difficulties persisted longer. CONCLUSION: This case highlights a rare presentation of the AMAN variant of GBS with unilateral claw hand and myositis. The findings suggest that elevated CPK levels in GBS may not directly indicate myositis but could be secondary to the syndrome. Prompt diagnosis and treatment of the patient for recovery have been emphasized. This report underlines the need to consider GBS in patients presenting with atypical motor impairments and elevated CPK levels.

Neurogenic Heterotopic Ossification of the Hip: a Case Report.

Heterotopic ossification (HO) denotes aberrant osteogenesis in extra-skeletal tissues, often associated with neurological disorders, total hip arthroplasty, and specific traumatic scenarios. Neurogenic heterotopic ossification manifests prominently subsequent to traumatic brain injury or spinal cord injury, with Guillain-Barre Syndrome presenting an infrequent etiological link. This article details the case of a 56-year-old female diagnosed with Guillain-Barre Syndrome, who developed neurogenic heterotopic ossification around both hips within two years of disease onset. The patient's medical history included mechanical ventilation, incomplete tetraplegia, and prolonged immobilization. A conclusive diagnosis of HO was established through radiological and clinical assessments. After neurogenic heterotopic ossification was confirmed, the patient had surgery to remove the lesions, radiation therapy, and medication treatments as planned. Physical therapy was introduced one week post-surgery, with subsequent follow-ups tracking improvements in pain levels, range of motion (ROM), and Activities of Daily Living scores. Key words: neurogenic heterotopic ossification, Guillain-Barre syndrome, hip, excision.

[Immune-mediated neuropathies: pathophysiology and management].

Immune-mediated neuropathies (IMN) are a heterogenous group of disorders affecting the peripheral nervous system, due to dysregulation of the immune system. It mainly includes Guillain-Barre syndrome, chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy and so on. Most of these diseases can be clinically improved by appropriate immunotherapy, but some patients still have unsatisfactory results. Therefore, studying the pathophysiology of the occurrence and development of diseases can reveal the nature of diseases and provide a theoretical basis for the prevention, diagnosis and treatment of diseases. In this paper, the pathophysiological mechanism of various IMNs is described in detail, with emphasis on immunological mechanism, and the progress of diagnosis and treatment of various IMNs is briefly introduced.

Risk Stratification and Management of Acute Respiratory Failure in Patients With Neuromuscular Disease.

OBJECTIVES: Guillain-Barré syndrome (GBS) and myasthenia gravis (MG) are the most common causes of acute neuromuscular respiratory failure resulting in ICU admission. This synthetic narrative review summarizes the evidence for the prediction and management of acute neuromuscular respiratory failure due to GBS and MG. DATA SOURCES: We searched PubMed for relevant literature and reviewed bibliographies of included articles for additional relevant studies. STUDY SELECTION: English-language publications were reviewed. DATA EXTRACTION: Data regarding study methodology, patient population, evaluation metrics, respiratory interventions, and clinical outcomes were qualitatively assessed. DATA SYNTHESIS: No single tool has sufficient sensitivity and specificity for the prediction of acute neuromuscular respiratory failure requiring mechanical ventilation. Multimodal assessment, integrating history, examination maneuvers (single breath count, neck flexion strength, bulbar weakness, and paradoxical breathing) and pulmonary function testing are ideal for risk stratification. The Erasmus GBS Respiratory Insufficiency Score is a validated tool useful for GBS. Noninvasive ventilation can be effective in MG but may not be safe in early GBS. Airway management considerations are similar across both conditions, but dysautonomia in GBS requires specific attention. Extubation failure is common in MG, and early tracheostomy may be beneficial for MG. Prolonged ventilatory support is common, and good functional outcomes may occur even when prolonged ventilation is required. CONCLUSIONS: Multimodal assessments integrating several bedside indicators of bulbar and respiratory muscle function can aid in evidence-based risk stratification for respiratory failure among those with neuromuscular disease. Serial evaluations may help establish a patient's trajectory and to determine timing of respiratory intervention.

Correlation of Clinical and Electrophysiological Criteria in the Diagnosis of Guillain-Barré Syndrome: A Prospective Observational Study.

INTRODUCTION: There are many criteria for diagnosing Guillain-Barré syndrome (GBS), and the yield of these diagnostic criteria varies. Each criterion requires some laboratory data and nerve conduction studies (NCS). Although supportive laboratory data are reassuring when present in suspected cases of GBS, when absent, they can potentially cause further delay in diagnosis and treatment. There is no gold standard test for the diagnosis of GBS, and there are multiple diagnostic criteria for GBS to date. The aim of the study is to know the sensitivity of different criteria, clinical and electrophysiological, for the diagnosis of GBS and to study the clinical spectrum and electrophysiological spectrum of GBS in our cohort of patients. MATERIALS AND METHODS: We studied a total of 43 cases who presented with one or more of the following symptoms: relatively symmetrical and progressive flail-type weakness of more than one limb, with or without ataxia and/or ophthalmoplegia, and were diagnosed with GBS according to clinical criteria at the time of admission to Government Stanley Medical College Hospital. GBS mimics were ruled out. In all patients, the demographic data, cerebrospinal fluid (CSF) analysis (if done), and electrophysiological findings fitting into the diagnostic criteria of National Institute of Neurological Disorders and Stroke (NINDS), Dutch, and Brighton criteria were recorded. The need for assisted mechanical ventilation, neurology intensive care unit (NICU) stay, any complications, and treatment outcome details were recorded in a structured proforma. RESULTS: Most of the patients in our study were in their fourth decade of life, with a mean age of 41.37 years, similar to previous studies from India. Men are more frequently affected compared to women, similar to what has been observed in most studies done previously worldwide. In our study, electrophysiological criteria by Dutch criteria (87.5%), Brighton criteria (87.5%), and NINDS criteria (85.6%) had low sensitivity compared to the clinical criteria. CONCLUSION: In the present study, electrophysiological criteria proposed by the NINDS, Dutch, and Brighton criteria are less sensitive compared to clinical criteria in diagnosing GBS at early stages. Clinical criteria alone may be useful in resource-poor countries and at peripheral healthcare systems where NCS are not always readily available.

Case Report: AMSAN variant of GBS complicating immediate postpartum period with severe dysautonomia leading to Posterior Reversible Encephalopathy Syndrome.

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.

Controversies in COVID-19: Diagnostic Evaluation and Management in Neuro-COVID.

SARS-CoV-2 is a virus in the coronavirus family originating out of Wuhan, China clinically known as COVID-19. While traditionally thought of as a respiratory virus, COVID-19 can have a multi-organ impact due to its invasion and widespread distribution throughout the body and via the angiotensin converting enzyme. Neurologic events due to COVID-19 are common, especially in the critically ill, and are called Neuro-COVID. Among these events are peripheral and central nervous system diseases such as Guillain-Barré, ischemic stroke, and various types of encephalitis. The impact of Neuro-COVID is devastating and is often far more severe than its non-COVID-19 form. Immunosuppressive or immunomodulatory therapy is often a mainstay of treatment, such as for encephalitis and Guillain-Barré, respectively, while management may fall in line with conventional processes in most cases, such as ischemic stroke. Much remains to be studied about the evaluation and management of Neuro-COVID.

Antibodies in immune-mediated peripheral neuropathies. Where are we in 2024?

Over the past 30 years, about 20 antibodies have been identified in immune-mediated neuropathies, recognizing membrane or intracellular proteins or glycolipids of neuron and Schwann cells. This article reviews the different methods used for their detection, what we know about their pathogenic role, how they have helped identify several disorders, and how they are essential for diagnosis. Despite sustained efforts, some immune-mediated disorders still lack identified autoantibodies, notably the classical form of Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy. The reasons for this are discussed. The article also tries to determine potential future developments in antibody research, particularly the use of omic approaches and the search for other types of biomarkers beyond diagnostic ones, such as those that can identify patients who will respond to a given treatment.

Complex Interplay of COVID-19 ARDS with Guillain-Barré Syndrome and Cerebral Infarction: A Case Study.

BACKGROUND Coronavirus disease (COVID-19) can cause various complications. We report a case of severe COVID-19 acute respiratory distress syndrome (ARDS) in a patient receiving veno-venous extracorporeal membrane oxygenation (V-V ECMO), complicated by Guillain-Barre syndrome (GBS) and cerebral infarction, as well as pulmonary embolism. CASE REPORT A 55-year-old Japanese man with a history of ulcerative colitis was admitted for COVID-19. His respiratory status worsened and progressed to ARDS, requiring intubation on hospital day (HD) 3. On HD 16, contrast computed tomography revealed PE. On HD 18, his respiratory condition worsened, and V-V ECMO was initiated. On HD 23, V-V ECMO was successfully discontinued. He regained consciousness on HD 44, but he had quadriplegia. Deep-tendon reflexes were absent in all limbs. Cranial nerve involvement, specifically bilateral facial nerve weakness, was noted. Magnetic resonance imaging showed bilateral scattered cerebral infarctions on HD 76. Nerve conduction studies indicated severe axonal neuropathy. Cerebrospinal fluid examination showed albuminocytologic dissociation. The antibody to the ganglioside GD1a was positive. These findings were consistent with the diagnosis of GBS. He received immunoglobulin treatment on HD 89, and his neurological findings slightly improved. CONCLUSIONS This study emphasized that in COVID-19, neurological complications are not rare, are difficult to diagnose, and are prone to delays in detection.

The Initial Clinical and Electrophysiological Characteristics of Different Subtypes of Guillain-Barré Syndrome Diagnosed Based on Serial Electrophysiological Examinations.

BACKGROUND: We aimed to identify different Guillain-Barré syndrome (GBS) subtypes, demyelination, axonal degeneration, and reversible conduction failure (RCF) as early as possible by analyzing the initial clinical and electrophysiological examinations. METHODS: This study retrospectively collected GBS patients between October 2018 and December 2022 at Beijing Tiantan Hospital. The diagnostic criteria for the initial electrophysiological study were based on Rajabally's criteria, and the criteria for the serial electrophysiological study were based on Uncini's criteria. All subjects underwent clinical and electrophysiological evaluations at least twice within 8 weeks. RESULTS: A total of 47 eligible patients with GBS were included, comprising 19 acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 18 axonal degenerations, and 10 RCFs. In the RCF group, 40%, 30%, and 30% patients were diagnosed as AIDP, axonal, and equivocal at the initial study, respectively. The AIDP group had significantly higher cerebrospinal fluid (CSF) protein than the RCF (123.8 [106.4, 215.1] mg/dL vs. 67.1 [36.8, 85.6] mg/dL, p = 0.002) and axonal degeneration (123.8 [106.4, 215.1] mg/dL vs. 60.8 [34.8, 113.0] mg/dL, p < 0.001) groups. The RCF group had significantly lower Hughes functional grades at admission (3 [2, 4] vs. 4 [4, 4], p = 0.012) and discharge (1.0 [1.0, 2.0] vs. 3.0 [2.0, 3.0], p < 0.001) than the axonal degeneration group and showed significantly shorter distal motor latency (DML), Fmin, Fmean, Fmax, and lower F% than the AIDP group (p < 0.05). DISCUSSION: The early identification of RCF from AIDP had relatively obvious features, including slightly elevated CSF protein levels and normal or slightly prolonged DML and F-wave latencies, contrasting with the apparent elevation and prolongation seen in AIDP. Differentiating RCF from axonal degeneration remains challenging. One potential distinguishing factor is that the motor function in RCF tends to be better than in the latter.

Dysphagia Revealing a Pharyngeal-cervical-brachial Variant of Guillain-Barré Syndrome in Pediatric Cases.

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.

Maternal and newborn outcomes in pregnancies complicated by Guillain-Barré syndrome.

OBJECTIVES: Guillain-Barré syndrome (GBS) is a rare autoimmune disorder that affects the peripheral nervous system. The purpose of our study was to evaluate maternal and fetal/neonatal outcomes among pregnancies complicated by GBS. METHODS: We performed a retrospective cohort study using the Healthcare Cost and Utilization Project - National Inpatient Sample from the United States. ICD-9 codes were used to identify all pregnant women who delivered between 1999 and 2015 and had a diagnosis of GBS. The remaining women without GBS who delivered during that time period constituted the comparison group. The associations between maternal GBS and obstetrical and fetal/neonatal outcomes were evaluated using multivariate logistic regression, while adjusting for the confounding effects of maternal characteristics. RESULTS: Of 13,792,544 births included in our study, 291 were to women with GBS, for an overall incidence of 2.1/100,000 births. A steady increase in maternal GBS was observed over the study period (from 1.26 to 3.8/100,000 births, p=0.02). Further, women with GBS were more likely to have pregnancies complicated by preeclampsia, OR 1.69 (95 % CI 1.06-2.69), sepsis, 9.30 (2.33-37.17), postpartum hemorrhage, 1.83 (1.07-3.14), and to require a transfusion, 4.39 (2.39-8.05). They were also at greater risk of caesarean delivery, 2.07 (1.58-2.72) and increased length of hospital stay, 4.48 (3.00-6.69). Newborns of women with GBS were more likely to be growth restricted, 2.50 (1.48-4.23). CONCLUSIONS: GBS in pregnancy is associated with maternal and newborn adverse outcomes. These patients would benefit from close follow-up throughout their pregnancy and in the postpartum period.