Course Of Guillain-Barré Syndrome Research Articles

Особливості маніфестації неврологічних симптомів гострих полінейропатій та їх диференційна діагностика в дітей при коронавірусній хворобі (COVID-19)

The relevance of the problem of diagnosis and treatment of polyneuropathy in children is due to the diverse clinical manifestations of this pathology, which are often acute and can lead to severe, life-threatening complications. Guillain-Barré syndrome (GBS) refers to acute or subacute, often post-infectious, immune-mediated polyneuropathies accompanied by axonal damage to nerve trunks and peripheral nerves. The present study is devoted to the assessment of neurological symptoms in the acute course of GBS, including its differential diagnosis with musculoskeletal lesions in coronavirus disease (COVID-19) in childhood. Purpose - to determine the features of the debut and characterize the neurological symptoms of acute polyneuropathies in children, including GBS in COVID-19. Materials and methods. We examined 10 children with confirmed GBS. Clinical-anamnestic, general clinical, clinical-neurological, clinical-instrumental and clinical-laboratory methods of examination were used. Results. In 1 of the examined patients aged 6 years, GBS developed in the acute period of COVID-19 and manifested as acute flaccid paresis in the distal, and after 4 days - severe weakness of the thigh muscles in combination with leg pain. In 8 of the examined patients, the debut of GBS occurred within 7-21 days after an acute respiratory viral infection. Of these, 6 children had the acute phase of COVID-19 14-21 days before the onset of GBS, which was confirmed by the results of an oropharyngeal PCR-test. The acute phase of COVID-19 in these patients was manifested by fever up to 38.0º, ague, hyposmia, lasting from 3 to 5 days. Severe pain in the legs was noted in all patients with GBS. The absence of elevated levels of creatine phosphokinase, alanine aminotransferase, aspartate aminotransferase according to biochemical blood tests in patients and the absence of myoglobin in the urine excluded rhabdomyolysis of skeletal muscles. Electroneuromyography (ENMG) confirmed the neural type of lesion in the legs. Conclusions. In most patients, the debut of GBS occurred 3 weeks after the acute phase of COVID-19. Pain syndrome and symmetrical flaccid paresis were the leading symptoms of the course of GBS in children. Reduced excitation conduction velocity along the motor fibres of the tibial and peroneal nerves, according to the results of ENMG, allowed confirming the diagnosis of GBS. Differential diagnosis of GBS with musculoskeletal lesions, in particular, the exclusion of the diagnosis of rhabdomyolysis in COVID-19 in children, allowed for timely prescription of adequate therapy. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of participating institutions. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

Postcovid Guillain-Barré syndrome with severe course - case series two patients including clinical evaluation of smell and examination of olfactory event-related potentials (OERPs).

We report a case series two patients of Guillain-Barré syndrome (GBS) associated with previous COVID-19 that both patients survived. GBS is an immune-mediated disease that affects peripheral nerves and can cause life-threatening complications. In both cases (53-year-old female and 59-year-old male) with severe GBS with complications, the smell of sense was investigated subjectively using Sniffin' sticks identification tests and objectively using objective olfactometry by the evaluation of olfactory event-related potentials (OERPs). Both patients had good results of the subjective Sniffin' sticks identification test without patholgical findings. Results of objective examination of OERPs: the P2-N1 wave complex was equipotent. No olfactory disturbance could be detected in either case, OERPs were plentiful in both cases. The presentation of a case series two patients of post-covid GBS are an example of one of the many complications of COVID-19 that can cause prolonged recovery. Despite the severe course of GBS and the long recovery time, both patients returned to normal life. An expanded prospective study is planned for the future to investigate post-covid olfactory impairment. The prevalence of GBS associated with COVID-19 is still unknown but it is evident that both mild and severe forms of GBS have been described in patients.

Detection of anti-ganglioside antibodies in Guillain-Barré syndrome.

Gangliosides are a class of glycosphingolipid molecules that are highly enriched in cellular membranes of the nervous system. The gangliosides associated with autoimmune diseases of the nervous system are mainly GM1, GD1a, GalNAc-GD1a, GM1b, GD3, CD1b, GT1a, and GQ1b. Multiple antibodies recognizing gangliosides are associated with some acute or chronic peripheral neuropathies, especially Guillain-Barré syndrome (GBS) and its clinical variants. Antibodies binding to gangliosides can activate complement system and recruit macrophages on the axolemma at the nodes of Ranvier of motor fibers, which are found in the course of GBS, causing axonal degeneration and reversible conduction block or conduction failure. Testing of anti-gangliosides autoantibodies is helpful for diagnosis of autoimmune peripheral neuropathies or support the diagnosis of the subtypes. These anti-gangliosides antibodies are usually detected by several qualitative or quantitative methods, particularly enzyme-linked immunosorbent assay (ELISA) and immunodot assays, which have been commercialized or established in-house worldwide. Herein, we introduce the methods and clinical applications of these assays in the diagnosis of autoimmune peripheral neuropathies. Anti-gangliosides antibodies are diagnostic markers of GBS subtypes. We use GBS as an example to explain the role of anti-gangliosides antibodies in the pathogenesis and diagnostic classification of neuropathies.

Radical radiotherapy for cervical cancer and meningioma with a history of Guillain-Barré syndrome: A case report

Guillain-Barré syndrome (GBS) is a rare autoimmune disease. Patients with cervical malignancies and intracranial meningiomas after the course of GBS are even rarer. There are no relevant reports at present. We report a patient who developed cervical cancer (CC) and intracranial meningioma simultaneously after the course of GBS. The history, pelvic enhanced magnetic resonance imaging (MRI) and pathology confirmed cervical squamous cell carcinoma, and the head enhanced MRI confirmed meningioma. After multi-disciplinary team, the patient received head stereotactic radiosurgery for meningioma and radical radiotherapy for CC. The follow up for 1 year after treatment revealed a complete remission of the cervical tumor, stable disease of the meningioma, and no signs of GBS recurrence. Up to now, the general condition of the patient is acceptable and the curative effect is satisfactory. This case report aims to improve the oncologists' understanding of malignant tumors with rare autoimmune diseases and provide treatment reference for similar diseases in the future.

Intravenous immunoglobulin treatment with prognosis for the first six months of Guillain-Barré Syndrome in Somalia: Case series.

Guillain-Barré Syndrome (GBS) is an acute, immune-mediated polyneuropathy that often leads to severe weakness. Intravenous Immunoglobulin (IVIG) is a proven effective treatment for GBS (class 1 evidence). The clinical course of GBS in individual patients is highly variable and difficult to predict. It is a retrospective observational study of 10 patients diagnosed with GBS by using nerve conduction studies and lumbar puncture. Fifty percent of the patients were under 40 years old, and ninety percent were male; all but one were treated with IVIG; and forty percent of the total mEGOS obtained was less than 5. The average predicted probability of being unable to walk unaided after 4 weeks was 47.7%, the average predicted probability of being unable to walk unaided after 3 months was 17%, and the average predicted probability of being unable to walk unaided after 6 months was 8.05%. Patients presenting with acute ascending weakness should be identified early, and early IVIG treatment for GBS improves disability as measured by The Modified Erasmus GBS Outcome Scale (mEGOS).

Natural course of Guillain-Barré syndrome.

In this retrospective study based on a registry, Kalita et al. [1] compared the outcomes of 189 patients with Guillain-Barré syndrome (GBS) who received intravenous immunoglobulin (IVIg), with 199 age- and peak disability-matched patients who did not receive any immunomodulation or plasmapheresis, thus representing the natural course of GBS. Numbers for in-hospital deaths and poor recovery at 3 months were similar between the two groups. At 6 months, 8.3% of the natural course subgroup, but only 2.2% of the IVIg group, had a poor outcome. After the patients had been divided into those with the acute motor axonal neuropathy (AMAN) phenotype and the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) phenotype, only those with the AIDP phenotype had a better 6-month outcome with IVIg. This study could not have been done in any setting where IVIg or plasmapheresis, the two established treatment modalities for GBS, are readily available. In fact, no clinical trial in GBS with a placebo arm would receive an ethics commission's approval for being carried out. Thus, data like the ones presented in this study are very valuable for our understanding of GBS treatment. The International Guillain-Barré Syndrome Outcome Study (IGOS) is collecting data on GBS patients around the world in a registry. Thus, here is another chance to compare outcomes of patients with and without immunomodulatory treatment. In one report from the IGOS group on current practice of GBS treatment, 193 patients from Bangladesh were excluded from the analysis, because 83% of them were not treated [2]. A study from Bangladesh reported a high mortality (12%) in GBS patients [3]. This was mainly due to lack of ventilator support. In this cohort, 71% of patients are reported as not receiving treatment. Lack of treatment was not a risk factor for mortality; however, the type of treatment is not specified in the report. Together, these data support the notion that intensive care support is more crucial for the survival of GBS patients than the immunomodulatory treatment [4]. We need to consider that the natural course subgroup in the study by Kalita et al. did not renounce treatment out of their free will or for medical reasons. The mere reason was that these patients could not afford the treatment. The group for natural course was matched to the IVIg group by the authors according to age and peak disability. Thus, any questions on whether disability did, in the end, influence the decision on treatment cannot be answered by the present study. However, the authors might further analyze their registry for this question. Claudia Sommer: Conceptualization (equal); writing – original draft (equal); writing – review and editing (equal). Open Access funding enabled and organized by Projekt DEAL. C.S. is a member of the International Guillain-Barré syndrome Outcome Study (IGOS) consortium. Data sharing is not applicable to this article as no new data were created or analyzed in this study.

Hyperacute Gullain Barre Syndrome (GBS); The Catastrophic Variant- A Rare Case Report

Guillain-Barré syndrome (GBS) also known as acute demyelinating polyradiculoneuropathy (AIDP) is an immunologically mediated rare neurological disorder. The basic pathogenic mechanism is regulated by molecular mimicry. Usually there is a history of preceding infection which occurs some weeks before the attack. The infections are gastroenteritis or upper respiratory. The clinical spectrum of ranges from mild weakness to devastating paralysis including respiratory failure. Majority of the cases recover but a few continue to have residual neurodeficit. The usual clinical course of GBS from the starting of weakness to development of maximum neurologic progression usually progresses over 4 weeks. Hyperacute GBS is a term used when the progression of weakness occurs within hours to days to maximum neurologic impairment. In this case report we present a 28 year old female who developed rapidly progressive, areflexic quadriparesis with respiratory muscle involvement requiring mechanical ventilatory support within nine hours. Clinical , laboratory and nerve conduction studies suggested a diagnosis of GBS.

Physical Therapy and Functional Motor Recovery in Patient with Guillain-Barré Syndrome - Case Report

Introduction: Guillain-Barré syndrome (GBS) is an autoimmune disease with sudden onset and progressive impairment of the peripheral nerves. Aim: To study the influence of applied physical therapy (PT) on functional recovery in patient with a prolonged complicated course of GBS, Landry ascending paralysis and assisted breathing and 4 months in-hospital stay. Case report: The study was conducted with a 34-year-old woman in subacute stage of GBS, over the course of 2 months in the patient’s home, after discharge. PT sessions were performed 3-4 times per week for 1-hour duration, moderate intensity, without reaching fatigue. Correct positioning in bed, passive-active exercises, analytical exercises, exercises for strength of abdominal muscles, breathing exercises, training balance and coordination of sitting and standing, and massage were applied. PT included correct positioning in bed, passive-active exercises, analytical exercises, exercises for strength of abdominal muscles, breathing exercises, training balance and coordination of sitting and standing, and massage. Tests for bed mobility and transfers, Five Times Sit-To-Stand Test (FTSTS) and 10 Meters Walking Test (10MW) were applied twice in order to monitor the effect of PT. Results and Discussion: The results showed an improvement in the speed of patient’s response for transfers. Time for performing FTSTS decreased 33 sec. Improvement in bed mobility, walking speed and cadence are also observed. Conclusion: After two months of treatment functional mobility of the patient was improved.

Guillain-Barré Syndrome Masquerading As Cervical Myelopathy

Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy. In typical cases, the first symptoms of GBS are pain, numbness, paresthesia, weakness in the limbs. Autonomic involvement is common and causes urinary retention and ileus. Most of these symptoms overlap with those of cervical myelopathy. Therefore, correct diagnosis of GBS in a patient with symptomatic cervical myelopathy or in a patient with atypical manifestations of GBS can be difficult, especially early in the course of GBS. We report a 60-year-old man who was admitted to the neurosurgery department with worsening neck pain, numbness and weakness in the hands initially thought to be secondary to progressive cervical myelopathy. However, his symptoms rapidly progressed to flaccid areflexic quadriparesis and respiratory difficulty within few days and shifted to ICU for ventilator support. Electrophysiological studies and cerebrospinal fluid analysis were consistent with an acquired demyelinating polyradiculoneuropathy. We planned for immunotherapy with intravenous immunoglobulin , but his condition was improving day by day with conservative treatment , so immunotherapy with intravenous immunoglobulin was not initiated. Any patient presenting as unexplain Cervical myelopathy, GBS should be kept in mind before planning any surgical intervention.Bangladesh Crit Care J September 2017; 5(2): 129-131

Gamunex® in Guillain-Barré Syndrome: A Postmarketing, Retrospective, Observational Study.

The objective of this retrospective study was to evaluate the effectiveness and safety of Gamunex® (immune globulin [human] 10%; hereinafter "Gamunex") when administered intravenously in the initial treatment of Guillain-Barré syndrome (GBS). The study was conducted as a postapproval commitment for Health Canada. A medical chart review for hospitalized patients diagnosed with GBS and treated with Gamunex (Gamunex 10% and IGIVnex® 10%; N=109; n=69 evaluable) was conducted at seven Canadian study centers in reverse chronological order. The primary endpoint for assessing effectiveness was the proportion of patients with treatment success compared with a prospectively defined historical effectiveness threshold for plasma exchange (PE) treatment (55.05%). Treatment success was assessed as ≥1 point improvement from baseline on the GBS Disability Scale or abbreviated GBS Disability Scale. Cases were not evaluable if treatment success, relapse, or treatment failure could not be determined by the available chart data. Applying a conservative estimate with all nonevaluable patients (n= 40) classified as treatment failures, Gamunex treatment success was estimated at 57.8% (63 of 109 patients), which exceeded the predefined historical PE effectiveness threshold. In the evaluable population of this study, Gamunex treatment was successful in 91.3% of patients (63/69). Some 23 (21.1%) of 109 Gamunex-treated patients experienced ≥1 adverse event; the profile and frequency were consistent with the adverse events reported for Gamunex in the product's labeling and with the natural clinical course of GBS. The effectiveness of Gamunex for treatment of GBS was comparable to PE therapy. Gamunex was observed to have an acceptable safety profile in this study population.

Diabetes mellitus exacerbates the clinical and electrophysiological features of Guillain-Barré syndrome.

It is known that underlying diabetes mellitus (DM) can affect the clinical and electrophysiological pattern of coexisting peripheral neuropathies of other etiologies. The aim of this study was to identify the effect of underlying DM on the clinical and electrophysiological features of Guillain-Barré syndrome (GBS) and on the prognosis of GBS with regard to functional outcome. This study prospectively included 27 GBS patients with DM (GBS-DM+) and 58 GBS patients without DM (GBS-DM-) from two university-based hospitals. The clinical and electrophysiological findings were compared between the two groups. The functional outcomes were quantified by measuring the Hughes grade, whose values were compared between the groups at 3 months after symptom onset. All three sudden deaths that occurred during the acute stage of GBS were GBS-DM+ patients. GBS-DM+ patients had a tendency toward more frequent sensory involvement, and specific electrophysiological patterns and calculated indices disclosed a distal accentuation of conduction abnormalities in these patients. In addition, multivariate analysis identified history of mechanical ventilation (odds ratio 10.057, 95% confidence interval 2.057-49.164, P = 0.04) and DM (odds ratio 9.049, 95% confidence interval 2.152-38.044, P = 0.003) as independent factors for poor functional outcome at 3 months. The findings of this study suggest that DM exacerbates the clinical and electrophysiological features of GBS and influences long-term disability. Both chronic inflammation and nerve ischaemia in DM may intervene in the disease course of GBS, which is a prototype of acute inflammatory neuropathy.

Serial cerebrospinal fluid neurofilament heavy chain levels in severe Guillain‐Barré syndrome

Proximal axonotmesis results in the release of neurofilament (Nf) proteins into the cerebrospinal fluid (CSF) in patients with Guillain-Barré syndrome (GBS). High CSF levels of the phosphorylated form of Nf-heavy chain (NfH(SMI35) ) at GBS onset have been reported to be a poor prognostic marker, but routine measurement of CSF NfH(SMI35) levels has not been done and the longitudinal profile of CSF NfH(SMI35) levels in GBS is not known. This prospective case series describes the clinical, neurophysiological, and biomarker characteristics of 3 patients with severe GBS. High and increasing levels of CSF NfH(SMI35) in serial CSF samples were associated with poor clinical and electrophysiological outcome. These data further suggest that CSF NfH(SMI35) could be a prognostic biomarker which might indicate the development of retrograde axonal degeneration or additional proximal axonal damage during the course of GBS.

Tumour Necrosis Factor α Enhances CCL2 and ICAM-1 Expression in Peripheral Nerve Microvascular Endoneurial Endothelial Cells

Recruitment and trafficking of autoreactive leucocytes across the BNB (blood–nerve barrier) is an early pathological insult in GBS (Guillain-Barré syndrome), an aggressive autoimmune disorder of the PNS (peripheral nervous system). Whereas the aetiology and pathogenesis of GBS remain unclear, pro-inflammatory cytokines, including TNFα (tumour necrosis factor α), are reported to be elevated early in the course of GBS and may initiate nerve injury by activating the BNB. Previously, we reported that disrupting leucocyte trafficking in vivo therapeutically attenuates the course of an established animal model of GBS. Here, PNMECs (peripheral nerve microvascular endothelial cells) that form the BNB were harvested from rat sciatic nerves, immortalized by SV40 (simian virus 40) large T antigen transduction and subsequently challenged with TNFα. Relative changes in CCL2 (chemokine ligand 2) and ICAM-1 (intercellular adhesion molecule 1) expression were determined. We report that TNFα elicits marked dose- and time-dependent increases in CCL2 and ICAM-1 mRNA and protein content and promotes secretion of functional CCL2 from immortalized and primary PNMEC cultures. TNFα-mediated secretion of CCL2 promotes, in vitro, the transendothelial migration of CCR2-expressing THP-1 monocytes. Increased CCL2 and ICAM-1 expression in response to TNFα may facilitate recruitment and trafficking of autoreactive leucocytes across the BNB in autoimmune disorders, including GBS.

Atypical Guillain-Barré Syndrome Misdiagnosed as Lumbar Spinal Stenosis

Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy. In typical cases, the first symptoms of GBS are pain, numbness, paresthesia, weakness in the limbs. Autonomic involvement is common and causes urinary retention and ileus. Much of these symptoms overlap with those of lumbar spinal stenosis. Therefore, correct diagnosis of GBS in a patient with symptomatic lumbar spinal stenosis or in a patient with atypical manifestations of GBS can be difficult, especially early in the course of GBS. Here, we report on a case of atypical GBS in a 74-year-old previously healthy patient with lumbar spinal stenosis and discuss the differential diagnosis of the GBS and lumbar spinal stenosis.

Isolated Bilateral Ptosis as an Early Sign of Guillain-Barré Syndrome

Background. Guillain-Barré syndrome (GBS) has many variants with distinct presentations. Ptosis as an initial presentation is rare. Case Report. We describe a young female with bilateral ptosis without ophthalmoplegia as the initial presentation of Guillain-Barré ptosis in an anti-GQ1b IgG antibody negative patient with a favorable outcome to intravenous immunoglobulins. Objectives. Our paper highlights the importance of recognizing GBS as a potential etiology in a patient presenting with isolated ptosis, particularly since the course of GBS can be more dramatic than in the anti-GBQ1b syndromes such as ophthalmoparesis without ataxia and Miller Fisher syndrome or ocular myasthenia. Conclusion. This is the first paper of anti-GBQ1b antibody negative GBS presenting with isolated ptosis without ophthalmoparesis. GBS should be included in the list of differential diagnosis of such presentations.

Serum and CSF levels of soluble interleukin-2 receptors in MS and other neurological diseases: a reappraisal.

Soluble interleukin-2 receptors (sIL-2-R) were determined longitudinally in sera from 59 patients with multiple sclerosis (MS), from 20 patients with Guillain-Barré syndrome (GBS) and from patients with other non-inflammatory disorders of the nervous system, as well as in 147 CSF samples collected for diagnostic purposes. Significantly increased serum levels of sIL2-R were observed early in the course of GBS and in progressive (relapsing or chronic) MS, but not in patients with clinically stable MS or with an acute relapse followed by complete remission. CSF levels were high in infections of the central nervous system and in meningeal carcinomatosis, as a result of an intrathecal production, but were normal in GBS and in most (94%) MS patients.

Guillain-Barré syndrome – Authors' reply

We thank Kountouras and colleagues, Lie and Xie, and Lo for their valuable comments in response to our Review. Guillain-Barré syndromeWe read with interest the Review by van Doorn and co-workers.1 These authors state that Campylobacter jejuni is the most commonly identified infection that precedes Guillain-Barré syndrome (GBS), and they discuss evidence that this infection contributes to the pathogenesis of GBS through molecular mimicry and a cross-reactive immune response. However, the authors did not mention Helicobacter pylori infection, a crucial antecedent infection that, like C jejuni, seems to be associated with the development of autoimmune sequelae observed in peripheral neuropathies and GBS. Full-Text PDF Guillain-Barré syndromeWe agree with van Doorn and co-workers that more trials are needed to study the effectiveness of treatments for Guillain-Barré syndrome (GBS) on the basis of GBS outcome prognostic scores because “the course of GBS in individual patients is highly variable and the effect of treatment can be shown only by comparing groups of patients”.1 We advocate the use of more comprehensive outcome measures and earlier randomisation of patients in trials. Full-Text PDF Guillain-Barré syndromeGuillain-Barré syndrome (GBS), including the Miller Fisher syndrome (MFS) subtype of GBS, and Bickerstaff's brainstem encephalitis (BBE) are immune-mediated disorders that have overlapping clinical features. Anti-GQ1b antibody is widely regarded to play a critical part in the pathogenesis of these disorders;1 this antibody is detected in 26% of cases of GBS, 66% of cases of BBE, and in up to 95% of cases of MFS.2 van Doorn and co-workers3 have comprehensively reviewed the clinical features, pathogenesis, and treatment of GBS, reminding us that, despite its self-limiting nature, there is still considerable mortality and morbidity from this disorder. Full-Text PDF

Tongue weakness is associated with respiratory failure in patients with severe Guillain-Barré syndrome

Swallowing impairment may worsen respiratory weakness and conduct to respiratory complications such as aspiration pneumonia in Guillain-Barré syndrome (GBS). We prospectively evaluate how tongue weakness could be associated to bulbar dysfunction and respiratory weakness in severe GBS patients. Tongue strength, dysphagia and respiratory parameters were measured in 16 GBS patients at intensive care unit (ICU) admission and discharge and in seven controls. Tongue strength was decreased in the GBS patients compared with the controls. At admission, patients with dysphagia and those requiring mechanical ventilation (MV) had greater tongue weakness. All the patients with initial tongue strength <150 g required MV during ICU stay. Tongue strength correlated significantly with respiratory parameters. This study confirms the strong association between bulbar and respiratory dysfunction in GBS admitted to ICU. Tongue weakness may be present in GBS, especially during the phase of increasing paralysis, and resolves during the recovery phase. Tongue strength and indices of global and respiratory strength vary in parallel throughout the course of GBS. Further studies are needed to assess if, when used in combination with other respiratory tests, tongue strength measurement could contribute to identify patients at high risk for respiratory complications.

Síndrome de Guillain-Barré en edad pediátrica. Experiencia de la Unidad de Neuropediatría de un hospital portugués

Guillain-Barré syndrome (GBS) is an acute inflammatory ascending polyradiculoneuropathy. Autoimmune mechanisms play a role in the demyelinating process. Clinically, progressive symmetric loss of motor strength, areflexia, sensitive and autonomic manifestations are observed. Albuminocytological dissociation and electrophysiological signs of demyelination are frequently found. It is the most common cause of acute flaccid paralysis in children. Retrospective review of all children with GBS admitted to Garcia de Orta Hospital in a 10 year period (1994-2003). 17 children (18 months to 14 years) were admitted during this period. Respiratory or gastrointestinal prodrome was identified in 15 children, with positive serologic studies in six. The main clinical manifestations were disturbances of gait, progressive muscular weakness, sensitive manifestations (pain, paresthesias) and osteotendinous areflexia. 13 children had albuminocytological dissociation and acute demyelinating neuropathy was identified in 12.64% of children were treated with immunoglobulins (2 g/kg). Clinical evolution was favourable in 16 cases, with a death secondary to autonomic dysfunction. Clinical presentation may be unspecific, particularly in young patients, with pain as a primary complaint, preceding muscular weakness and areflexia. Increased cerebrospinal protein and abnormal electrodiagnostic studies may be absent in the early course of GBS. Immunoglobulin therapy was efficacious and well-tolerated.

Dendritic cells in the cerebrospinal fluid and peripheral nerves in Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy

The role of antigen-presenting cells (APC) involved in induction of T and B cell mediated autoaggressive immunity in Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is poorly understood. We studied the numbers and phenotype of dendritic cells (DC) in blood and cerebrospinal fluid (CSF) over the course of GBS and CIDP before and after immunomodulatory treatment. Four out of seven GBS patients examined prior to treatment with high-dose intravenous immunoglobulins (IvIg) had elevated numbers of CD123 + plasmacytoid DC in the CSF, while both GBS and CIDP patients examined prior to treatment had elevated numbers of CD11c + myeloid DC in the CSF, as compared to patients with noninflammatory neurological diseases (OND). The percentages of blood DC expressing the cell surface marker CD1a, co-stimulatory molecules CD80 and CD86, adhesion molecule CD54, and chemokine receptors CCR1, CCR2, CCR5, and CXCR4 were not affected in GBS or CIDP. The immunohistochemistry of sural nerve biopsies revealed CD11c +CD83 −CD14 −CD16 − immature myeloid DC at low numbers, mostly in the perineurium, without difference between CIDP patients and controls. In contrast, the numbers of CD11c +CD14 +/CD16 + macrophages were higher within the endoneurium in CIDP patients compared with the controls. The recruitment of DC to CSF in GBS and CIDP may be important in capturing antigens released from inflamed spinal nerve roots into CSF and in transferring these antigens from CSF to local lymph nodes, where naïve T and B cells may be activated.