Severe Guillain-Barre Syndrome Research Articles

Potential advantage of Therapeutic Plasma Exchange over Intravenous Immunoglobulin in children with axonal variant of Guillain-Barré syndrome: A report of six paediatric cases

Guillain-Barre syndrome (GBS) is a disease entity described in literature since 1859. It is associated with various etiological, clinical and immunological factors with prognostic predictive value. Both Intravenous immunoglobulin (IVIG) and Therapeutic Plasma Exchange (TPE) have been regarded as the first-line treatment for GBS. Certain diagnostic tools help us in early identification of GBS subtypes that may aid clinical management. Here, we have discussed six paediatric cases of GBS of Acute Motor Axonal Neuropathy (AMAN) subtype that were considered for TPE. 5 out of 6 patients were eventually weaned from mechanical ventilation and discharged from the hospital. This study emphasizes the role of TPE in management of severe IVIG refractory GBS with axonal involvement that can be beneficial to the patient. TPE may be considered early in GBS cases with axonal involvement.

Guillain-Barre in the long-term acute care hospital setting: Ventilation does not prolong stay

Objective Guillain-Barre Syndrome (GBS) is a rare acute inflammatory demyelinating polyneuropathy, resulting in loss of muscle function and potentially respiratory failure requiring prolonged mechanical ventilation. Data describing the demographics and outcomes of patients with severe GBS requiring prolonged ventilation in the long-term acute care hospital (LTACH) setting is limited. We hypothesized that patients with GBS requiring prolonged mechanical ventilation require longer lengths of stay in an LTACH and are discharged with poorer functional status than GBS patients who do not require mechanical ventilation. Design, Setting, and Participants We conducted a retrospective study of GBS patients admitted over a 9-year period at an independent, large LTACH and compared ventilated versus non-ventilated GBS patients’ lengths of stay and functional ability at the time of admission and discharge. Interventions Not Applicable Main Outcome Measures and Results We found no significant difference in mean (standard error of means) lengths of stay between ventilated and non-ventilated GBS patients admitted to an LTACH (48.4 (± 8.0) vs. 38.8 (± 5.7) days, P 0.37). We also found that ventilated patients with GBS were discharged from an LTACH with similar functional ability than non-ventilated GBS patients (4.5 vs. 4.0, P 0.43 on 10 ft walk; 4.7 vs. 4.5 on P 0.70; 5.0 vs. 4.2, P 0.21 on 150 ft walk). Conclusion These findings suggest that GBS patients suffering from prolonged mechanical ventilation may expect similar lengths of stay in an LTACH as non-ventilated GBS patients and a similar or greater rate of functional improvement during their stay. These data support admission of chronically ventilated GBS patients to an LTACH for ongoing care after their acute care without the anticipation of greater length of stay or less rehabilitation outcomes with respect to non-ventilated GBS patients. Keywords: Guillain-Barre Syndrome, Assisted Ventilation, Pulmonary Rehabilitation, LTACH, Critical Care

The analysis of serum lipids profile in Guillain-Barre syndrome.

Guillain-Barre syndrome (GBS) is an immune-mediated inflammatory peripheral neuropathy. This study aimed to conduct a systematic analysis of the serum lipids profile in GBS. We measured the serum lipids profile in 85 GBS patients and compared it with that of 85 healthy controls matched for age and sex. Additionally, we analyzed the correlation between lipids and the severity, subtypes, precursor infections, clinical outcomes, clinical symptoms, immunotherapy, and other laboratory markers of GBS. Compared to the healthy controls, GBS exhibited significantly elevated levels of Apolipoprotein B (APOB), Apolipoprotein C2 (APOC2), Apolipoprotein C3 (APOC3), Apolipoprotein E (APOE), triglycerides (TG), and residual cholesterol (RC). Conversely, Apolipoprotein A1 (APOA1), Apolipoprotein A2 (APOA2), and high-density lipoprotein (HDL) were substantially lower in GBS. Severe GBS displayed noticeably higher levels of APOC3 and total cholesterol (TC) compared to those with mild disease. Regarding different clinical outcomes, readmitted GBS demonstrated higher RC expression than those who were not readmitted. Moreover, GBS who tested positive for neuro-virus antibody IGG in cerebrospinal fluid (CSF) exhibited heightened expression of APOC3 in comparison to those who tested negative. GBS with cranial nerve damage showed significantly reduced expression of HDL and APOA1 than those without such damage. Additionally, GBS experiencing limb pain demonstrated markedly decreased HDL expression. Patients showed a significant reduction in TC after intravenous immunoglobulin therapy. We observed a significant positive correlation between lipids and inflammatory markers, including TNF-α, IL-1β, erythrocyte sedimentation rate (ESR), white blood cells, monocytes, and neutrophils in GBS. Notably, APOA1 exhibited a negative correlation with ESR. Furthermore, our findings suggest a potential association between lipids and the immune status of GBS. The research demonstrated a strong connection between lipids and the severity, subtypes, clinical outcomes, precursor infections, clinical symptoms, immunotherapy, inflammation, and immune status of GBS. This implies that a low-fat diet or the use of lipid-lowering medications may potentially serve as an approach for managing GBS, offering a fresh viewpoint for clinical treatment of this condition.

Guillain-Barre Syndrome After COVID-19 Vaccination: A Secondary Analysis of Domestic Safety Data by the Japanese Government

Purpose The purpose of this study was to figure out the risk of Guillain-Barre syndrome (GBS) after coronavirus disease 2019 (COVID-19) vaccination, which has been reported as a rare adverse reaction. Methods Elucidating the characteristics, we performed a secondary analysis of the cases from February 2020through January 2022, based on the publicly available spontaneous adverse reaction reports in Japan. Results We identified 115 cases, and all were after messenger RNA (mRNA) vaccination. Of all the cases, 69 (60.0%) were female and 44 (38.2%) were older than 65 years old. Severe GBS was reported by 38 males (median age 61.5 years) and 51 females (median age 55 years). The median interval from vaccination to the onset of symptoms was eight days for males and four days for females. Sequelae were reported in 18 patients (7 males, median age 81 years; 11 females, median age 51 years), 11 of whom were older than 65 years old. The estimated incidence was about 0.0001% (0.000058% for the Pfizer vaccine and about 0.000046% for the Moderna vaccine, respectively). Conclusions Spontaneous reports would have various biases, the incidence of GBS after mRNA vaccination was as low as in other existing vaccination programs, and it is important not to interpret that risk expansively.

Guillain-Barré Syndrome and COVID-19: Two Case Reports

[b]BACKGROUND[/b] Since the outbreak of the coronavirus disease 2019 (COVID-19), which subsequently increased to a pandemic, the number of reports about neurological manifestations of the disease has increased. Guillain-Barre syndrome (GBS), which is acute paralytic neuropathy, is one such rare manifestation. [b]CASE REPORT[/b] We report 2 cases of GBS in 58-year-old men with typical manifestations of COVID-19; one of them is presumed COVID-19. The interval between the onset of COVID-19 symptoms and the first symptoms of GBS was 5 and 13 days, respectively. The patients had a more severe GBS than in general cases of GBS associated with COVID-19; they required mechanical ventilation, and both patients developed sepsis and acute kidney injury (AKI), which led to the death of one of them. Serum antiganglioside antibodies were tested in 1 patient and were absent. A nerve conduction study also was performed in 1 patient and confirmed an acute inflammatory demyelinating polyneuropathy. One patient was treated with intravenous immunoglobulin and had a good prognosis, and the other was treated with plasma exchange and died. [b]CONCLUSIONS[/b] Our cases demonstrate the clinical course of GBS in COVID-19, diagnostic findings, and treatment strategies. GBS associated with COVID-19 seems to share most features of other post-infectious GBS. Nevertheless, further research is required to fully understand the characteristics, pathogenesis, and management of GBS in COVID-19.

Disease progression velocity as predictor of severity in Guillain-Barre syndrome.

To identify the velocity of disease progression as a predictor of severity in patients with Guillain-Barre syndrome (GBS). Prospective observational study of patients with confirmed diagnosis of GBS between May and August 2019 in four hospitals in Peru. The disease progression velocity (DPV) was defined as the time since the onset of neurological symptoms and the maximum peak of neurological severity. Of 94 cases with GBS, the average age was 42 years; 73 (77.8%) patients presented severe GBS, the average hospital stay was 19 days; 45 (47.8%) patients haddiarrheal symptoms previously, in 63 (67.1%) patients the onset of motor weakness was located in the upper limbs and in 31 (32.9%) it was located in the lower limbs, 9 (10.0%) patients presented some type of dysautonomy; admission to mechanical ventilation was needed in 8 (8.5%) patients, and the deceased were 2 (2.0%). The DPV≤ 1 day has a 79% probability of developing severe disease, the two and three day DPV have the probability of 61% and 38% respectively of progressing to severe forms. DPV is a predictor of poor prognosis when it is less than 2 days and with a possible requirement for mechanical ventilation. The speed of progression of neurological disease is a practical and accessible clinical evaluation method that should be evaluated in patients with GBS.

Response and Clinical Outcome of Critically Ill Children with Guillain Barre Syndrome admitted to PICU of Children’s Hospital Lahore

Objective: To analyze patients with Guillain Barre Syndrome (GBS) requiring intensive care unit (ICU) admission, their course, response to treatment and outcome.
 Methods: A retrospective study conducted at ICU of The Children’s Hospital Lahore in 194 children with diagnosis of GBS from June 2018 to May 2020. Demographic profile, clinical features, treatment, duration of mechanical ventilation, length of ICU stay and outcome was noted. 
 Results: 
 Mean age of children was 5.95 ± 3.1 years with male predominance 136 (70 %). Major proportion were in range of 1-5 years 93(47.9%). Mean duration of ICU stay was 21.3±34.4 days. Out of 194 patients 125(64%) received IVIGs while 112 (57.7%) required mechanical ventilation because of rapidly progressive weakness within 24 hours of admission and those who remained on mechanical ventilation for longer period showed AMSAN (Acute motor sensory axonal neuropathy) type of GBS ( p value <0.001). Tracheostomy was performed in 22.7% of patients who were difficult to wean off from ventilator. Among nerve conduction studies AIDP (Acute inflammatory demyelinating polyneuropathy) was predominant 48% associated with p value <0.001 in those who showed complete recovery, followed by AMSAN 24%, AMAN ( Acute motor axonal neuropathy) 13.4% and miscellaneous 13.9 %. Regarding outcome complete recovery was seen in 28%, minimal residual disability in 70% and 1.5% remained ventilator dependent.
 Conclusion:
 Patients of severe GBS often require prolong respiratory support and ICU care. The indicators of poor outcome are rapidly progressive course, severe weakness and axonal neuropathy leading to longer duration on mechanical ventilation.
 KEYWORDS: 
 Guillain Barre Syndrome, Outcome, intensive care unit (ICU)

Impact of COVID-19 on the cerebrovascular system and the prevention of RBC lysis.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) uses Angiotensin- converting enzyme 2 (ACE2) receptors to infect host cells which may lead to coronavirus disease (COVID-19). Given the presence of ACE2 receptors in the brain and the critical role of the renin-angiotensin system (RAS) in brain functions, special attention to brain microcirculation and neuronal inflammation is warranted during COVID-19 treatment. Neurological complications reported among COVID-19 patients range from mild dizziness, headache, hypogeusia, hyposmia to severe like encephalopathy, stroke, Guillain-Barre Syndrome (GBS), CNS demyelination, infarcts, microhemorrhages and nerve root enhancement. The pathophysiology of these complications is likely via direct viral infection of the CNS and PNS tissue or through indirect effects including post- viral autoimmune response, neurological consequences of sepsis, hyperpyrexia, hypoxia and hypercoagulability among critically ill COVID-19 patients. Further, decreased deformability of red blood cells (RBC) may be contributing to inflammatory conditions and hypoxia in COVID-19 patients. Haptoglobin, hemopexin, heme oxygenase-1 and acetaminophen may be used to maintain the integrity of the RBC membrane.

Severe Guillain-Barr\xe9 syndrome associated with chronic active hepatitis C and mixed cryoglobulinemia: a case report

BackgroundWe describe a case of severe Guillain-Barre syndrome (GBS) associated with chronic active hepatitis C and mixed cryoglobulinemia (MC). To our knowledge, this association between GBS and hepatitis C virus (HCV) infection has been rarely reported.Case presentationA 56-year-old man developed symmetrical muscle weakness in all extremities, areflexia and sensorial disorder followed by acute respiratory failure associated with chronic active hepatitis C, which was confirmed by the presence of anti-HCV antibodies in the serum and persistence of HCV RNA viral load for more than 6 months. Chronic hepatitis C was further complicated by type 3 MC. Electromyography showed peripheral nerve injury (mainly in axon). A severe acute motor sensory axonal neuropathy (AMSAN) was diagnosed. After treatment with intravenous immunoglobulin and plasma exchange followed by antiviral therapy by direct-acting antiviral agent, patient showed progressive recovery and was transferred 3 months after his first admission to a rehabilitation center.ConclusionsOur case reported a severe GBS associated with HCV infection and MC. EMG classified for the first time the subtype of GBS (severe AMSAN) correlated with severe clinical form. HCV infection should be screened in high-risk patients to prevent silent progression of the chronic hepatitis C and its potentially severe extra-hepatic manifestations.

Severe Guillain-Barré Syndrome Following Shingrix® Vaccine Administration

Guillain-Barre syndrome (GBS) is a rare life threatening acute polyradiculoneuropathy with variable clinical presentations, including weakness, paresthesias, diminished deep tendon reflexes, hyponatremia, dysautonomia, and a cytoalbuminological dissociation on cerebral spinal fluid (CSF) analysis. The exact etiology remains unknown but is thought to occur as a post-infective immunemediated process. Vaccines and illnesses, including H1N1 Swine Flu vaccine, pneumococcal vaccine, Campylobacter jejuni bacteria, cytomegalovirus, and herpes zoster virus, have been associated with GBS. One vaccine that has not been identified to cause GBS is the newly released Shingrix® vaccine for herpes zoster infection prevention. We present a case of severe GBS following Shingrix® vaccine immunization.

A fulminant Guillain–Barrè syndrome mimicking brain death

We report a case of fulminant Guillain–Barre syndrome (GBS), mimicking brain death. A previously healthy 52-year-old male was admitted to hospital for rapidly progressive limb weakness. A GBS was diagnosed, based on the clinical picture and cerebrospinal fluid albumin-cytological dissociation; nerve conduction studies were scheduled for the following day and treatment with intravenous immunoglobulins was immediately started. The patient quickly developed respiratory failure and was admitted to Intensive Care Unit, requiring mechanical ventilation and deep sedation. The nerve conduction studies demonstrated motor and sensory responses unexcitable in upper and lower extremities. A week later, when the pharmacological sedation was withdrawn, the patient was found to have a coma with absent brainstem reflexes, resembling a state of brain death. An electroencephalogram showed quite a normal brain function, the brain MRI was unremarkable. On the following days, while the flaccid quadriplegia persisted, the ocular movements first reappeared, with signs of recovered consciousness, resembling a locked-in syndrome. Then, after six weeks, small amplitude and long duration motor response were detectable at upper limbs. The outcome was favorable, after long Intensive Care Unit and through rehabilitation program. Severe GBS may mimic brain death, probably by a de-afferentation/de-efferentation mechanism.

S33. Two case reports of ganglioside-associated Guillain-Barré syndrome with onset of respiratory muscle paralysis

Introduction Guillain–Barre syndrome (GBS) is an immune-mediated disorder of the peripheral nervous system presenting with inflammatory infiltration, typically sporadic, appearing following an infection. Since ganglioside preparations were introduced in clinical practice, in 1975, scholars from around the world have been reporting cases of exogenous ganglioside-associated GBS, with acute, severe and rapidly worsening peripheral nerve involvement, initially presenting with flaccid paralysis of the limbs. Methods We report two cases of ganglioside-associated GBS, for which the initial symptom was respiratory muscle paralysis. Results Exogenous gangliosides can cause severe GBS, with most electrophysiological studies showing damage to axons of motor nerves,and it is extremely rare that ganglioside-associated GBS present with initial respiratory muscle paralysis. Conclusion Immediate electrophysiological examination and detection of anti-ganglioside antibodies should be considered for patients presenting sudden dyspnea, after intravenous infusion of exogenous gangliosides. Once GBS is established, patients should be given IVIg or PE, which will aid in improving the patient’s neurological functions.

Guillain Barre Syndrome in the elderly: Experience from a tertiary-care hospital in India

Age influences incidence and prognosis of Guillain Barre Syndrome (GBS), common cause of ascending areflexic quadriparesis. Dedicated studies on elderly GBS are infrequent. This study aimed to describe clinical features and outcome at hospital-discharge in patients aged≥60years with GBS. Medical records of 70 elderly GBS over 15years were analysed. Mean symptom-duration was 5.78±4.5days and onset-to-peak 5.14±4.4days. Antecedent events preceded GBS by 8.07±9.9days and included: fever (n=19), respiratory infection (n=6), and gastroenteritis (n=5). Clinical features were weakness of facial (n=34), bulbar (n=13), extraocular (n=4) and respiratory (n=20) muscles and recurrence (n=4). Nine had Hughes disability score (HDS) of three or less. Sensory symptoms and signs included paresthesias (n=40), pain (n=24), and impaired kinaesthetic sensation (n=14). Laboratory abnormalities included albumino-cytological dissociation (n=50), hyponatremia (n=36) and elevated creatine kinase (n=18). Electrophysiological subtypes were: primary demyelinating (n=52), inexcitable (n=3), equivocal (n=2) and axonal (n=1). Fifty-seven patients treated with plasmapheresis (n=48) or intravenous immunoglobulin (n=9) had mean HDS of 3.53±0.7 at discharge. Twenty-one were ambulant (HDS≥3), one had persisting respiratory weakness and one died. Striking differences between the ‘elderly’ and 100 ‘adults’ seen over 20months were shorter symptom-duration, higher frequency of facial palsy and hyponatremia, lower frequency of pain, lower mean MRC sum score and worse HDS at study-entry and discharge (p<0.05). Requirement for mechanical ventilation and cardiac autonomic dysfunction was higher among elderly (p:0.02). In conclusion, in this cohort of elderly GBS, there was a higher frequency severe GBS and demyelinating electrophysiology.

Bilateral Ptosis due to Sympathetic Dysfunction as a Feature of Guillain-Barre Syndrome.

This case-control study demonstrates that bilateral ptosis due to ocular sympathetic dysfunction is a characteristic feature of Guillain-Barre syndrome (GBS) and apraclonidine can be helpful in unmasking this clinical feature. Five patients with GBS and 9 healthy controls were assessed for ocular sympathetic dysfunction through application of topical apraclonidine to 1 eye. Changes resulting from reversal of ptosis or miosis due to apraclonidine were compared with the eye on the other side with no apraclonidine using photographs. Ocular sympathetic dysfunction in the form of mild bilateral ptosis was found in all 5 patients with GBS recruited in this study. Consistent with previous reports, healthy subjects had no significant response to apraclonidine. Although there was evidence of concomitant pupillary dysfunction in the form of bilateral Horner syndrome in 2 of the patients with GBS with more severe GBS phenotype, this study did not have the statistical power to reach conclusions regarding pupillary dysfunction and disease severity in GBS.

Zika virus-induced neurological critical illness in Latin America: Severe Guillain-Barre Syndrome and encephalitis

Zika virus (ZIKAV) is classically described as causing minor symptoms in adult patients, however neurologic complications have been recognized. The recent outbreak in Central and South America has resulted in serious illness in some adult patients. We report adult patients in Latin America diagnosed with ZIKAV infection admitted to Intensive Care Units (ICUs). MethodsMulticenter, prospective case series of adult patients with laboratory diagnosis of ZIKAV in 16 ICUs in 8 countries. ResultsBetween December 1st 2015 and April 2nd 2016, 16 ICUs in 8 countries enrolled 49 critically ill patients with diagnosis of ZIKAV infection. We included 10 critically ill patients with ZIKAV infection, as diagnosed with RT-PCR, admitted to the ICU. Neurologic manifestations concordant with Guillain-Barre Syndrome (GBS) were present in all patients, although 2 evolved into an encephalitis-like picture. 2 cases died, one due to encephalitis, the other septic shock. ConclusionsDiffering from what was usually reported, ZIKAV infection can result in life-threatening neurologic illness in adults, including GBS and encephalitis. Collaborative reporting to identify severe illness from an emerging pathogen can provide valuable insights into disease epidemiology and clinical presentation, and inform public health authorities about acute care priorities.

The clinical characteristics and short-term prognosis in elderly patients with Guillain-Barré syndrome.

To investigate the clinical characteristics and short-term prognosis of elderly patients with Guillain–Barré syndrome (GBS).We retrospectively analyzed the clinical data of adult GBS. According to the age, the enrolled subjects were divided into 2 groups, that is, patients ≥60 years (elderly group) and those aged 18 to 59 years (nonelderly group). The clinical characteristics and short-term prognosis of the patients in the 2 groups were compared.In total, 535 patients were enrolled. There were 67 patients fell into the elderly group with a mean age of 69 years old; while 468 patients fell into the nonelderly group with a mean age of 39 years old. We found that the elderly patients had significantly lower incidence of antecedent infections (49.3% vs 66.2%, P < 0.01). The time from onset to admission (5 vs 4 days, P < 0.05) and time from onset to nadir (7 vs 6 days, P < 0.05) were significantly longer in the elderly patients. It was noteworthy that more elderly patients were found with lymphocytopenia (55.4% vs 37.3%, P < 0.01), hyponatremia (25.0% vs 10.2%, P < 0.01), hypoalbuminemia (9.0% vs 2.6%, P < 0.05), and hyperglycemia (34.3% vs 15.2%, P < 0.01). Importantly, the elderly patients had longer duration of hospitalization (17 vs 14 days, P < 0.05), higher incidence of pneumonia (29.9% vs 18.8%, P < 0.05), and poorer short-term prognosis (58.2% vs 42.7%, P < 0.05). In patients with severe GBS, no significant differences were observed in disease severity, treatment modality, incidence of pneumonia, and duration of hospitalization between the 2 groups. However, more patients in the elderly group showed poor short-term prognosis (84.1% vs 63.8%, P < 0.01). Further, old age (≥60 years) (OR = 2.906, 95% CI: 1.174–7.194, P < 0.05) and lower Medical Research Council (MRC) score at nadir (OR = 0.948, 95% CI: 0.927–0.969, P < 0.01) were risk factors for poor short-term prognosis in severe GBS patients.The clinical characteristics and short-term prognosis of elderly patients with GBS are distinct from nonelderly adults. Old age (≥60 years) and lower nadir MRC score serve as predictor for poor short-term prognosis in severe GBS patients.

P188 – 2864: Innovative concept in early neurological rehabilitation

Objective After acute neurological disease due to infection, trauma or other severe damage to the nervous system early neurological rehabilitation is mandatory. Rehabilitation should start during intensive care: early therapy improves the situation and increases the chances of optimal recovery. An interdisciplinary team works together with patients and their families to help regain consciousness, ensuring optimal medical treatment, for a good quality of life and to find their way back to life. We tried a novel way of supporting a patient through this hard, strenuous and emotionally difficult time. Methods Videorecording of steps of rehabilitation and progress in therapies by means of making a music video. Results We here present a music video that depicts the rehabilitation process of a 16 year old girl with severe Guillain-Barre syndrome. The patient was a former singer in a school rockband. On admission to our hospital, she was on mechanical ventilation without any voluntary movements, unable to talk, struck by severe neuropathic pain. Throughout the rehabilitation, even in ICU, she was videorecorded performing to her favourite popsong. All steps of rehabilitation are depicted in this video: first voluntary movements, weaning from the ventilator, enduring neuropathic pain, ability to sit and stand. Conclusion Recording the video was an additional incentive for our patient to work hard during rehabilitation to regain all lost abilities. Because of her passion for singing she could be motivated more easily.

G.P.299: Novel use of eculizumab in a patient with Guillain–Barré syndrome

Guillain–Barre syndrome (GBS) is a rare but life-threatening polyneuropathy. We describe the novel use of eculizumab in a patient with this disorder. Case A previously well 13 year old boy presented with an acute onset of ascending paralysis and was diagnosed with GBS. Despite receiving intravenous immunoglobulins on Day 3 of presentation, he continued to deteriorate and required ventilatory support and a subsequent tracheostomy. He remained non-responsive to a further course of 2 g/kg intravenous immunoglobulin. His GBS was complicated by severe life-threatening autonomic instability so plasmapheresis was not felt to be a suitable option. A single dose of eculizumab was given, after which the patient showed signs of improvement. He continued to convalesce over the next 8 weeks, at which stage he was mobilising independently. Discussion GBS poses a mortality rate of 10% despite medical advances. Intravenous immunoglobulins and plasmapheresis have been shown to improve outcome but beyond these, management is mainly supportive at present. Eculizumab, a monoclonal antibody, is currently used in patients with other immunological disorders but not GBS. Recent animal studies suggest that complement activation may play a role in the development of paralysis in GBS. Complement inhibitors such as eculizumab have been shown to have promising benefits in murine models. Further research is necessary but we believe eculizumab may have a role in improving outcome, especially in those with severe GBS. As far as we are aware, this is the first case report in literature of eculizumab being used in a patient with GBS. Our case suggests potentially favourable outcomes and clinical trials are needed to assess this further.

Pathogenetic and prognostic role of autoantibodies to gangliosides of the peripheral nerves in Guillain-Barre syndrome

Testing for IgG+IgM autoantibodies to gangliosides of peripheral nerves (asialo-GM1, GM1, GM2, GD1a, GD1a, GD1b, GQ1b) was performed in 95 patients with Guillain–Barre syndrome (GBS) in Moscow. In 70 patients (74%) acute inflammatory demyelinating polyneuropathy (AIDP) was diagnosed, and in 25 patients (26%) – either acute motor axonal neuropathy (AMAN) or acute motorsensory neuropathy (AMSAN). The role of antibodies to gangliosides for prognosis of the disease course and response to pathogenic treatment was assessed. Antibodies to gangliosides were found in 55 patients (57.9%) – more often in patients with AMAN/AMSAN (p<0.05). The whole range of antibodies was found both in patients with AIDP and AMAN/AMSAN. Anti-GM1 were found in both subtypes of GBS. Anti-GD1b were associated with AMAN/AMSAN (p<0.05). There was a correlation between anti-GM1 and diarrhea (p<0.05), anti-asialo-GM1 and campylobacteriosis (p<0.05). There wasn’t found significant correlation between anti-GD1 and axonal subtypes, diarrhea in anamnesis, and campylobacteriosis. Association of severe GBS and elderly age, camplylobacteriosis and axonal subtypes was confirmed. AMAN/AMSAN subtypes, severity of the disease, necessity in mechanical ventilation were characterized by insufficient efficacy of pathogenic therapy. The same factors and elderly age were associated with unfavorable prognosis in terms of absence of unassisted walking in 6 and 12months. Anti-GD1a were associated with sever course of the disease, including necessity in mechanical ventilation. Immunologic factors are not sufficient in prognosis of the treatment efficacy. Antibodies to GM1 were associated with unfavorable prognosis with absence of unassisted walking in 6 months. Clinical and neurophysiological data is crucial for making diagnosis and prognosis of the GBS course. Testing for autoantibodies to gangliosides could be helpful in diagnostics of GBS. It is possible to use that test for revealing axonal type of the disease (anti-GD1b), prognostication of severe course of GBS (anti-GD1a) and unfavorable rehabilitation of unassisted walking.

Clinical Usefulness of Serum Alpha2-Globulin in Guillain-Barre Syndrome To Predict Its Clinical Course (P06.143)

Objective: Serum alpha2-globulin on admission may be high in severe Guillain-Barre syndrome (GBS) cases. Background We can easily (and cheaply, quickly) obtain serum alpha2-globulin on admission. Serum alpha2-globulin reflects immunoreactive response. GBS has immunoreactive response to the ganglioside (for example,antiganglioside antibodies). Serum alpha2-globulin on admission may predict severity of GBS and its clinical course. Design/Methods: From January 2006 to September 2011, 36 GBS patients were admitted to our hospital. On admission we evaluated serum alpha2-globulin before the treatment. We also evaluated their final outcome by using modified Rankin Scale(mRS). Results: We can obtain serum alpha2-globulin on admission in 21 cases. Serum alpha2-globulin is significantly higher in high mRS (>3) group (n=6) than in low mRS( Conclusions: By using serum alpha2-globulin on admission, we can evaluate severity of GBS, and predict its final outcome. Disclosure: Dr. Mori has received personal compensation for activities with Kurashiki Central Hospital as an employee.