Guillain-Barre Syndrome Publications (7527)
Guillain-Barre Syndrome Publications
UCH-L1 protein levels were greater in patients with GBS than in controls. The patients with GBS whose UCH-L1 protein levels were higher than those of the controls presented with more severe symptoms at peak. UCH-L1 protein levels tended to become elevated as the total protein levels were increased; however, elevated UCH-L1 without an increase in total protein might be correlated with severe disease course (bedridden or ventilator supported). These results suggest that UCH-L1 could be a biomarker associated with the severity of the disease at the acute phase of GBS.
We assessed the clinical manifestations, results of electro-diagnostic tests, functional status, treatment instituted and outcome of 20 children diagnosed with GBS.
Of the 20 (male to female ratio = 2.3:1) children studied, all had motor weakness, 5 (25%) had sensory loss, 4 (20 %) had cranial nerve palsies and 4 (20%) had autonomic disturbances. Respiratory paralysis was found in 7 (35%) children requiring assisted ventilation. Antecedent illness preceding GBS was recorded in 50% children. The GBS subtype distribution as per electrodiagnostic studies was as follows: acute motor axonal neuropathy (AMAN) in 7 (38.9%), acute motor sensory axonal neuropathy (AMSAN) in 4 (22.2%), acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 4 (22.2%) and both axonal and demyelinating neuropathy in 3 (16.7%). Intravenous immunoglobulins (IVIG) constituted the treatment given in majority of the patients. Plasmapharesis was performed in one child in view of poor response to IVIG. Complete recovery was observed in 14 children and the remaining 3 children experienced only incomplete recovery.
Male preponderance and presence of antecedent illness in a majority of subjects was observed in our study. Regardless of the severity of illness at admission and electrophysiological subtypes, a majority achieved full recovery. Intravenous Immunoglobulin and supportive care form the cornerstone of management in childhood GBS.
The GBS rate per 100,000 person-years was estimated in the reference and pandemic periods.
The incidence of GBS was 0.63 (95% CI: 0.37-0.89) per 100,000 person-years in the reference period and 0.87 (0.49-1.26) in the pandemic period. During the vaccination season, the pandemic period GBS incidence rate was not significantly higher than the reference period incidence rate (Rate ratio: 1.52; 0.99-2.32), but difference was observed among persons aged 20-34 years. Rate of GBS increased after pH1N1 vaccination compared to the reference period (1.46, 1.26-1.68).
The incidence of GBS increased slightly but not significantly during the pandemic period, although pH1N1 vaccination increased the GBS rate. Therefore, mass influenza vaccination programs should not be precluded on the basis of GBS.
These include inflammatory myopathy, aseptic meningitis, severe meningo-radiculo-neuritis, temporal arteritis, Guillain-Barre syndrome, and posterior reversible encephalopathy syndrome. We report the first case of ipilimumab-induced progressive necrotic myelopathy.
Disease characteristics were determined from 15 historical case reports of patients presenting with isolated 'polyneuritis cranialis', 'cranial polyneuropathy', 'multiple cranial neuropathy' and 'multiple cranial neuropathies' due to GBS. Median age was 40 years. 80 % displayed antecedent infectious symptoms. In all cases, disease course was monophasic with clinical improvement within weeks or months. Initial symptoms were ocular (73 %) or bulbar (33 %). Mean number of cranial nerves involved was 6 (range, 3-10). 93 % displayed ocular signs, 73 % facial weakness or numbness and 87 % bulbar signs. In 3 patients (20 %), there was significant asymmetry. Cerebrospinal fluid albuminocytological dissociation was present in 67 % of cases. Serum anti-ganglioside antibodies were tested for in 8 of 15 patients and anti-GQ1b antibodies were found in 3 patients, whilst anti-GT1a antibodies were found in 1 patient. Polycranial neuritis (the oculopharyngeal subtype of GBS) can be defined in patients with disease characteristics of GBS who display ocular and pharyngeal weakness in the absence of limb weakness or ataxia. In half of cases tested for, anti-ganglioside antibodies were present and most frequently against GQ1b.
Other emerging indications include necrotizing fasciitis, toxic epidermal necrolysis/Stevens-Johnson syndrome, and toxic shock syndrome. The evidence for IVIg use in sepsis and septic shock remains controversial and insufficient to recommend its routine use. Intravenous immunoglobulin is expensive and also carries risks of adverse effects, including common and benign infusion-related reactions, as well as relatively rare and more serious problems, such as thromboembolic events, renal failure, and aseptic meningitis. In this article, we review the literature on conditions requiring ICU admission and IVIg, and we classify them as supported, emerging, or unsupported indications based on the available evidence and guidelines for clinical use of IVIg.
For these neuropathies, the classification in demyelinating and axonal is inadequate or even misleading, we therefore propose a new category of nodopathy that has the following features: (1) it is characterised by a pathophysiological continuum from transitory nerve conduction block to axonal degeneration; (2) the conduction block may be due to paranodal myelin detachment, node lengthening, dysfunction or disruption of Na(+) channels, altered homeostasis of water and ions, or abnormal polarisation of the axolemma; (3) the conduction block may be promptly reversible without development of excessive temporal dispersion; (4) axonal degeneration, depending on the specific disorder and its severity, eventually follows the conduction block. The term nodopathy focuses to the site of primary nerve injury, avoids confusion with segmental demyelinating neuropathies and circumvents the apparent paradox that something axonal may be reversible and have a good prognosis.
After receiving Flagyl for C. difficile colitis, the patient developed psychosis, ascending paralysis, and metabolic derangements. She underwent extensive neurological workup due to her congenital neurological abnormalities, most of which were unremarkable. As a differential diagnosis of Guillain Barré syndrome, acute porphyria was then considered and ultimately proved to be the diagnosis. After hematin administration and intense rehabilitation, the patient slowly recovered from the full-blown acute porphyria attack.
This case report, for the first time, documents acute porphyria attack as a result of a sequential combination of 3 common medications. This is the first case report of the concomitant presence of both acute porphyria and Arnold Chiari malformation, 2 genetic disorders with unclear association.
Specificity of the autoantibodies was confirmed by immunoblot assay, binding to contactin-1-transfected human embryonic kidney cells, binding to paranodes of murine teased fibres and preabsorption experiments. Paranodal pathology was investigated by immunofluorescence labelling of dermal myelinated fibres.
High reactivity to contactin-1 by ELISA was found in four patients with chronic inflammatory demyelinating polyradiculoneuropathy and in none of the patients with Guillain-Barré syndrome, which was confirmed by cell binding assays in all four patients. The four patients presented with a typical clinical picture, namely acute onset of disease and severe motor symptoms, with three patients manifesting action tremor. Immunofluorescence-labelling of paranodal proteins of dermal myelinated fibres revealed disruption of paranodal architecture. Semithin sections showed axonal damage but no classical signs of demyelination.
We conclude that anti-contactin-1-related neuropathy constitutes a presumably autoantibody-mediated form of inflammatory neuropathy with distinct clinical symptoms and disruption of paranodal architecture as a pathological correlate. Anti-contactin-1-associated neuropathy does not meet morphological criteria of demyelinating neuropathy and therefore, might rather be termed a 'paranodopathy' rather than a subtype of demyelinating inflammatory neuropathy.
g. chronic immune-mediated neuropathies, Guillain-Barré syndrome (GBS), and axonal/vasculitic neuropathies. We compared these data to 28 healthy controls. Based on these results an ultrasound pattern sum score (UPSS) with three sub-scores (UPS-A for the sensorimotor nerves, UPS-B for the cervical roots and the vagal nerve and UPS-C for the sural nerve) was developed. Afterwards, the applicability of the score was prospectively validated in 10 patients with chronic neuropathies and in 14 patients with unknown acute and subacute PNP before performing additional tests.
UPS-A and UPSS were significantly higher in CIDP than in other neuropathies and controls (p<0.001). UPS-B was significantly more often pathologic in GBS than in CIDP and other neuropathies (p<0.001). Using receiver operation characteristics curve analysis boundary values for each score were defined. Positive predictive value (PPV) of these scores for CIDP and GBS was >85%. Vasculitic neuropathies showed an intermediate type of UPSS compared to other axonal neuropathies (p<0.001). In the prospective application the pattern score could be used with good accuracy in several types of neuropathy.
UPS-A and UPSS operationalize to diagnose acute and subacute-onset CIDP and its variants with high sensitivity, specificity, and PPV. An increased UPS-B with normal UPSS and other sub scores may point to the diagnosis of GBS with high PPV and enables the differentiation from CIDP.
Using the UPSS and its sub-scores gives a new diagnostic power to the method of the peripheral nerve ultrasound.
Despite the favorable recovery of muscle weakness, he complained of severe needle-like pain in the thighs and buttocks and also painful numbness over the gastrocnemius regions. Acetaminophen and hydroxyzine therapy was ineffective for the pain control. Oral prednisolone therapy (0.7mg/kg/day) led to drastic pain-relief with favorable improvement of the weakness. Corticosteroid therapy is not typically used in the management of GBS patients. Although GBS-associated pain frequently occurs in children, only a few reports have indicated the analgesic utility of steroids in the treatment of pediatric GBS. This observation may suggest the alternative of this therapy as for the as the limited, but potentially rapid, control of GBS-associated acute radicular pain in pediatric patients.
LAIV4 reports in 2013-2014 were compared to LAIV3 reports from the previous three influenza seasons. Medical records were reviewed for non-manufacturer serious reports (i.e., death, hospitalization, prolonged hospitalization, life-threatening illness, permanent disability) and reports of selected conditions of interest. We conducted Empirical Bayesian data mining to identify disproportional reporting for LAIV4.
In 2013-2014, 12.7 million doses of LAIV4 were distributed and VAERS received 779 reports in individuals aged 2-49 years; 95% were non-serious. Expired drug administered (42%), fever (13%) and cough (8%) were most commonly reported in children aged 2-17 years when LAIV4 was administered alone, while headache (18%), expired drug administered (15%) and exposure during pregnancy (12%) were most common in adults aged 18-49 years. We identified one death report in a child who died from complications of cerebellar vascular tumors. Among non-death serious reports, neurologic conditions were common in children and adults. In children, seizures (3) and Guillain-Barré syndrome (2) were the most common serious neurologic outcomes. We identified three serious reports of asthma/wheezing following LAIV4 in children. Data mining detected disproportional reporting for vaccine administration errors and for influenza illness in children.
Our analysis of VAERS reports for LAIV4 did not identify any concerning patterns. The data mining finding for reports of influenza illness is consistent with low LAIV4 vaccine effectiveness observed for influenza A disease in children in 2013-2014. Reports of LAIV4 administration to persons in whom the vaccine is not recommended (e.g., pregnant women) indicate the need for education, training and screening regarding indications.
For CIDP, different treatments would be required according to the clinical subtypes; typical CIDP and asymmetric variabt). For multifocal neuropathy, maintainance treatment with immunoglobulin is necessary.
Here, we report the case of a 3-year-old child who was diagnosed with Bickerstaff's brainstem encephalitis presenting typical clinical features and interesting radiological findings. The patient showed ophthalmoplegia, ataxia, and subsequent stuporous mentality. Brain magnetic resonance imaging revealed high signal intensity in the pons and cerebellum around the 4th ventricle on a T2-weighted image. He was successfully treated with intravenous immunoglobulin. Differentiation of Bickerstaff's brainstem encephalitis and Miller Fisher syndrome is often difficult because they possess many overlapping features. Brain magnetic resonance imaging may be helpful in diagnosing Bickerstaff's brainstem encephalitis, especially when lesions are definitely found.
This review describes the characteristic US findings in PN reported to date and a classification of abnormal nerve US patterns in PN is proposed. Closer scrutiny of nerve abnormalities beyond assessment of nerve calibre may allow for more accurate diagnostic classification of PN, as well as contribute to the understanding of the intersection of structure and function in PN.
This was significantly lower than that reported in a recent Dutch study (56% vs 64%). Cytoalbuminologic dissociation was also lower in the Asian cohort (55% vs 64%), with a significantly higher proportion of patients with mild pleocytosis (26% vs 15%). A lower proportion of the 164 patients with MFS had elevated CSF protein level (38% vs 56%), mild pleocytosis (11% vs 26%) and cytoalbuminologic dissociation (41% vs 55%) compared to patients with GBS. In both conditions, cytoalbuminologic dissociation was linked to the timing of lumbar puncture. Cytoalbuminologic dissociation was only observed in half of the Asian patients with GBS and MFS, and it is strongly dependent on the timing of the lumbar puncture.
36/1 million vaccinations (95% credible interval -1.22 to 0.28) and -0.42/1 million vaccinations (95% credible interval, -3.68 to 2.44), respectively. These numbers represent a small absolute reduction in GBS risk with vaccination. Under typical conditions (e.g. influenza incidence rates >5% and vaccine effectiveness >60%), vaccination reduced GBS risk. These findings should strengthen confidence in the safety of influenza vaccine and allow health professionals to better put GBS risk in context when discussing influenza vaccination with patients.
AIM. To update the current indications of this technique in the treatment of neurological diseases. DEVELOPMENT. We conducted a thorough review of all articles about the efficacy of plasma exchange in the treatment of different neurological diseases published since the 80s. We have also carried out a detailed analysis of recommendations and evidence of the use of this procedure by analyzing the guidelines of different scientific societies. CONCLUSIONS. Plasma exchange has proven to be an effective alternative treatment with high grade scientific evidence in diseases such as Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy and myasthenia gravis. It has been effective in treating acute demyelinating episodes unresponsive to other therapies, neuromyelitis optica relapses and other central nervous system diseases induced by antibodies. In comparative studies with intravenous immunoglobulin efficacy of both therapies is similar. Comparative studies should continue to be conducted in order to better understand the mechanisms of action, prioritize indications and compare the cost-effectiveness ratio of both procedures.
This case implies that clinician should be on the alert to atypical sensory symptoms from the classical presentation of AMAN even if the patient is diagnosed with AMAN electrophysiologically and should consider proper treatment options based on clinical presentations.
The detailed mechanisms of axon degeneration itself have begun to be elucidated with studies of animal models with altered degeneration kinetics, including the slowed Wallerian degeneration (Wld(S)) and Sarm knockout animal models. These studies have shown axonal degeneration to occur through a programmed pathway of injury signaling and cytoskeletal degradation. Insights into the common disease insults that converge on the axonal degeneration pathway promise to facilitate the development of therapeutics that may be effective against other mechanisms of neurodegeneration.
We describe a case of pure laparoscopic augmentation ileocystoplasty in a patient affected by Guillain-Barre syndrome. Presentation: A 15-year-old female, complaining of voiding dysfunction, recurrent urinary tract infection and worsening renal function for three months. A previous history of Guillain-Barre syndrome on childhood was related. A voiding cystourethrography showed a pine-cone bladder with moderate post-void residual urine. The urodynamic demonstrated a hypocompliant bladder and small bladder capacity (190mL) with high detrusor pressure (54 cmH2O). Nonsurgical treatments were attempted, however unsuccessfully.
The host factors regulate a broad range of inflammatory processes that are involved in the pathogenesis of autoimmune diseases including GBS. Evidences suggest that systemically and locally released cytokines and their involvement in immune-mediated demyelination and axonal damage of peripheral nerves are important in the pathogenesis of GBS. Toll-like receptors (TLRs) link innate and adaptive immunity through transcription of several proinflammatory cytokines. TLR genes may increase susceptibility to microbial infections; an attenuated immune response towards antigen and downregulation of cytokines occurs due to mutation in the gene. Herein, we discuss the crucial role of host factors such as cytokines and TLRs that activate the immune response and are involved in the pathogenesis of the disease.