Guillain-Barre Syndrome Publications (7349)
Guillain-Barre Syndrome Publications
Patients and Methods A retrospective review of patients treated for EA between 1993 and 2013 was conducted. Both the patients were primarily treated by us, and referrals from elsewhere after two or more failed operations were included. Results In total, 23 patients were included (3/176 cases of EA treated primarily by us and 20 referrals). Of the 23 patients, 6 had type I EA, 15 type III (four long gaps), 1 type IV, and 1 type V. Cardiac anomalies were associated in seven cases, duodenal atresia in three, and Down syndrome in two patients. Primary anastomosis was initially achieved in 12 patients. Primary or secondary Foker lengthening was used in seven cases. The causes of the failure were anastomotic leaks in nine, unmanageable strictures in seven, and refistulization in five patients. These patients required 66 reoperations (median of 3 [2-7]) before inclusion in the study. Radical tertiary treatment consisted of 15 esophageal replacements (11 colonic grafts and 4 gastric pull-ups), and 1 esophageal-gastric disconnection. Five patients previously treated with esophageal replacement and referred for graft problems required 13 interventions. Two families did not give consent for one replacement and one disconnection. Complications appeared in 12 patients, and 9 additional operations were required in 7 patients. With a follow-up of 31 months (range, 4-139 months) 15 patients take all their meals per os, 5 occasionally use the gastrostomy, and 2 and 1 are fed exclusively via gastrostomy or jejunostomy. All tracheoesophageal fistulas were closed, but 15 cases are below p3 for weight and 12 for height. Three patients (13%) ultimately died 32 months (range, 9-56 months) after the first operation (due to aspiration in one, and for causes unrelated to it in the other two [tracheostomy obstruction and Guillain-Barré syndrome]). Conclusions When repeated complications appear after EA repair, radical surgical attitudes may be justified. If esophageal continuity cannot be reestablished, the native esophagus may have to be discarded and replaced. Many complications should be expected, but the end result can be good. These patients should be referred to centers with large experience in the management of this complex condition.
A total of 4974 cases of AFP were reported from Iraq during the study period, with an annual incidence of 2.5/100,000 population. Guillain-Barre syndrome represented more than half of the reported cases (N = 2611, 52.5%), followed by traumatic neuritis (N = 715, 14.4%), and other CNS infections (N = 292, 5.9%). Poliomyelitis accounted for 166 (3.3%) of cases, the last reported case being in January 2000. Surveillance performance showed that all, but two, indicators were below the required WHO recommended levels.
AFP surveillance remains the gold standard method for poliomyelitis detection. It witnessed dramatic changes over the last two decades. This has raised people's and clinicians' awareness to the importance of promptness in notifying suspected cases and timely transportation of stool specimens to the National Poliovirus Laboratory in Baghdad, or alternatively having more than one laboratory for poliovirus detection in the country, all of which are very useful measures to increase the surveillance performance in the country.
Herein, we report a case of GBS preceded by JEV infection in China. Case presentation: A 23-year-old male had generalized weakness, numbness in the extremities, and bilateral facial nerve paralysis. One week prior, he had a high fever with headache, and several days later, he developed facial diplegia and sensory disturbances. Physical examination revealed facial diplegia and a weak gag reflex, quadriparesis more pronounced distally, generalized hyporeflexia, and no Babinski sign. JEV IgM and hepatitis B surface antibody (HbsAb) tests were positive. Other tests for hepatitis B infection were negative. Nerve electrophysiology suggested an acute demyelinating sensorimotor polyradiculoneuropathy. His cerebrospinal fluid was clear, the leukocyte count was 5×10(6)/L (normal range: 0-5×10(6)/L), protein 0.62 g/L (normal range: 0.15-0.45 g/L), and JEV IgM was weakly positive. He was diagnosed with GBS associated with a recent JEV infection. Intravenous (IV) immunoglobulins combined with IV methylprednisone was administered for 5 days, and at the 3-month follow-up, a complete neurological recovery was noted. Conclusion: GBS may be associated with JEV infection. GBS exhibits a good response to intravenous immunoglobulin or plasma exchange and has a good prognosis making prompt diagnosis important.
also poses a major challenge. The authors review current knowledge on the microbiology of Campylobacter spp., complex pathogenetic as well as pathophysiological mechanisms in the development and course of campylobacteriosis and related complications such as Guillain-Barré syndrome.
He showed remarkable clinical and neurophysiological improvement after intravenous immunoglobulin and intensive physiotherapy. The occurrence of infantile acute hemiplegia as a presentation of GBS is rare. This report highlights the importance of considering GBS in the differential diagnosis so that early effective treatment may be started.
It appears from the published data that these patients, generally, have been left to their own devices, because no known cure is available, necessitating reliance on others for their daily activities and ambulation. We present a unique surgical option, with a follow-up period of 24 months, in which bilateral subtalar and ankle fusion allowed unassisted ambulation to a patient who might otherwise have never walked again.
INCAT score was assessed in patients with Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy. ALSFRS-R score was applied to MND patients and the modified Rankin (mRankin) scale compared disability among patient groups.
Mean 25(OH)D value in PNP patients was 40±16nmol/l, compared to 69±21nmol/l in healthy blood donors (p<0.001). MND patients had a higher mean 25(OH)D than PNP patients (59±26nmol/L; p=0.006) and comparable levels to healthy blood donors (p=0.15). Mean 25(OH)D value was not higher in PNP patients with pre-existing vitamin D3 supplementation of 800IU/day (N=6; 35±18nmol/L) than in unsupplemented PNP patients (42±16nmol). INCAT score ranged from 0 to 10 (mean 3.5) and ALSFRS-R ranged from 11 to 44 (mean 31). mRankin score was more severe in MND patients (mean 3.5) compared to PNP patients (mean 2.1).
All patients with primary immune-mediated PNP were diagnosed with vitamin D deficiency and they had significantly lower 25(OH)D values than healthy control persons and MND patients. We suggest monitoring of vitamin D status in patients with autoimmune PNP, since immune cells are responsive to the ameliorative effects of vitamin D.
Detailed history, clinical examination, and relevant investigations according to a pre-defined diagnostic algorithm were carried out. The patients were followed through their hospital stay till discharge or death.
The mean age was 33.27 (range 13-89) years with male preponderance (67.7%). The most common etiology was neuroparalytic snake envenomation (51.9%), followed by Guillain Barre syndrome (33.1%), constituting 85% of all patients. Hypokalemic paralysis (7.5%) and acute intermittent porphyria (4.5%) were the other important conditions. We did not encounter any case of acute polio mylitis in adults. In-hospital mortality due to respiratory paralysis was 9%.
Neuroparalytic snakebite and Guillain Barre syndrome were the most common causes of acute flaccid paralysis in adults in our study.
Simultaneous involvement of four limbs was the most common clinical feature, and cranial nerve involvement was common; however, previous infection, sensory nerve involvement and elevated proteins in cerebrospinal fluid occurred much less in the children with GBS than those in adult patients. Recruited children were classified as having acute inflammatory demyelinating polyneuropathy (AIDP; 41%), acute motor axonal neuropathy (AMAN; 38%), and were unclassified (21%). Electrophysiologic features and prognosis in these children were not different from those in adults. For children with AMAN, the efficacy of intravenous immunoglobulin (IVIg) was not different from that in adults; however, IVIg was not significantly effective for AIDP in these children.
Childhood GBS in Northeast China exhibits characteristics of clinical and electrophysiologic alternations; early diagnosis and appropriate treatments should be provided accordingly.
During Wallerian degeneration, lysosomes become secretory vesicles and are activated for lysosomal exocytosis. The lysosomal exocytosis triggers adenosine 5'-triphosphate (ATP) release from peripheral neurons and Schwann cells during Wallerian degeneration. Exocytosis is involved in demyelination and axonal degradation, which facilitate nerve regeneration following nerve degeneration. At this time, released ATP may affect the communication between cells in peripheral nerves. In this review, our description of the relationship between lysosomal exocytosis and Wallerian degeneration has implications for the understanding of peripheral nerve degenerative diseases and peripheral neuropathies, such as Charcot-Marie-Tooth disease or Guillain-Barré syndrome.
HIV screening was reactive and Paecilomyces lilacinus was isolated from her blood. The fungaemia did not resolve after one week of amphotericin B treatment. The addition of itraconazole was also unsuccessful in clearing the fungaemia. Accurate mycological diagnosis is important in the care of AIDS patients with fungaemia because of the risk of treatment failure with empirical therapy.
Prompt diagnosis is mandatory as GBS is a potentially life threatening disorder and needs timely treatment to ensure fast recovery and fewer complications.
Of the 93 patients, 53, 37, and 3 had GBS, FS, and overlapping GBS and FS, respectively. There were 6 recurrences in 4 patients, all of whom were women; their onset age ranged from 26 to 51 years, and the average time to recurrence ranged from 9 months to 25 years. The recurrence rate of FS was 10.8%. On the recurrence, 2 patients showed FS (5.4%) and 2 patients showed overlap of GBS and FS (5.4%). All patients with recurrence showed good prognosis and increased anti-GQ1b glycolipid antibody levels both at the initial episode and at recurrence. Immunological examinations, including those for detecting changes in anti-glycolipid antibodies, are important for clarifying the pathomechanism of recurrence in GBS and FS.
9±0.4 vs 3.6±1.0; 7.7±5.5 vs 36.9±14.5, both p<0.001), indicating a higher disease severity of ganglioside-associated GBS. A higher ratio of patients with ganglioside-associated GBS required mechanical ventilation (85.7% vs 15.6%, p<0.01). The short-term prognosis of ganglioside-associated GBS, as measured by the HFGS score and the MRC sum score at discharge, was poorer (4.3±0.5 vs 2.8±1.1; 17.3±12.9 vs 46.0±13.9, both p<0.001). All the patients in the ganglioside+ group presented an axonal form of GBS, namely acute motor axonal neuropathy (AMAN). When compared with the AMAN patients in the ganglioside- group, more severe functional deficits at nadir and poorer recovery after standard treatment were still prominent in ganglioside-associated GBS. Anti-GM1 and anti-GT1a antibodies were detectable in patients with AMAN while not in patients with the demyelinating subtype of GBS. The concentrations of these antibodies in patients with AMAN were insignificantly different between the ganglioside+ and ganglioside- groups. In sum, ganglioside-associated GBS may be a devastating side effect of intravenous use of gangliosides, which usually manifests a more severe clinical course and poorer outcome.
We compared the clinical, electrophysiological, and laboratory findings between groups and determined subgroups associated with poor outcome. The MS-AIDP and AMAN patterns more frequently exhibited prolonged recovery compared with the M-AIDP and minor abnormalities patterns and were associated with prolonged recovery (specificity, 100 %; sensitivity, 73 %; P < 0.001). The period of inability to walk independently was significantly longer in the MS-AIDP and AMAN patterns than in the M-AIDP and minor abnormalities patterns (median 85 vs. 10 days; P < 0.001). In conclusion, our classification of GBS using a single nerve conduction study in the early phase of disease is associated with outcomes. This classification can be used to counsel individual patients and guide decision-making with respect to treatment.
All countries contacted completed the survey. Each country used similar case definitions and processes for collecting AFP data. All countries used at least one of the WHO indicators for surveillance. No country consistently met the performance indicator for incidence or stool sampling. In all countries, at least one form of neurological testing was used to diagnose cases of AFP. Guillain-Barré syndrome was the most common final diagnosis in all countries for all years examined.
Industrialised countries surveyed do not consistently meet the WHO-recommended AFP surveillance performance indicators. An opportunity exists for INoPSU to suggest a standard way for member countries to collect AFP data in order to examine the potential for strengthening the current systems or introducing additional enterovirus surveillance or alternative/complementary neurological performance measures suitable for countries that have eliminated polio. INoPSU member countries are evaluating these possibilities.
GBS is subclassified into classic and localized forms (for example, pharyngeal-cervical-brachial weakness and bifacial weakness with paraesthesias), and MFS is divided into incomplete (for example, acute ophthalmoparesis, acute ataxic neuropathy) and CNS subtypes (Bickerstaff brainstem encephalitis). Diagnostic criteria based on clinical characteristics are suggested for each condition. We believe this approach to be more inclusive than existing systems, and argue that it could facilitate early clinical diagnosis and initiation of appropriate immunotherapy.
Stroke was considered related to VZV if it occurred within 6 months of VZV infection, the neuroimaging was characteristic, and other causes were excluded.
Clinical syndromes included acute cerebellar ataxia (n = 26), encephalitis (n = 17), isolated seizures (n = 16), stroke (n = 10), meningitis (n = 10), Guillain-Barré syndrome (n = 2), acute disseminated encephalomyelitis (n = 2), and Ramsay Hunt syndrome (n = 1). In those with acute complications (nonstroke), neurologic symptoms occurred a median of 5 days after rash onset (range -6 to +16). The time between rash onset and stroke ranged from 2 weeks to 26 weeks (median 16.0 weeks). Three children with encephalitis died. Residual neurologic sequelae at one year occurred in 9 of 39 (23%) of children with follow-up data. Only 4 children were reported to have received the varicella vaccine.
Neurologic complications of VZV infection continue to occur despite the availability of an effective vaccine. Neurologic symptom onset can predate the appearance of the VZV exanthem and in rare cases may occur in the absence of an exanthem.
Intravenous immunoglobulin was administered, and the patient gradually improved over subsequent months. This case is unique for its antiganglioside antibody profile associated with Miller Fisher syndrome.
Among 9349 women receiving MIV in any trimester, only one MAE occurred 0-3 days following MIV, an allergic reaction. No cases of Guillain-Barré syndrome, Bell's palsy, or transverse myelitis occurred 1-42 days after MIV. Compared to women receiving TIV and to unvaccinated women, risks for acute MAEs were not increased following MIV for any outcome. Hyperemesis was the most common adverse event in the MIV, TIV, and unvaccinated groups, occurring at a rate of about 4% over a 42-day period in all groups. Over a 42-day window, among all groups, incident gestational diabetes occurred at a rate of 3% and thrombocytopenia occurred at a rate of approximately 0.3%. Among women receiving MIV during pregnancy, increased risks for these and other less common obstetric events were not detected.
In this large cohort of pregnant women no acute safety signals were identified within 6 weeks of receipt of MIV.
Some of the genes that have been studied are immune related and some others have nervous system effects. The studies are limited by small numbers. Some of the genes show association with disease severity rather than disease susceptibility. The need for more detailed molecular studies of the role of HLA molecules and the need for modern genetic approaches to GBS and CIDP is explained.
The nerve conduction velocity was decreased, and anti-GM1 IgG antibodies were positive. The patient showed a temporary neurologic recovery following the administration of cancer chemotherapy, although he eventually died of progression of lung cancer. As a result of the almost simultaneous symptomatic development of lung cancer and Guillain-Barré syndrome, this case may be considered to involve a paraneoplastic neurologic syndrome.
We prospectively evaluated thumb reflex in Guillain Barré syndrome patients and age matched controls from April to September 2013.
There were 31 patients with Guillain Barré syndrome in whom thumb reflex could be elicited in all (24 brisk, 7 sluggish), whereas all the other muscle stretch reflexes were absent in 29 patients at presentation and the remaining two had sluggish biceps and quadriceps reflexes (P = 0.001). Serial examination revealed gradual diminution of the thumb reflex (P < 0.001). Rapid progression of weakness was associated with early loss of the thumb reflex.
Thumb reflex, a distal stretch reflex is preserved in the early phase of Guillain Barré syndrome.
Enzyme-linked immunosorbent assay showed titers of IgG and IgM anti-GM1, anti-GQ1bα, and anti-GT1aα antibodies did not differ among AD, vascular dementia, and normal controls, and being remarkably lower than those in Guilllain-Barré syndrome and multifocal motor neuropathy. The anti-ganglioside antibodies are not biological markers of AD.