Guillain-Barre Syndrome Publications (7349)

Guillain-Barre Syndrome Publications

2014Aug
Eur J Pediatr Surg
Eur J Pediatr Surg 2014 Aug 21. Epub 2014 Aug 21.
Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.

Aim The ideal repair of esophageal atresia (EA) is primary anastomosis with closure of the fistula if present. Long gap or local circumstances prompt other procedures that occasionally lead to disastrous complications. The aim of this study was to analyze the management of these complications in a tertiary referral center.Read More

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.

Acute flaccid paralysis surveillance (AFP) is an essential strategy of the WHO's Polio Eradication Initiative. This is the first study conducted to estimate the incidence, etiology, distribution, and surveillance performance of AFP in Iraq.
Surveillance data about the AFP cases under the age of 15 years reported from Iraq during January 1997 to December 2011 were depended in the current study.Read More


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.

2014Aug
Viral Immunol.
Viral Immunol 2014 Aug 20. Epub 2014 Aug 20.
1 Department of Neurology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital , Shanghai, China .

Abstract Background: Guillain-Barré syndrome (GBS) is preceded by an infection in about two-thirds of patients. However, the infectious organism is often not identified. GBS secondary to Japanese encephalitis virus (JEV) infection has been reported only in India.Read More

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.

2014Jun
Klin. Mikrobiol. Infekc. Lek.
Klin Mikrobiol Infekc Lek 2014 Jun;20(2):50-54
Department of Infectious Diseases, Faculty Hospital Brno, Czech Republic, e-mail: pavel.polak@fnbrno.cz.

Campylobacteriosis belongs to the most frequent bacterial gastrointestinal infections worldwide. In the past several years, an increasing trend in the prevalence of campylobacteriosis has been observed in many countries. The rapid spread of antibiotic resistance in Campylobacter spp.Read More

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.

2014Aug
Saudi Med J
Saudi Med J 2014 Aug;35(8):861-4
Department of Pediatrics, Faculty of Medicine, King Abdulaziz University, PO Box 80215, Jeddah 21589, Kingdom of Saudi Arabia. Fax. +966 (12) 6408353. E-mail. osamam@hotmail.com.

Guillain-Barre syndrome (GBS) usually presents in a symmetrical ascending fashion of weakness. We present a 6-month-old male infant who presented to our emergency room with acute left-sided limb weakness and head lag 3 days after a febrile upper respiratory tract infection. A diagnosis of GBS was established by confirming high cerebrospinal fluid protein, motor nerve reduced amplitude, and prolonged conductions, and MRI T2 high signal intensity affecting the ventral roots of the spinal cord.Read More

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. 

2014Aug
J Foot Ankle Surg
J Foot Ankle Surg 2014 Aug 13. Epub 2014 Aug 13.
Chief, Podiatric Surgery Department, PinnacleHealth System, Harrisburg, PA.

Guillain-Barré syndrome is a serious disorder that occurs when the body's immune system mistakenly attacks the peripheral nervous system. This leads to nerve inflammation and damage that can cause muscle weakness and/or paralysis, including foot drop. Therapy ranges from supportive measures, such as physical therapy, to surgical intervention.Read More

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.

2014Jul
J. Neurol. Sci.
J Neurol Sci 2014 Jul 26. Epub 2014 Jul 26.
Institute of Neuroscience, Department of Clinical Neurophysiology, Uppsala University, Uppsala, Sweden. Electronic address: anna.rostedt.punga@neuro.uu.se.

T cells are important in the immunopathology of immune-mediated peripheral neuropathies (PNP) and activated vitamin D regulates the immune response through increasing the amount of regulatory T cells. An association between vitamin D deficiency and polyneuropathy has been stipulated; hence we assessed whether patients with primary immune-mediated PNP have low vitamin D [25(OH)D] levels.
Plasma levels of 25(OH)D were analyzed in 26 patients with primary immune-mediated PNP, 50 healthy matched blood donors and 24 patients with motor neuron disease (MND).Read More

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.

2014Jul
J Emerg Trauma Shock
J Emerg Trauma Shock 2014 Jul;7(3):149-54
Department of Neurology, Post Graduate Institute of Medical Education and Research, Chandigarh, Punjab and Haryana, India.

Acute flaccid paralysis (AFP) is a complex clinical syndrome with a broad array of potential etiologies that vary with age. We present our experience of acute onset lower motor neuron paralysis.
One hundred and thirty-three consecutive adult patients presenting with weakness of duration less than four weeks over 12 months period were enrolled.Read More

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.

2014Sep
J. Formos. Med. Assoc.
J Formos Med Assoc 2014 Sep 8;113(9):634-9. Epub 2012 Sep 8.
Department of Neurology, First Hospital, Jilin University, Changchun, PR China.

Since little has been reported in previous studies, we aimed to find the clinical and electrophysiologic characteristics associated with childhood Guillain-Barré Syndrome (GBS) in Northeast China.
The clinical and electrophysiologic data were collected and reviewed retrospectively in 33 children and 105 adults with GBS during the period between 2006 and 2010 from the First Hospital of Jilin University.
Most of the children with GBS were older than 8 years of age and symptoms were severe at GBS onset.Read More

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.

2014Aug
Biomed Res Int
Biomed Res Int 2014 30;2014:936891. Epub 2014 Jun 30.
Department of Anatomy and Cell Biology, College of Medicine, Dong-A University, Busan 602-714, Republic of Korea.

Studies have shown that lysosomal activation increases in Schwann cells after nerve injury. Lysosomal activation is thought to promote the engulfment of myelin debris or fragments of injured axons in Schwann cells during Wallerian degeneration. However, a recent interpretation of lysosomal activation proposes a different view of the phenomenon.Read More

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.

2014Jul
Pak J Med Sci
Pak J Med Sci 2014 Jul;30(4):914-6
Petrick Periyasamy, Department of Medicine, Universiti Kebangsaan Malaysia Medical Centre, Jalan Yaacob Latif, Bandar Tun Razak, 56000 Kuala Lumpur, Malaysia.

Fungaemia due to Paecilomyces lilacinus is generally not considered in AIDS patients because this condition is not categorised as an AIDS-indicator illness. We report a case of a 25-year-old lady who presented to our hospital with Guillain-Barré Syndrome, with the subsequent development of refractory fungaemia, multi-organ failure and disseminated intravascular coagulopathy. Amphotericin B was given as empirical antifungal therapy.Read More

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.

2014Aug
J. Neurol. Neurosurg. Psychiatr.
J Neurol Neurosurg Psychiatry 2014 Aug 5. Epub 2014 Aug 5.
Departments of Medicine and Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore.
2014Aug
Acta Clin Belg
Acta Clin Belg 2014 Aug 4:1551214Z00000000085. Epub 2014 Aug 4.

We report the case of a 62-year-old man who developed Guillain-Barré syndrome (GBS) following Hantavirus infection. Only three similar cases have been described in the literature so far. GBS is an autoimmune disease characterized by progressive symmetrical weakness of lower limbs extending to upper limbs and face and low or absent tendon reflexes.Read More

Prompt diagnosis is mandatory as GBS is a potentially life threatening disorder and needs timely treatment to ensure fast recovery and fewer complications.

2014Aug
Rinsho Shinkeigaku
Rinsho Shinkeigaku 2014 ;54(7):577-80
Department of Neurology, Brain Attack Center Ota Memorial Hospital.

Recurrence of Guillain-Barré syndrome (GBS) and Fisher syndrome (FS) is uncommon. We retrospectively studied the cases of 93 consecutive patients with GBS and FS who were admitted to our hospital between January 2000 and March 2013. We analyzed the clinical features of and anti- glycolipid antibodies in patients who experienced recurrence.Read More

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.

2014Aug
PLoS ONE
PLoS One 2014 1;9(8):e104074. Epub 2014 Aug 1.
Neuroscience Center, Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China; Department of Neurobiology, Care Sciences and Society, Karolinska Institute, Stockholm, Sweden.

Ganglioside as a neurotrophic drug has been hitherto widely used in China, although Guillain-Barré syndrome (GBS) following intravenous ganglioside treatment was reported in Europe several decades ago. We identified 7 patients who developed GBS after intravenous use of gangliosides (ganglioside+ group) and compared their clinical data with those of 77 non-ganglioside-associated GBS patients (ganglioside- group) in 2013, aiming at gaining the distinct features of ganglioside-associated GBS. Although the mean age, protein levels in cerebrospinal fluid (CSF) and frequency of cranial nerve involvement were similar between the two groups, the Hughes Functional Grading Scale (HFGS) score and the Medical Research Council (MRC) sum score at nadir significantly differed (4.Read More

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.

2014Aug
J. Neurol.
J Neurol 2014 Aug 1. Epub 2014 Aug 1.
Department of Internal Medicine I, Osaka Medical College, Daigakumachi 2-7, Takatsuki, Osaka, 569-8686, Japan, in1237@poh.osaka-med.ac.jp.

Guillain-Barré syndrome (GBS) is an acute, post-infectious, inflammatory, autoimmune peripheral neuropathy with a highly diverse clinical course and outcome. We classified GBS on the basis of patients' first nerve conduction and validated this system to be associated with outcome on the basis of electrophysiological characteristics during the acute phase of GBS. We retrospectively evaluated 40 GBS patients who underwent their first electrophysiological study within 14 days of onset and classified GBS into four patterns: (1) acute inflammatory demyelinating polyneuropathy (AIDP) pattern with sensory nerve conduction abnormalities (motor-sensory AIDP: MS-AIDP), (2) AIDP pattern without sensory nerve conduction abnormalities (motor AIDP: M-AIDP), (3) acute motor axonal neuropathy (AMAN) pattern, and (4) minor abnormalities pattern.Read More

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.

2014Aug
BMJ Case Rep
BMJ Case Rep 2014 30;2014. Epub 2014 Jul 30.
Department of Neurology, St George's Hospital, London, UK.

Bickerstaff's brainstem encephalitis is a rare syndrome defined by the triad of ophthalmoplegia, ataxia and decreased consciousness. It is considered to be a variant of Miller Fisher syndrome and Guillain-Barré syndrome but is differentiated from the two by the presence of central nervous system involvement, commonly in the form of impaired consciousness. We present an unusual case of Bickerstaff's encephalitis, where the patient presented with pseudobulbar affect.Read More

2014Jul
J Paediatr Child Health
J Paediatr Child Health 2014 Jul 30. Epub 2014 Jul 30.
Centre for Immunization and Respiratory Infectious Diseases, Public Health Agency of Canada, Canada.

To compare acute flaccid paralysis (AFP) surveillance systems used by members of the International Network of Paediatric Surveillance Units (INoPSU) across the five AFP surveillance performance indicators recommended by the World Health Organization (WHO) for the maintenance of polio-free certification.
A survey was administered to AFP surveillance co-ordinators in five INoPSU member countries (Australia, Belgium, Canada, New Zealand and Switzerland). Data collected included information on surveillance system processes, WHO-recommended performance indicators, investigative practices and final diagnoses of cases from 2006 to 2010.Read More


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.

2014Jul
Nat Rev Neurol
Nat Rev Neurol 2014 Jul 29. Epub 2014 Jul 29.
Departments of Medicine and Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Unit 09-01, Centre for Translational Medicine, 14 Medical Drive, Singapore 117599, Singapore.

Guillain-Barré syndrome (GBS) and its variant, Miller Fisher syndrome (MFS), exist as several clinical subtypes with different neurological features and presentations. Although the typical clinical features of GBS and MFS are well recognized, current classification systems do not comprehensively describe the full spectrum of either syndrome. In this Perspectives article, GBS and MFS are classified on the basis of current understanding of the common pathophysiological profiles of each disease phenotype.Read More

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.

2014Jul
J Clin Neurosci
J Clin Neurosci 2014 Jul 22. Epub 2014 Jul 22.
Department of Neurology, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, 22, Gwanpyeong-ro 170 beon-gil, Dongan-gu, Anyang-si, Gyeonggi-do, Republic of Korea. Electronic address: ssbrain@hallym.ac.kr.

Miller Fisher syndrome is typically associated with a preceding infection, especially with Campylobacter jejuni. We describe a patient with Miller Fisher syndrome following Orientia tsutsugamushi infection, which to our knowledge has not been previously reported.Read More

2014Jul
J. Pediatr.
J Pediatr 2014 Jul 22. Epub 2014 Jul 22.
Division of Infectious Diseases, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada. Electronic address: ari.bitnun@sickkids.ca.

To describe the spectrum of central nervous system complications of varicella-zoster virus (VZV) in children admitted to The Hospital for Sick Children between January 1999 and December 2012.
Children aged 1 month to 18 years (n = 84) admitted with neurologic manifestations associated with a characteristic VZV rash or a confirmatory laboratory test (positive lesion scraping or cerebrospinal fluid polymerase chain reaction) were included in the study. Acute neurologic complications were included if they occurred within 4 weeks of VZV infection.Read More

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.

2014Jul
J Neuroophthalmol
J Neuroophthalmol 2014 Jul 24. Epub 2014 Jul 24.
Department of Ophthalmology (MLM, AEBP, US, SY, AGL), Houston Methodist Hospital, Houston, Texas; Baylor College of Medicine (NL, AGL), Houston, Texas; Departments of Ophthalmology, Neurology, and Neurosurgery (AGL), Weill Cornell Medical College, Houston, Texas; UTMB (AGL), Galveston, Texas and the UTMD Anderson Cancer Center (AGL), Houston, Texas; and University of Iowa Hospitals and Clinics (AGL), Iowa City, Iowa.

: We present a woman with acute onset of bilateral ophthalmoparesis and ataxia occurring 4 weeks after gastroenteritis. Serum antibody titers against asialo-GM1 and GD1a, typically associated with inflammatory axonal neuropathies, were elevated but titers against anti-GQ1b, the most commonly found antibody found in the Miller Fisher variant of Guillain-Barre syndrome were not. No other etiology for ophthalmoparesis was found despite extensive patient evaluation.Read More

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.

2014Sep
Vaccine
Vaccine 2014 Sep 18;32(39):4985-92. Epub 2014 Jul 18.
Center for Health Research Northwest, Kaiser Permanente Northwest 3800 N. Interstate Avenue, Portland, OR 97227 United States. Electronic address: Allison.naleway@kpchr.org.

To assess risks for acute adverse events and pregnancy complications in pregnant women following monovalent 2009 H1N1 inactivated influenza (MIV) vaccination.
Within the Vaccine Safety Datalink, we compared rates of pre-specified medically attended events (MAE) occurring within 42 days of MIV vaccination to those occurring in matched cohorts that at the same gestational age were either unvaccinated or received seasonal trivalent inactivated influenza (TIV) vaccine. Using generalized estimating equation method, with a Poisson distribution and log link, we calculated adjusted incident rate ratios (AIRR).Read More


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.

2014Jul
J. Peripher. Nerv. Syst.
J Peripher Nerv Syst 2014 Jul 7. Epub 2014 Jul 7.
The University of Queensland Centre for Clinical Research, Queensland, Australia.

Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are thought to be autoimmune diseases. There have been many attempts to find an HLA association with GBS and CIDP with little success. There have been studies of other plausible genes in GBS and CIDP and the role of these genes in GBS and CIDP and the data from these genetic studies is reviewed.Read More

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.

2014Jul
Intern. Med.
Intern Med 2014 15;53(14):1569-73. Epub 2014 Jul 15.
Department of Medicine, Mito Kyodo General Hospital, University of Tsukuba, Japan.

A 67-year-old man was admitted due to weakness, coughing, shortness of breath and fever. He had decreased breath sounds in the left lung and muscle weakness in the lower and upper extremities. Chest imaging showed a mass in the left lung, and a biopsy revealed small cell lung cancer.Read More

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.

2014Apr
Ann Indian Acad Neurol
Ann Indian Acad Neurol 2014 Apr;17(2):199-201
Department of Neurology, KLE University's Jawaharlal Nehru Medical College, Belgaum, Karnataka, India.

Areflexia is one of the cardinal clinical features for the diagnosis of Guillain Barré syndrome. However, some patients may have sluggish proximal muscle stretch reflexes. Presence of thumb reflex, a distal stretch muscle reflex has not been documented in Guillain Barré syndrome.Read More


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.

2014Jul
J. Alzheimers Dis.
J Alzheimers Dis 2014 Jul 11. Epub 2014 Jul 11.
Departments of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore Phsyiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore.

A few studies have reported the association of autoantibodies to GM1 or GQ1bα with Alzheimer's disease (AD) or vascular dementia. Here we investigated whether patients with AD or vascular dementia had high titers of the anti-ganglioside antibodies. Sera were obtained from patients with AD (n = 22), vascular dementia (n = 14), Guillain-Barré syndrome, and multifocal motor neuropathy as well as normal controls.Read More

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.