Acute motor axonal neuropathy following anti-rabies human diploid cell vaccine: A rare case and review

Rationale: Guillain Barre syndrome (GBS) is an acute neurological illness leading to quadriparesis with respiratory involvement. It can be triggered by infections, vaccinations, surgery, trauma, transplantation and drugs. Anti-rabies cell culture vaccines introduced to overcome the high rate of neurological complications associated with tissue based rabies vaccine, can be very rarely associated with GBS. Patient concerns: A 50-year-old female presented with acute severe upper back pain evolving into pure motor quadriparesis following administration of human diploid cell vaccine for rabies. Diagnosis: Acute motor axonal neuropathy variant of GBS following anti-rabies human diploid cell vaccine. Interventions: Intravenous high dose steroids. Outcomes: Patient recovered completely within 1 month. Lessons: Although anti-rabies cell culture vaccines are highly immunogenic and safe, they are rarely associated with GBS. Clinicians should be aware of this link because prompt diagnosis and treatment can result in complete recovery and avoid complications.

Guillain-barre Syndrome Complicating Dengue Fever

Guillain-Barre syndrome (GBS) is an immune mediated demyelinating polyradiculo-neuropathy manifesting as as-cending paralysis with loss of deep tendon reflexe. It has been seen more commonly as a post infectious complica-tion of Campylobacter jejuni and Cytomegalovirus infection but a rare neurological manifestation of Dengue infec-tion. Here we are presenting such a case of Guillain-Barre syndrome as a complication of Dengue infection.

Prediction of Functional Outcome in Axonal Guillain-Barre Syndrome

OBJECTIVE: To identify the factors that could predict the functional outcome in patients with the axonal type of Guillain-Barre syndrome (GBS). METHODS: Two hundred and two GBS patients admitted to our university hospital between 2003 and 2014 were reviewed retrospectively. We defined a good outcome as being "able to walk independently at 1 month after onset" and a poor outcome as being "unable to walk independently at 1 month after onset". We evaluated the factors that differed between the good and poor outcome groups. RESULTS: Twenty-four patients were classified into the acute motor axonal neuropathy type. There was a statistically significant difference between the good and poor outcome groups in terms of the GBS disability score at admission, and GBS disability score and Medical Research Council sum score at 1 month after admission. In an electrophysiologic analysis, the good outcome group showed greater amplitude of median, ulnar, deep peroneal, and posterior tibial nerve compound muscle action potentials (CMAP) and greater amplitude of median, ulnar, and superficial peroneal sensory nerve action potentials (SNAP) than the poor outcome group. CONCLUSION: A lower GBS disability score at admission, high amplitude of median, ulnar, deep peroneal, and posterior tibial CMAPs, and high amplitude of median, ulnar, and superficial peroneal SNAPs were associated with being able to walk at 1 month in patients with axonal GBS.

Guillain-Barre syndrome with preserved reflexes and normal cerebrospinal fluid: a case study.

Guillain-Barre syndrome (GBS) is an acute fulminant polyradiculopathy, which typically manifests as areflexic paralysis with variable sensory and autonomic involvement. Typical cerebrospinal fluid (CSF) picture consists of an elevated CSF protein without pleocytosis (albumin-cytologic dissociation). There have been many case reports of atypical presentations of GBS; with normo-reflexia or even hyper-reflexia from Chinese/Japanese and European population but only a few from Indian Subcontinent. Also the typical CSF picture if not found, makes the diagnosis of GBS even more difficult. A 24-year‑old man presented with weakness of all 4 limbs of 4 days duration with the antecedent history of loose stools and fever. On examination, there was flaccid paralysis involving all the 4 limbs (lower limb weakness more than the upper limb) with preserved reflexes, no sensory or cranial nerve deficit, no bladder-bowel involvement; and a normal CSF study at presentation, which 1 week later showed albumin-cytologic dissociation. On electro-diagnostic studies, it was proven as a case of acute motor axonal neuropathy. Patient was managed with routine empirical antibiotics and intravenous methyl prednisone; after 3 weeks, patient was discharged in a stable condition without any residual deficit. Our understanding about the GBS has changed manifolds over the last few decades with many atypical variants being reported across the world. This case study is to lay stress on the fact that even in the absence of typical clinical features and a normal CSF study the diagnostic possibility of GBS should be kept if there is strong clinical suspicion.

Antibodies to the Antiganglioside GD1b in a Guillain-Barre Syndrome Patient with Bilateral Cerebellar Lesions

No abstract available.

Prevalence of Anti-Ganglioside Antibodies and Their Clinical Correlates with Guillain-Barre Syndrome in Korea: A Nationwide Multicenter Study

BACKGROUND AND PURPOSE: No previous studies have investigated the relationship between various anti-ganglioside antibodies and the clinical characteristics of Guillain-Barre syndrome (GBS) in Korea. The aim of this study was to determine the prevalence and types of anti-ganglioside antibodies in Korean GBS patients, and to identify their clinical significance. METHODS: Serum was collected from patients during the acute phase of GBS at 20 university-based hospitals in Korea. The clinical and laboratory findings were reviewed and compared with the detected types of anti-ganglioside antibody. RESULTS: Among 119 patients, 60 were positive for immunoglobulin G (IgG) or immunoglobulin M antibodies against any type of ganglioside (50%). The most frequent type was IgG anti-GM1 antibody (47%), followed by IgG anti-GT1a (38%), IgG anti-GD1a (25%), and IgG anti-GQ1b (8%) antibodies. Anti-GM1-antibody positivity was strongly correlated with the presence of preceding gastrointestinal infection, absence of sensory symptoms or signs, and absence of cranial nerve involvement. Patients with anti-GD1a antibody were younger, predominantly male, and had more facial nerve involvement than the antibody-negative group. Anti-GT1a-antibody positivity was more frequently associated with bulbar weakness and was highly associated with ophthalmoplegia when coupled with the coexisting anti-GQ1b antibody. Despite the presence of clinical features of acute motor axonal neuropathy (AMAN), 68% of anti-GM1- or anti-GD1a-antibody-positive cases of GBS were diagnosed with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) by a single electrophysiological study. CONCLUSIONS: Anti-ganglioside antibodies were frequently found in the serum of Korean GBS patients, and each antibody was correlated strongly with the various clinical manifestations. Nevertheless, without an anti-ganglioside antibody assay, in Korea AMAN is frequently misdiagnosed as AIDP by single electrophysiological studies.

Guillain Barre Syndrome: Major Cause of Acute Flaccid Paralysis in Children and Adolescents of Nepal.

Introduction. Guillain Barre Syndrome (GBS) is a post infectious polyneuropathy involving mainly motor but sometimes sensory and autonomic nerves. It is an acquired disease of the peripheral nerves that is characterized by rapidly progressing paralysis, areflexia and albumino-cytological dissociation in CSF. Methodology: Prospective, descriptive, observational, hospital based study was carried out to find out the clinico-epidemiological features of GBS including existing treatment modalities and its outcome. All cases fulfilled the criteria for AFP (Acute flaccid Paralysis) surveillance was included. Cases were reviewed for full medical history and examinations. To confirm the diagnosis, necessary investigations were carried out and combined with clinical symptoms. Results: Thirty patients were included in the study during study period. Among them 90% were diagnosed as GBS, 7.4% patients of GBS were associated with hypokalemic paralysis, 7.4% diagnosed as transverse myelitis and 3.7% diagnosed as idiopathic neuropathy. Different types of GBS were classified as AIDP (Acute inflammatory demyelinating polyneuropathy) 62.96%, AMAN (Acute motor axonal neuropathy) - 25.52%, AMASAN (Acute motor and sensory axonal neuropathy) - 3.3% and MFS (Miller fisher’s syndrome) - 6.6% according to NCV result. Male female ratio is 1.7:1.0. There was 14.8% patients had relapse within 5 year. Associated diseases were URTI, pneumonia, sore throat and diarrhea. Facial Nerve palsy was commonest cranial nerve involvement.Sixty percentage of patients presented with sensory symptoms. There was transient bowel and bladder involvement in 20% of the cases. 69.2% patients became bed ridden at the nadir. There was albumin-cytological dissociation in 80% case. Majority of patients improved with supportive treatment alone, 19.5% patient required ventilator support among them 40% died. 7.4% of cases expired during treatment. Half of the patients fully recovered within 3 months. Conclusion: GBS is the commonest cause of AFP, AIDP being commonest subtype in our setting. We have to improve our existing treatment facilities and extend to different centers to detect and treat GBS. Most of the patients improve with supportive treatment alone. Ventilator support indicates grave prognosis.

Electrophysiologic Subtypes and Prognosis of Childhood Guillain-Barre Syndrome / 대한소아신경학회지

PURPOSE: Guillain-Barre Syndrome(GBS) is an inflammatory polyneuropathy characterized by progressive, symmetric weakness and areflexia. GBS is currently subdivided into AIDP(acute inflammatory demyelinating polyneuropathy), AMAN(acute motor axonal neuropathy), AMSAN(acute motor sensory axonal neuropathy), and other subtypes based on results from electrophysiological studies. Our study is intended to evaluate the clinical characteristics and prognosis of pediatric GBS and its subtypes. METHODS: From January 1999 to June 2009, We retrospectively reviewed of fifty patients with GBS the clinical manifestations and laboratory findings at the Asan Medical Center. Forty-six patients were classified into subtypes based on the results from electrophysiological studies. RESULTS: Forty-five(90.0%) patients exhibited lower limb weakness, 26(56.5%) patients had both lower and upper limb weakness, and eleven(23.9%) had sensory nerve involvement seen at their initial presentation. Of the 46 patients, 22(47.8%) had AIDP, seven had AMAN(15.2%), 12 were unclassified(26.0%), and five had normal nerve conduction studies. The AIDP and AMAN groups were not significantly different in age, gender, and clinical characteristics. Five patients(22.7%) in the AIDP group and one patient(14.3%) in the AMAN group had respiratory failure, which did not differ significantly according to their group. However, the AMAN group needed a significantly longer period of time on average to walk independently than the AIDP group(99.2+/-97.0 days vs. 39.1+/-29.7 days, P=0.022). CONCLUSION: Our electrophysiological data show that 15.2% of the pediatric patients with GBS had the AMAN-type disease, and these children underwent longer periods of recovery than children in the AIDP group.

Síndrome de Guillain Barré en pediatría / Guillain-Barré syndrome in pediatrics

Este trabajo revisa el conocimiento actual sobre el síndrome de Guillain-Barré (SGB) en niños. El SGB se define como una parálisis flácida arrefléxica aguda y se clasifica en 4 subgrupos: polirradiculopatía aguda inflamatoria desmielinizante (AIDP), neuropatía axonal sensitivo-motora aguda (AMSAN), neuropatía axonal motora aguda (AMAN) y síndrome de Miller-Fisher (SMF). La AIDP se asocia en un 30-50% a compromiso de pares craneales, lo cual no se observa en la AMAN. El SMF se caracteriza por ataxia, oftalmoplejía y arreflexia, pero puede presentar también compromiso de pares craneales. Datos recientes de la anatomía patológica y la fisiopatología del SGB destacan la importancia de la infección por Campylobacter jejuni en la generación de anticuerpos anti-gangliósidos (GM1 en AIDP, GQ1b en SMF y GD1a en AMAN) que lesionan la mielina en AIDP y SMF y el axón en AMAN. El diagnóstico diferencial debe descartar enfermedades del sistema nervioso central (SNC) (encefalitis, encefalomielitis, mielitis), síndromes miasténicos, neuropatías tóxicas por metales pesados, fármacos, substancias químicas o toxinas animales y cuadros miopáticos, especialmente la miositis aguda infecciosa benigna y la neuromiopatía del paciente en la unidad de cuidados intensivos. Es importante el tratamiento con inmunoglobulina en dosis total de 2 gramos por kilogramo a administrar en 48 horas. La plasmaféresis puede ser igualmente eficaz. El SGB tiene buen pronóstico en niños, con una recuperación total en el 85% de los casos. La rehabilitación es fundamental para lograr una recuperación más rápida e integral.

This paper reviews the current knowledge about Guillain- Barré syndrome (GBS). GBS is defined as an acute, areflexic, flaccid paralysis, which is classified into 4 subgroups: acute inflammatory demyelinating polyneuropathy (AIDP), acute motor-sensory axonal neuropathy (AMSAN), acute motor axonal neuropathy (AMAN) and Miller-Fisher syndrome (MFS). AIDP is associated in 30-50% of cases with cranial nerve involvement, which is not observed in AMAN. MFS is characterized by ataxia, ophthalmoplegia and areflexia, but it may also present cranial nerve dysfunction. Recent data on the pathology and pathophysiology of GBS emphasize the important role of Campylobacter jejuni infection in generating anti-ganglioside antibodies (GM1 in AIDP, GQ1b in MFS and GD1a in AMAN), which damage myelin in AIDP and MFS and axons in AMAN. The differential diagnosis must rule out other disorders of the central nervous system (encephalitis, encephalomyelitis, myelitis), myasthenic syndromes, toxic neuropathies induced by heavy meals, drugs, chemical substances or animal toxins, and myopathic conditions, especially acute benign infectious myositis and neuromyopathy of the intensive care unit patient. It is important the treatment with immune globulin, at a total dose of 2 grams per kilogram administered over 48 hours. Plasmapheresis can be equally effective. GBS has a good prognosis in children with a total recovery in 85% of cases. Rehabilitation is crucial to attain a more rapid and global improvement.

Clinical Characteristics of the Subtypes of Guillain-Barre Syndrome according to the Electrodiagnositic Criteria

BACKGROUND: Guillain-Barre syndrome (GBS) is defined as a recognizable clinical entity that is characterized by rapidly evolving symmetric limb weakness, loss of tendon reflexes, absent or mild sensory signs, and variable autonomic dysfunctions. Recently, GBS has been classified as a classical demyelinating (acute imflammatory demyelinating polyradiculoneuropathy, AIDP) and two axonal (acute motor axonal neuropathy, AMAN, and acute motor sensory axonal neuropathy, AMSAN) forms. The clinical pattern and prognosis according to type is not clear. METHODS: Forty-one patients clinically diagnosed as GBS were enrolled and classified as AIDP, AMAN, and AMSAN according to electrodiagnostic criteria. We analyzed the clinical data of each subgroup; age, sex, seasonal distribution, history of previous illness, cranial nerve involvement, respiratory involvement, and motor weakness. RESULTS: Forty-one patients with GBS were comprised of 19 patients (46.3%) with AIDP, 12 patients (29.2%) with AMAN, and 10 patients (24.3%) with AMSAN. AIDP was found more frequently in males and in winter (42.1%) while axonal forms of GBS showed neither gender nor seasonal predominance. Frequency of cranial nerve involvement was not different between the sub-groups of GBS, whereas respiratory involvement was more frequent in AMSAN (50%). Upper limbs were weaker in axonal than in demyelinating types of GBS. CONCLUSIONS: Axonal forms of GBS showed some clinical characteristics distinctive from the demyelinating forms, which might be useful in the differential diagnosis of subgroups of GBS. (J Korean Neurol Assoc 19(5):503~508, 2001)

Acute Motor Axonal Neuropathy Associated with Anti-GM1 Antibody: A case report

Guillain-Barre syndrme (GBS) has several subtypes that are divided by clinical, electro- physiological, and pathological findings. A novel form of GBS, that is termed acute motor axonal neuropathy (AMAN), is characterized by the selective involvement of motor fibers, and is associated with anti-GM1 antibodies. A 8-year-old male patient were developed ascending, symmetrical paralysis, and areflexia, but no sensory disturbance. Elevated titers of serum IgG anti-GM1 antibodies were detected. His thoracolumbar spine magnetic resonance imaging (MRI) revealed thickening of cauda equina and enhancement of anterior nerve roots of T12-L1 spinal level after Gd-DTPA infusion. Electrophysiological diagnosis was acute motor axonal neuropathy (AMAN). We report this case with review of the literature.

A Case of Acute Motor Axonal Neuropathy / 대한소아신경학회지

In northern China, annual epidemics of acute-onset flaccid paralysis diagnosed clinically Guillain-Barre syndrome have been observed for at least 20 years. These patients had a distinctive pattern that shares clinical and cerebrospinal fluid findings with demyelinating Guillain-Barr syndrome. But it was different from Guillain-Barr syndrome physiologically and pathologically. Electrodiagnostic studies showed normal motor distal latencies and limb conduction velocity, but reduced compound muscle action potential amplitude. When sensory nerve action potentials are elicitable, F waves are within the range of normal. This disorder was named acute motor axonal neuropathy characterized pathologically by motor nerve fiber degeneration of variable severity and by sparing of sensory fibers. Recovery is usually complete. We experienced a case of acute axonal motor neuropathy, and reported the case with a review of literature.