Pain abdomen and cardiac arrest as peculiar presenting symptoms in AIDP- AMSAN variety

AIDP (Acute Inflammatory Demyelinating Polyneuropathy) is a heterogenous condition encompassing several variants. It is a post infectious neurological disorder with an autoimmune pathogenesis with molecular mimicry mechanism. They present commonly with symmetrical ascending type paralysis and absent or diminished deep tendon reflexes. Cranial nerve palsies may or may not be present. The diagnosis is based on the clinical signs and symptoms, nerve conduction studies and cerebrospinal fluid analysis. We herewith report a rare case of AIDP, where the patient came walking to the emergency room with pain abdomen as the only complaint and had no neurological deficits at the time of presentation, hours later, the patient went into cardiac arrest, the cause of which was later thought to be dysautonomia and respiratory failure. The next day, patient developed motor weakness and multiple cranial nerve palsies which is an overlap of AMSAN and Acute Ophthalmoplegia. It is extremely uncommon to present with pain abdomen and cardiac arrest as presenting features in AIDP, AMSAN (Acute Motor Sensory Axonal Neuropathy) variety.

A rare presentation of Guillain-Barre syndrome with GQ1b positivity: A case report

Rationale: To report a case of cervicobrachial variant of acute inflammatory demyelinating polyneuropathy presenting with papilledema and GQ1b positivity. Patient concern: A 35-year-old female, 68 days postpartum, presented with headache, vomiting, and gait difficulty in swallowing with bilateral upper limb weakness and difficulty in walking, 13 days after ChAdOx1 nCoV-19 vaccination. Diagnosis: Guillain-Barre syndrome with GQ1b positivity. Intervention: Five cycles of plasmapheresis were given. Outcome: The patient's clinical condition improved. Palatal weakness improved and she could walk without support. There were mild sensory symptoms involving upper limbs which gradually improved. Lessons: AIDP should be considered in case of weakness following ChAdOx1 nCoV-19 vaccination. Albumino-cytological dissociation and anti-GQ1b positivity are needed to confirmed the diagnosis.

Clinical characteristics of patients with recurrent Guillain-barré syndrome / 中国神经精神疾病杂志

Objective To investigate the clinical features of recurrent Guillain-barré syndrome. Methods The data on demographic information and clinical features of 286 inpatients with Guillain-barré syndrome were collected. Patients were divided into recurrent group and non-recurrent group based on the number of episodes. The data from those patients with recurrent Guillain-barré syndrome whose information was missing was collected by telephone. Results we identified 22 (7.7%) cases with recurrent Guillain-barré syndrome including one case of Miller fisher syndrome. The total episodes were 55. Sixteen patients had 2 episodes, 3 patients had 3 episodes, 1 patients had 4 episodes and 2 patients had 5 episodes. Compared with non-recurrent group, patients with recurrent Guillain-barré syndrome had younger onset [(36.36 ±14.51)y vs. (45.72 ±16.13)y, t=-2.633, P=0.009], the shorter interval between the initial manifestations to peak [(2.18±0.73)d vs.(4.24±2.98)d, t=-8.537, P=0.000], the lower GBS disability score (68.2% vs. 31.8% , χ2=4.209, P=0.040) and less involvement in the cranial nerve (13.6% vs. 37.5% , χ2=5.040, P=0.025). Conclusion The recurrent risk may be considered in patients with Guillain-barré syndrome when younger patients, have lower GBS disability score and the shorter interval between the initial manifestations to peak.

Estudio electrodiagnóstico en síndrome de Guillain Barré en adultos / Electrophysiological assessment in Guillain-Barré syndrome in adults

El síndrome de Guillain Barré es una poliradiculoneuropatía aguda con varias formas de presentación y actualmente la principal causa de parálisis aguda arrefléctica en el mundo, su diagnóstico es clínico y los exámenes de apoyo como el electrodiagnóstico son utilizados para realizar el diagnóstico diferencial, clasificar entre las distintas variantes (desmielinizantes o axonales) y definir pronóstico. El propósito de este trabajo es realizar una revisión narrativa de la literatura disponible, haciendo énfasis en el estudio electrofisiológico, hallazgos precoces y describir los errores más frecuentes, como también destacar la importancia de realizar la clasificación del cuadro para orientar las acciones de rehabilitación hacia la recuperación funcional.

Guillain Barré syndrome is an acutepolyradiculoneuropathy with differentforms ofpresentation and is currently the leading cause ofacute arreflexicparalysis in the world, its diagnosis is clinical and additional assesment as electrodiagnosis are used for differential diagnosis, rank among the variants (demyelinating or axonal) and define prognosis. The purpose of this paper is to make a narrative review of the literature, emphasizing the electrophysiological study, describing early findings, the most common mistakes, while highlighting the importance of classification chart to guide actions towards rehabilitation and functional recovery.

Early Electrodiagnostic Features of Upper Extremity Sensory Nerves Can Differentiate Axonal Guillain-Barré Syndrome from Acute Inflammatory Demyelinating Polyneuropathy

BACKGROUND AND PURPOSE: Serial nerve conduction studies (NCSs) are recommended for differentiating axonal and demyelinating Guillain-Barré syndrome (GBS), but this approach is not suitable for early diagnoses. This study was designed to identify possible NCS parameters for differentiating GBS subtypes. METHODS: We retrospectively reviewed the medical records of 70 patients with GBS who underwent NCS within 10 days of symptom onset. Patients with axonal GBS and acute inflammatory demyelinating polyneuropathy (AIDP) were selected based on clinical characteristics and serial NCSs. An antiganglioside antibody study was used to increase the diagnostic certainty. RESULTS: The amplitudes of median and ulnar nerve sensory nerve action potentials (SNAPs) were significantly smaller in the AIDP group than in the axonal-GBS group. Classification and regression-tree analysis revealed that the distal ulnar sensory nerve SNAP amplitude was the best predictor of axonal GBS. CONCLUSIONS: Early upper extremity sensory NCS findings are helpful in differentiating axonal-GBS patients with antiganglioside antibodies from AIDP patients.

Clinical outcome of Gullian-Barre Syndrome in a tertiary care teaching hospital A prospective observational study

Background: Gullian-Barre Syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy (AIDP) with autoimmune background. The clinical management of GBS is by nerve conduction velocity (NCV) and supportive care, intravenous immunoglobulin’s (IVIG) and Plasmapheresis. We have studied the clinical outcome of Gullian-Barre Syndrome patients visiting to the tertiary care hospital in Andhra Pradesh. Material and methods: A cross sectional study was conducted in a tertiary care teaching hospital at Andhra Pradesh in 50 patients over the period of 2 years. Neurological examination like higher mental functions, cranial nerves, motor system, sensory system and autonomic system was done for all patients. Descriptive analysis of clinical presentation, type of GBS, occurrence of complications and final outcome was also done. Results: A total of 50 participants were included in the study. Majority (52%) of the study participants were aged below 40 years. Diabetes mellitus (DM) and hypertension (HTN) were the Vasa VK, Chowdary DB, Kalyani OM. Clinical outcome of Gullian-Barre Syndrome in a tertiary care teaching hospital – A prospective observational study. IAIM, 2016; 3(1): 105-109. Page 106 most common co-existing illnesses reported in 8% and 6% of study population respectively. Conclusion: The majority of the Guillain-Barre Syndrome patients recovered smoothly without going for complications. Prognostic outcome was poor in our study with increasing age and co-existing illness like diabetes mellitus or ischemic heart disease.

Catastrophic antiphospholipid syndrome – A case report.

Catastrophic antiphospholipid syndrome (CAPS) is a rare, potentially life-threatening condition, acute in onset , characterized by diffuse vascular thrombosis, leading to multiple organ failure in a short period of time in the presence of positive antiphospholipid antibodies (aPL). Lupus anticoagulant and anticardiolipin antibodies are the predominant antibodies associated with CAPS. Treatment options for CAPS include anticoagulation, steroids, plasma exchange, cyclophosphamide therapy, and intravenous immunoglobulin therapy. The high rate of mortality warrants greater awareness among clinicians for early diagnosis and treatment of CAPS. In this case report, 30-yearold post-partum female presented with progressive weakness, shortness of breath of grade IV and swelling of all the four limbs of 15 days duration with an episode of seizure. Investigations revealed MRV - cortical sinus venous thrombosis (CSVT) of transverse and sigmoid sinus, Raised anti-ds DNA anticardiolipin and lupus anticoagulant, 24 hour urinary proteins – 540 mg/day indicating clinical lupus nephritis. Weakness of all the limbs with areflexia indicated acute inflammatory demyelinating polyneuropathy (AIDP). 2D echocardiography- post partum dilated cardiomyopathy (DCMP).

Guillain-Barré Syndrome in the First Trimester of Pregnancy and its Improvement by Rehabilitation / General Medicine

A 31-year-old healthy mother of twins developed Guillain-Barré syndrome in her fourth gestational week of pregnancy. Impaired motor coordination, sensation, and joint position sense were observed. Immunoglobulin therapy was not performed given the early stage of pregnancy. She received rehabilitation to accommodate changes in her body shape and movements necessary for pregnancy, childbirth, and childcare. The patient delivered a healthy baby by cesarean section. By 42 weeks postpartum, she was capable of almost all housework activities and childcare. Family support was important in this case. Patient-oriented intervention, which included periodic confirmation and establishment of goals in each phase and continuity of intervention, was also essential.

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.