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).

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.