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.

Guillain-Barré syndrome and its variants: a case of acute motor-sensory axonal neuropathy in Jamaica / Síndrome de Guillain-Barré y sus variantes: un caso de la neuropatía axonal sensorial motor aguda en Jamaica

This paper reports a case of a Jamaican young woman who experienced flaccid quadriparesis and bulbar weakness over a three-week period after a gastrointestinal illness. Nerve conduction studies confirmed an axonal type neuropathy consistent with the acute motor-sensory axonal neuropathy variant of the Guillain-Barré syndrome. Recovery, although evident, was slow and was augmented after a course of intravenous immunoglobulin. The patient was discharged from hospital after three months but was re-admitted one week later and eventually succumbed to complications of the illness. This case serves as a reminder that Guillain-Barré syndrome is now the most common cause of acute flaccid paralysis and should be considered early in all patients presenting with flaccid quadriparesis.

El presenta trabajo reporta el caso de una joven jamaicana que experimentó debilidad bulbar y cuadriparesiaflácida por un período de tres semanas después de una enfermedad gastrointestinal. Los estudios de conducción nerviosa confirmaron una neuropatía de tipo axonal en correspondencia con la variante de la neuropatía axonal sensorial motora aguda del síndrome de Guillain-Barré. La recuperación, aunque evidente, fue lenta, y aumentó después de que se le aplicara inmunoglobulina intravenosa. La paciente fue dada de alta del hospital después de tres meses, pero fue ingresada de nuevo una semana más tarde, falleciendo finalmente a causa de las complicaciones de la enfermedad. Este caso sirve como recordatorio de que el síndrome de Guillain-Barré es ahora la causa más común de parálisis flácida aguda, y debe tenerse en cuenta temprano en todos los pacientes que acuden con cuadriparesia flácida.

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)