Acute Inflammatory Demyelinating Polyradiculoneuropathy (Guillain-Barre Syndrome)
Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is a primarily demyelinating neuropathy with autoimmune etiology. Motor axons are affected more than sensory axons.
Incidence in children is similar to that seen in adults. Children often have a prodromal respiratory or gastrointestinal infection occurring within one month of onset. Common precipitating infections include Mycoplasma, cytomegalovirus, Epstein-Barr virus, Campylobacter jejuni, and various vaccinations. Weakness generally begins distally in the lower extremity, with a progressive ascending paralysis ultimately involving the upper limbs. Pain and sensory symptoms are not uncommon. The most common cranial nerve abnormality is an ipsilateral or bilateral lower motor neuron facial paralysis. Objective sensory loss has been documented in the minority of children (85). In one series, only 15% required mechanical ventilation (86). The maximal degree of weakness generally reaches a peak within two weeks of onset, and time to maximum recovery was 7 months ± 5 months in one series (87). Complete recovery occurs in most children. Classic criteria for poor recovery in adults (low-median CMAPs and fibrillation potentials) may not apply to children (87).Disturbances of the autonomic nervous system are common in children, including transient disturbances of bowel and bladder, excessive sweating or vasoconstriction, mild hypertension or hypotension, and occasionally cardiac arrhythmias.
The acute motor axonal neuropathy (AMAN) involves predominantly motor nerve fibers with a physiologic pattern suggesting axonal damage, whereas the acute inflammatory demyelinating polyneuropathy (AIDP) involves both motor and sensory nerve fibers with a physiologic pattern suggesting demyelination. Another clinical variant is the Miller-Fisher syndrome characterized by acute onset ataxia, ophthalmopare- sis, and areflexia.
Diagnosis is generally confirmed by electrodiagnostic studies (see Chapter 7), and the CSF protein is characteristically elevated in a majority of children. Serum autoantibodies that may be elevated include IgM and IgG versus beta-tubulin and heparin sulfate. AMAN patients may show increased IgG antibodies to GM1 ganglioside. The Miller-Fisher syndrome is associated with a high frequency of the IgG GQ1b antibodies. The major considerations in differential diagnosis of AIDP or AMAN include transverse myelitis, toxic neuropathies, tick paralysis, infantile botulism, myasthenia gravis, and dermatomyositis.
Treatment has typically included corticosteroids, plasma exchange, or, more recently, intravenous immune globulin (88,89,90,91). AIDP patients respond to both plasma exchange and intravenous immunoglobulin (IVIG). Patients with AMAN respond preferentially to IVIG over plasma exchange. Recovery is often quite good in children without treatment. After standard intravenous immunoglobulin therapy, children with axonal forms of Guillain-Barre syndrome (GBS) recover more slowly than those with the demyelinating form, but outcome at 12 months appears to be equally favorable in two groups (92).
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