Redefining the Guillain-Barré Spectrum in Children: Neuroimaging Findings of Cranial Nerve Involvement

Abstract

SUMMARY: GBS and its MFS variant are acute polyneuropathies that are considered to represent a continuum rather than distinct entities, due to the overlap in their clinical features. Enhancement of the CE roots represents the neuroradiologic hallmark of GBS, while findings of neuroimaging studies in MFS are usually unremarkable. Our purpose was to evaluate the MR imaging findings of polyneuropathy in 17 children affected by GBS and its MFS variant. Fourteen of our 17 patients demonstrated CE enhancement, with predominant involvement of the anterior roots. Of 6 patients who underwent MR imaging of the brain, 5 had cranial nerve involvement. In children affected by GBS-MFS, involvement of the CE roots may be considered part of a more extensive autoimmune neuropathy, as demonstrated by enhancement of cranial nerves. Brain MR imaging should be considered in the routine evaluation in pediatric patients with GBS-MFS for the evaluation of the cranial nerves.

Emergency Neurological Life Support: Acute Non-traumatic Weakness

“…In children, the inability to walk may be due to weakness, pain, or ataxia and these distinctions can be a challenge to make in field or the Emergency Department. History Typically follows excessive magnesium administration in context of renal impairment More likely when supranormal magnesium levels targeted (e.g., in management of pre-eclampsia) Lethargy and confusion are most common neurologic manifestations As concentrations rise, generalized weakness develops, which progresses to involve muscles of respiration resulting in respiratory failure Common causes of acute, non-traumatic weakness in children include acute transverse myelitis, seizure, acute demyelinating encephalomyelitis, GBS, myasthenia gravis, and migraine [5][6][7][8][9]. While stroke in children is rare compared to adults, this should be considered early in the differential diagnosis for children with established risk factors including sickle cell, congenital heart disease, or a prothrombotic disorder.…”

The Child With Acute Weakness

“…[4][5][6][7]10,11 Presenting signs include weakness and areflexia or hyporeflexia in nearly all patients as well as neuropathy, with myalgias or leg pain and paraesthesias. 3,4,[6][7][8]10 Children experience pain to a much higher extent than adults. Ataxia may also be present due to weakness or associated with the Miller-Fisher variant.…”

“…ascending, essentially symmetric weakness and areflexia in a previously well child, 3 which peaks in less than 4 weeks, is followed by a plateau phase, and then has a slow recovery. 4 The disease is thought to be caused by the autoimmune-mediated destruction of the peripheral myelin sheath and inflammation of the nerve roots.…”

“…Ataxia may also be present due to weakness or associated with the Miller-Fisher variant. 3 Sensory symptoms and cranial neuropathies including facial palsy, ophthalmoplegia, or bulbar involvement occur in up to half of patients. [3][4][5][6] Patients may be nonambulatory or have bladder or bowel sphincter disturbance at presentation.…”

“…3 Sensory symptoms and cranial neuropathies including facial palsy, ophthalmoplegia, or bulbar involvement occur in up to half of patients. [3][4][5][6] Patients may be nonambulatory or have bladder or bowel sphincter disturbance at presentation. 4,8 Guillain-Barré syndrome must be differentiated from transverse myelitis (TM) and anterior spinal …”