The spectrum of polyneuropathies in childhood detected with electromyography

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Pediatr Neurol. 2007 Jun;36(6):393-6.

The spectrum of polyneuropathies in childhood detected with

electromyography.

Shabo G, Pasman JW, van Alfen N, Willemsen MA.

Department of Pediatric Neurology, Radboud University Nijmegen

Medical Centre, Nijmegen, The Netherlands.

Only a few studies have been reported describing polyneuropathies in

a series of children. To study the clinical and neurophysiological

spectrum of polyneuropathies in a large series of children and

obtain an overview of their etiologies, this retrospective study

reevaluated all electromyograms and electrophysiologic studies

performed between 1995 and 2004 in children under 17 years of age at

the Radboud University Nijmegen Medical Center, a tertiary

neuromuscular reference center.

Electromyograms revealing polyneuropathy were selected for further

analysis (n = 118), and the medical records were reviewed to

supplement electromyographic findings with the clinical diagnosis.

Hereditary polyneuropathies made up 68% of the total, and 54% of

these were isolated polyneuropathies; in the remaining 46%,

polyneuropathy was part of a more complex disorder. The acquired

polyneuropathies were primarily inflammatory.

Nerve biopsies had been performed in 22 of the 118 cases (19%) and

led to a diagnosis in 4 cases. Despite sophisticated investigation,

11 cases (9%) remained unclassified for underlying cause.

Hereditary motor and sensory neuropathies are the most common type

of polyneuropathy in childhood, followed by polyneuropathies as part

of an inborn error of metabolism and inflammatory polyneuropathies

(in patients in whom electromyography was used to diagnose the

neuropathy).

In the full series of patients, nerve biopsy did not play a

prominent role in the diagnostic work-up of childhood

polyneuropathies, due to the increasing availability of other

laboratory (genetic and metabolic) diagnostic tools. Nerve biopsy

nonetheless proved to have an important diagnostic yield in

selected, complex cases.