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CIDP: Childhood onset, Chronic

Note: Biopsies are all from children who

• Met strict criteria for CIDP
• Had weakness for more than 2 years
• Improved prominently in strength & ADL after corticosteroid treatment

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Nerve: Hypomyelination & Onion Bulbs

Toluidine blue stain Many thinly myelinated large axons Small onion bulbs

Toluidine blue stain Large axons surrounded by small onion bulbs

Toluidine blue stain

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Nerve: Onion bulbs, Large

Toluidine blue stain Large onion bulbs

Toluidine blue stain

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Toluidine blue stain Onion bulbs: Patchy distribution

Toluidine blue stain Onion bulbs: Around axons with thicker myelin sheath

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Nerve: Differential Axon Loss within a Fascicle

Toluidine blue stain Patchy involvement within one fascicle   More thinly myelinated axons in some areas

Toluidine blue stain Region of fascicle with relatively   abundant, thinly myelinated large axons

Toluidine blue stain Region of fascicle with few large axons Smaller myelinated and unmyelinated axons predominate

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Hypomyelinating neuropathy, Infant onset

Toluidine blue stain Larger axons: Very thin myelin sheath

Toluidine blue stain

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CIDP: Type 1 predominance is common in muscle

ATPase, pH 9.4 stain Type 1 muscle fiber predominance

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Return to Childhood CIDP

7/12/2017