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The clinical and pathological findings of a 21-year-old girl suffering from pseudopseudohypoparathyroidism (PPHP) with relapsing neuropathy are described. Episodic exacerbations were accompanied by intracranial hypertension and were relieved by the administration of corticosteroids. At autopsy, pathologic changes were almost restricted to the peripheral axons and showed distal dominant depletion of myelinated fibers without any active myelin breakdown or inflammatory changes. The neuropathy is thought to be similar to chronic inflammatory demyelinating polyradiculoneuropathy (CIDP); however, the relationship, if any, between PPHP and CIDP is unknown.
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