Kanda T, Nagashima T, Oda M, Hirose K, Tanabe H
Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan.
J Neurol Sci. 1991 May;103(1):42-7. doi: 10.1016/0022-510x(91)90281-b.
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.
本文描述了一名患有假性假甲状旁腺功能减退症（PPHP）并伴有复发性神经病变的21岁女孩的临床和病理表现。发作性加重伴有颅内高压，使用皮质类固醇后症状缓解。尸检时，病理变化几乎仅限于周围轴突，表现为有髓纤维远端为主的消耗，无任何活动性髓鞘破坏或炎症改变。这种神经病变被认为与慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）相似；然而，PPHP与CIDP之间的关系（如果有的话）尚不清楚。
