Fujita J, Kobari M, Takizawa K, Nakayama N
Department of Neurology, Tachikawa Hospital.
Rinsho Shinkeigaku. 2000 Jun;40(6):611-3.
We report a case of HTLV-1 associated myelopathy (HAM) with pseudopseudohypoparathyroidism (PPHP). A 57-year-old woman, without a history of blood transfusion, was admitted with numbness and pain in her legs. Pain sensation in both legs was reduced and tendon reflexes were exaggerated. She exhibited spastic gait. The diagnosis of HAM was made because of positive antibody for HTLV-1 in blood and CSF. She also had features of Albright syndrome (short stature, round face, and short fourth metatarsals) without abnormal calcium and phosphorus metabolism, leading to the diagnosis of PPHP. Brain CT revealed bilateral calcification of the basal ganglia. Although HAM rarely accompanies pseudohypoparathyroidism (PHP), there are few reported cases of HAM associated with PPHP. The exact mechanism of HAM is not known, but it is thought to involve an immune-related process. Since PHP is considered to induce a state of mild immunosuppression through reduction of 1,25-dihydroxyvitamin D3, it is possible that susceptibility to HAM could be augmented in the presence of PHP. If PPHP should be an incomplete form of PHP, a similar mechanism may be involved in the association of PPHP and HAM.
我们报告一例与假性甲状旁腺功能减退症（PPHP）相关的人类嗜T淋巴细胞病毒1型（HTLV-1）相关脊髓病（HAM）。一名57岁女性，无输血史，因双腿麻木和疼痛入院。双下肢痛觉减退，腱反射亢进。她表现出痉挛性步态。由于血液和脑脊液中HTLV-1抗体呈阳性，故诊断为HAM。她还具有Albright综合征的特征（身材矮小、圆脸和第四跖骨短），但钙磷代谢无异常，从而诊断为PPHP。脑部CT显示双侧基底节钙化。虽然HAM很少伴有假性甲状旁腺功能减退症（PHP），但HAM与PPHP相关的报道病例很少。HAM的确切机制尚不清楚，但认为涉及免疫相关过程。由于PHP被认为通过降低1,25-二羟维生素D3诱导轻度免疫抑制状态，因此在存在PHP的情况下，患HAM的易感性可能会增加。如果PPHP是PHP的不完全形式，则PPHP与HAM的关联可能涉及类似机制。
