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患有与甲状腺功能减退症和多发性颅内钙化相关的不可逆脑损伤的儿童。

Children with irreversible brain damage associated with hypothyroidism and multiple intracranial calcifications.

作者信息

Arii Junko, Tanabe Yuzo, Makino Michiko, Sato Hirokazu, Kohno Yoichi

机构信息

Chiba Rehabilitaiton Center, Department of Pediatrics, Graduate School of Medicine, Chiba University, Chiba-shi, Japan.

出版信息

J Child Neurol. 2002 Apr;17(4):309-13. doi: 10.1177/088307380201700416.

Abstract

Children who develop clinical hypothyroidism in early childhood have various degrees of irreversible brain damage, albeit less severe than cases detected by neonatal screening test for hypothyroidism in the first months of the life. We report three patients with hypothyroidism of childhood onset after a normal neonatal thyroid-stimulating hormone screening who showed deceleration in linear growth, spasticity in the lower limbs with deformity, mild intellectual impairment, and multiple calcifications in the basal ganglia and subcortical areas. The neurologic symptoms were not progressive but were irreversible in spite of thyroxine treatment. Motor disturbances commonly observed in postnatal-onset hypothyroidism are similar to those of cerebral palsy. Specific distribution of intracranial calcifications may result from metabolic derangement as a result of hypothyroidism, although the mechanism of calcification is not fully understood. We emphasize the need to re-evaluate thyroid function in diplegic patients with specific intracranial calcifications but normal neonatal thyroid-stimulating hormone screening.

摘要

在幼儿期出现临床甲状腺功能减退的儿童存在不同程度的不可逆脑损伤,尽管其严重程度低于出生后最初几个月通过新生儿甲状腺功能减退筛查检测出的病例。我们报告了3例新生儿甲状腺刺激素筛查正常但在儿童期发病的甲状腺功能减退患者,他们表现为线性生长减速、下肢痉挛伴畸形、轻度智力障碍以及基底神经节和皮质下区域多处钙化。尽管进行了甲状腺素治疗,神经症状并无进展但不可逆转。产后发病的甲状腺功能减退中常见的运动障碍与脑瘫相似。尽管钙化机制尚未完全明确,但颅内钙化的特定分布可能是由于甲状腺功能减退导致的代谢紊乱所致。我们强调,对于有特定颅内钙化但新生儿甲状腺刺激素筛查正常的双侧瘫患者,需要重新评估甲状腺功能。

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