The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma
The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma
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DOI：
10.3171/jns.1999.91.2.0212
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发表时间：
1999-08-01
影响因子：
4.1
通讯作者：
Nishi, Y
中科院分区：
文献类型：
--
作者：
Arita, K;Ikawa, F;Nishi, Y
Object.下丘脑错构瘤的诊断通常基于其磁共振（MR）成像特征和患者的临床症状，但神经放射学表现和临床表现之间的关系从未得到充分研究。在这项回顾性研究中，作者试图确定这种关系。作者根据MR表现将11例下丘脑错构瘤分为两类。7例为“下丘脑旁型”，其中错构瘤仅附着于第三脑室底部或通过蒂悬挂于底部。4例为“下丘脑内型”，其中错构瘤累及下丘脑或被下丘脑包裹，肿瘤扭曲了第三脑室; 6例为下丘脑旁型，表现为性早熟，由促黄体生成素释放激素类似物控制，1例无症状。在该组中未观察到癫痫发作或精神发育迟滞。所有下丘脑内型的患者都有药物难治性癫痫发作，其中1例出现性早熟。在两名患者中观察到严重的精神发育迟滞和行为障碍，包括攻击性。只有1例患者的癫痫发作得到控制，其中立体定向靶向照射的病灶performed.This拓扑结构/症状的关系，再次证实了61例报告的错构瘤，其中的MR表现清楚地描述了审查。下丘脑旁型通常与性早熟有关，但不伴有癫痫发作或发育迟缓，而下丘脑内型通常与癫痫发作有关。三分之二的后者患者经历发育迟缓，一半也表现出早熟青春期。根据MR结果将下丘脑错构瘤分为这两类，导致症状和随后的临床病程之间存在明显的相关性。
Object. Hypothalamic hamartoma is generally diagnosed based on its magnetic resonance (MR) imaging characteristics and the patient's clinical symptoms, but the relationship between the neuroradiological findings and clinical presentation has never been fully investigated. In this retrospective study the authors sought to determine this relationship.Methods. The authors classified 11 cases of hypothalamic hamartoma into two categories based on the MR findings. Seven cases were the "parahypothalamic type," in which the hamartoma is only attached to the floor of the third ventricle or suspended from the floor by a peduncle. Four cases were the "intrahypothalamic type," in which the hamartoma involved or was enveloped by the hypothalamus and the tumor distorted the third ventricle.Six patients with the parahypothalamic type exhibited precocious puberty, which was controlled by a luteinizing hormone-releasing hormone analog, and one patient was asymptomatic. No seizures or mental retardation were observed in this group. All patients with the intrahypothalamic type had medically intractable seizures, and precocious puberty was seen in one. Severe mental retardation and behavioral disorders including aggressiveness were seen in two patients. The seizures were controlled in only one patient, in whom stereotactically targeted irradiation of the lesion was performed.This topology/symptom relationship was reconfirmed in a review of 61 reported cases of hamartoma, in which the MR findings were clearly described. The parahypothalamic type is generally associated with precocious puberty but is unaccompanied by seizures or developmental delay, whereas the intrahypothalamic type is generally associated with seizures. Two thirds of patients with the latter experience developmental delays, and half also exhibit precocious puberty.Conclusions. Classification of hypothalamic hamartomas into these two categories based on MR findings resulted in a clear correlation between symptoms and the subsequent clinical course.