The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma

Citation
K. Arita et al., The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma, J NEUROSURG, 91(2), 1999, pp. 212-220
Citations number
63
Categorie Soggetti
Neurology,"Neurosciences & Behavoir
Journal title
JOURNAL OF NEUROSURGERY
ISSN journal
00223085 → ACNP
Volume
91
Issue
2
Year of publication
1999
Pages
212 - 220
Database
ISI
SICI code
0022-3085(199908)91:2<212:TRBMRI>2.0.ZU;2-X
Abstract
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 "parahypothalami c 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 "i ntrahypothalamic 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, w hich was controlled by a luteinizing hormone-releasing hormone analog, and one patient was asymptomatic. No seizures or mental retardation were observ ed in this group. All patients with the intrahypothalamic type had medicall y intractable seizures, and precocious puberty was seen in one. Severe ment al 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 report ed 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 intrahypot halamic type is generally associated with seizures. Two thirds of patients with the latter experience developmental delays, and half also exhibit prec ocious puberty. Conclusions. Classification of hypothalamic hamartomas into these two categ ories based on MR findings resulted in a clear correlation between symptoms and the subsequent clinical course.