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Hypocretin/orexins (Hcrt) are newly discovered hypothalamic peptides that have aroused interest after two recent reports linking the dysfunction of the Hcrt peptide/receptor system in animal models of narcolepsy.1,2⇓ Subsequently, Nishino et al.3 reported that CSF Hcrt-1/orexin A levels were decreased in idiopathic cases of human narcolepsy. However, the potential role of Hcrt in other sleep disorders remains unknown. Here, we describe a girl with mid- and posterior hypothalamic destruction that resulted in hypersomnolence and decreased CSF Hcrt-1 level.
Case report.
Discovery of a tumor in a 16-year-old girl with headache prompted a neurosurgical translamina terminalis resection to remove a suprasellar Grade 2 pilocystic astrocytoma. The surgical report described the tumor as an intra-axial, poorly circumscribed lesion arising in the left hypothalamus, occupying both sides of the hypothalamus; the thalamus and other surrounding brain structures were spared. The tumor and its gliotic rim were removed throughout the prechiasmatic cistern, except for the portion adhering to the third ventricle in the upper right hypothalamus. Tumor removal resulted in hypothalamic injury to both sides of the ventromedial, dorsomedial nuclei, and perifornical regions as well as right nucleus of the posterior hypothalamus …
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