Hypersomnia caused by isolated angiitis of the CNS.
A 42-year-old woman presented gradual development of hypersomnia and memory disturbance. T2-weighted and FLAIR MRI scans revealed a high intensity abnormality in the hypothalamus. Stereotactic brain biopsy specimen revealed prominent infiltration of the lymphocytes in the vessel walls. Since there was no evidence of systemic angiitis, the diagnosis of isolated angiitis of the CNS (IAC) was made. Polysomnographic study showed decreased REM sleep and increased stage I sleep. Orexin A in the CSF was decreased. Hypersomnia and a decrease of the CNS orexin A improved after corticosteroid therapy. Hypersomnia can be an initial symptom of IAC if it involves the hypothalamus.
Nakaji
Kayoko
K
Department of Neurology, Kyoto University Graduate School of Medicine, Japan.
Ikeda
Akio
A
Oka
Yasunori
Y
Tomimoto
Hidekazu
H
Shimohama
Shun
S
Kanbayashi
Takashi
T
Shibasaki
Hiroshi
H
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D001706:Biopsy; D006970:Disorders of Excessive Somnolence / Q000209:etiology; D005260:Female; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins / Q000134:cerebrospinal fluid; D008569:Memory Disorders / Q000209:etiology; D009479:Neuropeptides / Q000134:cerebrospinal fluid; D000068797:Orexins; D020293:Vasculitis, Central Nervous System / Q000134:cerebrospinal fluid / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy
D000305:Adrenal Cortex Hormones; C000596229:HCRT protein, human; D047908:Intracellular Signaling Peptides and Proteins; D009479:Neuropeptides; D000068797:Orexins