Report of a case of immunosuppression with prednisone in an 8-year-old boy with an acute onset of hypocretin-deficiency narcolepsy.
OBJECTIVE
To explore whether acute destruction of hypocretin cells in a patient with narcolepsy could be detected and if the course of the disease could be reversed or altered by the use of prednisone for immunosuppression.


METHODS
Case report.


METHODS
A sleep-clinic population in a tertiary-care hospital.


METHODS
An 8-year-old boy with a very acute recent (< 2 month) onset of sleepiness.


METHODS
Sleep studies; fluid-attenuated inversion recovery and gadolinium magnetic resonance imaging studies with a focus on the hypothalamus; examinations of cerebrospinal fluid for cytology, protein, and hypocretin-1 levels; and HLA typing were performed.


METHODS
A 3-week regimen of 1 mg x kg(-1) x day(-1) of prednisone was administered in an attempt to modify the course of the disease.


CONCLUSIONS
Sleep evaluations were consistent with a diagnosis of narcolepsy. Hypocretin-1 was absent in the cerebrospinal fluid, and HLA-DQB1*0602 was present. All other results were within normal limits, and prednisone did not have any noticeable effects. Clinical manifestation of narcolepsy might occur when the hypocretin cell damage is too advanced to be reversible.
Hecht
Marketa
M
Center For Narcolepsy, Department of Pediatrics, Stanford University School of Medicine, Stanford, Calif, USA.
Lin
Ling
L
Kushida
Clete A
CA
Umetsu
Dale T
DT
Taheri
Shahrad
S
Einen
Mali
M
Mignot
Emmanuel
E
D000208:Acute Disease; D001559:Benzhydryl Compounds / Q000627:therapeutic use; D002352:Carrier Proteins / Q000134:cerebrospinal fluid; D000697:Central Nervous System Stimulants / Q000627:therapeutic use; D002648:Child; D003511:Cyclohexanols / Q000627:therapeutic use; D006680:HLA Antigens / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D047908:Intracellular Signaling Peptides and Proteins; D008297:Male; D000077408:Modafinil; D009290:Narcolepsy / Q000175:diagnosis / Q000188:drug therapy* / Q000378:metabolism*; D009479:Neuropeptides / Q000134:cerebrospinal fluid / Q000172:deficiency*; D000068797:Orexins; D017286:Polysomnography; D011241:Prednisone / Q000627:therapeutic use*; D017367:Selective Serotonin Reuptake Inhibitors / Q000627:therapeutic use; D000069470:Venlafaxine Hydrochloride
D001559:Benzhydryl Compounds; D002352:Carrier Proteins; D000697:Central Nervous System Stimulants; D003511:Cyclohexanols; D006680:HLA Antigens; D007166:Immunosuppressive Agents; D047908:Intracellular Signaling Peptides and Proteins; D000077408:Modafinil; D009479:Neuropeptides; D000068797:Orexins; D011241:Prednisone; D017367:Selective Serotonin Reuptake Inhibitors; D000069470:Venlafaxine Hydrochloride; C000596229:HCRT protein, human