[Comparative analysis of patients with narcolepsy-cataplexy, narcolepsy without cataplexy and idiopathic hypersomnia].
OBJECTIVE
To evaluate the distribution of clinical, electrophysiological and biological variables, and their relationship with the CSF hypocretin-1 levels, in patients with central hypersomnias diagnosed as narcolepsy-cataplexy (NC), narcolepsy without cataplexy (NnC) and idiopathic hypersomnia (IH) based on the ICSD-2 criteria.


METHODS
We performed in all patients a clinical interview, a nocturnal polysomnogram and a multiple sleep latency test (MSLT), HLA analysis and measurement of CSF Hcrt-1 levels (low < or = 110 pg/mL).


RESULTS
Out of 51 patients, 31 were classified as NC, 11 as NnC and 8 as IH. 34 patients (66.7%) had low CSF Hcrt-1 levels (29 NC, 3 NnC and 1 IH). In the NC group, 96.1% were HLA DQB1*0602 positive and 91% had low CSF Hcrt-1 levels. The most frequent variables found in NC patients and in those with a low CSF Hcrt-1 levels were cataplexy, fragmented nocturnal sleep, short refreshing naps, automatic behavior, HLA DQB1*0602, and, in the MSLT, a short mean sleep latency, a higher number of REM sleep episodes and a short mean latency of REM sleep episodes. A long nocturnal sleep time and morning sleep drunkenness, 2 variables used in the ICSD-2 for the diagnosis of IH, were not different among the three groups of hypersomnias.


CONCLUSIONS
Central hypersomnias have a superposition of several clinical, electrophysiological and biological variables that makes sometimes difficult the differential diagnosis. The measurement of CSF Hcrt-1 levels may help in the diagnosis of those patients with unclear clinical or electrophysiological forms.
Martínez-Rodríguez
José E
JE
Servicio de Neurología, Hospital Clínic, Universitat de Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, España.
Iranzo
Alex
A
Casamitjana
Roser
R
Graus
Francesc
F
Santamaria
Joan
J
D000293:Adolescent; D000328:Adult; D000368:Aged; D000483:Alleles; D002385:Cataplexy / Q000134:cerebrospinal fluid / Q000150:complications / Q000503:physiopathology*; D003937:Diagnosis, Differential; D005260:Female; D020022:Genetic Predisposition to Disease; D006683:HLA-DQ Antigens / Q000235:genetics*; D059866:HLA-DQ beta-Chains; D006801:Humans; D020177:Idiopathic Hypersomnia / Q000134:cerebrospinal fluid / Q000503:physiopathology*; D047908:Intracellular Signaling Peptides and Proteins / Q000134:cerebrospinal fluid*; D008297:Male; D008875:Middle Aged; D009290:Narcolepsy / Q000134:cerebrospinal fluid / Q000150:complications / Q000503:physiopathology*; D009479:Neuropeptides / Q000134:cerebrospinal fluid*; D000068797:Orexins; D017286:Polysomnography; D012893:Sleep Wake Disorders / Q000145:classification; D012895:Sleep, REM
C000596229:HCRT protein, human; D006683:HLA-DQ Antigens; D059866:HLA-DQ beta-Chains; C069051:HLA-DQB1 antigen; D047908:Intracellular Signaling Peptides and Proteins; D009479:Neuropeptides; D000068797:Orexins