[Comparative analysis of patients with narcolepsy-cataplexy, narcolepsy without cataplexy and idiopathic hypersomnia].

Abstract

BACKGROUND AND 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.

PATIENTS AND METHOD:

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.

• Authors: José E Martínez-Rodríguez, Alex Iranzo, Roser Casamitjana, Francesc Graus, Joan Santamaria
• Journal: Medicina clinica (Med Clin (Barc)) Vol. 128 Issue 10 Pg. 361-4 (Mar 17 2007) ISSN: 0025-7753 [Print] Spain
• Vernacular Title: Análisis comparativo de un grupo de pacientes con narcolepsia-cataplejía, narcolepsia sin cataplejía e hipersomnia idiopática.
• PMID: 17386240 (Publication Type: Comparative Study, Journal Article)

Chemical References

• HCRT protein, human
• HLA-DQ Antigens
• HLA-DQ beta-Chains
• HLA-DQB1 antigen
• Intracellular Signaling Peptides and Proteins
• Neuropeptides
• Orexins

Topics

• Adolescent
• Adult
• Aged
• Alleles
• Cataplexy (cerebrospinal fluid, complications, physiopathology)
• Diagnosis, Differential
• Female
• Genetic Predisposition to Disease
• HLA-DQ Antigens (genetics)
• HLA-DQ beta-Chains
• Humans
• Idiopathic Hypersomnia (cerebrospinal fluid, physiopathology)
• Intracellular Signaling Peptides and Proteins (cerebrospinal fluid)
• Male
• Middle Aged
• Narcolepsy (cerebrospinal fluid, complications, physiopathology)
• Neuropeptides (cerebrospinal fluid)
• Orexins
• Polysomnography
• Sleep Wake Disorders (classification)
• Sleep, REM