![]() ![]() The HLA haplotype DQB1*0602 was present in 15 of 17 (88%) patients. Typical MSLT findings were found in 14 of 17 (82%) patients (MSL <5 min and ⩾2 sleep onset REM periods (SOREMPs)). UNS and SNS scores suggestive of narcolepsy were found in 13 of 14 (93%) and 10 of 13 (77%) patients, respectively. 8 The mean ESS score was 16.1 (standard deviation (SD) 3.6) and mean sleep latency (MSL) was 2.0 min (SD 1.7). Two of the latter three patients had a familial form of narcolepsy. Statistical analyses were performed using Student's t test, the Mann‐Whitney U test, and the Pearson correlation test.ĬSF hypocretin‐1 levels were undetectable in 12 patients, low (<320 pg/ml) in three patients (range 68–118), and normal in three patients (range 527–546). 3īecause of the large interassay variability of the radioimmunoassay and the still unknown pathophysiological significance of low or intermediate CSF hypocretin‐1 levels, we decided to group patients into those with and without detectable CSF hypocretin‐1 levels (detectable hypocretin: DH undetectable hypocretin: UH). 3, 9, 10 The ESS score was also reported in one of these studies. We identified 64 patients with narcolepsy‐cataplexy for whom detailed CSF hypocretin‐1 levels and sleep latencies on MSLT were reported. We also systematically reviewed the literature. Normal values (>320 pg/ml) were ascertained by determination of CSF hypocretin‐1 levels in a control group of 20 healthy subjects without signs of sleep‐wake or other neurological disorders. 8Ĭrude lumbar CSF from all patients was used for determination of hypocretin‐1 by radioimmunoassay with a detection limit of approximately 60 pg/ml and an intra‐assay variability of 4%. Two patients with a familial form of narcolepsy have been previously described. In all patients, pharmacological treatments were discontinued 7–14 days before hospital admission. Tests were conducted and scored according to standard criteria. A clinical/neurological examination (n = 18), a standardised sleep history (n = 18), polysomnography (n = 16), MSLT (n = 17), and HLA typing (n = 17) were available for most patients. The severity of cataplexy was graded as follows: 1, mild (partial, only one body region involved) 2, moderate (generalised, without falls) and 3, severe (generalised, with falls). 4 The frequency of cataplexy was graded as follows: 1, more than one attack per month but less than one per week 2, more than one attack per week but less than one per day and 3, more than one attack per day. Cataplexy was considered to be definite according to the following previously suggested criteria: (i) loss of muscle tone with a visible effect or involving other muscle groups in addition to leg muscles (ii) cataplexy occurring more than once a month (iii) duration of cataplexy (often or always) 14, SNS scores 7 are suggestive of narcolepsy. We studied 18 Caucasian patients (11 women and seven men, mean age 36 years, range 18–64) with narcolepsy and definite cataplexy.
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