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At least 199 records · Page 11Linked to original sources
PubMed1987

Complex event-related potentials (P300 and CNV) and MSLT in the assessment of excessive daytime sleepiness in narcolepsy-cataplexy.

The P300 and contingent negative variation (CNV) evoked potential (EP) paradigms were performed by 12 untreated narcoleptics and controls immediately prior to each nap of the Multiple Sleep Latency Test (MSLT) in order to assess whether they might hold promise as rapid quantitative techniques to assess excessive daytime sleepiness. The Stanford Sleepiness Scale (SSS) was also completed across test days and immediately before and after both the evoked potential recordings and MSLT naps. MSLT findings confirmed shorter sleep latencies and frequent SOREMPs in narcoleptics and a strong mid-afternoon increase in sleepiness based upon pressure for NREM sleep in both groups. On SSS narcoleptics were sleepier and they showed greater increase in sleepiness induced by the EP tests and greater sleepiness reduction by the MSLT naps. In the P300 paradigm, narcoleptics showed smaller component P3 amplitudes and larger P1 amplitudes. In the CNV paradigm, N1 latencies were greater in narcoleptics to both S1 and S2 and the post-CNV negative component was larger: but no significant differences were seen for the main CNV measures of negativity amplitude in the first or second halves of the response. The P300 paradigm but not the CNV, therefore, appeared to be a sensitive EP measure of sleepiness. Finally, EP components in both the P300 and CNV paradigms showed time-of-day (circadian) differences between narcoleptics and controls.

Acoustic Stimulation↗
PubMed1988

Narcolepsy-cataplexy in Israeli Jews is associated exclusively with the HLA DR2 haplotype. A study at the serological and genomic level.

Narcolepsy is a very rare disease among Israeli Jews with a frequency of 7/3 X 10(6). An investigation of the association of narcolepsy with the human leukocyte antigen system was conducted in Israeli Jews at the serologic and genomic levels. The human leukocyte antigen class I and class II antigen typing of 7 clinically diagnosed narcoleptics, 3 individuals suffering from sleep disorders other than narcolepsy, and 11 healthy matched controls revealed that all narcoleptic patients (100%) investigated in the present study carried the HLA-DR2 haplotype, whereas patients with other sleep disorders did not. The HLA-B7 and DR2 occurred jointly in 57% (4/7) of the narcoleptic patients, as compared to 2% in randomly selected Israeli healthy controls. Restriction fragment length polymorphism analysis was performed with several restriction enzymes and three cDNA probes for DQ alpha, DQ beta, and DR beta genes on genomic DNAs obtained from narcoleptics and patients with other sleep disorders, matched controls, and 3 homozygous typing cells representing the DR2 subtypes Dw2, Dw12, and DwAZH. The restriction fragment length polymorphism analysis showed that all narcoleptics (7 of 7) shared virtually identical restriction fragment length polymorphisms with one of the homozygous typing cells (GSO), which defines DR2,Dw2. The frequency of the DR2,Dw2 haplotype in the healthy Israeli population is 3.2%. Other non-narcoleptic patients did not share these restriction fragment length polymorphisms. These findings indicate that narcolepsy is associated worldwide with the HLA-DR2,Dw2 haplotype.

Adult↗
PubMed1999

A case of narcolepsy-cataplexy associated with distemper encephalitis.

Narcolepsy associated with localized brain lesions is described in a 10-month-old Argentine Dogo. Neurological examination and MRI study suggested an inflammatory lesion of the left frontal lobe. Postmortem examination revealed diffuse encephalitis in the forebrain and marked necrotic lesions in the ventral pontine area. Immunohistochemistry for distemper virus antigen showed positive staining of the cytoplasm of many neurones of the pons and cerebral cortex. The pathological pattern was suggestive of post-vaccinal distemper encephalitis and the localization of the lesions was consistent with the neurological syndrome shown by the animal. At any event, the possibility of coincidental findings of distemper encephalitis and idiopathic narcolepsy must be accounted for.

Animals↗
PubMed1988

A comparison of multiple and single sleep latency and cerebral evoked potential (P300) measures in the assessment of excessive daytime sleepiness in narcolepsy-cataplexy.

A direct comparison was made between the amplitude of evoked potential (EP) component P3 (by the P300 paradigm), a known sensitive EP correlate of sleepiness, and sleep latency measures (both to stage 1 or rapid eye movement [REM] and to stage 2 or REM) of the Multiple Sleep Latency Test (MSLT) in 11 untreated narcoleptics and matched controls. Repeated P3 measures were performed immediately prior to standard MSLT naps at 10:00 a.m., 12:00 noon, 2:00 p.m., 4:00 p.m., and 6:00 p.m. Using discriminant analysis and F tests, all three measures (P3 and both by MSLT) were found to distinguish the two groups for collapsed five-nap data, and all showed essentially parallel circadian time-of-day effects, with greatest sleepiness in the midafternoon. The MSLT, however, was somewhat more powerful for collapsed data. Both tests misclassified some subjects as belonging to the other group, with greater misclassification for both tests in the control group and more overall for the P3 measure. Adding the two sleep onset REM period (SOREMP) criteria on MSLT for narcolepsy, one patient was still classified as normal. Analysis of data from individual naps indicated that the MSLT was considerably more powerful in discriminating groups than was P3 amplitude, and it did so for all five naps.

Adult↗
PubMed1985

Propranolol: long-term treatment in narcolepsy-cataplexy.

Forty-eight sequential narcoleptic patients were treated with propranolol (80-240 mg/day) for an average period of 18.4 months. Initially all patients received single drug therapy; after 10 days or longer, however, 50% of patients also received tricyclics or stimulants because propranolol alone did not sufficiently suppress the narcoleptic symptoms. Fifty percent of patients judged the initial effectiveness of propranolol on daytime sleepiness to be good to very good; in these patients the effects seemed comparable to that of pemoline. Within 6 months, however, the effectiveness started to decrease, and after 26 months only 8% (2 out of 24) of those patients taking the single drug were satisfied with propranolol therapy alone. Side effects included disturbed night sleep, decreased blood pressure, increased lethargy, allergic skin rash, and asthma; 58% of the patients dropped out of the study after 26 months. Vigilance tests during the first 4 months (16 patients) showed significant improvements in all test criteria, including a shorter reaction time. All-night polygraphic electroencephalogram recordings of 14 patients on the eighth and ninth day of medication showed that average total sleep time decreased by 5.7%, but other sleep characteristics did not change significantly.

Arousal↗