Page 24 - The autoimmune hypothesis of narcolepsy and its unexplored clinical features M.S. Schinkelshoek
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Chapter 1
by laughter and being angry) started to occur. These attacks lasted several seconds, and up to several minutes when the trigger remained present. She also experienced hypnagogic hallucinations, disrupted nocturnal sleep, sleep paralysis and gained 7 kilograms in several months.
When she visited our institution in March 2017, she had high scores on both the Epworth Sleepiness Scale (ESS; 19/24) and the Fatigue Severity Scale (FSS; 6/7). HLA-typing showed HLA-DQB1*06:02 positivity. Sleep investigation results were also supportive for the diagnosis of NT1: a polysomnography with a sleep onset REM period (SOREMP) was followed by a mean sleep latency on MSLT of 2.9 minutes with SOREMPs in 3 out of 5 naps.
Figure 1.1. This figure visualizes the suggested steep and swift decrease in cerebrospinal fluid hypocretin-1 concentrations that is closely linked to symptom onset, based on this case report combined with previous reports (Savvidou et al., 2013, Kanbayashi et al., 2002, Kubota et al., 2003). Hypocretin-1 concentration was in the normal range only weeks before symptom onset.
We were allowed to use the previously stored CSF for hypocretin-1 measurement. A hypocretin-1 concentration of 271 pg/mL was detected. Hypocretin-1 concentrations were measured in duplicate with a iodine-125 hypocretin-1 radioimmunoassay (Phoenix Pharmaceuticals, Mountain View, CA, USA). This
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