Status Cataplecticus with Rapid Eye Movement Sleep Excess in Late-onset Narcolepsy Type 1

A 57-year-old man presented with difficulty speaking and walking along with increased daytime somnolence. His symptoms fluctuated throughout the day but never completely disappeared. A neurological examination revealed mild dysarthria, limb weakness, and staggering gait. Polysomnography showed rapid...

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Bibliographic Details
Published in:Internal Medicine 2022/10/01, Vol.61(19), pp.2951-2955
Main Authors: Ando, Takashi, Wakai, Masakazu, Kanbayashi, Takashi, Katsuno, Masahisa
Format: Article
Language:English
Subjects:
Case Report
cataplexy
Cerebrospinal fluid
Clomipramine
Gait
Histocompatibility antigen HLA
Internal medicine
Latency
Narcolepsy
Orexins
REM sleep
Sleep disorders
sleep latency
status cataplecticus
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Description
Summary:A 57-year-old man presented with difficulty speaking and walking along with increased daytime somnolence. His symptoms fluctuated throughout the day but never completely disappeared. A neurological examination revealed mild dysarthria, limb weakness, and staggering gait. Polysomnography showed rapid eye movement (REM) sleep excess (55.0%). Multiple sleep latency tests revealed a mean sleep latency of zero minutes with sleep-onset REM periods in all naps. The Orexin-A concentration in the cerebrospinal fluid was low (50.8 pg/mL). Human leukocyte antigen testing demonstrated DQB1*0602 positivity. His neurological symptoms were relieved by clomipramine. Thus, he was diagnosed with late-onset narcolepsy type 1 with status cataplecticus.
ISSN:0918-2918
1349-7235
DOI:10.2169/internalmedicine.8465-21