Prolonged daytime dissociative nrem3 sleep state in hypersomnolence with kleine levin symptomatology, a case report

Abstract

Introduction We present a case of recurrent hypersomnolence. A diagnostic approach was performed. Materials and methods Medical evaluation by neurologist, ENT-surgeon, Maxillofacial surgeon, orthodontist and expert in sleep medicine and epilepsy. Results History: 7 weeks: stenosis pylori, 10th year: head injury; 41: Narcolepsy? HLADQ2 DQ8: neg.; negative effect of modafinil and sodium-oxybate; 41 years: IGM: Monoclonal Gammopathy of Undertermined Significance (MGUS). Symptoms: staring at childhood. At 42 years: 3 days a week hypersomnolence during daytime: sleeps from 12 am to 4 pm, a feeling of unreality, works at home on automatic pilot, has no recollection of what he has done for several hours. In this period he is highly emotional and irritated, is sexually hyper aroused and has changed eating habits, with vomiting, automatic behaviour during eating, dysbasia and atonic jerks during defecation. Neurology: no abnormalities. Polysomnography: daytime sleep 1:30–4 p.m.: deep sleep with steep sws during 40 min. Nighttime sleep 0–6.30 am; SE = 91%; awakenings 24; nrem3 = 111 min (30%); rem = 66 min (18.4%); apnea-hypopnea-index = 18.3/h. MSLT: SL = 4.1 min, no sorem. Two days EEG and video monitoring: at start and next morning a normal background EEG. At 0 pm headache, double vision, faster speaking, followed by automatic behaviour for 3 h in the late afternoon, without recollection. EEG: frontal intermittent delta activity, changing into diffuse slowing in EEG to 1–2/s. and frontal regions zeta waves 1–3 s. Magnetic resonance imaging: no abnormalities in gray and white matter. CSF fluid: Hypocretine-1: 362 ng/l (range 224–653); liquor ery: 0. 0 × 106/l;leu,5.0 × 106/l; gluc 3.3; total protein 0.51 g/l; virology: DNA HSV-1, HSV-2 and VZV: neg. Hormonal axis: GH:5.4 mU/l; TSH:1.88 mU/l; Cortisol; 8 a.m.:411; 4 p.m:157 nmol/l; ACTH:3.3 pmol/l; Testosteron: 13.4 nmol/l; Vit D: 30 nmol/l. Blood sample; BSE 4 mm/h; CRP Conclusion Diagnosis: Kleine–Levin syndrome, OSAS. No epilepsy. Treatment of OSAS with MRA was started and Vit D supplied. Therapy of KLS stabilizing deep and REM sleep during nighttime with Valproate 300–150 mg, zopiclon 7.5 mg; clonazepam 1 mg an; Mirtazapine 18 mgr an, followed by stimulating daytime vigilance with methylphenidate 10 mg 4 dd. Nighttime sleep improved to 7 h, daytime nap lessened to 2 h, dissociative deep sleep state in afternoon to 1/2 h. Conclusion: This case suggests a possible auto-immune mediation. Acknowledgements To the co-workers of the Department of Clinical Neurophysiology, Epilepsy and Sleep Centre SEIN Zwolle.