Last modified: 2014-04-03
Abstract
OBJECTIVE:
Describe a patient with Kleine-Levin Syndrome (KLS) with rapid cycling of hypersomnia episodes and autonomic changes.
BACKGROUND: KLS is a rare disorder (1.5 cases/million), typically in adolescent males and preceded by a triggering event, such as infections or head trauma 1–4 It exists in the borderland between psychiatry and neurology, often misdiagnosed as depression, bipolar disorder, psychosis or seizures.
DESIGN/METHODS: A 14 yo boy with a previous history of absence epilepsy, off medications for 1 year, presented with hypersomnia after an upper airway infection. He slept over 15h/day, when awake exhibiting derealization and speaking in a regressed fashion. Family reported dishinhibition, bradyphrenia and alternating hyper/hypophagia.
Symptoms improved without treatment but he relapsed every 2-3 weeks, with cycles lasting 10-14 days. Investigation included two lumbar punctures, with non-inflammatory results and negative bacterial/viral studies. Long-term EEG and imaging were unrevealing. Toxicology, thyroid function, B12, Folate, Thiamine, serological, paraneoplastic and vasculitic/autoimmune panel were negative. Psychiatric evaluation did not elicit diagnostic criteria for mood, somatic or other mental health conditions.
RESULTS: Repeat EEG showed intermittent delta slowing. Cardiac monitoring displayed alternating bradycardia/tachycardia. HLA typing was positive for DQB1*0201, associated with KLS and suggesting autoimmune etiology3. He was started on modafinil with improved hypersomnia; preventive treatment with valproic acid and lithium was unsuccessful for the rapid cycling.
CONCLUSIONS: KLS has significant morbidity if unrecognized. We review and discuss updated diagnostic criteria1,5 , pathophysiology1,6–8 and management9,10. This case exemplifies an unusual rapid cycling pattern of hypersomnia episodes associated with classic KLS symptoms.
Keywords
References
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