Dados do Trabalho

Título

Hypersomnia, behavioral and cognitive alterations: report of Kleine Levin Syndrome

RESUMO

R.C.C., 29 years old, started episodic hypersomnolence at age 15. Sleep attacks lasted between days to three weeks. The first was preceded by bacterial pharyngitis. During these periods, he only slept and only fed or ingested liquids when he was awakened. During awakenings, aggressive behavior, disinhibition, polyphagia, visual and auditory hallucinations, as well as memory changes were observed. There were no mood disorders, no sleep disturbances, substance abuse, or previous developmental delays. The first physical examination performed after the third episode of hypersomnia was normal. However, there was a history of spontaneously appearing hematomas on the skin. During this period, magnetic resonance imaging of the brain, examination of cerebrospinal fluid, digital electroencephalogram and polysomnography were performed, with normal results. Other complementary tests were also requested. There was a change in the white series in the blood count. Bone marrow biopsy showed changes consistent with chronic myeloid leukemia (CML). The intensity and frequency of hypersomnia episodes, which reached four in a single year, decreased over the years. The last crisis was in 2020, lasting 1 week. Thus, the diagnosis of Kleine-Levin Syndrome (KLS) was made. Discussion: KLS is a rare sleep disorder, characterized by recurrent episodes of hypersomnia that last from days to weeks associated, during the period of awakening, with transient cognitive and behavioral changes. The frequency and intensity eventually decay over 8 to 12 years, and may become extinct. The estimated prevalence is 1-5:1,000,000 of cases, being more common in men. The average age of starting SKL is 16 years. The pathophysiology is still unknown. Autoimmune disorders, among others, appear as candidates for precipitating factors of the syndrome. However, we did not find CML among them in the scientific literature. The diagnosis is based on criteria established by the 3rd International Classification of Sleep Disorders (ICSD). Because it is a rare syndrome, there are no clinical trials and treatment guidelines. Currently, exclusively symptomatic therapy is chosen. Final comments: In addition to the rarity of the syndrome, the never mentioned coexistence with a lymphoproliferative disease and the occurrence of CML in an unusual age group may provide new insights into the pathophysiology of the disease.