Klein–Levine syndrome in an elderly female: A case report
Year: 2025
Type: article
Study design: Case Report
Abstract: Kleine–Levin syndrome (KLS) is a rare relapsing and remitting disease characterized by recurrent episodes of hypersomnia and, to various degrees, behavioral or cognitive disturbances, hyperphagia, and hypersexuality.[1] The disease typically affects adolescent boys, whereas it is less prevalent among females and older individuals.[2] Literature regarding KLS in elderly females is scarce. Currently, no Food and Drug Administration-approved medication is available for treating KLS. Here, we are reporting a case of a 60-year-old woman who exhibited improvement with oxcarbazepine. A 60-year-old female with a medical history of hypertension and hypothyroidism, stable on medications, with negative affective traits in premorbid personality, presented with a complaint of episodes of excessive sleepiness following interpersonal relationship difficulties with family members over minor matters. In 2019, her initial episode persisted for 2 days with approximately 17–18 h of sleep each day and improved spontaneously. Over the following ½-year period, she remained free from any episode and successfully regulated her biological activities, albeit with delayed initiation of sleep and an extended early morning awakening. She would also have on-and-off low mood or irritability sometimes. Occasionally, she would encounter fluctuating anxiety symptoms related to household tasks and interpersonal relationships. In 2020, she again experienced an episode after being exposed to similar stressors, which continued for 3 days. During this period, she slept for approximately 17–18 h/day and her appetite significantly increased during the episode. Before the episode, she typically consumed three meals per day, each consisting of two chapatis and curry. However, during the episode, her meal frequency increased to 4–5 meals per day. This increase was likely due to cognitive changes (forgetfulness) during the episodes, as she would often forget that she had already eaten. Moreover, she consumed 3–4 chapatis per meal during the episode, exceeding her usual intake. However, there were no changes in the choice of food. Over a year, from 2020 to 2021, she experienced 7–8 episodes with similar semiology of 18–24 h, lasting 5–6 days. During one of these episodes, she spoke in her sleep and abruptly woke up screaming in the middle of the night. However, she had no memory of the event afterward. Over a few months, she had about 3–4 episodes that lasted longer, around 10–12 days, compared to previous episodes. After waking up from excessive sleep, she started experiencing confusion. During one of the episodes, she had to be admitted to the intensive care unit for 4 days as she was not waking up even for her meals. In October 2021, during another episode, she was admitted to our department for a thorough evaluation and management. Diagnostic tests included a complete hemogram, liver function test, kidney function test, serum electrolytes, lipid profile, thyroid profile, cerebrospinal fluid study, magnetic resonance imaging brain with contrast, electroencephalogram, and sleep study. All tests were normal, but thyroid-stimulating hormone levels were found to be high, resulting in an increase of levothyroxine dosage from 50 μg to 100 μg by the endocrinology department. Her Glasgow Coma Scale score was E1M1V1 on the 1st day but gradually improved to E4V5M6 on the 6th day. The rest of her systemic examination revealed no abnormalities. On Kirby’s examination, she demonstrated no speech, response to commands, or reaction to painful stimuli. She was admitted for 8 days, during which the neurology consultation was sought, and conditions including temporal lobe epilepsy, narcolepsy, and autoimmune encephalitis were ruled out based on history and investigations and other psychiatric differentials of adjustment disorder, depressive disorder, catatonia, and dissociative disorder were ruled based on the history and examination a final diagnosis of KLS was made and she was commenced on tablet oxcarbazepine 300 mg BD. Along with psychoeducation, interpersonal relationship issues were addressed, and problem-solving coping strategies were taught to the patient to deal effectively with triggers. She was advised to follow strict sleep hygiene. The patient improved significantly at the time of discharge and remained symptom-free for almost a year until a brief episode occurred following a minor stressor lasting only 2 days. Currently patient is in regular follow-ups and maintaining well on oxcarbazepine 300 mg BD. The indexed patient met the diagnostic criteria for KLS as per the International Classification of Sleep Disorders-3, exhibiting recurrent hypersomnia, hyperphagia, cognitive dysfunction, confusion, and mood changes.[3] It mainly affects adolescent males.[4] Although patients over 30 years of age rarely experience their first episode,[5] the index case was a 60-year-old female with onset at 56 years of age. Before each occurrence, the index patient experienced mental stress and has a medical history of hypothyroidism (with the possibility of autoimmune etiology) which could be possible triggers as reported in the literature.[6] In the index case, over 3 years, she experienced 14–15 episodes of excessive sleepiness, with asymptomatic periods lasting from 1 to 2 months to 6 months. According to the literature review, the median duration of illness was 4–8 years, with a median of 7 episodes. The median interval between episodes was 3.5 months. Consistent with the review, episodes of excessive sleepiness lasted for 17–18 h.[7] The index case has hypothyroidism. Some secondary KLS cases may be primary KLS with incidentally coexisting medical diagnoses. In a large review, 18 of 186 KLS patients had symptoms linked to stroke or brain hematoma (n = 5), genetic or developmental diseases (n = 6), multiple sclerosis, hydrocephalus, paraneoplasia, autoimmune encephalitis, or severe infectious encephalitis (n = 3).[8] The efficacy of the treatment of KLS is inconsistent and questionable. No evidence indicates that pharmacological treatment for KLS is effective and safe. The stimulants have been utilized for treatment during symptomatic episodes to address hypersomnolence.[8] While stimulants were not effective in preventing episode recurrence, various mood stabilizers such as lithium and antiepileptics have been tried for it.[8] Lithium has been the most extensively studied medication, with reports of effectiveness in 25%–62% of cases, yet it is associated with significant risks such as thyroid and kidney problems.[8] However, the index patient, who has hypothyroidism, was started on oxcarbazepine and demonstrated a remarkable and enduring response. This contrasts with other studies where carbamazepine showed a response equivalent to a placebo. Patients have been advised to maintain a balanced sleep regimen to ensure their well-being during episodes. It is critical to guarantee their safety by limiting their activities, keeping them home, and preventing them from driving as suggested by Arnulf et al.[8] Recurrent hypersomnolence necessitates a comprehensive assessment using a multidisciplinary approach for prompt diagnosis and patient-centric treatment. Multiple pharmacological agents, including lithium and carbamazepine, have been tried in earlier cases, while our case responded to oxcarbazepine, reflecting the uncertainties related to the role of pharmacological management. Treatment is mainly supportive and needs to involve psychoeducation about the illness. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Institution: All India Institute of Medical Sciences Jodhpur
Language: English
Subfield: Cognitive Neuroscience
Field: Neuroscience
Domain: Life Sciences
Open Access status: diamond
OpenAlex ID: W4411710401