Kleine-Levin Syndrome

Kleine-Levin Syndrome [KLS] is often under-recognized and also misdiagnosed. When suspicion for KLS is raised, a thorough clinical evaluation should be performed, including detailed history from family members and a neurologic and psychiatric examination. Additional studies may include PSG, EEG, neuroimaging, as well as serological and CSF studies to rule out alternative diagnoses as clinically indicated. After arriving at a diagnosis of KLS, the foundation of care is supportive. Patients and their families should be provided with education about the disease. During symptomatic periods, patients should be allowed to rest at home under caregiver supervision. Caregivers should pay special attention to the patient’s eating habits and mood. Patients should not be allowed to drive or operate heavy machinery during these episodes. In between episodes, avoidance of reported triggers, such as alcohol and infection are encouraged as is maintenance of a regular sleep-wake cycle. Pharmacologic therapy has not been well-studied and for most patients is not necessary. For more severe cases, targeted symptomatic therapy, such as modafinil or amantadine for somnolence or risperidone for psychosis may be considered depending on the patient’s symptomatology. Lithium has the best data to support its use as a prophylactic agent and for patients with severe or frequent episodes, may be considered.