Three patients with symptomatic narcolepsy are reported from the Departments of Neurology and Psychiatry, university of Michigan Medical Center, Ann Arbor, MI. One was a girl who developed polyphagia, weight gain, decreased growth, headaches with visual blurring, and excessive daytime sleepiness with frequent, irresistible brief naps at age 7½ years. At age nine, she developed hyperprolactinemia and galactorrhea. At age 11 she had diabetic ketoacidosis, temperature dysregulation, hypothalamic hypothyroidism, partial diabetes insipidus, and hepatitis. The CT was normal. MRI showed mild diffuse brain substance loss. Following treatment with methylphenidate, sleepiness and irresistible sleep attacks improved. Tissue typing was positive for HLA-DR2 and HLA-DQwl. The authors refer to ten additional cases of symptomatic narcolepsy with documented brain lesions reported in the literature. [1]

COMMENT. In these cases the REM sleep abnormalities and excessive daytime sleepiness were documented by polysomnography and Multiple Sleep Latency Tests. Two of the three patients were HLA-DR2 positive while the third was negative. In an addendum, the authors note an additional report of symptomatic narcolepsy in a nine year old HLA-DR2-positive boy following removal of a craniopharyngioma [2]. Other brain lesions associated with narcolepsy have included midbrain glioblastoma, cerebral sarcoidosis, pontine infarcts, 3rd ventricle glioma, pituitary adenoma, 3rd ventricle colloid cyst, multiple sclerosis, encephalitis, ischemia, head trauma, as well as craniopharyngioma.