Pineal region tumours are characterised by a variety of clinical signs and symptoms resulting from compression and displacement surrounding structures ranging from raised intracranial pressure to visual field defects, cerebellar symptoms and endocrine disorders. Alongside these historically well-established features of pineal region tumors, behavioural disorders have only been described sporadically. To define the incidence of these disorders in a population of children and young adults (aged 4 to 20 years), we reviewed ten cases (7 males and 3 females) treated for pineal region tumours at the Department of Neurosurgery, Verona, between 1986 and 1995. Behavioural or cognitive disorders (hypersomnia, irritability, psychiatric disorders, impaired memory) were recognised before and after surgery in seven patients (70%). Although these disorders are usually deemed aspecific signs of expanding brain lesions or associated hydrocephalus in children, this was not supported by the relatively high age of our seven patients (14 years, 4-19). At diagnosis, seven patients presented hydrocephalus (70%). A review of around 200 cases reported in similar series (pineal region tumours, age < 20, analysis of symptoms, hydrocephalus determination) disclosed a lower incidence of behavioural disorders (10%), despite a high incidence of hydrocephalus (93%). We suspect that these symptoms are more common in patients with pineal region tumours than in other patients of the same age with tumours in other subtentorial locations. Other clinical and experimental observations are briefly discussed: in agreement with these findings we suggest that behavioural disorders are directly correlated to the involvement of anatomical structures like Nauta's mesencephalon-limbic circuit, the mesencephalic reticular formation, the premesencephalic grey matter and the pineal gland whose importance has been underestimated to date. These symptoms therefore merit greater attention in young patients with pineal region tumours.
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