Benefits of intervention in the Central Auditory Nervous System in individuals with Neurofibromatosis Type 1.

Objective: To verify the effectiveness of acoustically controlled auditory training in individuals with Neurofibromatosis Type 1.

Methods: The sample consisted of individuals with Neurofibromatosis Type 1, randomly distributed into two groups, making up the intervention group: individuals undergoing formal auditory training; and the comparison group: individuals who were not submitted to the intervention. Behavioral assessment of central auditory processing and electrophysiological evaluation, composed by Brainstem Auditory Evoked Potential and Long Latency-P300 Auditory Evoked Potential, were evaluated in three moments of evaluations: initial evaluation, reevaluation and after four months of reevaluation and comparing the performance between the groups.

Diencephalic syndrome-like presentation of brainstem tumor: a series case-based review.

Purpose: Russel described a rare clinical entity known as diencephalic syndrome (DS) in 1951, which was traditionally caused by a neoplasm in the hypothalamic-optic chiasmatic region. DS is characterized by severe emaciation despite adequate or slightly reduced caloric intake, locomotor hyperactivity, euphoria and other minor features. Current evidence suggests that a rare population of children with a similar phenotype may have their tumor located in the posterior fossa instead, defining the DS-like presentation, a rare entity with few cases reported in the literature.