Verbal short-term memory and cerebellum: evidence from a patient with congenital cerebellar vermis hypoplasia.

The impairment of phonological short-term memory has been reported in adults with cerebellar lesions. At the same time, a role of the cerebellum in speech production has been hypothesized. Cerebellar malformations have been related to developmental problems and language acquisition in children. We describe a 5-year-old male child with cerebellar vermis hypoplasia who presented a severe linguistic deficit. On language testing, verbal production was almost absent, while comprehension was partially spared. Digit span was markedly reduced. An extensive examination of phonological short-term memory confirmed a deficit at this level. Positron Emission Tomography revealed hypometabolism both in the cerebellum and the supratentorial areas involved in language function. This finding supports the hypothesis that the cerebellum is included in a cerebro-cerebellar network, that underlies the phonological short-term memory, whose integrity is necessary for language acquisition.

Neuropsychological Deterioration Predicts Tumor Progression in a Young Boy With Bithalamic Glioma.

A description is given of the clinical, neuropsychological, and neuroimaging evolution of a 12-year-old boy (CG) who presented with bilateral thalamic astrocytoma (World Health Organization Grade 2). CG underwent an extensive neuropsychological assessment immediately after biopsy prior to any medical therapies and was followed up for 3 years until death. Neuropsychological functioning was thoroughly investigated by means of a detailed battery that included intelligence and cognitive functions. Evolution was characterized by cognitive deterioration that preceded neuroimaging signs of tumor progression. Starting from normal cognitive organization, the child exhibited visuospatial memory deficits and, afterward, diffuse cognitive impairment. The role of neuropsychological assessment in detecting early disease progression is discussed, mainly for rare pathologies whose evolution may be extremely variable.

Use of a Virtual-Technological Sailing Program to Prepare Children With Disabilities for a Real Sailing Course: Effects on Balance and Quality of Life.

Sailing might produce a positive effect on a patient's general health and become an integrated part of rehabilitation. Our hypothesis was that a specific technological rehabilitation program might be used to prepare a group of disabled subjects for sailing. Seventeen patients (age range: 9-20) with impairments in motor coordination and balance and 15 healthy subjects participated in the study. The study was divided into the virtual-technological sailing phase, theory-practice phase, and sports phase. Proprioceptive platforms were used to evaluate balance, and the Child Health Questionnaire-PF50 was used to evaluate quality of life. Trunk displacement and the center of pressure velocity improved significantly after the virtual-technological sailing program. As regards quality of life, the physical and psychosocial score significantly improved at the end of the program. A technological rehabilitation training improved balance in disabled subjects and may be used to prepare them for a real sailing course. Sailing improves the quality of life of disabled subjects and could be used in the rehabilitation.

Expression of liquoral neuroprotection markers in children with acute lymphoblastic leukemia.

Brain damage related to intrathecal methotrexate in children with acute lymphoblastic leukemia (ALL) is still unclear. Neuroinflammatory mechanisms and intracerebral production of specific biomarkers, play a key role in determining neuroprotective mechanisms after brain injury. To determine whether the CSF concentrations of neuron-specific enolase (NSE), neurotrophic factors and doublecortin (DCX) are influenced by repeated intrathecal methotrexate administrations, we prospectively collected CSF samples from 10 children with ALL and 10 controls. Our results showed an increased expression of the liquoral markers. This up-regulation could be interpreted as a neuroprotective response of the brain against the neuronal damages induced by MTX.

Long-term cognitive outcome, brain computed tomography scan, and magnetic resonance imaging in children cured for acute lymphoblastic leukemia.

BACKGROUND: Prevention of meningeal recurrence achieved by intrathecal methotrexate (MTX) and systemic chemotherapy is part of standard acute lymphoblastic leukemia (ALL) treatment. Cranial irradiation has been a routine part of past protocol treatment but is currently reserved only for select subsets of patients. Central nervous system (CNS) prophylaxis may cause brain abnormalities such as intracerebral calcifications, cerebral atrophy, and white matter alterations. In addition, long-term neuropsychologic sequelae following CNS prophylaxis have been investigated marginally in children cured for ALL. METHODS: To explore possible correlations between neuroimaging findings and neuropsychologic outcome, we used detailed cognitive tests to evaluate 21 children with ALL who received cranial irradiation (range, 18-24 Gy) plus intrathecal MTX as CNS prophylaxis. All children were followed prospectively once a year by cerebral computed tomographic scan and magnetic resonance imaging. All patients had continuous complete disease remission for at least 4 years and cognitive tests were performed after neuroradiologic examinations. RESULTS: White matter abnormalities were associated with poor performance only in a task exploring visual motor integration in about 50% of patients. Intracerebral calcifications correlate with the number of intrathecal MTX doses and with low scores in total intellectual quotient, performance intellectual quotient, and significant impairment in attention and visual motor integration tests. Girls are more vulnerable to the effects of CNS prophylaxis, whereas age at treatment and radiotherapy dose are not relevant to neuropsychologic outcome. CONCLUSIONS: Our results indicate the need for careful follow-up of children's cognitive abilities because global intellectual measures often fail to detect specific disorders that may cause learning difficulties. Moreover, as the clinical implications of neuroimaging findings are often limited, periodic extensive evaluation by specific neurocognitive tests of mental abilities is recommended to detect early signs of cognitive impairment.