Pretreatment neuropsychological deficits in children with brain tumors.

Treatment of childhood brain cancer has been associated with long-term cognitive morbidity in children. In the present study, the cognitive status of children with brain tumors was examined prior to any treatment to single out the role of tumor and tumor-related factors in cognitive deficits. Eighty-three children with newly diagnosed brain tumors (mean age, 8.6 years; range, 7 months to 16.6 years; median, 9.4 years) completed an extensive battery of age-related tests to assess cognitive function before any therapeutic intervention. Magnetic resonance imaging (MRI) was used to determine tumor site and volume and tumor-related factors. Performance under test was compared with symptom duration, neurological status, epilepsy, and MRI. Cognitive difficulties are detected at diagnosis in as many as 50% of patients for some cognitive domains; 6% of patients present with true-diagnosed mental retardation. The location of the tumor is the principal determinant of cognitive deficits, with major impairment in children with cortical tumors. Symptom duration and the presence of epilepsy are significantly associated with neuropsychological disabilities, while neuroradiological tumor-related variables do not correlate clearly with neurocognitive performance. The knowledge of the pre-existing cognitive deficits is critical to evaluate the results of treatment, providing a baseline for assessing the true impact of therapy in determining cognitive decline. In addition, the study suggests that some clinical variables require careful monitoring, because they could be specifically implicated in the neuropsychological outcome; the efforts to reduce the impact of these factors could ameliorate long-term prognosis.

Clusters of cognitive and behavioral disorders clearly distinguish primary progressive aphasia from frontal lobe dementia, and Alzheimer's disease.

BACKGROUND/AIMS: Frontal lobe dementia (FLD) and primary nonfluent progressive aphasia (PnPA) are two forms of frontotemporal lobe degeneration. The relationship between these conditions remains unclear. Our study aimed to better define the behavioral and cognitive clusters characterizing PnPA patients. METHODS: We cognitively and behaviorally evaluated three groups of newly diagnosed patients affected by Alzheimer's disease (AD, n=20), FLD (n=22) and PnPA (n=10), in order to assess the cognitive-behavioral pattern of PnPA, compared to both FLD and AD. RESULTS: We found, as expected, worse performances in episodic memory in AD, of both the verbal fluency and naming tasks in PnPA, while FLD mainly showed behavioral disorders associated with an unremarkable deficit in the executive tasks. PnPA was not characterized by any significant behavioral disorders. Factor analysis-extracted three main factors ('mnesic', 'behavioral' and 'linguistic') clearly correlated to each group. A discriminant analysis based on the extracted factors correctly classified 84.6% of all patients. CONCLUSION: The evidence of a characteristics cognitive profile, without any significant behavioral changes, highlights that PnPA is different from other forms of frontotemporal lobe degeneration regarding both the cognitive and behavioral patterns; thus, it should be considered independently in further studies.

The role of brain infarcts and hippocampal atrophy in subcortical ischaemic vascular dementia.

We investigated if, in patients with vascular lesions, the variable that best discriminated demented from non-demented patients was the severity of the vascular pathology or the degree of hippocampal atrophy. A total of 39 patients multiple subcortical infarcts, who could be considered as possible vascular dementia with small vessel pathology, with underwent a neuropsychological study and brain magnetic resonance imaging (MRI) DSM IV criteria supported by neuropsychological data were used to distinguish demented from non-demented patients. The MRI study took into account the degree of hippocampal atrophy (hippocampal height and interuncal distance) and the severity of vascular pathology (number of brain infarcts). The distribution of lesions and a factor analysis showed that hippocampal atrophy is a better predictor of dementia than the number of brain infarcts. Multiple subcortical infarcts alone are probably not able to cause clinical dementia but the presence of vascular lesions increases the expression of concomitant Alzheimer's disease.

Visuospatial abilities in cerebellar disorders.

BACKGROUND: Cerebellar involvement in spatial data management has been suggested on experimental and clinical grounds. OBJECTIVE: To attempt a specific analysis of visuospatial abilities in a group of subjects with focal or atrophic cerebellar damage. METHODS: Visuospatial performance was tested using the spatial subtests of the WAIS, the Benton line orientation test, and two tests of mental rotation of objects-the Minnesota paper form board test (MIN) and the differential aptitude test (DAT). RESULTS: In the Benton line orientation test, a test of sensory analysis and elementary perception, no deficits were present in subjects with cerebellar damage. In MIN, which analyses the capacity to process bidimensional complex figures mentally, and in the DAT, which is based on mental folding and manipulation of tridimensional stimuli, subjects with cerebellar damage were impaired. CONCLUSIONS: The results indicate that lesions of the cerebellar circuits affect visuospatial ability. The ability to rotate objects mentally is a possible functional substrate of the observed deficits. A comparison between visuospatial performance of subjects with focal right and left cerebellar lesions shows side differences in the characteristics of the visuospatial syndrome. Thus cerebellar influences on spatial cognition appear to act on multiple cognitive modules.

Right side neglect in right cerebellar lesion.

A patient is described who developed right side hemineglect after a right cerebellar lesion. This spatial disorder was interpreted as a secondary effect of a deficit of the motor organisation in the right hemispace due to left frontal diaschisis. The pathological base may be the interruption of a highly integrated system which includes the lateral cerebellum and the contralateral frontal lobe.

Olivopontocerebellar atrophy: paraneoplastic syndrome of brain tumour?

We describe a patient who, three years after the onset of an olivopontocerebellar atrophy, developed a right cerebral tumour. The cerebellar symptomatology also included, as in other cerebellar patients previously described, a peripheral dysgraphia. Because this deficit of writing is generally reported in patients with right cerebral lesion, the authors hypothesized that functional alterations of supratentorial structures preceding the tumour by years may be able to damage the neural substrates connecting cerebral and cerebellar structures and to produce cerebellar atrophy.

Cerebellum and procedural learning: evidence from focal cerebellar lesions.

The aim of the present study was to investigate the influence of focal cerebellar lesions on procedural learning. Eight patients with cerebellar lesions and six control subjects were tested in a serial reaction-time task. A four-choice reaction-time task was employed in which the stimuli followed (or not) a sequence repeated 10 times, with the subjects aware (or not) of the item sequence. Learning was manifested by the reduction in response latency over the sequential blocks. Acquisition of declarative knowledge of the sequence was also tested. Reaction times displayed by patients with cerebellar lesions, even though they tended to be longer than those of control subjects in all testing conditions, significantly differed from control subjects only when the stimuli were presented in sequence. The reaction times in sequential trials were still statistically significant when simple motor response times were taken into account. Cerebellar patients were also significantly impaired in detecting and repeating the sequence. On the other hand, when the sequence was learned before testing, motor performances were significantly improved in all subjects. These data indicate that cerebellar lesions induce specific impairment in the procedural learning of a motor sequence and suggest a role of the cerebellar circuitry in detecting and recognizing event sequences.

Spatial dysgraphia and cerebellar lesion: a case report.

Spatial dysgraphia is a writing disorder that occurs in patients with right hemisphere lesion. We report a patient with cerebellar atrophy and spatial dysgraphia. To explain this finding, we hypothesize a discoordination between planning of the movement and performance due to a lack of the cerebellar modulation between supratentorial (premotor cortex) and peripheral (proprioceptive) afference during the ongoing handwriting movement.