[Cerebellar cognitive affective syndrome secondary to a cerebellar tumour]. / Síndrome afectivo-cognitivo cerebeloso secundario a tumor cerebeloso.

Cerebellar cognitive affective syndrome is characterized by disturbances of executive function, impaired spatial cognition, linguistic difficulties, and personality change. The case of an 11 year old boy is presented, with behavior problems, learning difficulties and social interaction problems. In the physical examination he had poor visual contact, immature behavior, reduced expressive language and global motor disability with gait dyspraxia, with no defined cerebellar motor signs. In the neuropsychological evaluation he has a full scale overall intellectual quotient of 84, with signs of cerebellar cognitive affective syndrome. A tumour affecting inferior cerebellar vermis was observed in the magnetic resonance imaging, which had not significantly grown during 5 years of follow up. The cerebellum participates in controlling cognitive and affective functions. Cerebellar pathology must be considered in the differential diagnosis of children with cognitive or learning disorder with associated behavioral and emotional components.

Neuroimaging findings in patient series with mucopolysaccharidosis.

INTRODUCTION: Mucopolysaccharidoses (MPS) are a group of inherited disorders due to lysosomal enzyme deficiencies. The aims of this study are to describe the neuroimaging findings in children evaluated in our hospital with this diagnosis, looking for a possible correlation of these alterations with the type of MPS and clinical severity, and finally to compare these findings with those previously reported. MATERIAL AND METHODS: We retrospectively analysed the medical records of 19 patients who had been diagnosed with MPS between 1992 and 2010: 7 had type I (5 with Hurler syndrome and 2 with Hurler-Scheie syndrome), 10 had type II or Hunter syndrome (4 with the severe form and 6 with the mild form), 1 had type III or Sanfilippo syndrome and 1 had type VI or Maroteaux-Lamy syndrome. We assessed the brain neuroimaging studies: computed axial tomography (CAT) in 5 patients, and magnetic resonance imaging (MRI) in 15. RESULTS: We observed a broad spectrum of neuroimaging anomalies. In CAT: mega cisterna magna (3/5, 60%). In brain MRI: dilated Virchow-Robin perivascular spaces (11/15, 73%), white matter abnormalities (11/15, 73%), and ventriculomegaly (5/15, 33%). CONCLUSIONS: Abnormal findings in neuroimaging studies are frequent in MPS (dilated Virchow-Robin perivascular spaces, white matter abnormalities and ventriculomegaly). Thus, given these abnormalities we should be aware of this possible diagnosis, particularly when typical signs and symptoms are present. However, we did not find a correlation between these findings and either any specific type of MPS or clinical severity.

[Presurgery neuropsychological evaluation in pediatric focal epilepsies]. / Evaluación neuropsicológica prequirúrgica en epilepsias focales pediátricas.

INTRODUCTION: Neuropsychological assessment is included in the protocols for evaluation of epilepsy surgery candidates, providing information about the patient's cognitive dysfunctions, allowing for prediction of possible cognitive deficits derived from surgery and yielding objective measures of any post-surgical changes. Neuropsychological disturbances constitute an important co-morbidity of medically intractable epilepsy. An early epilepsy onset in infancy may lead to cognitive dysfunctions that are atypical in terms of brain localization, due to the inherent plasticity and reorganization processes of the immature brain. The analysis of the neuropsychological profiles of paediatric focal epilepsies is much more complex than in the adult population. DEVELOPMENT AND CONCLUSIONS: In this paper, we review the neuropsychological disturbances associated to focal epilepsies (posterior cortex, temporal and frontal epilepsies), stressing the point that there is a considerable lack of rigorous studies on the topic in the literature, in spite of this being an essential part of the presurgical work-up in epilepsy patients.

[Differences between the neuropsychological profiles of Asperger's syndrome and non-verbal learning disorder]. / Diferencias entre los perfiles neuropsicológicos del síndrome de Asperger y del sindrome de dificultades de aprendizaje no verbal.

INTRODUCTION: The validity of the Asperger syndrome (AS) diagnose continues to be debated due to the lack of consensus on its definition, specially regarding higher-functioning autism. Previous research has suggested that the neuropsychological profile of the non verbal learning syndrome (NVLS) might be utilized to sustain a differential diagnose of the AS with other diseases associated with development. AIM: To analyze the neuropsychological profile of children diagnosed with AS and compare with those obtained from children with NVLS. SUBJECTS AND METHODS: All cognitive domains from 15 children have been evaluated with an extensive variety of tests; 5 were diagnosed with AS and 5 with NVLS, all of them submitted to the Nino Jesus Children's University Hospital. As a control group, another 5 children with no pathology were evaluated. RESULTS: Obtained data shows significant differences between the two groups under study. Profile from individuals under AS shows deficit in linguistic as well as non verbal skills and, when compared to the control group, executive functions seem to be generally more altered. CONCLUSIONS: Further investigation is suggested to determine that the NVLS could be an explanation model for AS. On the other hand, we stress the need to deepen the study of the executive functions and their relationship with the mind's theory, as an hypothesis to explain the AS.

[Attention process training application within an intervention project on attentional processes in children with cancer]. / Aplicacion del Attention Process Training dentro de un proyecto de intervencion en procesos atencionales en niños con cancer.

INTRODUCTION: Childhood cancer treatments have made a spectacular advance in recent years, obtaining survival rates of about 70%. These survival rates have permitted many children to reach adulthood, but also involve the appearance of previously unknown neurocognitive sequelae because of high mortality. DEVELOPMENT: Neuropsychological evaluation allows the detection of these deficits and the design of intervention. In children, rather than a loss of function there is a loss of the capacities that should develop in comparison with his/her peers. To obtain a base line to determine the affected and altered areas, it is equally vital to assess the acute and long term effects so as to evaluate the success of the intervention program. Attention Process Training (APT) is an individualized application program of attentional exercises of varying complexity in sustained, selective, alternating and divided attention. This program combines methods and techniques of cerebral damage rehabilitation, as well as educational and clinical psychology. It is completed with a self instruction training which is applied in situations of daily life. CONCLUSIONS: Child cancer treatment continues to carry long term neurocognitive sequelae. Neuropsychological evaluation is basic for its detection, allowing relevant information to be offered to parents and teachers, so as to facilitate design of individualized rehabilitation procedures. Attention training is basic for this type of population with generalized damage related to white matter, and forms part of a wider rehabilitation process that enhance the ecological validity of the program.

[Gerstmann's syndrome in a 9 year old boy]. / Síndrome de Gerstmann en un varón de 9 años.

INTRODUCTION: Gerstmann's syndrome encompasses the tetrad of finger agnosia, agraphia, acalculia and right-left confusion and is associated with lesions of the left angular gyrus, situated at the confluence of the temporal, parietal and occipital lobes. The localizing value of this syndrome has been questioned because multiple mechanisms can account for each of the four components of the syndrome. This clinical association is infrequent in children and it is impossible to diagnose in early stages of life because of parietal lobes have a slow functional development during childhood. CLINICAL CASE: We present the case of a learning disabled boy, 9 year old and right handed, who developed Gerstmann's syndrome. Acalculia, right-left disorientation, agraphia and finger agnosia were clearly identified by neuropsychological studies at this time, but there was no evidence of this dysfunction when he was first studied being 5 year old. This patient had perinatal asphyxia and suffered from focal clonic seizures in early neonatal period. In this case, a infarcted lesion was found at the confluence of parietal and occipital lobes in cranial CT an MRI scans. CONCLUSION: We conclude that is very important to identify this syndrome during childhood using a wide range of neuropsychological tests in order to diminish learning disorders with an early psychopedagogic supervision.