We tried to establish possible correlations between clinical data and MRI in a group of patients with Wilson's disease. Eleven patients (6 male, 5 female), aged between 11 and 50 years old, with a duration of illness from 5 months to 32 years, were submitted to MRI on a 1.5 T System. Three patients were asymptomatic, two had mild neurological disturbances, two were moderately affected and the remaining four had a severe form of the disease. All were receiving D-penicillamine at the time of the study. In the most symptomatic patients there were abnormalities in five or more sites on MRI. The putamen was affected in all symptomatic patients, including five with dystonia. A striking feature was the peripheral location of high signal putaminal lesions on T2-weighted images. In five cases, lesions in the corpus striatum or substantia nigra explained the patient's Parkinsonian features. MRI is an efficient method for studying involvement of the central nervous system in Wilson's disease, and allows some interesting anatomoclinical correlations.

No abstract

We have recently shown ipsilateral dynamic deWcits in trajectory control are present in left hemisphere damaged (LHD) patients with paresis, as evidenced by impaired modulation of torque amplitude as response amplitude increases. The purpose of the current study is to determine if these ipsilateral deWcits are more common with contralateral hemiparesis and greater damage to the motor system, as evidenced by structural imaging. Three groups of right-handed subjects (healthy controls, LHD stroke patients with and without upper extremity paresis) performed single-joint elbow movements of varying amplitudes with their left arm in the left hemispace. Only the paretic group demonstrated dynamic deWcits characterized by decreased modulation of peak torque (reXected by peak acceleration changes) as response amplitude increased. These results could not be attributed to lesion volume or peak velocity as neither variable diVered across the groups. However, the paretic group had damage to a larger number of areas within the motor system than the non-paretic group suggesting that such damage increases the probability of ipsilesional deWcits in dynamic control for modulating torque amplitude after left hemisphere damage.

No abstract

No abstract

extraparenchymal NCC is more frequent than previously thought. Because clinicians outside the Southwest United States are often unfamiliar with NCC as a cause of chronic meningitis, chronic ventriculitis, or hydrocephalus without obvious cysts, the diagnosis of extraparenchymal NCC often depends on the correct interpretation of neuroimaging.

Introduction  The cochlear implants centers in the world are studying the cochlear implantation in children with multiple handicaps. Objective  To develop a questionnaire to analyze the subjective benefits of the cochlear implantation in multiple handicapped children according to their parent's perspective. Methods  A questionnaire was applied to 14 families of multiple handicapped children, aging from 2–12 years old and having from 11 months to 11 years of implant use. Results  The social-emotional abilities were improved because of many factors, such as: auditory exposure, which happened in 84% of the children; recognition of their own names, which increased in 56%; and development of eye contact, in 28% of the subjects. Other benefits appeared to be: music appreciation and more attention and adherence to other therapies and school activities. Besides, some children became interested in objects, playing with other children, and more adapted to daily routines. Thirty-five percent of the children acquired oral language, mainly the bilaterally implanted, while 14% of them were engaged in sign language. Although all of the children showed a significant improvement in communication, the emotional issues of some families and the severity of the handicaps negatively impacted the outcomes. In spite of the families' acknowledgement of some benefits, the diagnosis of autism spectrum disorder caused frustration, requiring a readjustment of the expectations. Conclusion  The questionnaire turned out to be an adequate tool to reveal the social-emotional benefits of cochlear implantation. Although oral language was not the major outcome in these cases, the cochlear implant benefits involved the whole family. All of the families recommended the implant to other children in a similar situation.

No abstract