Patrizia D'Alessandro

Attention Problems in Epilepsy: Possible Significance of the Epileptogenic Focus

Summary: Investigation of the relation between epilepsy and cognition presents serious methodologic problems because several factors may contribute to impair neuropsychological performances in epileptic persons. Benign epilepsy of childhood with rolandic paroxysmal discharges (EPR) may be a very useful model of investigation in relation to opportunity to examine subjects without brain damage, therapy, and negative environmental influences. Thus, neuropsychological dysfunction in patients with EPR may support the hypothesis that epilepsy itself plays a specific role in the genesis of cognitive disturbances. We assessed the impact of the laterality of the epileptogenic focus on cognition of children with EPR. All subjects performed a figure cancellation task, a test used to evaluate mainly attention mechanisms and abilities in processing visuospatial information. Results showed that children with right‐sided (or bilateral) focus scored worse, whereas children with left‐sided focus performed as well as the control subjects. Our data agree with those of studies suggesting that focal discharges may be related to poor cognitive performance. Evidence of a concordance between neurophysiologic and neuropsy‐chologic findings may have great practical and theoretical implications in management of epileptic patients.

Language lateralization in children with benign partial epilepsy.

Summary: To investigate the relationship between epilepsy and hemispheric asymmetries for language, a dual‐task procedure was used to assess language lateralization in children with benign rolandic childhood epilepsy. In the sample selection, care was taken to include factors believed to influence both the mental capabilities of epileptic patients and the individual functional cerebral organization. Results suggest that the interhemispheric prevalence pattern is related to the focus site. Controls as well as epileptic patients with a right hemispheric focus showed the expected left language lateralization; conversely, children with a left unilateral focus showed a different pattern of functional representation, suggesting an involvement of the right hemisphere in language mechanisms. It is emphasized that this atypical cerebral organization is found in subjects with no structural lesion and no therapy. It seems likely that the presence of a focal epileptic activity itself can alter the cerebral mechanisms underlying cognitive functions. A relationship between this modified hemispheric specialization and subtle neuropsychological dysfunctions observed in the children with focal epilepsy is suggested.

Epilepsy with Bilateral Occipital Calcifications: Sturge‐Weber Variant or a Different Encephalopathy?

Summary: A series of cases of epilepsy with associated bilateral occipital calcifications (EBOC) without signs of phakomatosis and without any disorders known to produce cerebral calcifications have been reported. It is unclear whether EBOC is an incomplete variant of Sturge Weber disease (SWD) or if it is a different, as yet undefined encephalopathy. We describe four new cases of EBOC that are different clinically by age of onset, type, course, severity of epilepsy, and associated cognitive deficits but that are linked by similar neuroradiologic findings. Similar to cases described in the literature, there is convincing evidence in favor of the hypothesis that these cases belong to an encephalopathy different from SWD and frequently associated with celiac disease.

Cerebral Organization for Language in Down's Syndrome Patients

Language lateralization was assessed with a dual task procedure in 10 male right-handed patients with Downs Syndrome and relatively preserved linguistic skills. Their performance was compared with that of two control groups, with and without mental retardation, matched with Downs Syndrome individuals for sex, handedness and I.Q. Results did not support the hypothesis that mental retardation is related to specific pattern of cerebral lateralization.

Early frontal impairment as a predictor of dementia in Parkinson's disease

To the Editor: The very interesting article of Jacobs et al. [8] on Neuropsychological characteristics of dementia in Parkinsons disease (PD) suggests several ideas. A preclinical phase of parkinsonian dementia can be identified, and this preclinical phase presents a specific neuropsychological pattern that can be distinguished from the pattern seen in Alzheimers disease. Verbal fluency represents the type of cognitive performance that can best be correlated with subsequent development of dementia, and poor performance on these tests would reflect an executive dysfunction due to an involvement of the frontal-subcortical circuits. On the basis of our own results, an early frontal dysfunction can be considered the best predictive factor for the development of dementia in PD. [2] In particular, in an otherwise unselected sample of parkinsonian patients, we found that four different types of relationships can be observed between a frontal syndrome, dementia and PD: (1) no cognitive abnormalities are revealed by neuropsychological evaluation; (2) the frontal symptomatology is associated with a more generalized cognitive impairment; (3) signs of frontal dysfunction constitute the sole cognitive alteration that can be noted; and (4) the neuropsychological examination reveals abnormal cognitive functions that cannot be attributed to a frontal dysfunction. The first pattern can be …

Neuropsychological Follow-Up of Parkinsonian Patients with and without Cognitive Impairment

In order to evaluate possible progression in the severity of their cognitive impairment, 34 parkinsonians with intellectual impairment were followed longitudinally for 7 years. Each patient was matched for age, sex, severity and duration of illness, and pharmacological treatment, with a parkinsonian patient without cognitive impairment. Results suggest that cognitive deficits are not static but rather there is a progression in the severity. Furthermore, patients suffering from severe dementia are more likely to die during the follow-up period. The prognosis of Parkinsons disease seems to be changed substantially by the occurrence of dementia. The natural history of parkinsonian dementia does not seem to differ from the history of other forms of dementia with a progressively disabling course leading to a complete loss of autonomy.

Slowly Progressive Apraxia without Dementia

A patient is described who showed slowly progressive apraxia not associated with other neuropsychological dysfunctions. The onset of symptoms began at age 60 years, and the duration of illness was 5 y