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Right cerebral hemiatrophy: neurocognitive and electroclinical features
Asli Demirtas-Tatlidede1, A Destina Yalcin, Ender Uysal
1Department of Neurology, Sisli Etfal Research and Training Hospital, Istanbul, Turkey. aslidemirtas@yahoo.com
Right cerebral hemiatrophy (Dyke-Davidoff-Masson syndrome) often causes intellectual disability and significant visuospatial impairments. Seizures are common, and EEG shows reduced activity in the affected right hemisphere.
Area of Science:
- Neuroscience
- Neurology
- Cognitive Science
Background:
- Right cerebral hemiatrophy, also known as Dyke-Davidoff-Masson syndrome (DDMS), is a rare neurological condition.
- Understanding its cognitive and electroclinical profile is crucial for diagnosis and management.
Purpose of the Study:
- To retrospectively evaluate the cognitive and electroclinical characteristics of individuals with right-sided DDMS.
- To analyze neuroimaging findings in relation to cognitive and electroclinical features.
Main Methods:
- Retrospective analysis of five patients with confirmed right-sided DDMS.
- Cognitive assessments including intelligence tests and neuropsychological evaluations focusing on visuospatial functions.
- Analysis of electroclinical data (seizure types, EEG) and neuroimaging characteristics.
Main Results:
- All but one patient exhibited mental retardation.
- Consistent impairments were observed in visuospatial tasks (spatial processing, orientation discrimination).
- Seizure types included simple partial motor, complex partial, and secondarily generalized seizures; EEG showed reduced amplitude and slow activity in the affected hemisphere.
Conclusions:
- Cognitive deficits in DDMS are broad, affecting multiple domains.
- Findings support the hypothesis that dorsal visual pathways, crucial for spatial processing, may be right-hemisphere lateralized.
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