Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Mental deterioration in Lafora's disease.

A Cukiert1, M M Vilela, H B Scapolan

  • 1Department of Neurology, São Paulo University, School of Medicine, Brasil.

Arquivos De Neuro-Psiquiatria
|June 1, 1990
PubMed
Summary

Lafora disease, a progressive myoclonic epilepsy, causes early dementia even before social life is disturbed. Neuropsychological testing reveals significant cognitive decline, particularly in non-dominant hemisphere functions.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Fellowship education in epilepsy in Latin America and Africa: Results of a survey.

Epilepsy & behavior : E&B·2024
Same author

Doctors' awareness concerning primary immunodeficiencies in Brazil.

Allergologia et immunopathologia·2015
Same author

Compound heterozygous RAG2 mutations mimicking hyper IgM syndrome.

Journal of clinical immunology·2013
Same author

Pituitary-adrenal dynamics after ACTH-secreting pituitary tumor resection in patients receiving no steroids post-operatively.

Journal of endocrinological investigation·2005
Same author

[Evolution of hematological parameters in a group of children with human immunodeficiency virus infection - HIV 1]

Jornal de pediatria·2003
Same author

[Serum immunoglobulins in children perinatally exposed to human immunodeficiency virus]

Jornal de pediatria·2003

Area of Science:

  • Neuroscience
  • Neurology
  • Epilepsy Research

Background:

  • Lafora disease is a rare, fatal neurodegenerative disorder.
  • It is classified as a progressive myoclonic epilepsy.
  • Dementia is a hallmark symptom, but its early progression is understudied.

Observation:

  • Two early-diagnosed Lafora disease cases underwent comprehensive neuropsychological testing.
  • Testing occurred before patients reported mental deterioration.
  • Subsequent tests documented rapidly progressive dementia.

Findings:

  • Severe impairment of right parietal lobe functions was consistently observed.
  • Cognitive deficits included constructional praxis, memory, and abstract processing.
  • Language and intellectual functions were relatively preserved.

Related Experiment Videos

  • Dominant hemisphere cognitive functions showed less impairment than non-dominant ones.

    • Implications:

    • Mental deterioration is an early, potentially presymptomatic, manifestation of Lafora disease.
    • The disease causes asymmetrical cognitive deficits, suggesting complex neuropathology.
    • Further research is needed to understand the mechanisms behind this asymmetry.