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Rett's syndrome with Lennox-Gastaut pattern.

G Olmos Garcia de Alba1, J D Gamboa Marrufo, O Rengifo Ramos

  • 1Department of Electroencephalography, Hospital Infantil de Mexico, Mexico, D.F.

Clinical EEG (Electroencephalography)
|October 1, 1987
PubMed
Summary

Three female patients with Rett's Syndrome showed normal imaging but abnormal EEGs. This highlights the electroencephalogram's (EEG) importance in diagnosing this rare neurological disorder.

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Area of Science:

  • Neurology
  • Genetics
  • Pediatrics

Background:

  • Rett's Syndrome is a rare neurodevelopmental disorder primarily affecting females.
  • Clinical diagnosis relies on specific criteria including developmental regression and characteristic motor and behavioral features.

Observation:

  • Three Latin American female patients presented with clinical symptoms consistent with Rett's Syndrome.
  • Studies were conducted across two centers: Mexico City and Houston, Texas.

Findings:

  • All patients exhibited normal laboratory and neuroimaging results (CT and MRI).
  • Electroencephalograms (EEGs) consistently revealed abnormalities, specifically the Lennox-Gastaut pattern.
  • This electrographic pattern was notably associated with the clinical presentation of Rett's Syndrome.

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Implications:

  • The electroencephalogram (EEG) plays a crucial role in the differential diagnosis of Rett's Syndrome.
  • The consistent association of the Lennox-Gastaut pattern warrants further investigation in Rett's Syndrome patients.
  • This finding emphasizes the utility of EEG in identifying specific electrophysiological markers for rare neurodevelopmental disorders.