Decreased cortical excitability in Unverricht-Lundborg disease in the long-term follow-up: a consecutive SEP study.
Clin Neurophysiol
; 122(8): 1617-21, 2011 Aug.
Article
em En
| MEDLINE
| ID: mdl-21353634
ABSTRACT
OBJECTIVE:
To delineate long-term change of cortical excitability by measuring somatosensory evoked potentials (SEPs) in patients with Unverricht-Lundborg disease (ULD).METHODS:
SEPs to median nerve stimulation were repeatedly examined in two genetically proven ULD patients manifesting stable condition over 16 years, namely disabling but non-progressive myoclonus and cessation of generalised tonic-clonic seizures.RESULTS:
In both patients, five sets of early cortical components were identified 16 years ago two tangential components of N20-P20 and P30-N30 and three radial components of P25, N35 and N40. Cortical SEPs were regarded as abnormally enhanced 'giant' based on the N35 amplitude (>mean+3 SD of normal controls). The bimodal negative peaks of N35 and N40 showed different spatial distribution N35 maximum in the central area and N40 in the centro-parietal area. At present, N35 remained giant while N40 disappeared in both patients.CONCLUSIONS:
It is possible that currently preserved giant SEPs at least at N35 reflect disabling cortical myoclonus and that disappearance of N40 might reflect a lesser degree of increased cortico-cortical connectivity and/or decreased cortical hyperexcitability in the association cortices. It might possibly have resulted in the disappearance of GTCSs.SIGNIFICANCE:
We delineated long-term change of giant SEP in ULD.
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Córtex Cerebral
/
Síndrome de Unverricht-Lundborg
/
Potenciais Somatossensoriais Evocados
Idioma:
En
Ano de publicação:
2011
Tipo de documento:
Article