TY - JOUR
T1 - Non-convulsive status epilepticus of frontal origin in mucopolysaccharidosis type II successfully treated with ethosuximide
AU - Bonanni, Paolo
AU - Gubernale, Marco
AU - Martinez, Federica
AU - Randazzo, Giovanna
AU - Milantoni, Luca
AU - Martinuzzi, Andrea
AU - Boniver, Clementina
AU - Vecchi, Marilena
AU - Scarpa, Maurizio
PY - 2012/10
Y1 - 2012/10
N2 - At 7years of age, a female with mucopolysaccharidosis type II (MPS II) showed a sudden deterioration in neurological function, a sleep disorder, and progressive behavioural impairment. Electroencephalography was performed 1year and 8months after the onset of the neurological regression and revealed continuous ictal activity in the frontal regions. The female was diagnosed as having frontal non-convulsive status epilepticus. After 5weeks of therapy with ethosuximide, the ictal electroencephalographic activity disappeared. At the same time, her sleep and cognitive and behavioural functions were observed to improve. This is the first case of MPS type II reported in an individual with non-convulsive status epilepticus. Two main forms of MPS II can be recognized: attenuated and severe. Severe MPS II is characterized by neurodegeneration. No study has explored the relationship between epilepsy and neurological deterioration in MPS II. Our observation shows that epilepsy may be a treatable cause of neurological regression in individuals with MPS II.
AB - At 7years of age, a female with mucopolysaccharidosis type II (MPS II) showed a sudden deterioration in neurological function, a sleep disorder, and progressive behavioural impairment. Electroencephalography was performed 1year and 8months after the onset of the neurological regression and revealed continuous ictal activity in the frontal regions. The female was diagnosed as having frontal non-convulsive status epilepticus. After 5weeks of therapy with ethosuximide, the ictal electroencephalographic activity disappeared. At the same time, her sleep and cognitive and behavioural functions were observed to improve. This is the first case of MPS type II reported in an individual with non-convulsive status epilepticus. Two main forms of MPS II can be recognized: attenuated and severe. Severe MPS II is characterized by neurodegeneration. No study has explored the relationship between epilepsy and neurological deterioration in MPS II. Our observation shows that epilepsy may be a treatable cause of neurological regression in individuals with MPS II.
UR - http://www.scopus.com/inward/record.url?scp=84866382580&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84866382580&partnerID=8YFLogxK
U2 - 10.1111/j.1469-8749.2012.04228.x
DO - 10.1111/j.1469-8749.2012.04228.x
M3 - Article
C2 - 22414067
AN - SCOPUS:84866382580
VL - 54
SP - 961
EP - 964
JO - Developmental Medicine and Child Neurology
JF - Developmental Medicine and Child Neurology
SN - 0012-1622
IS - 10
ER -