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Sturge-Weber syndrome: Cerebral haemodynamics during seizure activity

Sturge-Weber syndrome: Cerebral haemodynamics during seizure activity
Sturge-Weber syndrome: Cerebral haemodynamics during seizure activity

The aim of this studs was to examine the haemodynamic response to seizures in three infants with Sturge-Weber syndrome by measuring regional cerebral blood how using transcranial Doppler sonography and 99mTC HMPAO SPECT. Time-locked video/digital EEG recording was carried out for ictal studies. MRI was performed in all subjects. SPECT showed hemispheric hypoperfusion interictally in all three patients and also ictally in one of the three; a small region of hyperperfusion was seen on the same ictal scan in the latter, ie. the patient with interictal and ictal hypoperfusion. In the two older children middle cerebral artery velocity (MCAV) was reduced by between 29 and 62% in the middle cerebral artery of the predominantly affected hemisphere compared with the contralateral side. During seizures, increases of 6 to 30% in MCAV were recorded for the clinically seizing hemisphere compared with 24 to 170% for the contralateral side in four of the seizures recorded. In one infant, MCAV fell bilaterally during a seizure that generalized (-18 and -43% in the predominantly affected and contralateral side respectively). Sequential recordings in one infant suggested that, with time, the haemodynamic response to seizures of the unaffected hemisphere may decrease. These findings suggest that the venous malformation in SWS is associated with an impairment of the cerebral haemodynamic response to seizure activity.

0012-1622
480-485
Aylett, S. E.
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Neville, B. G.R.
f955179d-844d-49cb-8d88-0eb6a1f76d8a
Cross, J. H.
3c10596a-3f6e-418d-9957-8ec0a4e5078c
Boyd, S.
84c9a3f4-62e9-4573-b815-5eb287f1d570
Chong, W. K.
bb7bc2ce-1866-4fb0-8b82-5a6d2381b67a
Kirkham, F. J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Aylett, S. E.
a944fa5a-e1f1-45ca-9879-2056d2946438
Neville, B. G.R.
f955179d-844d-49cb-8d88-0eb6a1f76d8a
Cross, J. H.
3c10596a-3f6e-418d-9957-8ec0a4e5078c
Boyd, S.
84c9a3f4-62e9-4573-b815-5eb287f1d570
Chong, W. K.
bb7bc2ce-1866-4fb0-8b82-5a6d2381b67a
Kirkham, F. J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58

Aylett, S. E., Neville, B. G.R., Cross, J. H., Boyd, S., Chong, W. K. and Kirkham, F. J. (1999) Sturge-Weber syndrome: Cerebral haemodynamics during seizure activity. Developmental Medicine and Child Neurology, 41 (7), 480-485. (doi:10.1017/S0012162299001036).

Record type: Article

Abstract

The aim of this studs was to examine the haemodynamic response to seizures in three infants with Sturge-Weber syndrome by measuring regional cerebral blood how using transcranial Doppler sonography and 99mTC HMPAO SPECT. Time-locked video/digital EEG recording was carried out for ictal studies. MRI was performed in all subjects. SPECT showed hemispheric hypoperfusion interictally in all three patients and also ictally in one of the three; a small region of hyperperfusion was seen on the same ictal scan in the latter, ie. the patient with interictal and ictal hypoperfusion. In the two older children middle cerebral artery velocity (MCAV) was reduced by between 29 and 62% in the middle cerebral artery of the predominantly affected hemisphere compared with the contralateral side. During seizures, increases of 6 to 30% in MCAV were recorded for the clinically seizing hemisphere compared with 24 to 170% for the contralateral side in four of the seizures recorded. In one infant, MCAV fell bilaterally during a seizure that generalized (-18 and -43% in the predominantly affected and contralateral side respectively). Sequential recordings in one infant suggested that, with time, the haemodynamic response to seizures of the unaffected hemisphere may decrease. These findings suggest that the venous malformation in SWS is associated with an impairment of the cerebral haemodynamic response to seizure activity.

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Published date: 1999

Identifiers

Local EPrints ID: 428118
URI: http://eprints.soton.ac.uk/id/eprint/428118
ISSN: 0012-1622
PURE UUID: a4ed5e03-cd24-4bf4-ade3-58f5beabfaa6
ORCID for F. J. Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

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Date deposited: 12 Feb 2019 17:30
Last modified: 07 Oct 2020 01:46

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Contributors

Author: S. E. Aylett
Author: B. G.R. Neville
Author: J. H. Cross
Author: S. Boyd
Author: W. K. Chong
Author: F. J. Kirkham ORCID iD

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