Answer:
Hemiconvulsion-hemiplegia-epilepsy syndrome
Clinical data:
3 yr old girl with prolonged partial seizures followed by left hemiplegia.
Findings:
There is diffuse cortical edema in entire right cerebral hemisphere not confining to any specific vascular territory. DWI and ADC maps reveal cytotoxic edema in these areas as well in right thalamus. MR angiography shows dilatation of right middle cerebral artery representing hyperperfusion.
Discussion:
Hemiconvulsion-hemiplegia-epilepsy syndrome (HHE) was first described by Gastaut and colleagues almost 50 years ago. It is defined by a prolonged unilateral febrile seizure or seizures followed by the development of a transient or permanent hemiparesis ipsilateral to the side of the convulsion occurring in a young child (<4 years of age). The imaging and pathologic features of HHE include acute edema of the affected hemisphere, often followed by the development of volume loss in that hemisphere. Following initial presentation, children may then develop partial epilepsy at a time remote from the initial presentation.
References:
1. Hemiconvulsion-hemiplegia-epilepsy syndrome Another case for epilepsy surgery Neurology May 27, 2008
2.Diffusion-Weighted MR Imaging of the Brain Second Edition T. Moritani S. Ekholm,P.-L. Westesson
Contributed By: Dr. Arun Chelladurai MD, Dr. Karunakaran M. Kalathi MD
Consultant Radiologists, Aarthi Scans, Thirunelveli, Tamilnadu, India
Hemiconvulsion-hemiplegia-epilepsy syndrome
Clinical data:
3 yr old girl with prolonged partial seizures followed by left hemiplegia.
Findings:
There is diffuse cortical edema in entire right cerebral hemisphere not confining to any specific vascular territory. DWI and ADC maps reveal cytotoxic edema in these areas as well in right thalamus. MR angiography shows dilatation of right middle cerebral artery representing hyperperfusion.
Discussion:
Hemiconvulsion-hemiplegia-epilepsy syndrome (HHE) was first described by Gastaut and colleagues almost 50 years ago. It is defined by a prolonged unilateral febrile seizure or seizures followed by the development of a transient or permanent hemiparesis ipsilateral to the side of the convulsion occurring in a young child (<4 years of age). The imaging and pathologic features of HHE include acute edema of the affected hemisphere, often followed by the development of volume loss in that hemisphere. Following initial presentation, children may then develop partial epilepsy at a time remote from the initial presentation.
References:
1. Hemiconvulsion-hemiplegia-epilepsy syndrome Another case for epilepsy surgery Neurology May 27, 2008
2.Diffusion-Weighted MR Imaging of the Brain Second Edition T. Moritani S. Ekholm,P.-L. Westesson
Contributed By: Dr. Arun Chelladurai MD, Dr. Karunakaran M. Kalathi MD
Consultant Radiologists, Aarthi Scans, Thirunelveli, Tamilnadu, India