The association of focal motor seizures with cerebral hemiatrophy is a recognised rare paediatric syndrome known as 'hemiconvulsion, hemiatrophy and epilepsy' (HHE). To date, HHE has not been reported in adults. We present four adult patients with striking similarities to HHE, following alcohol withdrawal in chronic alcoholics. We document the imaging findings in the acute and subacute phases, discuss the underlying mechanisms and present a hypothesis regarding the pathophysiology.
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http://dx.doi.org/10.1111/imj.16162 | DOI Listing |
J Neurosurg Case Lessons
September 2023
Departments of1Cerebrovascular Surgery, and.
Background: Brain arteriovenous malformations (AVMs) usually manifest as hemorrhages or seizures. They rarely present with ischemic symptoms, especially in young patients. We present a case of an epileptogenic AVM that led to cerebral infarction due to paradoxical embolic occlusion of the middle cerebral artery (MCA) involving the main feeder of the lesion.
View Article and Find Full Text PDFIntern Med J
July 2023
The University of Western Australia (M570), Perth, Western Australia, Australia.
The association of focal motor seizures with cerebral hemiatrophy is a recognised rare paediatric syndrome known as 'hemiconvulsion, hemiatrophy and epilepsy' (HHE). To date, HHE has not been reported in adults. We present four adult patients with striking similarities to HHE, following alcohol withdrawal in chronic alcoholics.
View Article and Find Full Text PDFEpilepsia Open
December 2022
Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy.
We describe a case of epileptic encephalopathy in a young woman with undiagnosed medium-chain acyl-coenzyme A dehydrogenase deficiency (MCADD), who presented with an early-onset focal motor status epilepticus (SE) then followed by permanent left hemiplegia and drug-resistant epilepsy with neurodevelopmental delay. Throughout her clinical history, recurrent episodes of lethargy, feeding difficulties, and clustering seizures occurred, progressing into a super refractory SE and death at the age of 25 years. Although epilepsy is not a distinctive feature of MCADD, we advise considering this metabolic disease as a possible etiology of epileptic encephalopathy and hemiconvulsion-hemiplegia-epilepsy syndrome of unknown origin, on the chance to provide a timely and targeted treatment preventing development delay and evolution to SE.
View Article and Find Full Text PDFEur J Neurol
February 2022
Department of Neurology, Christian Doppler University Hospital, Center for Cognitive Neuroscience, Paracelsus Medical University, Salzburg, Austria.
Background And Purpose: New-onset refractory status epilepticus (NORSE) is a clinical presentation, neither a specific diagnosis nor a clinical entity. It refers to a patient without active epilepsy or other pre-existing relevant neurological disorder, with a NORSE without a clear acute or active structural, toxic or metabolic cause. This study reviews the currently available evidence about the aetiology of patients presenting with NORSE and NORSE-related conditions.
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