Spotlight on Lennox-Gastaut syndrome

Lennox-Gastaut syndrome is diagnosed in individuals with intractable epilepsy characterized by multiple seizure types, slow spike-wave pattern on EEG, and cognitive deterioration typically after first seizure onset. Seizure types include tonic seizures that mainly occur in sleep, atonic seizures, atypical absences, and myoclonic seizures. The condition may follow West syndrome, but has also been associated with various genetic and neurocutaneous syndromes, metabolic diseases, and early infectious or ischemic insults, or may have an unknown etiology. Numerous interventions as well as medications have been studied in treating this condition. Clobazam has been studied in controlling drop attacks and cannabidiol is being researched as an adjunctive antiepileptic agent in patients with Lennox-Gastaut syndrome and other intractable epilepsy syndromes.

In this article, Dr. Mary Spiciarich of Albert Einstein College of Medicine and Montefiore Medical Center and Dr. Solomon Moshe of Albert Einstein College of Medicine discuss the clinical manifestations, diagnosis, and management of Lennox-Gastaut syndrome.

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Spotlight on Antibody-mediated epilepsies

In this article, Dr. Sarosh Irani and Angela Vincent of Oxford University Hospitals and Dr. Bethan Lang of John Radcliffe Hospital summarize the recent advances in the field of antibody-mediated epilepsies. This entity began to be defined with the frequent observation of seizures in encephalitides associated with autoantibodies directed against surface antigens. The seizures in the antibody-positive cases are often best controlled with immunotherapies, which simultaneously reduce the levels of autoantibodies.

Recently, a new distinctive epileptic syndrome with frequent and brief dystonic episodes, typically involving the face and arm, has been recognized and found to be associated with VGKC-complex antibodies, almost always directed against LGI1. Patients with these “faciobrachial dystonic seizures” have a relatively poor response to antiepileptic drugs, but their seizures often respond better to immunotherapies. Emerging data suggest that patients who present with faciobrachial dystonic seizures progress to develop cognitive impairment, which may be prevented by treatment of the faciobrachial dystonic seizures. In addition, a few patients with status epilepticus and classical temporal lobe epilepsy have been described with these and other autoantibodies. Finally, although glutamic acid decarboxylase (GAD) is an intracellular enzyme, antibodies directed against GAD are found in patients with acute and chronic focal epilepsies and may be a marker of an autoimmune tendency. The antibody-mediated epilepsies are a growing field with important etiologic and therapeutic implications for both children and adults.

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MedLink Neurology authors are always at work to bring you broad and up-to-date coverage of neurology topics. We are pleased to highlight clinical summaries that have been recently added or updated and to introduce the authors who write these authoritative articles. We hope you enjoy these overviews and appreciate the contributions of our more than 450 authors who keep MedLink Neurology the premier information resource for neurologists.

Spotlight on Hyperkinetic seizures

Hyperkinetic seizures are characterized by motor seizures involving predominantly proximal limb or axial muscles in irregular sequential ballistic movements. The origin of hyperkinetic seizures is more commonly localized in the mesial frontal or orbitofrontal regions. Seizures often occur during sleep and recur repeatedly, with intervals of a few seconds. Exclusively nocturnal hyperkinetic seizures are the typical spells in autosomal dominant nocturnal frontal lobe epilepsy. In this clinical summary, Drs. Ana Carolina Coan and Fernando Cendes of the Department of Neurology at University of Campinas – UNICAMP in Brazil present recent data and discussion about the causes, syndromes, diagnosis, and management of hypermotor seizures.

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MedLink Neurology authors are always at work to bring you broad and up-to-date coverage of neurology topics. We are pleased to highlight clinical summaries that have been recently added or updated and to introduce the authors who write these authoritative articles. We hope you enjoy these overviews and appreciate the contributions of our more than 450 authors who keep MedLink Neurology the premier information resource for neurologists.

Spotlight on Startle epilepsy

Startle epilepsy is characterized by seizures triggered by unexpected sudden sensory stimuli, usually sound or touch. Most patients have intractable seizures, evident static encephalopathy, and neurologic deficits (infantile hemiplegia is common) with corresponding abnormal imaging. The startle response consists of axial tonic posturing, frequently causing falls that can often be traumatic. Concurrent symptoms, such as marked autonomic manifestations, automatisms, laughter, and jerks, may occur. Less commonly, startle-induced seizures may be atonic or myoclonic, particularly in patients with cerebral anoxia. Seizures are frequent, occurring many times a day, and sometimes progress to status epilepticus.

The prognosis is often poor, particularly for those with severe pre-existing encephalopathies. Startle epilepsy is often resistant to medical treatment. Epilepsy surgery may be beneficial to those with focal etiology of seizures.

Dr. C P Panayiotopoulos, consultant emeritus in St. Thomas’ Hospital, London, UK, details clinical manifestations, etiology (including inborn errors of metabolism), investigative procedures, pathophysiology, and management issues (including neurosurgery) of startle-induced epileptic seizures.

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MedLink Neurology authors are always at work to bring you broad and up-to-date coverage of neurology topics. We are pleased to highlight clinical summaries that have been recently added or updated and to introduce the authors who write these authoritative articles. We hope you enjoy these overviews and appreciate the contributions of our more than 450 authors who keep MedLink Neurology the premier information resource for neurologists.