Absence-to-bilateral-tonic-clonic seizure: A generalized seizure type.

OBJECTIVE: To test the hypothesis that absence seizures can evolve to generalized tonic-clonic seizures, we documented electroclinical features of this novel seizure type. METHODS: In 4 large video-EEG databases, we identified recordings of seizures starting with impaired awareness that, without returning to baseline interictal state, evolved to generalized tonic-clonic seizures. We extracted the detailed semiologic and electrographic characteristics of these seizures, and we documented the clinical background, diagnoses, and therapeutic responses in these patients. RESULTS: We identified 12 seizures from 12 patients. All seizures started with a period of impaired awareness and bursts of generalized spike or polyspike and slow-wave discharges, the hallmark of absence seizures. Without returning to baseline, the nonmotor (absence) phase was followed by tonic-clonic convulsions. We called this novel generalized seizure type absence-to-bilateral-tonic-clonic seizure. Most patients had idiopathic generalized epilepsies, although with a high incidence of unusual features and poor therapeutic response. CONCLUSIONS: Absence-to-bilateral-tonic-clonic seizures are a novel generalized seizure type. Clinicians should be aware of this seizure for correctly diagnosing patients. This novel seizure type may further elucidate generalized ictogenesis

Epilepsy and Cerebral Palsy

Abnormalities of muscle tone, movement, and motor skills are the hallmark of cerebral palsy (CP) which results from injury to the developing brain. Clinically, the syndrome evolves over time and may only be apparent after 3–5 years of age, although suggestive signs and symptoms may be present at an earlier age. Epilepsy is common in CP and occurs in about 30% of patients. Generally, the onset is within the first 2 years of life. Epilepsy is commonly observed in children with spastic hemiplegia, followed by quadriplegia and diplegia. Significant risk factors for the development of epilepsy in patients with CP are family history, neonatal seizure, structural abnormalities, low Apgar scores, and mental retardation. Focal to bilateral tonic-clonic seizures are the most prominent seizure types, followed by focal aware or impaired awareness seizures, while infantile spasms and myoclonic jerks are seen in 25% of cases. Mental retardation is a predisposing factor for early onset of seizures and more severe epilepsy. The overall outcome of seizures in children with CP is generally poor, requiring prolonged course of antiepileptic medication, usually polytherapy with higher incidence of refractory seizures, side effects, comorbidities, and hospital admissions for drug-resistant seizures or status epilepticus

Status Epilepticus in Children

Status epilepticus (SE) is an acute, one of the most common, life threatening emergency condition in children and adolescence. According to the ILAE commission, SE is defined as a condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which lead to abnormally, prolonged seizures. Regarding duration, ILAE defines convulsive SE as bilateral tonic–clonic lasting longer that 5 minutes, and absence and focal status epilepticus as exceeding 10 minutes. Children compared to adults are more prone to epileptic seizures and as a consequence to SE, mainly due to age related maturational imbalance between excitatory and inhibitory synaptic mechanisms. The classification into generalised or focal, convulsive and nonconvulsive differ widely in aetiology, management and outcome. Early prehospital intervention with appropriate protective measures and emergency medication of seizure disorder, may prevent perpetuation of seizure recurrence. If first line treatment fails and or event, emergency hospital admission should be provided for more aggressive intravenous therapy, assessment-support

Panayiotopoulos syndrome: a consensus view

The aim of this paper is to promote the correct classification of, and provide guidelines on, the diagnosis and management of panayiotopoulos syndrome (ps). an international consortium of established researchers in the field collaborated to produce a consensus document. the resulting document defines ps, characterizes its electro-clinical features, considers its likely pathogenesis, and provides guidance on appropriate management. we conclude that ps is a common idiopathic, benign seizure disorder of childhood, which should be classified as an autonomic epilepsy, rather than an occipital epilepsy.</p

Periodic EEG Patterns in the Intensive Care Unit (ICU): Definition, Recognition and Clinical Significance

Periodic electroencephalographic (EEG) patterns are frequently recorded during ICU EEG monitoring in patients with altered mental status; these EEG features represent electrical discharges, ictal in appearance, occuring at regular intervals. They are known as lateralized periodic discharges (LPDs), bilateral independent periodic discharges (BIPDS), generalized periodic discharges (GPDs), continuous 2/s GPDs with triphasic morphology or triphasic waves (TWs) and Stimulus Induced Evolving Lateralized Rhytmic delta activity or Si-Evolving LRDA (previously SIRPIDS); other periodic, rhythmic patterns are Occasional frontally predominant brief 2/s GRDA (FIRDA previously), Lateralized rhythmic delta activity (LRDA) and Brief potentially ictal rhythmic discharges or B (I)RDs. The role of most (not all) of these EEG patterns is controversial; there is no consensus on which patterns are associated with ongoing seizure injury, which patterns need to be treated, and how aggressively they should be treated. Many authors consider these patterns as an unstable state on an ictal-interictal EEG continuum; the aim of the present chapter is to gain knowledge of these EEG features, show their association with known neurologic pathologies/syndromes and finally how to manage them

Jeavons syndrome – updated review

Introduction. Eyelid myoclonia and absences (ELMA) was first described by Jeavons in 1977 as a separate type of photosensitive epilepsy