Epilepsy. Presented By: Stan Andrisse

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Transcription:

Epilepsy Presented By: Stan Andrisse

What Is Epilepsy Chronic Neurological Disorder Characterized by seizures Young children or elderly Developing countries

Famous Cases Socrates Muhammad Aristotle Joan of Arc Julius Caesar Multiple Saints Napoleon Beethoven Pythagoras Isaac Newton

Classification First Cause Observable manifestations Origination location in the brain Identifiable medical syndromes Triggering event/age of onset

Seizure Types Partial/Focal Onset (Localized ) Simple Partial Consciousness affected Complex Partial Psychomotor Unknown Localization Single Seizures Provoked Seizures Febrile Convulsions Generalized (Distributed) Loss of consciousness Multifocal/Circuits Types Absence (petit mal) Myoclonic Clonic Tonic Atonic (Vendrame 2011)

Seizure Types (cont) Partial (focal) vs Generalized

Seizure Types (cont) Over 40 different types Triggers Reflex (reading) Photosensitive Hyperventilation Emotional stress Sleep deprivation Alcohol Menstrual cycle Common Mistaken Behaviors Blind Stares Shudders Self gratification Nodding, rocking, etc

Causes Idiopathic Genetic abnormalities Ring Chromosome 20 Syndrome Altered neuronal regulation Symptomatic Epileptic lesion Focal (tumor) Defect in metabolism Widespread Brain injury Cryptogenic Presumptive lesion Difficult diagnosis

Causes (cont) CNS infections/abnormalities Stress Metabolic disorders Brain tumor Head trauma Illicit drug use Hypoxic encephalopathy Alcohol withdrawal Age related onset

Most common adult epilepsy Hippocampal Sclerosis (HS) Recurrent Excitation/Inhibition Religious/mystic hallucinations Post status epilepticus mice Latency Motor seizures Similar lesions

PathoPhysiology of MTLE A) Bregma 3.12 Cranial Hippocampus B) Bregma 5.28 Caudal Hippocampus C) Sub anatomic Hippocampus D) Hippocampal network unidirectional

Idiopathic genetic abnormality Cognitive Impairment Absence of dysmorphology Birth defects Pathogenicity unknown 8 r(20) patients FISH characterization Florescence in situ hybridization

West Syndrome Trademarks Infantile spasms Developmental Delay Hypsarrthmia Abnormal EEG Prognosis Continued seizures Cognitive impairment Onset 8 9 months Causes Idiopathic, symptomatic, or cryptogenic Tuberous sclerosis

Rasmussen s Encephalitis Trademarks Symptomatic localization Progressive Inflammatory lesions Cause Immunological attack on glutamate receptors Excitatory transmitter Onset Before age 10 Pathology Inflammatory encephalitis on one side of brain

Pathophysiology Channel Mutations Voltage gated Ligand gated Epileptogenesis Epilepsy after trauma Kindling Electrical stimulus Increased seizure susceptibility Mechanisms Constitutively open Na + channels (hyper excite) Release of glutamate Excessive Ca 2+ release Neurotoxic Hippocampal NMDAR GABA mutations Chemical stimulation Pesticides Excitotoxicity

Management Medications Anticonvulsant Lifelong Decreased quality of life 20 FDA approved Goal Lyrica (pregabalin) Valium (Diazepam) Pentobarbital (My lab uses) Many black market (Mexico) No seizures Minimal side effects Surgery Focal localization Anticonvulsant resistance Epileptic vs non epileptic Long term video EEG Resection of lesions (Tumors) Anterior Temporal Lobectomy Palliative Remove front of temporal Amygdala and hippocampus Decreased health care cost

Management Ketogenic Diet High fat, low carb Vagus Nerve Stimulation Cyberonics Mechanism unknown Similar to pacemaker Connects to vagus in neck

Literature Cited ALOK K. SHARMA, RACHEL Y. REAMS, WILLIAM H. JORDAN, MARGARET A. MILLER, H. LEON THACKER, AND PAUL W. SNYDER. Mesial Temporal Lobe Epilepsy: Pathogenesis, Induced RodentModels and Lesions. Toxicologic Pathology, 35:984 999, 2007. Tilelli, C. Q., Del Vecchio, F., Fernandes, A., and Garcia Cairasco, N. Different types of status epilepticus lead to different levels of brain damage in rats. Epilepsy Behav 7, 401 10, 2005. Vendrame M, Zarowski M, Alexopoulos AV, Wyllie E, Kothare SV, Loddenkemper T. Localization of pediatric seizure semiology. Clin Neurophysiol. 2011. Daniela Giardino et al. Genetic investigations on 8 patients affected by ring 20 chromosome syndrome. BMC Medical Genetics 2010, 11:146.

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