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Case Reports Patient 1 Childhood onset- age 5 years old Characterized as staring off Responded to Zarontin Adulthood onset – 49 years old Characterized.

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Presentation on theme: "Case Reports Patient 1 Childhood onset- age 5 years old Characterized as staring off Responded to Zarontin Adulthood onset – 49 years old Characterized."— Presentation transcript:

1 Case Reports Patient 1 Childhood onset- age 5 years old Characterized as staring off Responded to Zarontin Adulthood onset – 49 years old Characterized as staring off, malfunction of right arm and fluttering of eyes Failed zarontin, depakote, celontin, lamictal, and topamax Responded to keppra Patient 2 Childhood onset- NONE Adulthood onset – 60 years old Characterized as aphasia followed by GTC activity, funny feelings in head Failed Keppra, Lamictal, Dilantin, Depakote Responded to Ativan prn to prevent GTC Patient 3- Childhood onset- NONE Adulthood onset – 47 years old Characterized as mental lapse or freezing up Failed Keppra, Lamictal, and Dilantin Responded to Depakote Consciousness and Seizure Characteristics in Adults with Recurrent Absence Status and Generalized Epilepsy Patricia S. Mireles MD, Cormac A. O’Donovan MD Dept. of Neurology, Wake Forest University School of Medicine Introduction The clinical features of absence seizures including consciousness and attention can show wide degrees of heterogeny between patients in similar generalized epilepsy syndromes. Recent literature showed that absence persisting into adult life shows particular clinical and EEG patterns, distinct from those in childhood.(1) Atypical electroclinical characteristics may be associated with refractoriness to AEDs and persistence into adulthood. Due to the fact that absence seizure remit in up to 90% in childhood, data in adults is lacking. VEEG monitoring recording in these patients is even more limited (2) This has led to the suggestion that absence seizures with polyspike and wave may predict refractoriness despite this being associated with JME which often remits at 20 years of age. We describe three adult patients with presumed primary generalized epilepsy persisting into the 4 th to 6 th decades who showed a spectrum from one with short absences responsive to medication, another with yearly bouts of absence status evolving to tonic-clonic status, and another with unusual electrographic generalized status for days during which complex neuropsychological testing was performed without difficulty.Methods Three adult patients age ranging from 40 to 65.with recurrent absence as prominent seizure type ranging from frequent brief seizures to prolonged runs of generalized status pattern. Seizure characteristics and frequency, EEG features as well as response to drug therapy were reviewed.Discussion 1. The lack of correlation between epileptiform patterns on EEG and precise manifestations of altered awareness and seizure frequency rates in these adult patients is further evidence of the heterogeneous nature of generalized epilepsy syndromes with absence seizures. 2. Our cases showed some similarities to other series such as polyspike and wave on EEG but many differences. These included amongst others male preponderance, amnesia without altered awareness and failure to respond to many drugs including Valproate 2. Our cases showed some similarities to other series such as polyspike and wave on EEG but many differences. These included amongst others male preponderance, amnesia without altered awareness and failure to respond to many drugs including Valproate. 3. Patient 3 had focal abnormalities referred for surgery reported on outside EEGs which were normal variants on review. The persistence of EEG continuous spike and wave for several days which was subclinical as confirmed by neuropsychological testing and amnesia is very unusual from our literature review. 4. Functional imaging, genetic testing and measurement of awareness in other domains may help characterize these patients better to understand differences from typical primary generalized epilepsy. 5.The multicenter study by Glauser showing lower efficacy rates for drugs previously thought to highly effective for absence seizures highlights that many refractory cases exist and probably undiagnosed in adult. Preliminary studies in animal models show that untreated absence seizures may have long term structural consequences. References: 1.Tatum WO, Ho S, Benbadis SR. Polyspike ictal onset absence seizures. J Clinical Neurol Physiol. 2010 April:27(2):93-9 2.Bleck, T P (08/1998). "Idiopathic generalised epilepsy in adults manifested by phantom absences, generalised tonic-clonic seizures, and frequent absence status". Journal of neurology, neurosurgery and psychiatry (0022-3050), 65 (2), p. 282. 3.Marini C, King MA, Archer JS, Newton MR, Berkovic SF. Idiopathic generalised epilepsy of adult onset: clinical syndromes and genetics. J Neurol Neurosurg Psychiatry. 2003 Feb;74(2):192-6. 4.Glauser TA, Cnaan A, Shinnar S, Hirtz DG, Dlugos D, Masur D, Clark PO, Capparelli EV, Adamson PC. Ethosuximide, valproic acid, and lamotrigine in childhood absence epilepsy. Engl J Med. 2010 Mar 4;362(9):790-9. 5.Nguyen-Michel VH, Ourabah Z, Sebban C, Lavallard-Rousseau MC, Adam C. Nguyen-Michel VH, Ourabah Z, Sebban C, Lavallard-Rousseau MC, Adam C. Idiopathic generalised epilepsies in the elderly: the viewpoint of a geriatrician. Rev Neurol (Paris). 2009 Nov;165(11):924-32. Epub 2009 Mar 13 5.Nguyen-Michel VH, Ourabah Z, Sebban C, Lavallard-Rousseau MC, Adam C. Nguyen-Michel VH, Ourabah Z, Sebban C, Lavallard-Rousseau MC, Adam C. Idiopathic generalised epilepsies in the elderly: the viewpoint of a geriatrician. Rev Neurol (Paris). 2009 Nov;165(11):924-32. Epub 2009 Mar 13.Nguyen-Michel VHOurabah ZSebban CLavallard-Rousseau MC Adam CNguyen-Michel VHOurabah ZSebban CLavallard-Rousseau MC Adam C 6.Hamandi, K, et al., (2006) EEG-fMRI of idiopathic and secondarily generalized epilepsies. NeuroImage. 1700-1710.


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