Epilepsy & Seizures Flashcards
What is deja vu?
Perception of familiarity with previously unfamiliar people or events
Perception of unfamiliarity with previously familiar people or events
Deja entendu
What is jamais entendu?
Perception of unfamiliarity with previously familiar auditory stimulus
Perception of familiarity with previously unfamiliar auditory stimulus
jamai vu
Neonatal Epilepsy Syndromes 5
Benign familial neonatal seizures
Early myoclonic encephalopathy
Ohtahara syndrome
Migrating partial seizures of infancy
Infantile Epilepsy Syndromes
Mnemonic:
DraW-A-BEBE
West’s syndrome Aicardi’s syndrome Benign myoclonic epilepsy of infancy Benign infantile seizures Dravet syndrome
Childhood Epilepsy Syndromes
Benign childhood epilepsy with centrotemporal spikes
Early-onset benign childhood occipital epilepsy
Late-onset childhood occipital epilepsy
Epilepsy with myoclonic absences
Myoclonic-astatic epilepsy of childhood
Lennox-Gastaut syndrome
Landau-Kleffner syndrome
Epilepsy with continuous spike and waves during slow wave
sleep
Childhood absence epilepsy
Progressive myoclonic epilepsies
Also called “fifth-day fits”
Benign neonatal seizures
Benign familial neonatal convulsions is an autosomal
dominant disorder caused by mutation of
KCNQ2 or KCNQ3
mutations impair potassium-dependent repolarization resulting in hyperexcitability
West’s syndrome is a nonspecific diagnosis referring to
the triad of
1 infantile spasms
2 hypsarrhythmia
3 developmental arrest
Aicardi’s syndrome is the X-linked triad of
1 infantile spasms
2 agenesis of corpus callosum
3 retinal malformations
Early myoclonic encephalopathy is characterized by
newborn infants with migrant focal myoclonic epilepsy
and progressive psychomotor abnormalities
Early myoclonic encephalopathy EEG findings
- Generalized or focal epileptiform discharges
- burst suppression which may evolve to hypsarrythmia later
- Myoclonus has no EEG counterpart
Epilepsy Syndromes which show burst suppression on EEG
Early myoclonic encephalopathy
Ohthara Syndrome
Epilepsy Syndromes which show hypsarrythmia
Infantile Spasms
West Syndrome
How to differentiate Early myoclonic encephalopathy from Ohthara Syndrome?
no myoclonic seizures in Ohtahara syndrome
Migrating partial seizures of infancy
Multifocal seizures, shift from hemisphere to hemisphere
Pyridoxine (vitamin B6)-dependent seizures (congenital
dependency on pyridoxine) is said to be because of
diminished activity of glutamic acid decarboxylase (GAD); leading to increased glutamic acid
Severe myoclonic epilepsy in infancy also known as
Dravet Syndrome
Dravet syndrome
progressive myoclonic seizures (begin mild, worsen over time, partial seizures develop later
progressive neurologic deterioration that may be secondary to recurrent seizures
refractory to treatment
benign rolandic epilepsy of childhood
Centrotemporal spikes on EEG
Early-onset benign childhood occipital epilepsy also known as?
Panayiotopoulos syndrome
Panayiotopoulos syndrome
Autonomic seizures and status epilepticus: commonly,
ictal vomiting, eye deviation; often progress to partial
clonic or generalized tonic-clonic seizures (often
nocturnal)
Visual seizures
Panayiotopoulos syndrome EEG
bursts or trains of high-voltage rhythmic occipital 1 to 3 Hz spikes & spike-wave complexes, localized to uni or bilateral occipital, w normal background, increases during NREM sleep, disappears with eye opening
Panayiotopoulos syndrome first line of treatment
carbamazepine
AEDs that can worsen abscence seizure and why
Carbamazepine
Phenytoin
AEDs that can worsen abscence seizure and why
Carbamazepine
Phenytoin
Absence seizures are driven by T-type calcium channels of the thalamus, which are promoted by GABAergic drugs
Therefore, GABAergic anticonvulsants may promote
absence seizures
Rasmussen’s encephalitis pathogenesis
antibodies to GLUR3 (glutamate receptor-3)
Progressive Myoclonic Epilepsies
Lafora body disease Unverricht-Lundborg syndrome Neuronal ceroid lipofuscinosis Myoclonic epilepsy with ragged-red fibers Sialidoses
Autosomal dominant partial epilepsy with auditory
features is an example of a monogenic temporal lobe
epilepsy, caused by a mutation of the
LGI1 (leucine-rich,
glioma-inactivated 1) gene
Features of a TYPICAL Absence Seizure
Adams
Rapid onset and offset
Typical 3 per second spike and wave
Complete loss of awareness
What is ATYPICAL Absence Seizure
Adams
Long runs of SLOW spike and wave activity, usually with NO apparent loss of consciousness
Most common underlying conditions of Lennox- Gestaut Syndrome (Adams)
Prematurity, perinatal injury and metabolic diseases of infancy
Most common form of idiopathic generalized epilepsy in older children and young adults (Adams)
Juvenile Myoclonic Epilepsy
EEG findings in JME
4- to 6- Hz irregular polyspike activity
Focal seizures with sensory or motor features at the onset most often arise from the SENSORIMOTOR CORTEX.
Where does seizures with impairment of consciousness mostly arise from?
Limbic and Autonomic areas or in the Temporal Lobe
The FENCING POSTURE in frontal lobe seizures have been associated with what specific area of the frontal lobe?
High medial frontal region corresponding to Area 8 and Supplementary Motor Cortex
Olfactory hallucinations are associated with which area?
Inferior and medial parts of the temporal lobe (parahippocampal convolution) or Uncus (Uncinate Seizures)
Gustatory hallucinations are associated with which area?
Temporal Lobe> Insula and Parietal operculum
Vertiginous sensations are associated with which area?
Superoposterior Temporal region or junction between parietal and temporal lobes
Particular combination seen in Hypothalamic Hamatoma?
Gelastic Seizures
Precocious Puberty
What is the risk of sudden death among untreated patients with epilepsy?
as high as 20 times greater
True or False: Benign Epilepsy of Childhood with Centrotemporal spikes is self- limited.
True.
Common viral precipitant in Febrile Seizures probably due to its tendency to cause high fever
Herpesvirus 6
True or False: Prophylactic AED has been found to be helpful in preventing Febrile Seizures
False
