JLE

Epileptic Disorders

MENU

How to diagnose and classify idiopathic (genetic) generalized epilepsies Volume 22, issue 4, August 2020

TEST YOURSELF

(1) What is idiopathic (genetic) generalized epilepsy? Which syndromes are regarded as IGE?

 

(2) What are the seizure types encountered in IGE?

 

(3) How can you increase sensitivity of EEG in suspected IGE cases?

 

(4) Which one of the following is not an indispensable step in the work-up for IGE?

A. Detailed history of the seizures

B. Family history

C. Neurological examination

D. EEG tailored to “A”

E. Neuroimaging

 

(5) Which one of the following is false regarding generalized tonic-clonic seizures?

A. Their onset is gradual

B. Recovery after GTCS is gradual

C. They may accompany any IGE syndrome

D. GTCSs may follow successive absences or myoclonic jerks

E. Short-lived focal features at onset may be encountered

 

(6) Which of these statements about absence seizures is true?

A. Impairment of consciousness starts and ends abruptly

B. A myoclonic component may be observed during absences

C. Automatisms are not exceptional during absence seizures

D. Hyperventilation triggers absence seizures

E. All of the above

 

(7) Which one of the following is false regarding myoclonic seizures?

A. They may be singular or occur in rhythmic or arrhythmic clusters

B. Consciousness is usually preserved

C. They are exclusively seen in JME patients

D. Jerks may be asymmetrical or focal

E. They are more frequent upon awakening

 

(8) EEG is a vital part in the evaluation of IGE patients, however, EEG has some limitations. Which one of these indications below is not appropriate?

A. To aid the diagnosis of IGE

B. To help exclude other items in the differential diagnosis list

C. To help optimise antiepileptic drug selection

D. To detect specific seizure triggers

E. To predict possible relapses after antiepileptic drug discontinuation

 

(9) The hallmark EEG finding for IGE is:

A. Generalized photoparoxysmal responses

B. Occipital intermittent rhythmic delta activity

C. Epileptic (spiky) K complexes

D. Generalized delta wave paroxysms

E. Generalized spike-wave discharges

 

(10) Activation methods are of utmost importance in IGE patients. Which of the following is false regarding activation methods?

A. Photoparoxysmal responses are exclusively seen in IGE syndromes

B. Photoparoxysmal responses are most commonly encountered in JME patients

C. Existence of photoparoxysmal responses warrants lifestyle changes

D. Hyperventilation effectively induces absences in most patients with absence seizures

E. Sleep and sleep deprivation are very important activation methods

 

(11) Which one of the following is not expected in JME?

A. Onset between ages 8 to 25

B. Myoclonic jerks alone or in combination with absence seizures and generalized tonic-clonic seizures

C. Generalized spike-wave and polyspikes-wave discharges

D. Focal spikes not consistent over one particular area

E. Slow-spike-wave discharges

 

(12) Which one of the following is true regarding IGE with GTCS alone?

A. GTCS is the only seizure type

B. Rarely, absences or myoclonic seizures may accompany the clinical picture

C. The onset is usually within the first year of life

D. Sleep deprivation and awakening are not important seizure facilitators

E. Diagnosis can be made without EEG findings

 

(13) Which one of the following is false regarding absence epilepsies?

A. The age at onset for childhood absence epilepsy is generally 6-8 years and multiple absences occur daily

B. The age at onset for juvenile absence epilepsy is generally 10-12 years and the frequency of absences is lower than in CAE

C. Their EEG hallmark is the 3-Hz generalized spike-wave discharge

D. Children with absence epilepsy may be mistakenly diagnosed with learning difficulties due to lapses of attention

E. GTCS are unexpected in the disease course and their existence should lead to exclusion of absence epilepsy in the differential diagnosis

 

 

 

 

 

 

 

 

 

 

 

See answers

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Answers

(1) Idiopathic or genetic generalized epilepsy is a type of epilepsy manifesting with generalized seizures, and without any causal brain lesion or demonstrable aetiology other than genetic tendency. IGEs consist of four well-established syndromes and some other rarer phenotypes. The main four IGEs are childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy and IGE with generalized tonic-clonic seizures alone.

 

(2) Three main seizure types are encountered either alone or in combination in IGE; these are generalized tonic-clonic seizures, typical absences and myoclonic seizures.

 

(3) Many methods can be applied in order to increase the sensitivity of EEG. Sleep deprivation and sleep can enhance generalized spike-wave discharges (GSWDs), effectively triggering GSWDs and TA; photic stimulation can reveal photosensitivity; and other triggers gathered from the patient history can be tested. Scheduling the EEG appointment to early morning may disclose GSWDs and MS, while 24-hour EEG or longer telemetry may allow registration of GSWD when routine or sleep EEG is unremarkable.

 

(4) E

 

(5) A

 

(6) E

 

(7) C

 

(8) E

 

(9) E

 

(10) A

 

(11) E

 

(12) A

 

(13) E

 

 

 

 

 

 

 Back to questions