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Symptomatic epilepsy – identifiable cerebral cause. Cryptogenic epilepsy – non-identifiable cause in a patient with neurological deficits or cognitive impairment Infantile spasms LGS (Lennox-Gastaut syndrome) Myoclonic astatic epilepsy of Doose Idiopathic epilepsy – non-identifiable cause in an entirely normal patient juvenile myoclonic epilepsy benign partial epilepsy of childhood with centrotemporal spikes benign partial epilepsy of childhood with occipital paroxysms
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Symptomatic epilepsy – identifiable cerebral cause. Cryptogenic epilepsy – non-identifiable cause in a patient with neurological deficits or cognitive.

Dec 18, 2015

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Merryl Walsh
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Page 1: Symptomatic epilepsy – identifiable cerebral cause. Cryptogenic epilepsy – non-identifiable cause in a patient with neurological deficits or cognitive.

Symptomatic epilepsy – identifiable cerebral cause.• Cryptogenic epilepsy – non-identifiable cause in a

patient with neurological deficits or cognitive impairment– Infantile spasms– LGS (Lennox-Gastaut syndrome)– Myoclonic astatic epilepsy of Doose

• Idiopathic epilepsy – non-identifiable cause in an entirely normal patient– juvenile myoclonic epilepsy– benign partial epilepsy of childhood with centrotemporal

spikes– benign partial epilepsy of childhood with occipital

paroxysms 

Page 2: Symptomatic epilepsy – identifiable cerebral cause. Cryptogenic epilepsy – non-identifiable cause in a patient with neurological deficits or cognitive.

• Important Epilepsy Syndromes:• West syndrome – Triad of:

– hypsarrhythmia on EEG– infantile spasms– developmental retardation or regression

• Presents in 6-18 month infants and is treated with ACTH or vigabatrin.• Lennox-Gastaut Syndrome (LGS) – Infantile spasms and West syndrome frequently transform into LGS

becoming a life-long epileptic encephalopathy. It consists of:– atonic seizures– tonic seizures– atypical absence seizures associated with mental retardation and a characteristic EEG pattern.

• Typical onset of LGS is between 3-5 years of age. EEG shows an abnormally slow background and diffuse slow spike and slow wave (<2.5 Hz) activity due to generalized encephalopathy.

• Childhood Absence Epilepsy – occurs between 3-5 years of age and remits by ages 10-12 years. Frontally dominant EEG showing normal background for age and 3-Hz generalized spike and wave discharges. Usually 4 Hz at the onset of the absence seizures and may slow to 2.5 Hz at the end of a seizure.

• Benign partial epilepsy syndromes of childhood – there are two important types:– Benign rolandic epilepsy (BRE) or Benign partial epilepsy of childhood with centrotemporal spikes – onset is

between 3-10 years with history of orobuccal numbness on one side of the mouth or with a tingling sensation on one side of the face. EEG shows frequent spike and wave discharges in the centrotemporal region.

– Benign partial epilepsy of childhood with occipital paroxysms (BPEOP) – similar to BRE but the discharges are located in the occipital part of brain.

• Juvenile Myoclonic Epilepsy (JME)- the most common epilepsy syndrome presenting with generalized tonic-clonic seizures in an other-wise neurologically normally patient aged 12-30 years. Photosensitivity is present in at least 30% of patients.

• Clue words for the boards: • slow spike and wave activity = LGS• diffuse 3-Hz spike and wave activity = benign absence epilepsy• fast spike and wave (>2.5 Hz) activity = benign myoclonic types of epilepsy• hyperarrhythmia = infantile spasms

Page 3: Symptomatic epilepsy – identifiable cerebral cause. Cryptogenic epilepsy – non-identifiable cause in a patient with neurological deficits or cognitive.

• Syncope;• Transient ischaemic attack (TIA);• Transient global amnesia;• Panic attack;• Episodic vertigo;• Stroke;• Migraine;• Memory disturbance and/or confusion; and• Restless legs syndrome

Page 4: Symptomatic epilepsy – identifiable cerebral cause. Cryptogenic epilepsy – non-identifiable cause in a patient with neurological deficits or cognitive.

Seizure Syncope TIA Transient global

amnesia

Panic attack

Premonition

Symptoms

None or Aura symptoms None; or LightheadednessNauseaVomitingDiaphoresisPallor

Palpitations None LightheadednessNauseaVomitingDiaphoresisPanic, fear

Precipitating

Factors

Sleep deprivationFlashing lights

Postural changeNeck movementsProlonged standing

Exercise None Stress Social situations

Onset Acute Variable Acute Acute Acute

Duration 1-2 minutes Seconds-minutes Minutes-hours Hours Variable

Movements Variable tonic clonic movements depending upon where seizure affects brain

Loss of toneClonic jerking

Physical deficits dependant upon where occurs

None None orPacingAgitationRapid breathingStiffening of hands

Incontinence Variable Occasional None None None

Heart rate Increased or decreased Variable Normal Normal Increased

Postictal Confusion Sleep Alert or mild confusion Alert Alert Alert

Page 5: Symptomatic epilepsy – identifiable cerebral cause. Cryptogenic epilepsy – non-identifiable cause in a patient with neurological deficits or cognitive.

• 50% of patients • Depression, Anxiety and Psychotic

disturbances• These psychiatric disturbances can be

classified according to how they relate in time to seizure occurrence,

• ictal• periictal(preictal/prodromal, postictal)• interictal.

Page 6: Symptomatic epilepsy – identifiable cerebral cause. Cryptogenic epilepsy – non-identifiable cause in a patient with neurological deficits or cognitive.

• Treatmentrelated psychiatric problems

• Antiepileptic drugs

• most commonly depression, anxiety, behavioural or cognitive problems and, in rare cases, psychosis.

• Phenobarbitone, primidone, tiagabine, topiramate, vigabatrin and felbamate have

• been associated with depression.

• Psychosis is a rare complication of a number of AEDs such as vigabatrin and topiramate.

• Improved seizure control has been associated with the emergence of psychiatric symptoms.

• Landolt introduced the term ‘forced normalisation’ which refers to a dramatic reduction in epileptiform activity on EEG being associated with the emergence of psychosis or sometimes behavioural/mood disturbances.

• This phenomenon has been reported with most AEDs and therefore any new drug should be started at low doses and increased slowly. The risk may be higher in patients who are on polytherapy, become seizure free abruptly, or if there is a past psychiatric history.

Page 7: Symptomatic epilepsy – identifiable cerebral cause. Cryptogenic epilepsy – non-identifiable cause in a patient with neurological deficits or cognitive.

• Epilepsy surgery

• Transient mood disturbances (emotional lability, depression and anxiety) have been reported following temporal lobe surgery for epilepsy (about 25%) in the first 6-12 weeks.

• in some patients (10%), symptoms, particularly depression, may persist and require psychiatric treatment.

• There are also reports of de novo psychosis arising after surgery and it has been suggested that it is more common with right sided temporal lobe surgery.

• It is therefore important for pre and postsurgical psychiatric evaluation to form part of the assessment/management for epilepsy surgery.

Page 8: Symptomatic epilepsy – identifiable cerebral cause. Cryptogenic epilepsy – non-identifiable cause in a patient with neurological deficits or cognitive.

MAGNETIC RESONANCE IMAGING (MRI): Each patient withnew-onset epilepsy should have an MRI (temporal angulation,T1,T2, FLAIR, coronal and axial) to detect structural lesions causedby for example cortical malformation, traumatic brain injury, braintumor, and cerebrovascular disease, which are the most commoncauses of symptomatic epilepsy. Contrast media, inversion recovery,fast field echo and 3D only in special cases. Even in idiopathicepilepsy, MRI is recommended to diagnose unsuspected dualpathology as discussed above.ELECTROENCEPHALOGRAM (EEG): Each patient with newonsetepilepsy should have an EEG. EEG is most valuable within 24h of the seizure. Information gain is optimal up to the 4th EEG, if noparoxysmal interictal discharges are found, repeat EEG duringsleep. 24-hour EEG most meaningful in a patient with frequentseizures who can be expected to have seizures during the 24 hrecording. Interictal EEG discharges may support the diagnosis ofthe epilepsy syndrome.COMPUTER TOMOGRAM (CCT): Computer tomograms areobsolete, except to detect fractures or hemorrhage in an emergencysituation.HEAD X-RAY: obsoleteCLINICAL CHEMISTRY: routine work-up, creatinkinase, VitaminB6 if seizures are unresponsive to AEDs, even in adults. CSF, onlywhen infectious disorders are suspected. Creatinkinase increasedwithin 12-24 h, prolactin increased within 30 min.SINGLE-PHOTON- EMISSION -TOMOGRAM (SPECT) andPOSITON- EMISSION-TOMOGRAM (PET), MAGNETOENCEPHALOGRAPHY (MEG): only for presurgical work-up orscientific studies.

Page 9: Symptomatic epilepsy – identifiable cerebral cause. Cryptogenic epilepsy – non-identifiable cause in a patient with neurological deficits or cognitive.

Suggested MRI protocols for epilepsy patients (standard investigations)Temporal lobe epilepsy1. Hippocampal oriented T2-weighted (coronal + axial)2. Hippocampal oriented fluid-attenuated inversion recovery (FLAIR) (coronal + axial)3. Isotropic T1-weighted three-dimensional sequence (MPRage)4. (Gadolinium contrast-enhanced T1-weighted image if non-contrast-enhanced image is inconclusive)5. T2*-weighted sequenceExtratemporal lobe epilepsy1. AC–PC oriented T2-weighted (coronal + axial)2. AC–PC oriented FLAIR (coronal + axial)3. Isotropic T1-weighted three-dimensional sequence (MPRage)4. (Gadolinium contrast-enhanced T1-weighted image if non-contrast-enhanced image is inconclusive)5. T2*-weighted sequenceSpecial protocols: Rationale1. T2 relaxometry hippocampal signal abnormalities2. Magnetic resonance spectroscopy detection of metabolic abnormalities3. Diffusion tensor imaging (DTI) investigation of fiber tracts4. Functional MRI investigation of eloquent cortical areas5. Three-dimensional sequences automated voxel-based analyses6. Magnetic resonance angiography investigation of brain vascularization

Page 10: Symptomatic epilepsy – identifiable cerebral cause. Cryptogenic epilepsy – non-identifiable cause in a patient with neurological deficits or cognitive.