SURGICAL MANAGEMENT OF EPILEPSY

Eric R. Trumble, MD
NEUROSURGEONS FOR KIDS

 

 

 

Definition of Epilepsy

  • Epilepsy implies a periodic recurrence of seizures with or without convulsions
  • Seizures result from an excessive synchronous discharge of cortical neurons and is characterized by changes in electrical activity
  • A convulsion implies violent, involuntary contraction(s) of the voluntary muscles

 

 

 

Diagnosis of Epilepsy

  • Clinical history is key
    • Experience at onset
    • Report of observers
    • Postictal experience
  • Electroencephalogram
    • Generalized syndrome: commonly abnormal
    • Partial seizures: rarely abnormal on first recording
  • Imaging study: MRI

 

 

 

Classification of Seizures
Generalized Seizures

  • Absence seizures
  • Tonic-clonic seizures
  • Myoclonic seizures
  • Tonic seizures
  • Clonic seizures
  • Atonic seizures

 

 

 

Classification of Seizures:
Absence (Petit Mal)

  • Brief alterations of awareness
  • Activity arrest, starring, automatisms
  • Pick-up where they left-off
  • Average age 8-10years
  • Classic EEG Pattern of 3Hz/sec
  • Females slightly more than males
  • Hyperventilation provokes onset

 

 

 

Classification of Seizures:
Tonic-Clonic

  • Any age onset and M=F
  • Stiff extension of body/extremities followed by violent, rhythmic, symmetric movements
  • Alteration of consciousness followed by confusion and sleepiness
  • Changes in respiration, bladder and bowel

 

 

 

Classification of Seizures:
Myoclonic

  • Brief, random, lightning-like movements
  • May occur singly or in clusters
  • Seconds in duration, no post-ictal changes
  • Various age onset
  • Can be resistant to therapy

 

 

 

Classification of Seizures:
Tonic

  • Sustained flexion and/or contraction of major muscle groups
  • Component of Lennox-Gastaut syndrome
  • Often associated with respiratory arrest
  • May be associated with coarse tremor

 

 

 

Classification of Seizures:
Clonic

  • Symmetric, rhythmic movements of extremities as in Tonic/Clonic seizures
  • Often occur out of sleep

 

 

 

Classification of Seizures:
Atonic

  • Abrupt loss of tone
  • Frequently associated with trauma
  • Positive alteration consciousness with variable post-ictal changes

 

 

 

Classification of Seizures

  • Partial seizures
  • Simple partial seizures
  • Complex partial seizures
    • Impaired consciousness at outset
    • Simple partial evolving to lost consciousnes
  • Partial seizures evolving to general tonic-clonic seizures (GTCS)

 

 

 

Classification of Seizures:
Simple Partial

  • Often called Benign Rolandic
  • Hallmark is preservation of consciousness
  • Classic pattern on EEG
  • M=F, average age onset 7-8
  • +/- therapy necessary
  • Often “outgrown”
  • May not be “so benign”

 

 

 

Classification of Seizures:
Complex Partial

  • Change in consciousness
  • Most common epilepsy in children
  • Automatisms, deja vue, auras or alice in wonderland changes seen
  • Focal motor movements of any limb
  • Variable duration and severity
  • Any age onset

 

 

 

Monotherapy AED

 

 

Classic Versus Newer Anticonvulsants

Classic AEDs

  • Phenobarbital
  • Phenytoin (Dilantin®)
  • Primidone (Mysoline®)
  • Carbamazepine (Tegretol®)
  • Valproate      (Depakote®/Depacon®)
  • Ethosuximide (Zarontin®)

Newer AEDs

  • Felbamate (Felbatol®)
  • Gabapentin (Neurontin®)
  • Lamotrigine (Lamictal®)
  • Levetiracetam (Keppra®)
  • Oxcarbazepine (Trileptal®)
  • Tiagabine (Gabitril®)
  • Topiramate (Topamax®)
  • Vigabitrin (Sabril®)
  • Zonisamide (Zonegran®)

 

 

 

Mechanisms of Action
Antiepileptic Drugs

  • Sodium channel effects
  • Potassium channels and GABA release
  • GABAergic effects
    • Precursors, mimicry, and transporters
  • Glutamate regulation-neuroprotection
  • Calcium channels and transmitter release

 

 

 

Anticonvulsants:
Mechanisms of Action

 

 

 

Choice and Use of Drugs

 

 

 

Pharmacoresistant Epilepsy

Previously Untreated Epilepsy Patients (n=470)1

 

 

 

Patient Typically Not Considered
Surgical Epilepsy Candidate Unless:

  • They have failed at least 3 adequate anti-convulsant trials
    • Definition of failure varies
  • EEG confirming epilepsy
  • Recent MRI
  • Family/patient willing to consider surgery

 

 

 

Etiology

  • Focal
    • Lesion
    • Anatomically Normal
    • Temporal
    • Extra-Temporal
  • Multi-Focal/Generalized

 

 

 

Epilepsy Associated with Cortical Malformation

  • 20% of epilepsy patients
  • Mental retardation
  • Autism
  • Neuropsychiatric syndromes

 

 

 

Cell Migration Disorders

  • Heterotopias - A failure of neuronal migration from the periventricular matrix to the cortex; asymptomatic or mental retardation or seizure
  • Agyria - No gyri (lissencephaly) or a few malformed gyri (pachygyria)
  • Polymicrogyria - Excessive number of gyri are formed with shallow sulci; sporadic (congenital ischemia/hypoxia, infection) or familial; asymptomatic, mental retardation or seizure
  • Focal cortical dysplasia

 

 

 

HEMIMEGALENCEPHALY

 

 

 

Cell Migration Disorders

  • Heterotopias - A failure of neuronal migration from the periventricular matrix to the cortex; asymptomatic or mental retardation or seizure
  • Agyria - No gyri (lissencephaly) or a few malformed gyri (pachygyria)
  • Polymicrogyria - Excessive number of gyri are formed with shallow sulci; sporadic (congenital ischemia/hypoxia, infection) or familial; asymptomatic, mental retardation or seizure
  • Focal cortical dysplasia

 

 

 

Lissencephaly

 

 

 

Lissencephaly

 

 

 

Cell Migration Disorders

  • Heterotopias - A failure of neuronal migration from the periventricular matrix to the cortex; asymptomatic or mental retardation or seizure
  • Agyria - No gyri (lissencephaly) or a few malformed gyri (pachygyria)
  • Polymicrogyria - Excessive number of gyri are formed with shallow sulci; sporadic (congenital ischemia/hypoxia, infection) or familial; asymptomatic, mental retardation or seizure
  • Focal cortical dysplasia

 

 

 

Polymicrogyria - Diffuse

 

 

 

Polymicrogyria - Localized

 

 

 

Polymicrogyria - Localized

 

 

 

Cell Migration Disorders

  • Heterotopias - A failure of neuronal migration from the periventricular matrix to the cortex; asymptomatic or mental retardation or seizure
  • Agyria - No gyri (lissencephaly) or a few malformed gyri (pachygyria)
  • Polymicrogyria - Excessive number of gyri are formed with shallow sulci; sporadic (congenital ischemia/hypoxia, infection) or familial; asymptomatic, mental retardation or seizure
  • Focal cortical dysplasia

 

 

 

Tuberous
Sclerosis -Tubers

 

 

 

Surgical Management
of Epileptiform Lesions

TAKE THEM OUT!!!

Consider monitoring, either with surface EEG or with implanted grids.  However, once lesion is confirmed to be source of epileptiform discharges, TAKE THEM OUT!!!

 

 

 

Corticectomy

  • Work-up includes: Stage I
    • Surface EEG-Video Monitoring
    • Head MRI with and without contrast
    • Neuropsychological testing
  • Stage II
    • Invasive EEG
      • Grids, strips, and depth electrodes
    • Functional Imaging
      • Wada, PET, SPECT, fMRI

 

 

 

Success

  • Temporal lobe epilepsy            
    • 90% were seizure free
    • 98% had a >90% reduction in seizures1
  • Extra-temporal lobe epilepsy
    • 70% were seizure-free2

1 Cukiert et al: Neurosurg Focus. 2002 Oct 15;13(4):ecp2
2 Kazemi et al: Epilepsia. 1997 Jun;38(6):670-7.

 

 

 

Multi-focal Epilepsy

  • Corpus Callosotomy    
    • More commonly used in patients with Lennox-Gastaut or drop attacks
    • 30% of patients were seizure-free1
  • Vagus Nerve Stimulation
    • 88% of patients had a >50% reduction in seizures
    • 20-30% of patients became seizure-free
    • Average reduction in anti-convulsant dose was 1/3
    • Utilization increasing, with 7,000 VNS implanted/year for epilepsy

1 Phillips et al: Br J Neurosurg. 1996 Aug;10(4):351-6.

 

 

 

VNS Therapy

  • Implantable pulse generator and lead
  • Mild electrical pulses applied to the left vagus nerve in the neck send signals to the brain
  • Automatic intermittent stimulation
  • Magnet use allows patient/caregiver
    • On-demand stimulation
    • On-demand side effect control
  • Simple in-office programming
  • Assured compliance

 

 

 

Vagus Nerve Stimulation Implantation

 

 

 

VNS Surgical Technique

  • Creation of left chest sub-cutaneous pocket
  • Cut-down to left vagus nerve
  • Attachment of lead to nerve
  • Tunneling on lead
  • Lead test
  • Programming
  • Closure

 

 

 

VNS System Implant:
The Exposed Carotid Sheath

 

 

 

Final Electrode/Anchor Tether Placement

Use of the integrated anchor tether helps prevent force transfer to the electrodes.

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Pulse Generator:
Programmable Parameters