Multidisciplinary Presurgical Evaluation for Epilepsy Surgery


Introduction

When we decide that surgery is an option for treating epilepsy, there are a number of considerations we have to make. First and most important, the decision of operating on a patient with seizures does not reside in one person, it is the result of a multidisciplinary team: epileptologist, neurophysiologist, neuropsychologist, neuroradiologist, and neurosurgeons play an important role in selecting, studying, and, above all, tailoring the best surgery for the patient.

Despite the advances in medical treatment, 30% of epileptic patients do not respond to adequate antiepileptic treatment. We consider that a patient has medically uncontrollable (or refractory) epilepsy when satisfactory seizure control cannot be achieved with any of the potentially available effective antiepileptic drugs (AEDs), alone or in combination, at doses or levels not associated with unacceptable side effects. On the other hand, surgery has a success rate of about 75%, depending on the site of surgery, the type of surgery performed, and the underlying etiology. In spite of this, surgical treatment of medically uncontrollable epilepsy is often delayed or withheld. Referral for epilepsy surgery takes an average of 20 to 25 years, resulting in a number of avoidable seizure-related deaths, including drowning, motor vehicle accident, fatal status epilepticus, and sudden unexpected death in epilepsy. In children, appropriate timing for surgical procedures is critical, as seizure control may interfere with consolidation of cognitive and motor functions in a developing brain. On the other hand, brain maturation may decrease epileptogenesis, and a nonprogressive brain lesion like a scar or dysplasia may be associated to transient epileptogenesis.

Other than lack of efficacy, causes of poor response to medical treatment are noncompliance, pseudo-epileptic seizures or a combination of epileptic and pseudo-epileptic seizures, incorrect classification of seizures, and incorrect pharmacological treatment (either drug or dosage). So, the first action in evaluating surgical candidates is to confirm that a proper diagnosis and an appropriate medical treatment have been offered.

A patient’s satisfaction with the results achieved with medical treatment often depends on their professional and social circumstances, as well as the type of seizures and the time of day in which they occur. Some patients seek surgical treatment for reasons other than medical intractability; intolerable side effects of AEDs, avoidance of congenital malformations in women who desire to get pregnant, and avoidance of the social stigmata of the disease are some of the reasons patients have expressed when coming to our clinic. Self-assessment of quality of life is an important step in evaluating the patient’s perception of their disease. At our clinic we use the questionnaires QOLIE-31 for adults , and QOLIE-48-AD for adolescents. ,

When epilepsy surgery is planned, we have three goals:

  • 1.

    Eliminate or decrease epileptic seizures,

  • 2.

    Prevent neurologic deficit due to surgery,

  • 3.

    Improve the quality of life.

In order to be able to reach the best surgical choice for the epileptic patient, a multidisciplinary team has to work to solve the surgical questions: Where do the patient’s seizures start? Are there functional or eloquent areas involved? What is the prognosis after surgery? Answering these questions will permit us to know what type of surgery is indicated and to be able to customize the procedure according to our patient’s needs. Nowadays, we have a great number of studies that can be performed, but it is important to remember that there is not a single one that can be considered the golden standard for diagnosis by itself. It is the clinician’s ability to interpret and find the concordance between studies that will lead us to a correct diagnosis and a successful surgery.

The diagnostic workup can be divided into noninvasive and invasive phases:

Phase I Testing: Noninvasive Studies

Clinical Diagnosis: Seizure Description

Probably the most important part of the noninvasive studies is the seizure description. We must try to compile data that will lead us to the most probable diagnosis. Special attention should be paid to what both family and patient have to report.

Aura: specific auras have been described in different types of seizures. For example, the manifestations of fear and ascending epigastric sensation direct us toward mesial temporal epilepsy; simple visual auras toward occipital foci, levitation to somatosensory areas; insular foci manifest as sympathetic symptoms such as perspiration, difficulty in swallowing, and salivation. The sequence of symptoms that the patient has is also important; epigastric and psychic auras followed by behavioral arrest and automatisms (ocular, oral, hands, ambulatory) indicate a mesial temporal onset of seizures. A different sequence or visual, auditory, and somatic auras at the beginning orient us to extratemporal foci with propagation to the mesial temporal lobe. Dystonic positions of hands as an initial symptom can orient us to the contralateral frontal area, while if present after behavioral arrest and automatisms, they lose their localizing precision. If a tonic-clonic seizure occurs after auras or the symptoms previously mentioned, they are probably the result of propagation of a partial seizure, but if it is not preceded at all by any symptom, it is reasonable to presume that they are primary generalized seizures.

Postictal symptoms have to be taken into consideration; for example, if there is amnesia and sleepiness during several hours, we suspect mesial temporal lobe seizures. If, on the contrary, there is an immediate recovery, frontal origin is more probable.

Interictal behavioral changes must also be explored, since conduct abnormalities such as perseverance and aggressiveness (frontal lobe symptoms), depression, anxiety, and memory problems (mesial temporal lobe symptoms) can orient us to a specific area. Together with the neuropsychology team we can design the appropriate testing for each patient.

Surface Electroencephalogram

The electroencephalogram (EEG) is considered the most important test for diagnosis since its initial description. Indeed, when abnormal, EEG provides valuable information.

Interictal data: Focal discharges consisting of spikes and acute waves, usually localized to the epileptic area are suggestive of focus location; if a focal discharge occurs at or near one of the electrodes common to two channels, we will observe a phase reversal, pointing to the focus localization. If a discharge occurs midway between two electrodes, each will be equipotential and the channel connected between them will not show an abnormality.

Some patients have bilateral secondary synchrony. This occurs when seizure activity starts in a cortical or mesial focus and spreads by the way of the thalamic reticular formation. A careful analysis will show that activity starts in a specific area and then spreads to other areas.

Ictal data: Ictal recordings usually show acute and rhythmic theta activity, which starts in the affected region and afterward propagates to other areas. The postictal activity can be slow and unilateral indicating the affected hemisphere.

A normal EEG does not mean that a patient does not have epilepsy; it is also true that the same patient might have different data in several EEG recordings taken on different days. One patient can have a normal EEG one day, abnormal right data another day, and contralateral data in another recording. This is part of the challenge of the epileptologist, to find concordance.

Ictal recordings usually show acute and rhythmic theta activity, which starts in the epileptic zone (EZ, area where seizures are initiated) and afterward propagates to other areas. The postictal activity can be slow and unilateral indicating the affected hemisphere.

Continuous Video/Electroencephalogram Monitoring

This technique consists in recording simultaneously the EEG as well as the behavior of the patient for a period that can last up to several days. This method allows for the confirmation of the clinical type of seizures and their correlation with the onset of abnormal EEG activity, particularly ictal activity, and detection of those cases with pseudo-epileptic seizures.

Both EEG and video/EEG recordings have their limitations. In spite of the length of the registration period, sometimes seizures do not occur, or multiple movement artifacts do not permit proper visualization of the abnormalities. The spatial resolution is not specific enough to permit the precise localization of the EZ and the clinical seizure type and EEG recordings do not always coincide so that sometimes intracranial recording becomes necessary.

Neuropsychological Assessment in Surgical Candidates

The overall objective of neuropsychological assessment in candidates to surgery for epilepsy is to seek evidence of cognitive deficits and their relationship with other studies (clinical and electrophysiological). ,

Neuropsychological testing answers specific questions that other specialists of the epilepsy clinic have made and whose response has a significant impact on surgical decision. (1) What is the patient’s overall neuropsychological status? (2) Which is the hemispheric dominance for language? (3) If cognitive deficit exists, is it lateralized? (4) Is there concordance between neuropsychological findings and structural and electrophysiological results? (5) Should the patient have any sequelae from surgery, can we predict the degree of recovery?

There are specific tests for each of the above questions. We will focus on questions two and three of the above, with particular attention to those adult patients with temporal lobe epilepsy (TLE).

Hemispheric Dominance for Language

Determining the lateralization of language can have a significant impact in surgical planning. It has been established , that 95% to 99% of right-handed subjects and 15% to 19% of left-handed have a left cerebral hemispheric dominance for language. Due to the small percentage of subjects who may have a right hemisphere dominance, it is very important to clarify each case. ,

This statement brings up another important fact: a right-handed patient who presents with difficulties of language might be mistakenly classified as having a left hemisphere affection when in fact he could have dysphasia of the dominant hemisphere, which is not necessarily the left.

For decades, the Wada test has been the gold standard for determining hemispheric language dominance (HLL). , , , , , , Recently, a growing number of publications report that other techniques such as functional magnetic resonance imaging (fMRI) may have the same degree of reliability , , , without the disadvantages of cost, risk, and complexity of other techniques. , found a 91.3% concordance between the Wada test and fMRI. Other authors have also found correlation between these two methods, even in cases with bilateral representation of language. New noninvasive techniques are evolving to substitute Wada test, however, they still present limitations.

To determine the HLL using fMRI, most authors use the task of evoking words silently , based on a semantic category (e.g., names of animals) to activate temporal regions, or a phonological category (words beginning with same letter) for activating frontal regions of the dominant hemisphere. , Fig. 99.1 illustrates a fMRI study using a phonological category.

FIGURE 99.1, Functional magnetic resonance imaging study showing left cerebral hemispheric dominance for language in a healthy subject using a phonological category (see text).

Dichotic listening technique (DLT) has also been proved useful in determining the HLL. , , , It consists of simultaneous presentation of two words (one in each ear) with the same characteristics in terms of sound, number of syllables, and common use, with the intention to present some competition for processing stimuli between the two hemispheres. With this methodology it has been shown that the majority of right- and left-handed subjects have right ear advantages as a reflection of a left HLL. Fig. 99.2 shows the variability in the index of HLL according to the dichotic listening test in right-handed healthy subjects. The lateralization index language (LI) is a predictor of postoperative verbal memory deficit as shown by some studies.

FIGURE 99.2, Variability in language laterality in right-handed healthy subjects using the dichotic-listening test. The laterality index (LI) is obtained as follows (36): (L − R)/(L + R) = LI, where L = number of words heard with the left ear, R = number of words heard with the right ear. A positive LI corresponds to a left-predominant lateralization and a negative LI corresponds to a right-predominant lateralization, as follows: SLL = strong left lateralization (+0.50 to +1). WLL = weak left lateralization (+0.25 to +0.50). B = bilateral representation (+0.25 to −0.25). WRL = weak right lateralization (−0.25 to −0.50). SRL = strong right lateralization (−0.50 to −1).

Lateralization of Memory Deficits

Lateralization of memory deficits (LMDs) is another objective of preoperative neuropsychological assessment. Table 99.1 presents some of the available memory tests. At our Clinic we use the Battery of Learning and Memory and the Visuospatial Learning and Memory tests. When the LMD is found in the same hemisphere where the EZ has been localized through clinical, EEG, and imaging data, the decision for surgery is strengthened, otherwise the complete testing described in Table 99.1 is applied. , ,

Table 99.1
Most Frequently Used Tests in Epilepsy
General Batteries Problem Solving, Flexibility
Wechsler Intelligence Scale Wisconsin Card Sorting Test
Halstead-Reitan Battery Word Fluency
Hemispheric lateralization Stroop Test
Wada Test Battery of Learning and Memory
Dichotic Listening Wechsler Memory Scale
Functional Magnetic Verbal Learning and Memory
Resonance Story Recall
Imaging Paired Word Learning
Attention Rey Auditory Verbal Learning Test
Trail Making Test California Verbal Learning Test
Cancelation Test Visuospatial Learning and Memory
Language Simple Designs Recall
Boston Diagnostic Aphasia Rey-Osterrieth Complex Figure
Examination Benton Visual Retention Test
Boston Naming Test Others
Token Test Beck Depression Inventory
Visuospatial and Perceptual Quality of Life in Epilepsia (QOLIE-31)
Hooper Visual Organization Test International Neuropsychiatric Interview (MINI)
Constructional Apraxia
Benton Judgment of Line
Orientation
Motor and Reaction Time
Finger Oscillation
Hand Dynamometer

Evaluation of memory is performed by well-established tests ( Fig. 99.3 ) that may vary slightly from one center to another. In approximately 50% of patients it is not possible to determine the LMD through neuropsychological studies alone, but that percentage decreases information provided by neuropsychological testing is concordance with other studies.

FIGURE 99.3, (A and B) Axial and coronal images, showing migration and sulcation disorders, with gray matter nodular heterotopy. Notice the abnormal localization of gray matter in deep white matter lateral to right ventricle with ependymal extension (arrows) .

A major problem in LDM is that there is a high error rate due to language dominance, as discussed above: that is, verbal memory deficit does not always indicate affection of the left cerebral hemisphere.

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