Corpus Callosotomy: Indications and Techniques


In 1940, William P. van Wagenen and R. Yorke Herren described a small series of patients with various disruptive lesions of the corpus callosum. Their report highlighted the phenomenologic distinction between generalized convulsive seizures in individuals with tumors near the intact callosum and only partial seizures following insidious invasion of the corpus callosum months to years later. The hypothesis that interruption of the corpus callosum could alter the bihemispheric spread of seizure activity in epileptic individuals led to the simple yet elegant conclusion that surgical division of the corpus callosum might confine epileptogenic foci to a single hemisphere and thus ameliorate secondary seizure generalization. At the time of their writing, the practice of excising regions of brain tissue to eliminate seizures had been firmly established, although with varying rates of success. Nevertheless, the historical significance of van Wagenen and Herren’s careful observations cannot be overstated. They set the stage for the first corpus callosotomy lesions performed with the intent of stopping seizure propagation to the contralateral hemisphere in individuals with intractable epilepsy, marking a critical advancement in the field of modern epilepsy surgery.

In the 10 cases of corpus callosotomy that constituted van Wagenen and Herren’s landmark report, there was significant reduction in seizure frequency, with few permanent adverse effects. Curiously, however, the practice of corpus callosotomy for treatment of epilepsy was rarely used until more than 20 years later, when the next case reports of corpus callosotomy in humans appeared and the procedure began to gain acceptance within the neurosurgical community. The procedure itself has undergone various technical modifications, including total versus partial callosotomy, anterior versus posterior approaches, single versus multistaged operations and even radiosurgical callosotomy. , Furthermore, 21st-century technology for less invasive neurosurgical approaches, such as vagal nerve stimulation, and the advent of novel antiepileptics have led to reconsideration of the role of surgical corpus callosotomy for patients having intractable seizures without a focally resectable lesion. However, the safety and efficacy of this procedure have been substantiated in more than 60 years of empiric and retrospective studies in humans and experimental studies in animals. , , , Van Wagenen and Herren’s seminal work directly contributed to the first theoretical interpretation of “split-brain” syndromes by Andrew J. Akelaitis and eventually culminated in the awarding of a Nobel Prize to the neuropsychologist Roger Sperry for his innovative tachistoscopic experiments on disconnected patients. Sperry’s insights subsequently led to more detailed anatomic and physiologic studies of callosal function, which have helped shape our contemporary understanding of the functional lateralization of the cerebral hemispheres.

Modern Indications

Corpus callosotomy was developed specifically for treatment of generalized seizures, which can include numerous abnormal movement phases (tonic, clonic, and atonic). Of these variations, drop attacks (both tonic and atonic) remain the primary indication for corpus callosotomy, as repeated studies have shown superior efficacy for this seizure type. , Nevertheless, all forms of generalized seizures, including tonic, clonic, tonic-clonic, myoclonic, and absence seizures, have been reported to respond favorably to corpus callosotomy. In some studies, corpus callosotomy has been reported to reduce partial seizures as well, although at significantly lower rates. In addition, callosotomy has been reported to reduce seizures in several childhood epilepsy disorders including Rasmussen encephalitis , and Lennox-Gastaut syndrome, , as well as infantile hemiplegia and frontal lobe epilepsy.

In most modern epilepsy surgery centers, corpus callosotomy is not considered a primary surgical treatment in patients who might benefit from focal resection of epileptogenic regions. Controversy remains regarding whether forms of mental retardation should preclude patients from callosotomy, , although intelligence quotient (IQ) has not been found to significantly predict outcome following callosotomy. Patients with crossed dominance have been reported to have less favorable outcomes, presumably because these patients require cortical interconnections for routine function to a more significant extent than those without crossed dominance.

Preoperative Evaluation

The preoperative assessment of patients considered for corpus callosotomy is comprehensive and should adhere to three general principles. First, seizure intractability with multiple anticonvulsants used in combination should be established. Second, a resectable epileptogenic focus should be excluded because alternative procedures such as lesionectomy or lobectomy have higher seizure control rates. Third, it should be communicated to patients that callosotomy is considered a palliative procedure, and patient expectations and goals for the surgery should be carefully explored. Other specific components of the workup should include a complete physical examination, battery of neuropsychological testing, magnetic resonance imaging of the brain to evaluate structural lesions and corpus callosal anatomy, and electroencephalography with video monitoring of ictal and interictal states. ,

Surgical Approaches

Rationale

The topographic organization of the corpus callosum follows a bilaterally connected arrangement in the anterior-posterior plane. The anterior portion of the corpus callosum carries fibers connecting frontal cortical regions, including anterior cingulate, premotor, motor, and anterior insular areas. Secondary generalization is therefore dependent on anterior motor callosal fibers during tonic-clonic and atonic seizures. Posterior fibers transmit somatosensory and visual information from the parietal and occipital lobes, as well as information from association cortices. Hence severing these posterior connections can produce a perceptual disconnection syndrome, which is the rationale for preserving the splenium. Nevertheless, posterior callosotomy may still be reserved for seizures refractory to anterior section alone. Specific patients, such as those with severe, bilateral seizures, may even benefit from initial complete callosotomy, although this has not been proven. It should be pointed out that the corpus callosum is one of several midline commissures. Others include the anterior commissure, the dorsal hippocampal commissure, and the massa intermedia of the thalamus. The callosum is the primary pathway through which seizures become secondarily generalized. However, seizure propagation may also be transmitted through these alternative pathways. Presumably, in such cases, these alternate pathways may be the basis for residual seizure activity following callosotomy.

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