Abstract
Tuberous sclerosis complex is a genetic systematic disorder characterized by hamartomas in multiple organs. Cortical tubers, the hamartomas in the cerebrum, cause multifocal refractory seizures. In certain cases, epileptic foci potentially involve language areas, and hence, extra- and intraoperative cortical mapping can help identify anterior and posterior areas, thus avoiding postsurgical language impairment. We report on a 21-year-old female with tuberous sclerosis complex experiencing refractory partial seizures due to two epileptic foci in the left hemisphere overlapping anterior and posterior language areas. To completely evaluate both language areas, we performed stepwise resections beginning from the anterior to the posterior epileptic focus. Although the patient presented with expressive aphasia following anterior resection, it was possible to conduct language tests during every resection. Postoperatively, she presented with expressive aphasia, comprehension deficits, left-right disorientations, and arithmetic deficits. The language dysfunctions almost disappeared at 5 weeks after the surgery and were completely resolved at 6 months after surgery. At postoperative 9 months, she was free from seizures.
Highlights
PatientClinical Course A 21-year-old Japanese female, who was right handed and a university student of English literature, presented with multiple hypomelanotic macules since birth
Observations of multiple cortical tubers on magnetic resonance imaging (MRI), facial angiofibroma, and hypomelanotic macules led to a diagnosis of tuberous sclerosis complex (TSC)
Anterior language area was considered to be associated with expressive language function and distribute across Brodmann’s areas 44 and 45, while posterior language area was considered to be associated with comprehension and understanding language, and correspond to Brodmann’s areas 22 and 39 [6,7,8]
Summary
Clinical Course A 21-year-old Japanese female, who was right handed and a university student of English literature, presented with multiple hypomelanotic macules since birth. We identified anterior language area at five electrodes on the middle and inferior frontal gyrus that excluded pars triangularis and pars opercularis, and posterior language area at three electrodes in the inferior parietal lobule including the supramarginal gyrus and angular gyrus She represented retardations or disruptions at picture naming and responsive naming and mistakes at token test in both language areas, more severe disturbances and paraphasia occurred during the stimulations to posterior language area (Figure 2, red and white shaded areas). We avoided resection of the posterior language area, which was confirmed via extraoperative mapping After the surgery, she presented expressive aphasia, inclu ding phonemic and verbal paraphasia, disturbed recalling and repetition, neology, agrammatism, prolonged speech, and dysgraphia. The SLTA scores recovered completely (100%) at 6 months after the surgery and postoperatively at the 9-month follow-up, she was free from seizures
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