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Porphyrias are rare genetic disorders which cause a deficiency in the enzymes involved in the biosynthesis of heme. The treatment of epilepsy in patients with acute intermittent porphyria can be difficult since many anticonvulsants can increase heme synthesis and trigger porphyric attacks. We report a patient with focal epilepsy successfully treated with eslicarbazepine without exacerbation of porphyria.

Summary: In this review, the semiology, and characteristics of noninvasive investigations suggestive of anterior cingulate and anterior midcingulate epilepsy are detailed by the authors. The clinical presentation is representative of a recently recognized rostrocaudal gradient of functional connectivity with seizures of the anterior cingulate cortex manifesting emotional and interoceptive aura followed by a hyperkinetic or complex motor seizures. The few reports of anterior midcingulate epilepsy show a trend toward a higher proportion of sensory auras and premotor semiology. Ictal pouting, vocalizations, and, in particular, laughter are strong indicators of epilepsy arising or spreading to this region. Although scalp EEG was traditionally thought to provide little information, the data provided in this review demonstrate that most patients will have abnormalities over the frontal or frontotemporal regions. Frontotemporal abnormalities at least interictally provide valuable information regarding lateralization. The etiology of epilepsy arising from the anterior cingulate region seems to be most frequently secondary to focal cortical dysplasia (FCD), followed by neoplasms and vascular lesions, particularly cavernomas, although one cannot rule out a publication bias. Findings of nuclear medicine imaging is seldomly reported but both positron emission tomography and ictal single-photon computed tomography can identify the generator or the network often showing abnormalities extending to the frontal regions. The few available magnetoencephalography (MEG) studies reveal mixed results, sometimes providing false lateralization of the focus. Anterior cingulate epilepsy is difficult to recognize, but the features summarized in this review should prompt suspicion in clinical practice.