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Different surgical approaches have been described for selective amygdalohippocampectomy in patients with pharmacoresistant temporal lobe epilepsy. The aim of this study was to report the results of the innovative anterior trans-superior temporal gyrus approach in a single-center series. Patients' characteristics, postoperative outcomes, and complications were reviewed in a series of 8 consecutive patients with temporal lobe epilepsy operated on using the anterior trans-superior temporal gyrus approach between November 2015 and April 2017. Over a mean 2.5-year follow-up, 7 of 8 patients (87.5%) remained seizure-free (Engel class I). Only 1 patient (12.5%) was not cured (Engel class III) with no clear explanation for treatment failure. Mean operative time was 237 minutes, which was 80 minutes shorter compared with the classic transsylvian approach. No perioperative deaths were recorded and there were no visual field defects or visual acuity impairments secondary to the approach. One patient experienced a left posterior thalamocapsular stroke. The anterior trans-superior temporal gyrus approach is feasible, fast, and safe for selective amygdalohippocampectomy in patients with drug-refractory temporal lobe epilepsy. This approach allows preservation of the optic radiation but cuts part of the uncinate fasciculus and potentially the anterior aspect of the anterior bundle of the middle longitudinal fasciculus.

Liver disease, occurring during pediatric or adult age, is often of undetermined cause. Some cases are probably related to undiagnosed inherited metabolic disorders. Hepatic disorders associated with fructose-1,6-bisphosphatase deficiency, a gluconeogenesis defect, are not reported in the literature. These symptoms are mainly described during acute crises, and many reports do not mention them because hypoglycemia and hyperlactatemia are more frequently in the forefront. Herein, the liver manifestations of 18 patients affected with fructose-1,6-bisphosphatase deficiency are described and the corresponding literature is reviewed. Interestingly, all 18 patients had liver abnormalities either during follow-up (hepatomegaly [n = 8/18], elevation of transaminases [n = 6/15], bright liver [n = 7/11]) or during acute crises (hepatomegaly [n = 10/17], elevation of transaminases [n = 13/16], acute liver failure [n = 6/14], bright liver [n = 4/14]). Initial reports described cases of liver steatosis, when liver biopsy was necessary to confirm the diagnosis by an enzymatic study. There is no clear pathophysiological basis for this fatty liver disease but we postulate that endoplasmic reticulum stress and de novo lipogenesis activation could be key factors, as observed in FBP1 knockout mice. Liver steatosis may expose patients to severe long-term liver complications. As hypoglycemia becomes less frequent with age, most adult patients are no longer monitored by hepatologist. Signs of fructose-1,6-bisphosphatase deficiency may be subtle and can be missed in childhood. We suggest that fructose-1,6-bisphosphatase deficiency should be considered as an etiology of hepatic steatosis, and a liver monitoring protocol should be set up for these patients, during lifelong follow-up.

Although seizures in neonates are common and often due to acute brain injury, 10-15% are unprovoked from congenital brain malformations. A better understanding of the risk of neonatal-onset epilepsy by the type of brain malformation is essential for counseling and monitoring. In this retrospective cohort study, we evaluated 132 neonates with congenital brain malformations and their risk of neonatal-onset epilepsy. Malformations were classified into one of five categories based on imaging patterns on prenatal or postnatal imaging. Infants were monitored with continuous video EEG (cEEG) for encephalopathy and paroxysmal events in addition to abnormal neuroimaging. Seventy-four of 132 (56%) neonates underwent EEG monitoring, and 18 of 132 (14%) were diagnosed with neonatal-onset epilepsy. The highest prevalence of epilepsy was in neonates with disorders of neuronal migration/organization (9/34, 26%; 95% confidence interval [CI] = 13-44%), followed by disorders of early prosencephalic development (6/38, 16%; 95% CI = 6-31%), complex total brain malformations (2/16, 13%; 95% CI = 2-38%), and disorders of midbrain/hindbrain malformations (1/30, 3%; 95% CI = 0-17%). Of neonates with epilepsy, 5 of 18 (28%) had only electrographic seizures, 13 of 18 (72%) required treatment with two or more antiseizure medicines (ASMs), and 7 of 18 (39%) died within the neonatal period. Our results demonstrate that disorders of neuronal migration/organization represent the highest-risk group for early-onset epilepsy. Seizures are frequently electrographic only, require treatment with multiple ASMs, and portend a high mortality rate. These results support American Clinical Neurophysiology Society recommendations for EEG monitoring during the neonatal period for infants with congenital brain malformations.

The role of neuroinflammation in epileptogenesis is extensively investigated, but short-term effects of seizures on established CNS pathologies are less studied and less predictable. We describe the case of a woman with previous recurrent episodes of focal cerebral haemorrhage of unknown cause who developed a pseudo-tumoural oedema triggered by provoked focal status epilepticus. A brain biopsy revealed that the underlying condition was primary angiitis of the CNS. Ictal-induced blood-brain barrier dysfunction allows the entry of water and inflammatory molecules that, in the context of CNS inflammatory diseases, may trigger a self-reinforcing process. Caution should be observed when tapering antiepileptic drugs in patients with such conditions.

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Visuospatial impairments have long been reported in Severe Alcohol Use Disorder but remain poorly understood, notably regarding the involvement of magnocellular (MC) and parvocellular (PC) pathways. This empirical gap hampers the understanding of the implications of these visual changes, especially since the MC and PC pathways are thought to sustain central bottom-up and top-down processes during cognitive processing. They thus influ- ence our ability to efficiently monitor our environment and make the most effective decisions. To overcome this limitation, we measured PC-inferred spatial and MC-inferred temporal resolution in 35 individuals with SAUD and 30 healthy controls. We used Landolt circles displaying small apertures outside the sensitivity range of MC cells or flickering at a temporal frequency exceeding PC sensitivity. We found evidence of preserved PC spatial resolution combined with impaired MC temporal resolution in SAUD. We also measured how spatial and tem- poral sensitivity is influenced by the prior presentation of fearful faces – as emotional content could favor MC processing over PC one – but found no evidence of emotional modulation in either group. This spatio-temporal dissociation implies that individuals with SAUD may process visual details efficiently but perceive rapidly updating visual information at a slower pace. This deficit has implications for the tracking of rapidly changing stimuli in experimental tasks, but also for the decoding of crucial everyday visual incentives such as faces, whose micro-expressions vary continuously. Future studies should further specify the visual profile of individuals with SAUD to incorporate disparate findings within a theoretically grounded model of vision.

Visuo-perceptive deficits in severe alcohol use disorder (SAUD) remain little understood, notably regarding the respective involvement of the two main human visual streams, i.e., magnocellular (MC) and parvocellular (PC) pathways, in these deficits. Besides, in healthy populations, low-level visual perception can adapt depending on the nature of visual cues, among which emotional features, but this MC and PC pathway adaptation to emotional content is unexplored in SAUD. To assess MC and PC functioning as well as their emotional modulations in SAUD. We used sensitivity indices (d') and repeated-measures analyses of variance to compare orientation judgments of Gabor patches sampled at various MC- and PC-related spatial frequencies in 35 individuals with SAUD and 38 matched healthy controls. We then explored how emotional content modulated performances by introducing neutral or fearful face cues immediately before the Gabor patches and added the type of cue in the analyses. SAUD patients showed a general reduction in sensitivity across all spatial frequencies, indicating impoverished processing of both coarse and fine-scale visual content. However, we observed selective impairments depending on facial cues: individuals with SAUD processed intermediate spatial frequencies less efficiently than healthy controls following neutral faces, whereas group differences emerged for the highest spatial frequencies following fearful faces. Altogether, SAUD was associated with mixed MC and PC deficits that may vary according to emotional content, in line with a flexible but suboptimal use of low-level visual content. Such subtle alterations could have implications for everyday life's complex visual judgments.