[Cognitive Development in Children with Benign Rolandic Epilepsy of Childhood with Centrotemporal Spikes - Results of a Current Systematic Database Search]. / Kognitive Entwicklung von Kindern mit benigner Epilepsie des Kindesalters mit zentrotemporalen Spitzen (Rolando-Epilepsie) ­ Ergebnisse einer aktuellen systematischen Literaturrecherche.

Benign Rolandic Epilepsy (BRE) is one of the most common epilepsy syndromes in childhood. Although global intellectual performance is typically normal in BRE-patients, problems were found in specific cognitive domains. To summarize recent empirical findings concerning cognitive development in children with BRE a systematic literature search of clinical studies published between 2009 and 2015 was performed. 19 studies of relevance were found.In most recent studies children with BRE consistently showed general intellectual performance within the normal range. However, in two of the studies patients showed a significantly poorer (but still normal) performance in comparison to controls. The studies provide clear indications for a high prevalence of impairments in language (10 out of 12 studies) and academic performance (6 out of 8 studies) in children with BRE. Regarding deficits in other cognitive domains (attention, memory, visual/auditory perception, executive functions) current findings are inconsistent. In addition, no clear results are found in studies examining cognitive development after remission of BRE. Studies on the relationship between selected clinical/electroencephalographic characteristics (e. g. EEG-patterns, focus lateralization) and cognitive performance and studies on potential benefits of anti-epileptic therapy for cognitive functions also have not yielded consistent results. Studies using fMRI and evoked potentials provide evidence for functional reorganization of neural networks in BRE.Due to the developmental risks in children with BRE early cognitive assessment, early treatment and follow-up assessments are important.

Unexpected death of a 12 year old boy with monosomy 1p36.

Monosomy 1p36 may result in a clinically recognizable chromosomal microdeletion syndrome. We report the unexpected death of a 12 year old boy with mildly dysmorphic facial features, short stature at 138 cm (3rd centile), moderate mental retardation and a history of seizures, obesity, transient muscle weakness of the right arm and leg and episodes of transient atonic hemiparesis of the right side of the body. Despite the relatively few congenital anomalies and normal karyotype, the 1p36 deletion was suspected on clinical grounds and was demonstrated by fluorescent in situ hybridisation (FISH). Two months after diagnosis and following a short history of a mild upper airway infection, high fever and severe diarrhea, the patient had a massive circulatory shock and asystolia, resulting in deep coma, brain edema, apallic syndrome and death. To our knowledge there has been no previous report of episodes of transient unilateral muscle weakness and atonic hemiparesis, circulatory shock and sudden death associated with monosomy 1p36.

Transient lesion in the splenium of the corpus callosum: three further cases in epileptic patients and a pathophysiological hypothesis.

OBJECTIVE: Focal lesions limited to the splenium of the corpus callosum (SCC) are rare and little is known about their aetiology. Three patients were examined for presurgical evaluation in epilepsy with a transient lesion in the SCC and a pathophysiological hypothesis is presented. METHODS: Three patients were identified with a circumscribed lesion in the centre of the corpus callosum. Follow up MRI was performed, the medical records examined retrospectively, and the literature reviewed. RESULTS: The patients showed identical lesions in the SCC with reduced T1 and increased T2 signal intensity and an unaffected marginal hemline of a few mm. Patients were asymptomatic and control MRIs showed complete normalisation within 2 months. Patients had been treated with antiepileptic drugs (AEDs) without signs of toxicity. In all patients AEDs were rapidly reduced for diagnostic purposes, but only one had psychomotor seizures, 5 days before imaging. CONCLUSIONS: A transient lesion in the SCC has so far only been described in 13 patients with epilepsy and has been interpreted either as reversible demyelination due to AED toxicity or transient oedema after secondary generalised seizures. The data confirm neither of these hypotheses. A transient lesion in the SCC seems to be a non-specific end point of different disease processes leading to a vasogenic oedema. This suggests, in these patients, a multifactorial pathology triggered by transient effects of AEDs on arginine vasopressin and its function in fluid balance systems in a condition of vitamin deficiency. The complete and rapid reversibility in all cases without specific intervention is emphasised and any invasive diagnostic or therapeutic approach is discouraged.