Ictal crying in epileptic seizures and psychogenic nonepileptic seizures: What are the hints to differentiate them?

OBJECTIVES: Ictal crying (IC) is a quite rare semiological manifestation of epileptic seizures (ESs) and it has been mostly reported in psychogenic nonepileptic seizures (PNESs). However, labeling IC as a pathognomonic sign of PNES can be harmful. We first aimed to investigate IC frequency in ES and PNES and highlight the differences of IC between ES and PNES. Secondly, we aimed to analyze etiology, detailed semiology, treatment options, and outcome of patients with IC in ES in more detail. METHODS: We retrospectively screened all video-EEG monitoring unit reports from Hacettepe University Hospitals' Epilepsy Center over a 20-year period (1996-2017) for the diagnosis of IC. We included the patients with IC who had at least one documented seizure. Patients who had IC with both facial expression and vocalization compatible with crying with or without weeping and subjective feeling of sadness, were included in the study. We classified patients with IC as ES and PNES. Demographic, historical, clinical, neuroimaging, electrophysiological parameters, video-EEG data, treatment options, and prognosis of all patients were recorded. Demographic, clinical, and video-EEG data were compared between ES and PNES. RESULTS: During the study period, 1983 patients were investigated. Six patients (all female) with ES and 37 patients (33 female) with PNES were identified. When we compared patients with PNES and ES with IC, the number of ASMs taken and duration of disease were significantly higher in patients with ES than PNES. Longer duration of seizure, longer duration of crying component, late onset of crying component in seizure, early responsiveness after seizure, not occurring during sleep, accompanied by eye closure and weeping, were found significantly higher in patients with PNES. Besides, if we analyze ES group in more detail, all had medical treatment refractory focal epilepsy and two of them whose IC was seen as an early semiological manifestation of their seizures had good outcome after nondominant anterior temporal lobectomy (ATL)+amygdalohippocampectomy (AH). However, three patients had various cortical lesions apart from temporal lobe on MRI and one patient had focal epilepsy with frontal lobe semiology with negative MRI. CONCLUSION: Although the most common etiology for IC is PNES and it is rarely seen in ES, it can be harmful to label ictal crying as a pathognomonic sign for PNES. We proposed that there are some semiological differences in terms of IC between PNES and ES. These differences may help to distinguish IC in PNES and ES in daily practice. Moreover, it can be speculated that nondominant temporal lobe involvement may be associated with IC in ES.

Gelastic seizures not associated with hypothalamic hamartoma: A long-term follow-up study.

OBJECTIVE: The objective of the study was to describe the electroclinical features, seizure semiology, and the long-term evolution of gelastic seizures (GS) not associated with hypothalamic hamartoma (HH). METHODS: We reviewed video-electroencephalogram (video-EEG) recordings from pediatric patients with GS without HH admitted to 14 Italian epilepsy centers from 1994 to 2013. We collected information about age at onset, seizures semiology, EEG and magnetic resonance imaging (MRI) findings, treatment, and clinical outcome in terms of seizure control after a long-term follow-up. RESULTS: A total of 30 pediatric patients were stratified into two groups according to neuroimaging findings: group 1 including 19 children (63.3%) with unremarkable neuroimaging and group 2 including 11 children with structural brain abnormalities (36.7%). At the follow-up, patients of group 1 showed better clinical outcome both in terms of seizure control and use of AED polytherapy. Our patients showed remarkable clinical heterogeneity, including seizure semiology and epilepsy severity. Electroencephalogram recordings showed abnormalities mainly in the frontal, temporal, and frontotemporal regions without relevant differences between the two groups. Overall, carbamazepine showed good efficacy to control GS. CONCLUSIONS: Patients with nonlesional GS have a more favorable outcome with better drug response, less need of polytherapy, and good long-term prognosis, both in terms of seizure control and EEG findings.

Crying with sorrow evoked by electrocortical stimulation.

Dacrystic seizures are rare and have been reported in patients with hypothalamic hamartoma as well as fronto-temporal epilepsy, involving the non-dominant hemisphere. We describe the first reported case of cortical stimulation of the left posterior orbito-frontal gyrus, generating consistent and reproducible crying with affective content in a 41-year-old woman with medically intractable left temporal lobe epilepsy, who underwent extraoperative intracranial video-EEG monitoring for resective non-lesional epilepsy surgery.

A familial syndrome of hypothalamic hamartomas, polydactyly, and SMO mutations: a clinical report of 2 cases.

Hypothalamic hamartomas are benign tumors known to cause gelastic or dacrystic seizures, precocious puberty, developmental delay, and medically refractory epilepsy. These tumors are most often sporadic but rarely can be associated with Pallister-Hall syndrome, an autosomal dominant familial syndrome caused by truncation of glioblastoma transcription factor 3, a downstream effector in the sonic hedgehog pathway. In this clinical report, the authors describe two brothers with a different familial syndrome. To the best of the authors' knowledge, this is the first report in the literature describing a familial syndrome caused by germline mutations in the Smoothened (SMO) gene and the first familial syndrome associated with hypothalamic hamartomas other than Pallister-Hall syndrome. The authors discuss the endoscopic endonasal biopsy and subtotal resection of a large hypothalamic hamartoma in one of the patients as well as the histopathological findings encountered. Integral to this discussion is the understanding of the hedgehog pathway; therefore, the underpinnings of this pathway and its clinical associations to date are also reviewed.

Ictal laughter and crying: Should they be classified as automatisms?

Gelastic seizures (GS) describe ictal laughter and are associated with hypothalamic lesions, as well as other cortical areas. Dacrystic seizures (DS), characterized by ictal crying, also have been reported in hypothalamic lesions and focal epilepsy. We describe a young girl with drug resistant focal dyscognitive seizures associated with gelastic and dacrystic features. However, neither laughter nor crying was correlated with a stereotyped electroencephalographic (EEG) pattern or involvement of a particular brain region. Additionally, based on the variety of epileptogenic foci associated with GS and DS in the literature, laughter and crying appear to represent ictal or peri-ictal automatisms.