A case of frontal lobe seizures with 'dancing-like' semiology.

BACKGROUND AND PURPOSE: 'Dancing-like' semiology is extremely rare and described in few case reports. It is characterized by rhythmic, oscillatory movements of the pelvis and/or limbs during which the subject appears to be dancing. It has been associated with both the frontal and temporal epileptic zone; however, the possible network involved in these fascinating seizures is unclear. METHODS: The case of a 45-year-old woman suffering from drug-resistant focal epilepsy with multi-day seizures of bizarre semiology is described. A structural and perfusion magnetic resonance imaging study (interictal and peri-ictal) and video-electroencephalograms were carried out, and several home videos were employed. A vagal stimulator was implanted. RESULTS: Home videos documented the 'dancing' semiology of seizures better than video- electroencephalogram recordings. The imaging study revealed a focal frontal polymicrogyria with a peri-ictal cerebral blood flow increase at the perisylvian lesion foci. The combination of add-on cenobamate and vagal nerve stimulation resulted in complete seizure freedom. CONCLUSION: The unusual and complex dancing-like semiology observed during our patient's seizures adds to the repertoire of fascinating complex motor manifestations of frontal lobe epilepsy.

Specificity of electroclinical features in the diagnosis of ring chromosome 20.

BACKGROUND: Ring chromosome 20 (R20) syndrome is a chromosomal disorder characterized mainly by drug-resistant frontal lobe seizures, recurrent nonconvulsive status epilepticus (NCSE), and typical EEG features. The aim of this study was to investigate if this triad is common and specific to all patients with R20. METHODS: In this cross-sectional study (from 2000 to 2011), we selected patients who fulfilled at least two out of three criteria: drug-resistant frontal lobe seizures, recurrent NCSE, and characteristic electroencephalography (EEG) features. In all patients, diagnosis was based on karyotype analysis of at least 100 metaphases. RESULTS: We identified 36 patients who met at least two of the selected criteria: six patients (16.7%) with R20 and 30 (83.3%) without R20 (non-R20). All patients with R20 met all three criteria. Eleven (36.7%) patients without R20, however, also displayed the full triad. In 19 patients without R20 (63.3%), one of the three clinical features was missing: frontal lobe seizures were not resistant to antiepileptic drugs (AED) in four (13.3%), recurrent NCSE was missing in six (20%), and nine (30%) patients did not have typical EEG features. Based on this data, specificity was 63.3%, positive predictive value was 35.3%, and sensitivity and negative predictive values were 100%. Additionally, a review of all publications describing the R20 phenotype revealed that 81.98% of patients with R20 display the full electroclinical triad. CONCLUSIONS: In our study, all patients with R20 displayed the three electroclinical characteristics. This is in line with previous reports (presenting high sensitivity and negative predictive value). However, these features can also be observed in other epilepsies and are not specific to R20. Our findings suggest that in the presence of the full triad of symptoms, karyotype analysis focused on chromosome 20 should be conducted.

Morphological Description of Frontal EEG Interictal and Ictal Discharges in an Adult Cohort of 175 Patients.

Clinical and electroencephalogram (EEG) features in frontal lobe epilepsy (FLE) vary considerably among patients, making the diagnosis a challenge. The objective of this study was to describe interictal and ictal EEG activity, identifying variables that could help to differentiate and diagnose frontal lobe epilepsy cases. A prospective cross-sectional study from patients with frontal interictal epileptiform discharges (IED) referred to the Vall d'Hebron University Hospital (Barcelona, Spain) after a clinical event compatible with epileptic seizures was designed. The interictal and ictal activity were analyzed to provide a detailed EEG description of the cases, using different statistical analyses. The morphological seizure pattern at the ictal onset remained globally unchanged over time in seizures arising from the frontal lobe for each patient. Isolated sharp waves were the most frequent waveforms in the expression of IED. Frontal lobe seizures are frequently short and sometimes appear grouped in clusters within the same recording. Often the ictal expression of the electrical activity in frontal lobe seizure is subtle and challenging to interpret. A description of the main findings is summarized to identify seizures arising from the frontal lobe and avoid false negatives findings in EEG interpretations.

Family history and frontal lobe seizures predict long-term remission in newly diagnosed cryptogenic focal epilepsy.

PURPOSE: Cryptogenic focal epilepsy (CFE) is a heterogeneous clinical disorder including patients with severe refractory forms and patients with a fairly good prognosis. Predictors of prognosis in CFE are poorly understood. The aim of this retrospective study is to identify long-term (5-year) prognostic predictors in patients with newly diagnosed CFE. METHODS: Subjects with cryptogenic focal epilepsy (CFE) seen from April 1987 to September 2011 in two twin Epilepsy Centres located in Reggio Calabria and Catanzaro, Calabria, Southern Italy, were screened. Patients were excluded if they had psychogenic seizures, major psychiatric disorders presence of brain lesions except for non-specific white matter T2-hyperintensities, short follow-up (less than five years) or for having received the diagnosis of CFE elsewhere. One hundred and eighty-six patients, firstly diagnosed in our Centres, constituted the study sample. Survival curves were generated according to the Kaplan-Meier method and compared with the log-rank test. The endpoint was the cumulative time-dependent chance of 5-year remission after treatment start. Independent predictors of remission were tested by multivariate analysis using Cox proportional hazards function models. The accuracy of the resulting model was tested with Receiver Operating Characteristics (ROC) curve analysis. KEY FINDINGS: The cumulative incidence of remission was 23%. At Kaplan-Meier analysis, the only factor predicting remission was family history of epilepsy or febrile seizures (FS; p=0.02). At Cox regression, family history and frontal lobe epilepsy showed to be independent predictors of outcome (p=0.02 and 0.03, respectively). The accuracy of these predictors was good (area under ROC curve 0.648, 95% CI 0.575-0.716). Interestingly, we also found a considerable (7 years) diagnostic delay that did not result in a worse prognosis. SIGNIFICANCE: About one quarter of subjects with newly diagnosed CFE attains 5-year seizure remission during follow-up. Family history of epilepsy or FS and frontal localization are independent prognostic predictors.

A case of periodic catatonia, due to frontal lobe epilepsy.

A case history is presented in which acute periodic catatonia was caused by frontal lobe epilepsy. This manifestation of frontal lobe epilepsy has not, to the authors' knowledge, been described before. The authors mention the problems of diagnosis and discuss a possible pathogenetic mechanism for the capricious presentation in this patient.