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Objective: We aimed to clarify the association between magnetic resonance imaging (MRI)-lesion patterns, including cortices and white matters, and the development, occurrence, and intractableness of West syndrome in patients with tuberous sclerosis complex (TSC), using visual analysis., Methods: We collected data for 44 patients with TSC who had undergone brain MRI and developmental evaluation after the ages of 2 and 3 years, respectively. Fluid-attenuated inversion recovery (FLAIR) and T1-weighted images were used to analyze the number of cyst-like tubers, the number of cyst-like subcortical lesions, and the presence of diffuse lesions involving the cortices and white matter., Results: Developmental delays were observed in 28 patients. Nineteen patients had a history of West syndrome. Cyst-like tubers (range: 1-10), cyst-like subcortical lesions (range: 1-4), and diffuse lesions (range: 1-6 areas) were observed in 15, 9, and 14 patients, respectively. In the univariate analyses, all MRI findings were associated with development and/or history of West syndrome. However, in the multivariate analyses, only the diffuse lesion was associated with severe development (p = 0.003) and history of West syndrome (p = 0.012). In the subanalysis of patients with West syndrome, the diffuse lesions were also associated with pharmacological intractableness. Patients with diffuse lesions had a history of West syndrome with sensitivity of 68% and specificity of 96%. Patients with two or more areas of diffuse lesions had history of pharmacologically intractable West syndrome with sensitivity of 89% and specificity of 91%., Conclusions: Diffuse lesions may help to predict the poor neurological outcomes in patients with TSC., Competing Interests: Declaration of competing interest The authors declare no competing interests., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: Postictal generalized electroencephalographic suppression (PGES) has been associated with sudden unexpected death in epilepsy (SUDEP) in adults. Decreased heart rate variability (HRV) is one clinical marker of SUDEP in adults with epilepsy. The objective of this study was to analyze the characteristics of HRV associated with generalized convulsive seizures (GCS) ± PGES in children., Methods: Nine hundred and seventy-seven consecutive children who underwent prolonged scalp video-EEG (vEEG) and 1-lead electrocardiogram (ECG) monitoring at the Hospital for Sick Children, Toronto, Ontario, Canada were reviewed retrospectively from 2009 to 2011. Thirty-five children had GCS captured during their vEEG with or without PGES and met inclusion criteria. Children were subdivided into three age groups and compared with age-matched controls: 3-6 years; 7-12 years; and 13-18 years. Interictal HRV was measured at 5 min during N2 sleep. Preictal HRV was measured at 1 h prior to GCS onset, and postictal HRV was measured at 3 min post-GCS cessation. Low frequency (LF: ms 2 , 0.04-0.15 Hz) and high frequency (HF: ms 2 , 0.15-0.4 Hz) bands of heart rate oscillations were analyzed during the interictal and preictal periods. The root mean square of successive differences (RMSSDs) was analyzed during the following time points: interictal; preictal; and postictal., Results: Thirty-five children had GCS: 18 children with PGES [3-6 years (n = 2); 7-12 years (n = 6); 13-18 years (n = 10)] and 17 children without PGES [3-6 years (n = 6); 7-12 years (n = 5); 13-18 years (n = 6)]. Seventeen additional age-matched controls were identified [3-6 years (n = 3); 7-12 years (n = 5); 13-18 years (n = 9)]. Seventy-four GCS were captured consisting of 36 GCS + PGES and 38 GCS - PGES. There was no difference of interictal HRV among children with GCS ± PGES and controls. The preictal LF and HF in 36 GCS + PGES were significantly higher compared with 38 GCS - PGES (p < 0.01). The postictal RMSSD in 36 GCS + PGES was significantly higher compared with 38 GCS - PGES (p < 0.01). The pre- to postictal RMSSD change was significantly lower in children with GCS + PGES than in those with GCS - PGES (p = 0.035)., Conclusions: In summary, the preictal HRV in GCS + PGES was significantly higher than in children with GCS - PGES. The higher remaining postictal RMSSD in children with GCS + PGES is a potential indicator of autonomic dysregulation. In certain children with epilepsy, autonomic dysregulation may contribute to poor recovery from a GCS with subsequent PGES, thereby contributing to SUDEP. Heart rate variability and autonomic regulation in children with epilepsy should be further studied prospectively in order to better understand the mechanism by which PGES may lead to SUDEP., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Corpus callosotomy (CC) has recently been adopted for the treatment of drug-resistant epileptic spasms and tonic spasms. In the present study, we investigated CC outcomes among patients with drug-resistant epileptic spasms or tonic spasms associated with tuberous sclerosis complex (TSC)., Methods: We retrospectively collected data from seven patients (3 women, 4 men) with diagnosed TSC and who were treated using CC at Seirei Hamamatsu General Hospital in Japan. All patients had experienced drug-resistant epileptic spasms (<3 s of muscular contraction) or tonic spasms (>3 s) prior to CC, which were confirmed via video-electroencephalogram monitoring., Results: All patients exhibited multiple bilateral cortical tubers on brain magnetic resonance imaging. The main seizure types were epileptic spasms in four, tonic spasms in one, and both seizure types in two patients. Patients underwent total CC between the ages of 25 months and 21.5 years. Additional resection or disconnection was performed in two patients. The follow-up period after CC ranged between 9 months and 3.5 years. Three patients achieved remission from spasms following CC alone. Two other patients became free from spasms several months after CC but required an additional focus disconnection or medical treatment. The remaining two patients continued to show spasms or asymmetrical tonic seizures., Conclusion: Total CC resulted in freedom from drug-resistant epileptic or tonic spasms in several patients with TSC. Stepwise progression from CC to additional resection or disconnection surgery may aid in the treatment of spasms secondary to TSC., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: Sudden death in the bathtub occurs relatively frequently in Japan, particularly among elderly people. We hypothesize that sudden death in epilepsy occurring in the bathtub (SDEPB) can be distinguished from sudden death in nonepilepsy occurring in the bathtub (SDnonEPB), but is identical to sudden unexpected death in epilepsy (SUDEP)., Methods: Tokyo Medical Examiner's Office conducts postmortem examinations for all sudden and unexpected deaths in Tokyo. Clinical, social, and autopsy findings of 43 SDEPB were compared with 76 SDnonEPB, 50 SUDEP outside the bathtub, and Japanese forensic autopsy data as controls., Results: Extension of the leg(s) outside the bathtub was seen in 33% of SDEPB, but none of SDnonEPB. Sitting position was seen less frequently in SDEPB (37%) than in SDnonEPB (64%). Lung weight and pleural effusion volume were significantly lower in SDEPB than in SDnonEPB. Age at death in SDEPB was significantly younger than that in SDnonEPB. Sudden death in epilepsy occurring in the bathtub showed no differences in lung weight and pleural effusion volume from SUDEP. Living with family was more frequent in SDEPB (73%) than in SUDEP (48%). Few antiepileptic drugs, infrequent seizures, and low rate of mental retardation were identical between SDEPB and SUDEP. Lung weight was significantly heavier in all three groups than in age- and sex- matched autopsy controls., Conclusions: Leg extension outside the bathtub, lower lung weight, and absence of pleural effusion distinguish SDEPB from SDnonEPB in elderly people. Sudden death in epilepsy occurring in the bathtub may represent a form of SUDEP occurring in the bathtub, rather than drowning despite submergence in the bathtub at discovery. Conditions for bathing require careful attention from physicians and relatives, even for patients with epilepsy with few medications and infrequent seizures, and without mental retardation., (Copyright © 2019 Elsevier Inc. All rights reserved.)