The full spectrum of MRI findings in 18 patients with Canavan disease: new insights into the areas of selective susceptibility.

INTRODUCTION: Canavan disease (CD) is a rare autosomal recessive neurodegenerative disorder caused by a deficiency of aspartoacylase A, an enzyme that degrades N-acetylaspartate (NAA). The disease is characterized by progressive white matter degeneration, leading to intellectual disability, seizures, and death. This retrospective study aims to describe the full spectrum of magnetic resonance imaging (MRI) findings in a large case series of CD patients. MATERIALS AND METHODS: MRI findings in 18 patients with confirmed CD were investigated, and the full spectrum of brain abnormalities was compared with the existing literature to provide new insights regarding the brain MRI findings in these patients. All the cases were proven based on genetic study or NAA evaluation in urine or brain. RESULTS: Imaging analysis showed involvement of the deep and subcortical white matter as well as the globus pallidus in all cases, with sparing of the putamen, caudate, and claustrum. The study provides updates on the imaging characteristics of CD and validates some underreported findings such as the involvement of the lateral thalamus with sparing of the pulvinar, involvement of the internal capsules and corpus callosum, and cystic formation during disease progression. CONCLUSION: To our knowledge, this is one of the largest case series of patients with CD which includes a detailed description of the brain MRI findings. The study confirmed many of the previously reported MRI findings but also identified abnormalities that were previously rarely or not described. We speculate that areas of ongoing myelination are particularly vulnerable to changes in CD.

Clinical and genetic characterization of neuronal ceroid lipofuscinoses (NCLs) in 29 Iranian patients: identification of 11 novel mutations.

Neuronal ceroid lipofuscinoses (NCLs) are neurodegenerative lysosomal storage diseases which are considered among the most frequent causes of dementia in childhood worldwide This study aimed to identify the gene variants, molecular etiologies, and clinical features in 23 unrelated Iranian families with NCL. In total, 29 patients with neuronal ceroid lipofuscinoses (NCLs), diagnosed based on clinical manifestations, MRI neuroimaging, and electroencephalography (EEG), were recruited for this study. Through whole-exome sequencing (WES), functional prediction, Sanger sequencing, and segregation analysis, we found that 12 patients (41.3%) with mutations in the CLN6 gene, 7 patients (24%) with the TPP1 (CLN2) gene variants, and 4 patients (13.7%) with mutations in the MFSD8 (CLN7) gene. Also, mutations in each of the CLN3 and CLN5 genes were detected in 2 cases and mutations of each PPT1 (CLN1) and CLN8 gene were observed in only 1 separate patient. We identified 18 different mutations, 11 (61%) of which are novel, never have been reported before, and the others have been previously described. The gene variants identified in this study expand the number of published clinical cases and the variant frequency spectrum of the neuronal ceroid lipofuscinoses (NCLs) genes; moreover, the identification of these variants supplies foundational clues for future NCL diagnosis and therapy.

Prone extension views in cervical MRI: A case-driven Novel approach.

The cervical spinal canal has a wide range of motion and specific biomechanics involved with different pathologies that may cause dynamic cord compressions. This study has introduced new protocol for acquiring an extension view of cervical MRI to assess dynamic cervical spinal canal compromise. We posit that dynamic MRI comprising extension view in prone position could be a practical option when deciding the best approach in treating challenging patients.

Bumetanide reduces seizure frequency in patients with temporal lobe epilepsy.

Alterations in the balance of K-Na-2Cl cotransporter (NKCC1) and Na-Cl cotransporter (KCC2) activity may cause depolarizing effect of γ-aminobutyric Acid (GABA), and contribute to epileptogenesis in human temporal lobe epilepsy. NKCC1 facilitates accumulation of chloride inside neurons and favors depolarizing responses to GABA. In the current pilot study we provide the first documented look at efficacy of bumetanide, a specific NKCC1 antagonist, on reduction of seizure frequency in adult patients with temporal lobe epilepsy. According to our results, seizure frequency was reduced considerably in these patients. Furthermore, epileptiform discharges decreased in two of our patients. If the efficacy of bumetanide is proven in large scale studies, it can be used as a supplemental therapy in temporal lobe epilepsy.

Hippocampal body changes in pure partial onset sleep and pure partial onset waking epileptic patients.

The aim of the current study was to evaluate for the first time the hippocampal changes in patients with pure sleep and pure waking epilepsy. A total of 35 patients with pure partial onset sleep epilepsy and 35 patients with pure partial onset waking epilepsy matched for age and sex ratio were enrolled. MR images were analyzed to determine hippocampal body changes. Rounding ratio of hippocampal body was defined as short axis divided by long axis and hippocampal bodies with ratios ≥ 0.70 were considered rounded. Hippocampal sclerosis and atrophy were found in nine (25.7 %) and seven (20.0 %) patients with pure sleep epilepsy, and in 12 (34.3 %) and 11 (31.4 %) patients with pure waking epilepsy, respectively (P > 0.05 for the comparison between sleep and waking epilepsy). However, proportion of subjects with rounded hippocampal bodies (15, 42.9 % vs. 3, 8.6 % for patients with sleep and waking epilepsy, respectively) and rounding ratios of both left and right hippocampal bodies (0.66 ± 0.13 and 0.61 ± 0.12, respectively for left and right hippocampal bodies in sleep epileptic patients vs. 0.57 ± 0.11 and 0.55 ± 0.11, respectively for left and right hippocampal bodies in waking epileptic patients) were increased in patients with sleep epilepsy (P < 0.05). Further, in sleep epileptic patients with left sided hippocampal body rounding, epileptiform discharges were more readily lateralized to the left temporal lobe (P < 0.05). In conclusion, hippocampal sclerosis and atrophy are not different between pure partial onset sleep and waking epileptic patients. However, rounding ratio and frequency of hippocampal body rounding are increased in sleep epileptic patients.

Magnetic resonance imaging findings in children with intractable epilepsy compared to children with medical responsive epilepsy.

Objective: Epilepsy is a common brain disorder characterized by a persistent tendency to develop seizures in neurological, cognitive, and psychological contents. Magnetic Resonance Imaging (MRI ) is a neuroimaging test facilitating the detection of structural epileptogenic lesions. This study aimed to compare the MRI findings between patients with intractable and drug-responsive epilepsy. Material & Methods: This case-control study was conducted from 2007 to 2019. The research population encompassed all 1-16-year-old patients with intractable epilepsy referred to the Shafa Neuroscience Center (n=72) (a case group) and drug-responsive patients referred to the pediatric neurology clinic of Baqiyatallah Hospital (a control group). Results: There were 72 (23.5%) patients in the intractable epilepsy group and 200 (76.5%) patients in the drug-responsive group. The participants' mean age was 6.70± 4.13 years, and there were 126 males and 106 females in this study. Normal brain MRI was noticed in 21 (29.16%) patients in the case group and 184 (92.46%) patients in the control group.Neuronal migration disorder (NMD) was also exhibited in 7 (9.72%) patients in the case group and no patient in the control group. There were hippocampal abnormalities and focal lesions (mass, dysplasia, etc.) in 10 (13.88%) patients in the case group and only 1 (0.05%) patient in the control group.Gliosis and porencephalic cysts were presented in 3 (4.16%) patients in the case group and no patient in the control group. Cerebral and cerebellar atrophy was revealed in 8 (11.11%) patients in the case group and 4 (2.01%) patients in the control group. Corpus callosum agenesis, hydrocephalus, brain malacia, and developmental cyst were more frequent in the case group; however, the difference between the groups was not significant. Conclusion: The MRI findings such as hippocampal abnormalities, focal lesions (mass, dysplasia), NMD, porencephalic cysts, gliosis, and atrophy are significantly more frequent in children with intractable epilepsy than in those with drug-responsive epilepsy.

Neuroimaging Findings of the High-risk Neonates and Infants Referred to Mofid Children's Hospital.

Objectives: Neuroimaging in high-risk neonates and infants is done to help child neurologists predict the future neurodevelopmental outcome of these children. In this study, we assessed high-risk neonates and infants admitted to the NICU or neonatal wards of Mofid children's Hospital, especially regarding clinical development and brain imaging. Materials & Methods: This cross-sectional study was conducted on 170 patients admitted to the neonatal and NICU ward of Mofid children's Hospital. Considering the inclusion criteria, 112 patients were included in this project. Brain ultrasonography was performed on almost all of these babies by a single radiologist. Some patients underwent a brain CT scan, and brain MRI without contrast was done on the others. These images were interpreted and compared by a single pediatric neuro-radiologist blinded to clinical data. All of these babies were followed up until 18 months of age. Results: In this study, 57.1% of the patients were male and 42.9% were female. Of 44 patients who obtained Electroencephalogram (EEG) during the hospitalization period with probable seizure, 25 (56.8%) had normal EEGs. Of 89 babies who were examined by ultrasound, 19 (21.3%) had abnormal findings; ventriculomegaly and then germinal matrix hemorrhage (GMH) were the most common abnormalities. Also, 27 cases (71.1%) of 38 patients undergoing a CT scan had abnormal findings. The most common findings were a hypodense area in the white matter and ventriculomegaly. Of 41 patients who underwent MRI between 1 and 27 months, 34 cases (82.9%) had an abnormal MRI. The most common findings were periventricular hyperintensities in 17 cases (41.5%), mildly delayed myelination in 15 cases (36.6%), and severe brain atrophy or thinning of corpus callosum or white matter volume loss in seven cases (17.1%). During the follow-up period, which was 18.55 ± 6.56 months, 79 (70.5%) of the children had normal development and 33 (29.5%) were suffering from a global neurodevelopmental delay. More precisely, 49 (43.7%) and 35 (31.2%) patients had motor development delay and delayed verbal development, respectively. The abnormal findings of brain imaging in the ultrasound, CT scan, and MRI were all significantly associated with an adverse neurodevelopmental outcome (P <0.001, P = 0.02, and P <0.001, respectively). Conclusion: In this study, we showed that at any time before six months or after one year of age, the result of brain MRI was a strong predictor of the patient's outcome.

Contribution of altered corticospinal microstructure to gait impairment in children with cerebral palsy.

OBJECTIVE: Corticospinal tract (CST) injury may lead to motor disorders in children with Cerebral Palsy (CP). However, the precise underlying mechanisms are still ambiguous. We aimed to characterize the CST structure and function in children with CP and determine their contributions to balance and gait impairments. METHOD: Twenty-six children with spastic CP participated. Transcranial magnetic stimulation (TMS) and diffusion tensor imaging (DTI) were utilized to characterize CST structure and function. Common clinical measures were used to assess gait speed, endurance and balance, and mobility. RESULTS: CST structure and function were significantly altered in children with CP. Different abnormal patterns of CST structure were identified as either abnormal appearance of brain hemispheres (Group-1) or semi-normal CST appearance (Group-2). We found significant correlations between the DTI parameters of the more affected CST and gait features only in Group-1. CONCLUSION: CST structure and function are abnormal in children with CP and these abnormalities may contribute to balance and gait impairment in some children with CP. SIGNIFICANCE: Our findings may lead to the development of further investigations on the mechanisms underlying gait impairment in children with CP and on decision-making for more effective rehabilitation.

Giant Basal Cell Carcinoma of the Scalp with Intracranial Invasion: MRI Findings with Tract Visualisation.

A rare case of recurrent basal cell carcinoma in the scalp that infiltrated multiple intracranial structures is presented. Basal cell carcinoma represents one of the most frequent malignant nonmelanotic skin neoplasms, but the majority of them have no aggressive and recurrent behaviour. The aim of this case report is to provide an overview of the main clinical and radiologic features of basal cell carcinoma, focusing on the conventional and advanced (tractography) MRI findings and providing an overview of treatment and prognosis.

The Causal Role of Right Frontopolar Cortex in Moral Judgment, Negative Emotion Induction, and Executive Control.

INTRODUCTION: Converging evidence suggests that both emotional and cognitive processes are critically involved in moral judgment, and may be mediated by discrete parts of the prefrontal cortex. The current study aimed at investigating the mediatory effect of right Frontopolar Cortex (rFPC) on the way that emotions affect moral judgments. METHODS: Six adult patients affected by rFPC and 10 healthy controls were included in the study. Participants made judgements on moral dilemmas after being shown either neutral or emotional pictures. The role of rFPC in executive control and emotional experience was also examined. RESULTS: The study results showed that inducing an emotional state increased the number of utilitarian responses both in the patients and controls. However, no significant differences were observed between the patients and controls in response time or the number of utilitarian responses. Also, no significant differences were observed in personal and impersonal dilemmas before and after the emotion induction in intergroup comparisons. Results of the executive control tasks showed reduced performance in patients affected by rFPC compared with the controls. CONCLUSION: The results of the current study suggested that rFPC might not have a direct role in mediating emotional processes during moral judgments, but possibly this region is important in a network supporting executive control functions.

SZT2 mutation in a boy with intellectual disability, seizures and autistic features.

The seizure threshold 2 (SZT2) gene has been shown to confer a low seizure threshold and may enhance epileptogenesis in mice. However, its biological function is still not known. Mutations in SZT2 have been reported in very few patients and features range from mild to moderate intellectual disability without seizures to severe intellectual disability with epileptic encephalopathies with severe developmental delay. Here, we report a six-year-old boy with a novel homozygous mutation in SZT2 gene with intellectual disability, seizures, absent speech and autistic features. We are reporting the first patient with autistic features including very little or no eye contact, arm flapping and repetitive behaviour.

Outcome of lesional epilepsy surgery: Report of the first comprehensive epilepsy program in Iran.

BACKGROUND: We investigated the utility of epilepsy surgery and postoperative outcome in patients with lesional epilepsy in Iran, a relatively resource-poor setting. METHODS: This prospective longitudinal study was conducted during 2007-2017 in Kashani Comprehensive Epilepsy Center, Isfahan, Iran. Patients with a diagnosis of intractable focal epilepsy, with MRI lesions, who underwent epilepsy surgery and were followed up ≥ 24 months, were included and evaluated for postoperative outcome. RESULTS: A total of 214 patients, with a mean age of 26.90 ± 9.82 years (59.8% men) were studied. Complex partial seizure was the most common type of seizure (85.9%), and 54.2% of the cases had auras. Temporal lobe lesions (75.2%) and mesial temporal sclerosis (48.1%) were the most frequent etiologies. With a mean follow-up of 62.17 ± 19.33 months, 81.8% of patients became seizure-free postoperatively. Anticonvulsants were reduced in 86% of the cases and discontinued in 40.7%. In keeping with previous studies, we found that seizure freedom rates were lower among patients with longer follow-up periods. CONCLUSIONS: We found high rates of seizure freedom after surgery in lesional epilepsy patients despite limited facilities and infrastructure; antiepileptic medications were successfully tapered in almost half of the patients. Considering the favorable outcome of epilepsy surgery in our series, we believe that it is a major treatment option, even in less resource-intensive settings, and should be encouraged. Strategies to allow larger scale utility of epilepsy surgery in such settings in the developing world and dissemination of such knowledge may be considered an urgent clinical need, given the established mortality and morbidity in refractory epilepsy.

Long-term Video-EEG Monitoring Findings in Children and Adolescents with Intractable Epilepsy.

OBJECTIVE: Long Term Video-EEG Monitoring (LTM) may give us important information in the preoperative assessment of these patients. We performed this study for the first time in pediatric age group in Iran. MATERIALS AND METHODS: In this cross-sectional study, 43 children between 4 to 18 yr, with intractable epilepsy referred to Shefa Neuroscience Research Center, Tehran, Iranfrom2007-2012, were enrolled to study in order to evaluate their long-term video EEG findings. RESULTS: The patients mean age was10.07 yr, from which 24(65.9%) were boys.Seven patients with definite epileptogenic zone were advised to perform lesionectomy surgery.In two patients, there was not any seizure onset focus but corpus callosotomy was advised to control their frequent falling.Eight cases were recommended to perform electrocorticography or invasive EEG monitoring and26 cases to adjust medical treatment. In three cases, there was not any electrical seizure activity during clinical attacks, so discontinuing anti-epileptic drugs were recommended fordiagnosis of conditions that mimic epilepsy. CONCLUSION: It is necessary to perform LTM in patients with refractory epilepsy in order to determine their treatment strategy. If there is any doubt about pseudoseizureLTM can help to differentiate epilepsy from conditions that mimic epilepsy.

An Undiagnosed Case of Hypothalamic Hamartoma with a Rare Presentation.

Background. Hypothalamic hamartomas (HHs) are rare tumor-like malformations that may present with complex partial seizures refractory to anticonvulsants in adulthood. The condition may be misdiagnosed because of rarity. Case Presentation. We report a 25-year-old man with complaint of seizures presented by falling, tonic spasm of limbs, oral automatism, vocalization, and hypermotor activities. His seizures started at the age of one month and presented as eye deviation and upper limbs myoclonic jerk, followed by frequent seizures with variable frequency. The patient had delayed developmental milestones and was mentally retarded. He was hospitalized and underwent video-EEG monitoring and neuroimaging, and the diagnosis of HH was made. The patient became candidate for surgery after that. Conclusion. In this case, the underlying etiology of seizures was diagnosed after 25 years. HH is a rare condition and neurologists may encounter very small number of these cases during their practice. Therefore, they should consider it in patients who present with suspected signs and symptoms.

Anatomical Variations in Aortic Arch Branching Pattern.

BACKGROUND: The branch anatomy of the aortic arch varies widely between individuals. These are likely due to alterations in the development of aortic arch arteries during the embryonic period. The purpose of this study is to determine the frequency of the aortic arch branch variations in the local population and provide useful data to intervention radiologists, neck and thoracic surgeons. METHODS: In this study, branching pattern of the aortic arch in 226 patients was retrospectively evaluated by MR angiography. MRA performed on a high field 3 Tesla MRI scanner using 3D flash sequence. RESULTS: The normal aortic arch branching pattern was observed in 192 patients (84.9%). Also, three variations of the aortic arch branching pattern were observed. The aortic arch in 12.4% of the patients had two arterial branches. In 0.9% of cases, the left vertebral artery originated directly from the aortic arch. In 1.8% of cases, the right subclavian artery originated as the fourth aortic arch branch. CONCLUSION: Although the number of cases with aortic arch branches variation in our study is similar to other studies, the Bovine aortic arch variation is more common than other variations of aortic arch branches.

Comparative Evaluation between Diameter Difference of the Thumb and Asymmetry of Lateral Cerebral Ventricles in Children with Developmental Delay: A New Finding.

OBJECTIVE: Anthropometry (measurement of body dimensions) has been used for clinical diagnosis of growth and developmental disorders during pregnancy and after birth. Different brain volumes have also been shown in abnormal developmental disorders. This study compares the different horizontal diameters of the left- and right-hand thumbnails and asymmetry of lateral cerebral ventricles in children with developmental delays. MATERIALS & METHODS: This retrospective case control study was carried out in the Pediatric Neurologic Outpatient of a university hospital in Tehran, Iran (2009-2011). Twenty-eight patients with motor developmental disorders (case) and 28 healthy individuals (control) had brain MRIs and volume of lateral cerebral ventricles size had been studied. The maximum horizontal diameters of the left and right thumbnails were measured by calipers during physical and neurological exams by a pediatric neurologist. Finally, we compared and analyzed different horizontal diameters of the left and right hand thumbnails and asymmetry of lateral cerebral ventricles. RESULTS: There was a significant correlation between asymmetry of brain lateral ventricles size and mean difference of horizontal diameter of thumb nails (P = 0.0001). A meaningful relation between brain hemispheres asymmetry and developmental delay (P = 0.04) was seen. CONCLUSION: The asymmetry of thumbnails can be a marker for asymmetry of lateral ventricles and child developmental delays.

The Prevalence of Magnetic Resonance Imaging Hyperintensity in Migraine Patients and Its Association with Migraine Headache Characteristics and Cardiovascular Risk Factors.

OBJECTIVES: To determine the frequency of hyperintense foci in migraine patients and the relationship with migraine headache characteristics and cardiovascular risk factors. METHODS: Ninety patients with migraine headache (70 without aura and 20 with aura) were enrolled and interviewed. Information on their headache (severity, frequency, and mean disease duration) and other related data was obtained by completing a clinical checklist. Subsequently, brain magnetic resonance imaging (MRI) was performed and each patient was then evaluated for hyperintense lesions. RESULTS: Of the 90 patients, 29 (32%) had silent hyperintense lesions on their MRI. The mean age of the patients with hyperintense foci was 41 years while those with no lesions was 33 years (p<0.010). Supratentorial hyperintense lesions represented the majority of lesions in the patients (n=46, 63%). Moreover, 56.3% of the lesions (n=41) were located within the right hemisphere. Cardiovascular risk factors such as smoking, serum cholesterol, oral contraceptive pills use, and body mass index (BMI) were not significantly different in these two groups (p>0.050). The lesions were found significantly more frequently in the patients who experienced chronic migraine (p=0.032). CONCLUSION: Our study adds weight to the theory that disease duration has a key role in the formation of hyperintense brain lesions. Certain cardiovascular risk factors such as sex, smoking, serum cholesterol, and BMI, do not affect the presence or absence of such lesions, suggesting that the relationship between migraine and these lesions may be directly due to the effects of migraine itself.

The clinical features and diagnosis of Metachromatic leukodystrophy: A case series of Iranian Pediatric Patients.

OBJECTIVE: Metachromatic leukodystrophy disorder (MLD) is one of the rare neurometabolic diseases caused due to lack of saposin B and arylsulfatase A enzyme deficiency. MATERIALS & METHODS: Eighteen patients diagnosed as metachromatic leukodystrophy in the Neurology Department of Mofid Children's Hospital in Tehran, Iran between 2010 and 2014 were included in our study. The disorder was confirmed by clinical, EMG-NCV, arylsulfatase A enzyme checking and neuroimaging findings along with neurometabolic and genetic assessment from reference laboratory in Iran. We assessed age, gender, past medical history, developmental status, clinical manifestations, and neuroimaging findings of 18 patients with metachromatic leukodystrophy. RESULTS: From 18 patients, 80% were offspring from consanguineous marriages. A family history of metachromatic leukodystrophy disease was positive for four patients. Twelve patients had late infantile form of this disorder and six patients had juvenile form. A history of tonic type seizure was positive in 20% of the patients and tonic spasm was confirmed with clinical information. Electromyographgraphy (EMG) in 96% of patients was abnormal with demyelinating sensorimotor neuropathy pattern. MRI in all patients showed the leukodystrophic pattern as arcuate fibers sparing and subcortical rim in white matter and periventricular involvement. Our diagnosis was confirmed by EMG-NCV findings with sensorimotor neuropathy pattern and the assessment of arylsulfatase A enzyme function. CONCLUSION: MLD is an inheritance metabolic disorder, which was confirmed by the assessment of arylsulfatase A enzyme function, peripheral blood leukocyte that assessed in a referral laboratory in Iran.

The Clinical Features and Diagnosis of Canavan's Disease: A Case Series of Iranian Patients.

OBJECTIVE: Canavan's disease is a lethal illness caused by a single gene mutation that is inherited as an autosomal recessive pattern. It has many different clinical features especially in the non-Ashkenazi Jewish population. MATERIAL & METHODS: 45 patients were referred to the Pediatric Neurology Department of Mofid Children's Hospital in Tehran-Iran from 2010-2014 with a chief complaint of neuro developmental delays, seizures, and neuroimaging findings of leukodystrophy were included in this study. Magnetic Resonance Spectrometry (MRS) and neuro metabolic assessment from a referral laboratory in Germany confirmed that 17 patients had Canavan's disease. RESULTS: Visual impairment, seizure, hypotonia, neuro developmental arrest, and macrocephaly were the most consistent findings in the patients in this study. Assessments of neuro developmental status revealed that 13 (76%) patients had neuro developmental delays and 4 (24%) patients had normal neuro development until 18 months of age and then their neuro developmental milestones regressed. In this study, 100% of cases had macrocephalia and 76% of these patients had visual impairment. A history of seizures was positive in 8 (47%) patients and began around 3 months of age with the most common type of seizure was tonic spasm. EEGs were abnormal in all epileptic patients. In ten of the infantile group, we did not detect elevated level of N-acetylaspartic acid (NAA) in serum and urine. However, the MRS showed typical findings for Canavan's disease (peaks of N-acetylaspartic acid). CONCLUSION: We suggest using MRS to detect N-acetylaspartic acid as an acceptable method for the diagnosis of Canavan's disease in infants even with normal serum and urine N-acetylaspartic acid levels.

Cardiac arrest associated with epileptic seizures: A case report with simultaneous EEG and ECG.

Ictal asystole is a rare, probably underestimated manifestation of epileptic seizures whose pathophysiology is still debated. This report describes two patients who had cardiac asystole at the end of their seizure. The first patient was a 13-year-old boy with complex partial seizures.. His MRI showed symmetrical signal abnormality in the bilateral parietooccipital lobe accompanied by mild gliosis and volume loss. During a 3-day long-term video-EEG monitoring, he had cardiac arrest at the end of one of his seizures that was secondarily generalized. The second one was a 42-year-old veteran with penetrating head trauma in the left frontal lobe due to shell injury. During long-term video-EEG monitoring, he had one generalized tonic-clonic seizure accompanied by bradycardia and cardiac asystole. Asystoles could have a role in the incidence of sudden unexpected death in epilepsy (SUDEP), meaning that the presence of ictal bradycardia is a risk factor for SUDEP. In cases of epileptic cardiac dysrhythmia, prolonged simultaneous EEG/ECG monitoring may be required. Cardiological investigation should be included in epilepsy management.