Epilepsy with eyelid myoclonia (Jeavons syndrome): Generalized, focal, or combined generalized and focal epilepsy syndrome?

Epilepsy with eyelid myoclonia (EM) or Jeavons syndrome (JS) is an epileptic syndrome related to the spectrum of genetic generalized epilepsies (GGE). We report two untreated children on which EEGs were performed several hours after a generalized tonic-clonic seizure (GTCS). These showed a unilateral, nearly continuous posterior slowing. This slow-wave activity was associated with contralateral epileptiform activity in one case, while in the second case, it was associated with an ipsilateral activity. However, in the latter child, a few months later an independent focus on the contralateral side was observed. A diagnosis of focal occipital lobe epilepsy was proposed in both cases, and one child underwent a left occipital lobectomy at 3.5 years of age. Despite surgery, absences with EM persisted in this child, and a marked photosensitivity to photic stimulation was observed two years later. The focal slow wave activity of one occipital lobe several hours after a GTCS in these two subjects was in favor of a focal onset preceding the generalization. The EEG evidence for independent left and right posterior focus in these two cases, the persistence of EM, and the development of a marked photosensitivity to photic stimulation in the child who underwent an occipital lobectomy, allow us to suggest that JS is associated with a network of bi-occipital hyperexcitability that rapidly engages bilaterally to produce generalized seizures.

Epilepsy in Children With Congenital Heart Disease: Risk Factors and Characteristic Presentations.

BACKGROUND: Children with a congenital heart disease (CHD) are at a higher risk of developing epilepsy than the general population, but detailed characteristics of CHD-associated epilepsy have not been clarified. The purposes of this study were to determine the risk factors for developing epilepsy associated with CHD and to elucidate the characteristics of such epilepsy. METHODS: We performed a retrospective cohort study based on medical records of pediatric patients with CHD who were born between January 2006 and December 2016, underwent cardiac surgery at Okayama University Hospital, and were followed up until at least age three years. Multivariate logistic regression analysis was used to determine factors particularly associated with epilepsy occurrence. In patients who developed epilepsy, clinical data on seizure characteristics were further investigated. RESULTS: We collected data from 1024 patients, and 41 (4.0%) developed epilepsy. The presence of underlying disease (odds ratio [OR]: 2.413; 95% confidence interval [CI]: 1.150 to 4.883) and the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery score category 2 (OR: 4.373; 95% CI: 1.090 to 29.150) and category 5 (OR: 10.385; 95% CI: 1.717 to 89.016) were significantly related to epilepsy occurrence. Of the 41 patients with epilepsy, 15 (including nine with hypoplastic left heart syndrome) had focal impaired awareness seizures specified as autonomic seizures with vomiting, which tends to escape detection. CONCLUSIONS: We clarified the risk factors for developing epilepsy in children with CHD. We also found that autonomic seizure with vomiting is an important symptom in these children.

A Case of Diagnosis of Occipital Lobe Epilepsy Complicated by Right Hemianopsia Associated with Left Occipital Lobe Cerebral Infarction.

We report a case of occipital lobe epilepsy (OLE) in a patient with occipital lobe stroke whose diagnosis was complicated by homonymous hemianopsia. An 81-year-old woman presented with a complaint of "blurred vision" on the right side and was kept under outpatient observation at the Hirabayashi Eye Clinic for homonymous lower right hemianopsia, glaucoma, and post-cataract surgery. Her past medical history included hypertension, angina pectoris, atrial fibrillation, diabetes mellitus, and left occipital lobe cerebral infarction. The corrected visual acuity and intraocular pressure were 20/16 and 12 mm Hg and 20/20 and 13 mm Hg in the right and left eye, respectively, and no change was observed in the fundus and visual field defect; hence, the patient was placed under observation. Two days later, the patient voluntarily visited a neurosurgical hospital and underwent magnetic resonance imaging. No abnormalities were detected other than the left obsolete occipital lobe stroke. Five days later, she returned to our clinic because she felt "something wobbly" on her right side. Upon examination, we suspected a transient ischemic attack based on the wobbling, closed eyelids, and loss of consciousness, and referred her to the same neurosurgical hospital. Electroencephalography (EEG) revealed spikes and waves with occipital lobe predominance, and the diagnosis of OLE was made. The patient had right-sided homonymous hemianopsia owing to left occipital lobe cerebral infarction and "blurred vision" on the same side. Thus, it is inferred that EEG is imperative for ruling out epileptic seizures.

Resection of bilateral occipital lobe lesions during a single operation as a treatment for bilateral occipital lobe epilepsy.

BACKGROUND: Neurosurgical treatment of severe bilateral occipital lobe epilepsy usually involves two operations several mos apart. AIM: To evaluate surgical resection of bilateral occipital lobe lesions during a single operation as a treatment for bilateral occipital lobe epilepsy. METHODS: This retrospective case series included patients with drug-refractory bilateral occipital lobe epilepsy treated surgically between March 2006 and November 2015. RESULTS: Preoperative evaluation included scalp video-electroencephalography (EEG), magnetic resonance imaging, and PET-CT. During surgery (bilateral occipital craniotomy), epileptic foci and important functional areas were identified by EEG (intracranial cortical electrodes) and cortical functional mapping, respectively. Patients were followed up for at least 5 years to evaluate treatment outcome (Engel grade) and visual function. The 20 patients (12 males) were aged 4-30 years (median age, 12 years). Time since onset was 3-20 years (median, 8 years), and episode frequency was 4-270/mo (median, 15/mo). Common manifestations were elementary visual hallucinations (65.0%), flashing lights (30.0%), blurred vision (20.0%) and visual field defects (20.0%). Most patients were free of disabling seizures (Engel grade I) postoperatively (18/20, 90.0%) and at 1 year (18/20, 90.0%), 3 years (17/20, 85.0%) and ≥ 5 years (17/20, 85.0%). No patients were classified Engel grade IV (no worthwhile improvement). After surgery, there was no change in visual function in 13/20 (65.0%), development of a new visual field defect in 3/20 (15.0%), and worsening of a preexisting defect in 4/20 (20.0%). CONCLUSION: Resection of bilateral occipital lobe lesions during a single operation may be applicable in bilateral occipital lobe epilepsy.

Cognitive and perceptual functions in patients with occipital lobe epilepsy, patients with migraine, and healthy controls.

This study was conducted to compare cognitive and perceptual functions among patients with occipital lobe epilepsy, patients with migraine, and healthy individuals, in relation to the moderating roles of gender and educational level. Participants included 93 individuals from Mashhad City, Khorasan-e-Razavi province, Iran. A demographic questionnaire and Bender-Gestalt II (BGT-II; Brannigan & Decker, 2003) were used for data collection in this study. Results showed significant group differences for copy, recall, motor, and perceptual subscales of BGT-II in these samples, where patients with occipital lobe epilepsy and patients with migraine having significantly lower scores than healthy individuals. Also, patients with occipital lobe epilepsy had significantly poorer scores in all subscales of the BGT-II in comparison with the patients with migraine. There were no significant differences with regard to gender and educational level when considering dependent variables in the present study.

"Mini" occipital seizures with accompanying ultra-slow waves (DC shift) on SEEG in a 14-year old child.

Occipital lobe epilepsies can present with complex or elementary visual hallucinations and oculomotor symptoms like contraversive tonic or clonic deviation of eyes and head. Brief occipital seizures can be difficult to diagnose. We report a child with occipital plus epilepsy who was investigated with stereo-EEG.

Tonic Upward Eyeball Deviation Mimicking Non-Convulsive Occipital Lobe Status Epilepticus That Was Induced by Hydrocephalus.

Several seizure-like symptoms are difficult to differentiate from epileptic convulsion, and then if they were misdiagnosed, they could be led to grave prognosis. A 41-year-old man was referred to the emergency room due to unconsciousness. Brain computed tomography (CT) revealed acute subdural hemorrhage along the left frontal lobe, and intraparenchymal hemorrhage in the left temporo-occipital lobe. After emergent decompressive craniectomy, he recovered an alert mental state but became progressively drowsy. Four days later, virtually continuous tonic upward eyeball deviation was observed. He had been taking antiepileptic drugs following an occipital lobectomy 20 years prior due to intractable epilepsy, and we assumed these upward eyeball deviations were symptoms of non-convulsive occipital lobe status epilepticus. Hence, doses and classes of antiepileptic drugs were modified, but clinical manifestations did not improve. Follow-up brain CT revealed newly developed hydrocephalus and compression of the mesencephalon. His symptoms fully resolved after a ventriculo-peritoneal shunt operation. In this case report, we describe the case of a patient exhibiting tonic upward eyeball deviation induced by hydrocephalus that was not associated with a seizure.

Electroclinical and prognostic characteristics of epilepsy patients with photosensitivity.

BACKGROUND AND PURPOSE: Epilepsy with photosensitivity (PSE) is one of the reflex epilepsy types with pathophysiology still unexplained. In our study we aimed to evaluate the clinical, electroencephalogram (EEG) and prognosis of patients with PSE diagnosis. METHODS: A total of 44 patients with PSE diagnosis according to international classification were included in this retrospective and cross-sectional study. The age, gender, syndrome, clinical and EEG characteristics of patients, and treatment response were investigated. RESULTS: The mean age was 22.09±6.49 years for 28 females and 16 males included in the study. Of patients, 17 had idiopathic photosensitive occipital lobe epilepsy (IPOLE), 11 had juvenile myoclonic epilepsy (JME), 11 had other PSE and 5 had juvenile absence epilepsy (JAE), with the most common visual trigger factors television and sunlight. In terms of seizure type, the most common was generalized tonic clonic seizure (GTCS), with myoclonus, absence and other seizure types observed. There was family history present in 17 patients and valproic acid was most commonly used for treatment. CONCLUSION: As noted in the literature, our data show that PSE has defined age group and clinical presentation, good prognosis but requires correct choice of medication for treatment. It is thought that good description of these epilepsy types will reduce misdiagnosis and mistreatment rates.

Relationship between cortical resection and visual function after occipital lobe epilepsy surgery.

OBJECTIVE In this study, the authors investigated long-term clinical and visual outcomes of patients after occipital lobe epilepsy (OLE) surgery and analyzed the relationship between visual cortical resection and visual function after OLE surgery. METHODS A total of 42 consecutive patients who were diagnosed with OLE and underwent occipital lobe resection between June 1995 and November 2013 were included. Clinical, radiological, and histopathological data were reviewed retrospectively. Seizure outcomes were categorized according to the Engel classification. Visual function after surgery was assessed using the National Eye Institute Visual Functioning Questionnaire 25. The relationship between the resected area of the visual cortex and visual function was demonstrated by multivariate linear regression models. RESULTS After a mean follow-up period of 102.2 months, 27 (64.3%) patients were seizure free, and 6 (14.3%) patients had an Engel Class II outcome. Nineteen (57.6%) of 33 patients had a normal visual field or quadrantanopia after surgery (normal and quadrantanopia groups). Patients in the normal and quadrantanopia groups had better vision-related quality of life than those in the hemianopsia group. The resection of lateral occipital areas 1 and 2 of the occipital lobe was significantly associated with difficulties in general vision, peripheral vision, and vision-specific roles. In addition, the resection of intraparietal sulcus 3 or 4 was significantly associated with decreased social functioning. CONCLUSIONS The authors found a favorable seizure control rate (Engel Class I or II) of 78.6%, and 57.6% of the subjects had good visual function (normal vision or quadrantanopia) after OLE surgery. Lateral occipital cortical resection had a significant effect on visual function despite preservation of the visual field.

Respiratory arrest at the onset of idiopathic childhood occipital epilepsy of Gastaut.

Occipital lobe epilepsy of childhood includes two entities: Panayiotopoulos syndrome in pre-school children, and idiopathic childhood occipital epilepsy of Gastaut (ICOEG) in school-age children. The typical initial manifestation of the former is vomiting, and that of the latter is visual hallucinations. Ictal cardiopulmonary arrest at initial presentation has been reported for Panayiotopoulos syndrome, but not for ICOEG. We document a 7-year-old previously healthy girl who experienced an acute elemental visual hallucination of seeing insects, followed by a new-onset generalized seizure. Upon arrival at the local hospital, she was unconscious and soon thereafter, developed respiratory arrest. She was resuscitated and initiated on mechanical ventilation. An electroencephalogram taken three days after seizure cessation showed frequent occipital spikes, consistent with the diagnosis of ICOEG. The sequence of acute elementary visual hallucination followed by a motor seizure, and then witnessed respiratory arrest illustrated occurrence of life-threatening autonomic involvement at initial onset in ICOEG. We speculate that the epileptic propagation from the occipital lobes eventually compromised the respiratory center in the brainstem. The possibility of occipital lobe epilepsy should be considered in school-age children presenting with acute visual hallucination followed by respiratory arrest. Such a presentation should prompt an urgent electroencephalogram and initiation of antiepileptic treatment if indicated.

Improvement of visual field defects after focal resection for occipital lobe epilepsy: case report.

Improvement of visual field defects after surgical treatment for occipital lobe epilepsy is rare. Here, the authors report on a 24-year-old man with a 15-year history of refractory epilepsy that developed after he had undergone an occipital craniotomy to remove a cerebellar astrocytoma at the age of 4. His seizures started with an elementary visual aura, followed by secondary generalized tonic-clonic convulsion. Perimetry revealed left-sided incomplete hemianopia, and MRI showed an old contusion in the right occipital lobe. After evaluation with ictal video-electroencephalography, electrocorticography, and mapping of the visual cortex with subdural electrodes, the patient underwent resection of the scarred tissue, including the epileptic focus at the occipital lobe. After surgery, he became seizure free and his visual field defect improved gradually. In addition, postoperative 123I-iomazenil (IMZ) SPECT showed partly normalized IMZ uptake in the visual cortex. This case is a practical example suggesting that neurological deficits attributable to the functional deficit zone can be remedied by successful focal resection.

Understanding the Epilepsy in POLG Related Disease.

Epilepsy is common in polymerase gamma (POLG) related disease and is associated with high morbidity and mortality. Epileptiform discharges typically affect the occipital regions initially and focal seizures, commonly evolving to bilateral convulsive seizures which are the most common seizure types in both adults and children. Our work has shown that mtDNA depletion-i.e., the quantitative loss of mtDNA-in neurones is the earliest and most important factor of the subsequent development of cellular dysfunction. Loss of mtDNA leads to loss of mitochondrial respiratory chain (MRC) components that, in turn, progressively disables energy metabolism. This critically balanced neuronal energy metabolism leads to both a chronic and continuous attrition (i.e., neurodegeneration) and it leaves the neurone unable to cope with increased demand that can trigger a potentially catastrophic cycle that results in acute focal necrosis. We believe that it is the onset of epilepsy that triggers the cascade of damage. These events can be identified in the stepwise evolution that characterizes the clinical, Electroencephalography (EEG), neuro-imaging, and neuropathology findings. Early recognition with prompt and aggressive seizure management is vital and may play a role in modifying the epileptogenic process and improving survival.

Cognitive dysfunctions in occipital lobe epilepsy compared to temporal lobe epilepsy.

OBJECTIVE: To compare cognitive profiles of occipital lobe epilepsy (OLE) and temporal lobe epilepsy (TLE) and to investigate whether impairment of visuospatial functions is a specific deficit of OLE. METHOD: Eighteen patients with OLE, 18 patients with TLE, and 18 controls underwent a neuropsychological battery assessing memory, visuospatial functions, and frontal/executive functions. RESULTS: Multivariate analysis evidenced poorer performance of patients with TLE and patients with OLE relative to controls on tasks assessing verbal and non-verbal long-term memory, frontal functions, and visuospatial functions. Patients with OLE had poorer performance than patients with TLE on visuospatial tasks, whereas patients with TLE performed worse than patients with OLE on verbal long-term memory test. Discriminant analysis identified two canonical discriminant functions: The first explained 53.3% of the variance, and the second explained 46.7% of the variance. The first function included verbal and non-verbal memory tests distinguishing controls from both OLE and TLE, whereas the second factor including a visuoconstructional test distinguished OLE from TLE and controls. CONCLUSIONS: The results demonstrate that visuoconstructional dysfunction is related to OLE and support the idea that alterations of occipito-parietal stream may be specific to patients with OLE.

Localization diagnosis and surgical treatment of intractable occipital epilepsy / 局解手术学杂志

Objective To explore the preoperative localization diagnosis and surgical techniques of intractable occipital lobe epilepsy.Methods Retrospectively studied 37 patients diagnosed as occipital lobe epilepsy and underwent focal occipital resections for epilepsy.The semiology,scalp electroencephalography,MRI,fluorodeoxyglucose-positron emission tomography(FDG-PET),and intracranial EEG monitoring were used to localize the epileptogenic zones.The long-term seizure outcomes were assessed according to the Engel classification scheme.Results Visual symptoms were present in 25 patients preoperatively in this series.MRI displayed occipital lobe lesions in 15 patients,and FDG-PET revealed hypometabolism in or adjacent to epileptogenic zones.And 30 patients' epileptogenic zones and functional areas were defined by intracranial EEG monitoring.Visual field deficits were present in 35.3% of patients preoperatively,and 61% had new or aggravated visual field deficits after surgery.After a mean follow-up of 41 months,81.1% of the patients were seizure free or rarely had seizures.Conclusion The curative effect of the surgery on the medically intractable occipital lobe epilepsy is good.Intracranial EEG monitoring with electrodes extensively covering the occipital lobe and adjacent areas can be useful to demarcate the epileptogenic zones and the visural cortex,and it may prevent aggravation of the visual field deficits as much as possible.

A Case Report of Occipital Lobe Epilepsy and Related Optical Coherence Tomography Findings

PURPOSE: In the present case report, visual pathway damage confirmed by retinal ganglion cell layer (GCL) damage on optical coherence tomography (OCT) in occipital lobe epilepsy was described. CASE SUMMARY: A 25-year-old female with idiopathic generalized epilepsy developed visual blurring followed by a generalized seizure. On brain magnetic resonance imaging (MRI), very subtle changes of the cortex in the left parietooccipital lobe were observed. Two days after the attack, even after the disappearance of epileptiform wave on electroencephalogram (EEG), visual acuity in both eyes was 0.5 and a perimetry revealed nearly complete visual defect in both eyes. OCT showed severe thinning of GCL and mild thinning of retinal nerve fiber layer (RNFL). No additional seizure attack occurred thereafter. One month after the attack, her visual acuity was recovered to 1.0 in both eyes and her left visual hemifield defect was recovered. However, even 6 months after the attack, her right visual hemifield defect and GCL damage persisted in both eyes. CONCLUSIONS: We reported a case in which the visual pathway damage caused by occipital lobe epilepsy was identified using OCT, despite very subtle changes in brain imaging. This case indicated GCL thinning is an objective and prognostic index for the irreversible visual field defect in occipital lobe epilepsy.

Occipital Lobe Epilepsy with Hemicrania Epileptica

No abstract available.

Epilepsy classification and additional definitions in occipital lobe epilepsy.

AIM: To evaluate epileptic children with occipital lobe epilepsy (OLE) in the light of the characteristics of Panayiotopoulos syndrome and late-onset occipital lobe epilepsy of Gastaut (OLE-G). METHODS: Patients were categorized into six groups: primary OLE with autonomic symptoms (Panayiotopoulos syndrome), primary OLE with visual symptoms (OLE-G), secondary OLE with autonomic symptoms (P-type sOLE), secondary OLE with visual symptoms (G-type sOLE), and non-categorized primary OLE and non-categorized secondary OLE according to characteristic ictal symptoms of both Panayiotopoulos syndrome and OLE-G, as well as aetiology (primary or secondary). Patients were compared with regards to seizure symptoms, aetiology, cranial imaging, EEG, treatment and outcome. RESULTS: Of 108 patients with OLE (6.4±3.9 years of age), 60 patients constituted primary groups (32 with Panayiotopoulos syndrome, 11 with OLE-G, and 17 with non-categorized primary OLE); the other 48 patients constituted secondary groups (eight with P-type sOLE, three with G-type sOLE, and 37 with non-categorized sOLE). Epileptiform activity was restricted to the occipital area in half of the patients. Generalized epileptiform activity was observed in three patients, including a patient with Panayiotopoulos syndrome (PS). Only one patient had refractory epilepsy in the primary groups while such patients made up 29% in the secondary groups. CONCLUSION: In OLE, typical autonomic or visual ictal symptoms of Panayiotopoulos syndrome and OLE-G do not necessarily indicate primary (i.e. genetic or idiopathic) aetiology. Moreover, primary OLE may not present with these symptoms. Since there are many patients with OLE who do not exhibit the characteristics of Panayiotopoulos syndrome or OLE-G, additional definitions and terminology appear to be necessary to differentiate between such patients in both clinical practice and studies.

A comparison of occipital and temporal lobe epilepsies.

PURPOSE: Differentiating between occipital lobe epilepsy (OLE) and temporal lobe epilepsy (TLE) is often challenging. This retrospective case-control study compares OLE to TLE and explores markers that suggest the diagnosis of OLE. METHODS: We queried the Jefferson Epilepsy Center surgery database for patients who underwent a resection that involved the occipital lobe. For each patient with OLE, three sequential case-control patients with TLE were matched. Demographic characteristics, symptoms, electrophysiological findings, imaging findings, and surgical outcome were compared. RESULTS: Nineteen patients with OLE and 57 patients with TLE were included in the study. Visual symptoms were unique to patients with OLE (8/19) and were not reported by patients with TLE (P < 0.0001). Occipital interictal spikes (IIS) were found only in one-third of the patients with OLE (6/19) and in no patients with TLE (P < 0.0001). IIS in the posterior temporal lobe were found in five of 19 patients with OLE vs one of 57 patients with TLE (P = 0.003). IIS involved more than one lobe of the brain in most patients with OLE (11/19) but only in nine of 57 the TLE group. (P = 0.0003) Multilobar resection was needed in most patients with OLE (15/19), typically including the temporal lobe, but in only one of the patients with TLE (P < 0.0001). CONCLUSION: Occipital lobe epilepsy is difficult to identify and may masquerade as temporal lobe epilepsy. Visual symptoms and occipital findings in the EEG suggest the diagnosis of OLE, but absence of these features, does not exclude the diagnosis. When posterior temporal EEG findings or multilobar involvement occurs, the diagnosis of OLE should be considered.