Analysis of the aetiology of epilepsy in 3,216 adult patients attending a tertiary referral center enabled by an electronic patient record.

PURPOSE: The aim of this study was to review the causes of the epilepsies in our institution, an adult tertiary referral center for neurology and neurosurgery in Dublin, Ireland. Data was obtained from a bespoke epilepsy electronic patient record (EPR). METHODS: Predetermined search parameters of well-established broad categories of epilepsy aetiology were used to identify patients with a diagnosis of epilepsy attending Beaumont Hospital, Dublin. There were 3216 patients that met the inclusion criteria for this study. We included living patients with epilepsy attending our institution. We then excluded patients with a diagnosis of pure non-epileptic attack disorder and patients found to have idiopathic generalised epilepsy (IGE) (n = 382) from our final cohort. We excluded IGE due to the complex polygenic basis underlying this patient group. RESULTS: An aetiology was identified in 54.3 % (n = 1747) of the total number of patients studied. Of the symptomatic epilepsies, 41.08 % (n = 1321) were acquired and 13.3 % (n = 426) were predominantly of genetic or developmental aetiology. The most common causes of the acquired epilepsies were hippocampal sclerosis (n = 380; 28.75 %), cerebral tumor (n = 279; 21.06 %), traumatic brain injury (n = 248; 18.77 %), stroke and cerebrovascular disease (n = 151; 11.43 %) and perinatal causes (n = 138; 10.45 %). The leading causes in the genetic / developmental category included cavernous haemangiomas (n = 62, 22.22 %), arteriovenous malformations (n = 59; 21.15 %) and cortical dysplasia (n = 55; 19.71 %). The aetiology of a patient's epilepsy was undetermined in 45.68 % (n = 1469) of individuals. CONCLUSION: This study emphasizes the clinical utility of the ILAE's 2017 revised classification of the epilepsies and highlights the evolving dynamic nature of attributing causality in epilepsy. This is the largest single centre analysis of the aetiology of the epilepsies described in the literature. It is also the first large scale study examining aetiology utilising a bespoke electronic patient record in epilepsy.

Reversible cerebral vasoconstrictive syndrome preceded by minor head trauma.

Reversible cerebral vasoconstriction syndrome is a heterogeneous and under-recognised neurovascular disorder. Our knowledge with regards to specific syndrome triggers and optimal management is limited. The delay in diagnosis can be deleterious to the patient due to intracerebral sequelae causing temporary or permanent morbidity. Prompt identification of this syndrome is vital to reverse neurological deficits while appropriately managing and supporting patient recovery.

Historical perspectives and definitions of the postictal state.

The postictal state and its features were recognized by physicians from Babylonian times through to the advent of modern neurology in the late 19th century. Among varied descriptions and definitions lies one of the best known and still used eponyms in medicine, Todd's paralysis. Despite a relative lack of biological insight, many key observations were made in an era mostly devoid of treatments for epilepsy.

Ictal bigeminy.

Seizures can influence cardiac autonomic function and cause abnormalities in the electrocardiogram (EKG). A case of stereotypical bigeminy during left temporal lobe seizures recorded in the epilepsy monitoring unit is presented. The mechanism is likely due to spread of the ictal discharge to primary visceromotor regions in the left insula (Island of Reil). This case illustrates the potential influence of ictal brain activity on cardiac electrophysiology.

Subdural electrode analysis in focal cortical dysplasia: predictors of surgical outcome.

OBJECTIVE: Patients undergoing epilepsy surgery for focal cortical dysplasia (FCD) guided by subdural EEG generally have a poor surgical outcome. Our objective was to identify predictors of postoperative seizure recurrence in this patient cohort. METHODS: We retrospectively reviewed 48 consecutive surgeries guided by subdural electrode recordings between 1990 and 2004 in patients with a pathologic diagnosis of isolated FCD. Using survival analysis, we analyzed results of the noninvasive evaluation, MRI, subdural interictal and ictal EEG patterns, extent of resection, proximity to eloquent cortex, and postoperative EEG. RESULTS: After a median follow-up of 2.7 years, 45% of patients were completely seizure-free. Most seizures recurred in the first 6 months or between years 2 and 3 after surgery. On univariate analysis, seizure recurrence was associated with bilateral EEG abnormalities, multiple semiologic seizure types, and incomplete resection of the ictal onset zone. The absence of an MRI lesion did not affect outcome, nor did proximity to eloquent cortex. Interictal paroxysmal fast and runs of repetitive spikes correlated with the ictal onset zone, whereas isolated spikes did not. The 6-month EEG predicted ultimate surgical failure in patients seizure-free at that stage. An ictal spread pattern from the edge of the subdural grids was an independent predictor of seizure recurrence on multivariate analysis. CONCLUSIONS: We have identified specific predictive factors that may guide the surgical evaluation of patients with focal cortical dysplasia and intractable epilepsy requiring subdural EEG monitoring. Successful surgical results can be obtained utilizing subdural EEG in carefully selected patients.

Multiple auras: clinical significance and pathophysiology.

BACKGROUND: Patients with partial epilepsy may report multiple types of aura during their seizures. The significance of the occurrence of multiple auras in the same patient is not known. METHODS: The clinical and electrophysiologic characteristics of patients with more than one aura type (abdominal, auditory, autonomic, gustatory, olfactory, psychic, somatosensory, and visual auras), evaluated in the Cleveland Clinic epilepsy monitoring unit between 1989 and 2005, were studied. RESULTS: Thirty-one patients experienced multiple aura types during a seizure. Ninety percent of patients with at least two aura types (n = 31) and 100% percent of patients with at least three aura types (n = 12) had seizures arising from the right/nondominant hemisphere. EEG seizures remained restricted in all patients during their auras. nineteen [corrected] patients had epilepsy surgery with seizure freedom in 53%. Subdural EEG recordings in six patients showed either a march of sequential auras, or in one case, several ictal onset zones resulting in separate isolated auras. Ictal SPECT in six patients with right-sided seizures showed a lack of activation in brainstem structures. CONCLUSIONS: Most patients who report multiple aura types have localized epilepsy in the nondominant hemisphere, and are good surgical candidates. A common mechanism for multiple auras may be a spreading but restricted EEG seizure activating sequential symptomatogenic zones, but without the ictal activation of deeper structures or contralateral spread to cause loss of awareness and amnesia for the auras.

Predictors of outcome after temporal lobectomy for the treatment of intractable epilepsy.

To assess short- and long-term seizure freedom, the authors reviewed 371 patients who underwent anterior temporal lobectomy to treat pharmacoresistant epilepsy. The mean follow-up duration was 5.5 years (range 1 to 14.1 years). Fifty-three percent of patients were seizure free at 10 years. The authors identified multiple predictors of recurrence. Results of EEG performed 6 months postoperatively correlated with occurrence and severity of seizure recurrence, in addition to breakthrough seizures with discontinuation of antiepileptic drugs.

October 2004: a 49-year-old man with progressive dementia.

October 2004. A 49-year-old right-handed man developed progressive cognitive difficulties over a 4-month period. There was impairment in recent memory, calculations and language. He also developed fatigue, weight loss, gait imbalance and urinary incontinence. Past history included transfusion-associated Hepatitis C. Neurologic exam showed mild dysarthria, dysnomia, left sided neglect, bilateral Babinski signs, and a prominent grasp reflex. Laboratory testing provided no positive etiologic data. An EEG showed generalized intermittent slowing suggestive of a diffuse encephalopathy and decreased background in the right hemisphere, suggestive of a structural lesion. MRI showed multiple areas of high signal on FLAIR imaging and patchy enhancement. FDG-PET showed multi-focal areas of increased uptake, correlating with the abnormal areas on MRI, on a background of decreased uptake. A 4-vessel cerebral angiogram showed no abnormalities. A brain biopsy showed diffuse infiltrates of large malignant cells that were immunoreactive with antibodies to CD20, diagnostic of diffuse large B cell lymphoma. In summary, the clinical presentation suggested bilateral hemispheric involvement, which was supported by physical examination, EEG, MRI, and PET scans. The differential diagnosis for this presentation is limited to demyelinating disease such as multiple sclerosis, vascular dementia, and infiltrating neoplasm such as glioblastoma multiforme or lymphoma. Diagnosis was made by morphologic and immunohistochemical analysis of brain tissue.