Epilepsy with anterior temporal encephaloceles: Baseline characteristics, post-surgical outcomes, and comparison to mesial temporal sclerosis.

OBJECTIVE: To review clinical and neuropsychological characteristics and natural history of a series of patients with temporal lobe epilepsy (TLE) and anterior temporal encephaloceles (ATE) and compare them to a similar series of TLE patients with mesial temporal sclerosis (MTS) to identify characteristics suggestive of ATE-related epilepsy. METHODS: Patients with epilepsy and ATE were identified via clinic encounters and consensus epilepsy surgery conference at a Level 4 epilepsy center. The drug-resistant subset of these patients who underwent epilepsy surgery (twenty-two of thirty-five) were compared to age- and laterality-matched patients with MTS. Clinical, neuropsychological, electrophysiologic, and surgical data were abstracted through chart review. RESULTS: In comparison with MTS, ATE patients were more often female, had significantly later onset of epilepsy, and did not have prior febrile seizures. In addition, ATE patients were more likely to have chronic headaches and other historical features consistent with idiopathic intracranial hypertension (IIH). Failure to identify ATE on initial imaging was common. Most patients had limited temporal cortical resections sparing mesial structures. Of the twenty ATE patients who had a long-term postsurgical follow-up, seventeen (85%) had International League Against Epilepsy (ILAE) Class 1 or 2 outcomes. SIGNIFICANCE: A shorter duration of epilepsy, female gender, and lack of history of febrile seizures may suggest ATE as an etiology of refractory TLE in adults. Targeted encephalocele resections can result in seizure freedom, underscoring the importance of encephalocele identification.

Video-EEG results and clinical characteristics in patients with psychogenic nonepileptic spells: The effect of a coexistent epilepsy.

RATIONALE: Epilepsy and psychogenic nonepileptic spells (PNES) can coexist, often posing diagnostic and therapeutic challenges. We sought to identify clinical and historical characteristics of two groups of patients, those with coexisting epilepsy and PNES and those with PNES alone, and determine the prevalence of coexisting epilepsy/PNES with strict diagnostic criteria in a large group of epilepsy monitoring unit (EMU) patients. METHODS: We reviewed the medical records of all consecutive patients admitted to the Vanderbilt University Medical Center Adult EMU between July 1, 2007 and June 30, 2012. We identified patients with recorded PNES and classified them as having coexisting epilepsy/PNES or PNES alone and then systematically compared the clinical characteristics of these two groups. RESULTS: A total of 1567 patient medical records were reviewed. The prevalence rate of coexisting epilepsy/PNES was 5.2% among all EMU admissions (12.3% of all patients with epilepsy and 14.8% of all patients with PNES). These rates were lower when patients with interictal epileptiform activity (IEA) alone and no recorded ictal discharges were not included in the group with epilepsy (2.6%, 6.2%, and 7.4%, respectively). The accuracy of pre-EMU clinical suspicion was significantly higher in the group with PNES-only. Patients with epilepsy/PNES were significantly more likely to require more than one EMU admission for definitive diagnosis. The first PNES event preceded an epileptic seizure (ES) in 94.4% of patients with epilepsy/PNES. The group with PNES-only had significantly higher suggestibility, and the group with epilepsy/PNES had a significantly higher presence of epilepsy risk factors. Abnormal neurological examination and abnormal brain MRI were also significantly more common in the group with epilepsy/PNES. CONCLUSIONS: Our study defined the prevalence of coexisting epilepsy/PNES in a large cohort with strict diagnostic criteria and outlined specific clinical and historical characteristics differentiating the two groups of patients with coexisting epilepsy/PNES and PNES-only. These findings should help guide clinicians to reach the correct diagnosis faster and provide appropriate treatment earlier.

Temporal lobe origin is common in patients who have undergone epilepsy surgery for hypermotor seizures.

RATIONALE: Hypermotor seizures are most often reported from the frontal lobe but may also have temporal, parietal, or insular origin. We noted a higher proportion of patients with temporal lobe epilepsy in our surgical cohort who had hypermotor seizures. We evaluated the anatomic localization and surgical outcome in patient with refractory hypermotor seizures who had epilepsy surgery in our center. METHODS: We identified twenty three patients with refractory hypermotor seizures from our epilepsy surgery database. We analyzed demographics, presurgical evaluation including semiology, MRI, PET scan, interictal/ictal scalp video-EEG, intracranial recording, and surgical outcomes. We evaluated preoperative variables as predictors of outcome. RESULTS: Most patients (65%) had normal brain MRI. Intracranial EEG was required in 20 patients (86.9%). Based on the presurgical evaluation, the resection was anterior temporal in fourteen patients, orbitofrontal in four patients, cingulate in four patients, and temporoparietal in one patient. The median duration of follow-up after surgery was 76.4months. Fourteen patients (60%) had been seizure free at the last follow up while 3 patients had rare disabling seizures. CONCLUSIONS: Hypermotor seizures often originated from the temporal lobe in this series of patients who had epilepsy surgery. This large proportion of temporal lobe epilepsy may be the result of a selection bias, due to easier localization and expected better outcome in temporal lobe epilepsy. With extensive presurgical evaluation, including intracranial EEG when needed, seizure freedom can be expected in the majority of patients.

Does adherence to epilepsy quality measures correlate with reduced epilepsy-related adverse hospitalizations? A retrospective experience.

In 2011, the American Academy of Neurology (AAN) established eight epilepsy quality measures (EQMs) for chronic epilepsy treatment to address deficits in quality of care. This study assesses the relationship between adherence to these EQMs and epilepsy-related adverse hospitalizations (ERAHs). A retrospective chart review of 475 new epilepsy clinic patients with an ICD-9 code 345.1-9 between 2010 and 2012 was conducted. Patient demographics, adherence to AAN guidelines, and annual number of ERAHs were assessed. Fisher's exact test was used to assess the relationship between adherence to guidelines (as well as socioeconomic variables) and the presence of one or more ERAH per year. Of the eight measures, only documentation of seizure frequency, but not seizure type, correlated with ERAH (relative risk [RR] 0.343, 95% confidence interval [CI] 0.176-0.673, p = 0.010). Among patients in the intellectually disabled population (n = 70), only review/request of neuroimaging correlated with ERAH (RR 0.128, 95% CI 0.016-1.009, p = 0.004). ERAHs were more likely in African American patients (RR 2.451, 95% CI 1.377-4.348, p = 0.008), Hispanic/Latino patients (RR 4.016, 95% CI 1.721-9.346, p = 0.016), Medicaid patients (RR 2.217, 95% CI 1.258-3.712, p = 0.009), and uninsured patients (RR 2.667, 95% CI 1.332-5.348, p = 0.013). In this retrospective series, adherence to the eight AAN quality measures did not strongly correlate with annual ERAH.

Aphasic or amnesic status epilepticus detected on PET but not EEG.

PURPOSE: To describe five patients with ictal aphasia and one patient with ictal amnesia, who had focal positron emission tomography (PET) hypermetabolism but no clear ictal activity on electroencephalography (EEG). METHODS: (18)F-Fluorodeoxyglucose (FDG)-PET scans with concomitant EEG were obtained in five patients with suspected ictal aphasia or ictal amnesia without ictal activity on EEG. We reviewed medical history, EEG, imaging data, and treatment outcome. RESULTS: Brain magnetic resonance imaging (MRI) showed no structural abnormalities in any of the patients. EEG showed left temporal irregular delta activity in three patients, with aphasia and nonspecific abnormalities in two other patients, all without clear ictal pattern. All patients demonstrated focal hypermetabolism on PET scan. The hypermetabolism was in the left frontotemporal region in patients with ictal aphasia and in the bilateral hippocampal region in the patient with amnesia. Three patients who received intravenous benzodiazepines during their episodes had transient clinical improvement. With antiepileptic drug (AED) treatment, symptoms gradually resolved in all patients. Concomitant resolution of PET hypermetabolism was documented in three patients who had follow up scans. One patient with ictal aphasia later developed recurrent episodes, each with recurrent PET hypermetabolism. This patient and one other patient required immune-modulating therapy in addition to AEDs. DISCUSSION: FDG-PET imaging should be considered as a diagnostic tool in patients with suspected ictal aphasia or amnesia, who fail to show clear evidence of ictal activity on EEG.

Ictal flatulence: seizure onset in the nondominant hemisphere.

We describe the case of a 45-year-old woman with frequent simple partial seizures consisting mainly of autonomic manifestations including flatulence. The ictal onset and interictal EEG epileptiform abnormalities were localized to the right frontotemporal region. This localization was supported by structural brain imaging showing recurrence of a right frontotemporal oligodendroglioma with involvement of the insula. We therefore suggest that ictal flatulence is one of several autonomic and gastrointestinal symptoms and signs that may be used clinically in lateralizing to the nondominant hemisphere.

Localization value of ictal turning prone.

PURPOSE: Ictal semiology complements ictal EEG in identifying the likely epileptogenic zone. Ictal turning prone (ITP) with body turning of 90 ° or more can be seen with frontal lobe epilepsies. The aim of our study was to evaluate the localizing value of ITP in a general population of patients undergoing long term video-EEG monitoring. METHODS: We reviewed our epilepsy monitoring unit database for adult patients with recorded habitual seizures with ITP. All 16 patients identified had continuous video-EEG monitoring using standard scalp electrodes; eight patients also had intracranial EEG monitoring. We only included focal seizures without evolution to bilateral tonic-clonic activity. RESULTS: We identified 16 patients with ITP, mean age of 32.5 years (range 18-50). ITP was consistently seen in at least one focal impaired awareness seizure of all patients. Ictal onset zone on scalp EEG was left temporal in five, right temporal in three, left frontal convexity in two, right frontal convexity in two, probable right medial frontal in three and probable left medial frontal in one patient. Direction of ITP was uni-directional in 12 patients while 4 patients had ITP in opposite direction in different seizures. Nine patients underwent epilepsy surgery; five patients had Engel class I outcome and four patients had Engel class III outcome. CONCLUSIONS: Ictal turning prone does not have a consistent single localizing or lateralizing value and can be seen with various epileptogenic zones including medial frontal, lateral frontal or temporal. ITP direction can vary even with a single epileptogenic zone.

Communicating the diagnosis of psychogenic nonepileptic seizures: The patient perspective.

Psychogenic nonepileptic seizures (PNES) are a common cause of refractory seizures. Video-electroencephalographic (EEG) monitoring has allowed PNES to be effectively distinguished from epileptic seizures. Once the diagnosis of PNES is established, neurologists face the challenge of explaining it to patients. Patients may not always receive the diagnosis well. The aim of this study is to evaluate how effectively patients receive and perceive the diagnosis of PNES. This prospective study was conducted in an eight-bed epilepsy monitoring unit (EMU). Adult patients with newly confirmed PNES were included. After receiving written consent, a self-administered questionnaire was given to patients after the attending physician had communicated the diagnosis of PNES. A total of 75 patients were recruited. All patients had their typical seizures recorded on video-EEG (range 1-12, mean 2.18). Seventy patients were satisfied with the diagnosis of PNES. Nine patients did not agree that PNES has a psychological cause. Nineteen patients thought that the EMU doctors had no clue as to the cause of their seizures and 20 thought that there was no hope for a cure of their seizures. A significant number of patients with PNES feel that there is no hope for cure of their seizures. Thorough education about PNES, properly preparing patients before discussing the diagnosis of PNES, and preferably earlier diagnosis may prevent this miscommunication and result in better outcomes. A comprehensive approach including psychological counseling and psychiatric input, evaluation and treatment, in order to bring the illness from the subconscious to the conscious level, and effective follow-up may be helpful.

Temporal Lobe Encephaloceles: A Potentially Curable Cause of Seizures.

OBJECTIVE: Temporal lobe encephaloceles are characterized by protrusion of brain parenchyma through a structural defect in the floor of the middle fossa. They have been reported to cause cerebrospinal fluid (CSF) leaks, conductive hearing loss, meningitis, and seizures. The association between temporal encephaloceles and epileptiform activity is particularly rare. PATIENTS: All patients who presented to a single tertiary referral center between 2011 and 2014 with intractable seizures and radiographic evidence of a middle cranial fossa encephalocele were evaluated. Five patients from this subset who underwent surgical repair of their encephalocele are presented. INTERVENTION(S): Middle cranial fossa approach for encephalocele repair. MAIN OUTCOME MEASURE(S): Postoperative epileptiform activity. RESULTS: Five patients underwent a craniotomy for resection of a temporal lobe encephalocele with repair of a middle fossa floor defect. After surgery, CSF rhinorrhea resolved, when present, and all patients remained seizure-free through their last available follow-up. Range of follow-up time was 3.5 months to 4 years. Average follow-up time was 19.7 months. CONCLUSION: Temporal lobe encephaloceles are an infrequent cause of seizures. Given that these lesions can be missed with standard imaging modalities, they are likely underdiagnosed upon initial medical evaluation. This diagnosis should be considered in patients with intractable seizures. If an encephalocele is found, focused resection of epileptogenic tissue associated with herniation and repair of the temporal floor defect can provide definitive treatment.

Patients visiting the emergency room for seizures: insurance status and clinic follow-up.

PURPOSE: Epilepsy is a chronic condition that is best treated in the outpatient clinic setting. However, many epilepsy patients use the hospital emergency room (ER) as a primary resource for seizure management. We studied characteristics of these patients in comparison with patients attending an epilepsy clinic. METHODS: We reviewed ER data of patients seen in 2002 and 2003 for seizures, in Vanderbilt University Hospital (VUH) and Metro Nashville General Hospital (MNGH), seeking to identify patients who had visited the emergency room more than once. We collected demographic and insurance information on these patients and identified those who followed up in the epilepsy clinic. RESULTS: There were 1005 patients who visited the VUH ER and 205 the MNGH ER for seizures. Patients visiting the ER for seizures were less likely to be insured than epilepsy patients followed in the clinic, in both institutions. The proportion of patients visiting the ER more than once was 15.2% at VUH and 29.2% at MNGH. Among these patients, 3.2% at VUH and 26.7% at MNGH were uninsured. Clinic follow-up occurred in 68.6% of VUH and 13.3% of MNGH repeat ER visitors. Combining institutions, insured patients were much more likely to follow-up in the clinic. DISCUSSION: Repeated use of the ER for seizures was more common in the county hospital, where the proportion of uninsured patients was also higher. Patients visiting the county hospital ER repeatedly tend not to follow-up in the neurology clinic. This element of disparity of care requires further attention.

Rhythmic ictal nonclonic hand (RINCH) motions: a distinct contralateral sign in temporal lobe epilepsy.

PURPOSE: To describe a new ictal sign in temporal lobe seizures-rhythmic ictal nonclonic hand (RINCH) motions and to determine its lateralizing significance and other ictal manifestations associated with it. METHODS: We identified 15 patients with temporal lobe epilepsy who demonstrated RINCH motions and reviewed video-EEG recordings of all their seizures. We analyzed the epilepsy characteristics and all clinical features of recorded seizures, with particular attention to RINCH motions. RESULTS: RINCH motions were unilateral, rhythmic, nonclonic, nontremor hand motions. RINCH motions were usually followed by posturing, sometimes with some overlap. They involved the hand contralateral to the temporal lobe of seizure onset in 14 of 15 patients. CONCLUSIONS: RINCH motions are a distinct ictal sign that could be considered a specific type of automatism. They appear to be a lateralizing contralateral sign and are associated with dystonic posturing in temporal lobe epilepsy.