The cognitive phenotype of idiopathic generalized epilepsy.

OBJECTIVE: Dysexecutive traits have been described in idiopathic generalized epilepsy (IGE), but studies mainly focused on juvenile myoclonic epilepsy (JME). To better understand the neuropsychology of IGE, more research is needed on syndromes other than JME, controlling potential confounding factors as the cognitive effects of valproate and epileptic discharges (ED). We describe the neuropsychological profile of a group of patients with different syndromes of IGE including simultaneous video electroencephalography (EEG). METHODS: We performed a comprehensive cognitive and neuropsychiatric evaluation with video-EEG on 61 adults with IGE (JME 19; IGE with generalized tonic-clonic seizures [GTCS] alone [IGE-GTCS] 22; childhood absence epilepsy [CAE] or juvenile absences epilepsy [JAE] persisting in adulthood 20). We compared results between patients (globally and by syndrome) and a control group of 21 individuals (similar age, educational level); p-values were adjusted for multiple testing according to a 0.05 false discovery rate. RESULTS: Patients obtained significantly lower results than controls on visuospatial working memory, processing speed, cognitive flexibility and strategy, abstract visuospatial reasoning, arithmetic, and acquired knowledge. While CAE/JAE showed the lowest scores on cognitive assessment and highest anxiety index, IGE-GTCS showed the most favorable scores. Most tests were not influenced by valproate intake, and the dose did not correlate with cognitive performance in the test that yielded differences between patients and controls. Epileptic discharges during assessment were not frequent (10 patients, 1-4 tests). SIGNIFICANCE: Our findings suggest that patients with IGE have significantly lower abilities in various executive functions and acquired knowledge, compared to population of same age and education. The low frequency of ED on simultaneous video-EEG and absence of correlation of scores with valproate dose reinforce that the obtained results are due to a cognitive phenotype in IGE. This phenotype may be influenced by syndrome, and patients with CAE/JAE persisting in the adult may have a wider neuropsychiatric impairment.

Extreme delta brush in a patient with anti-NMDAR encephalitis.

Anti-N-methyl-D-aspartate receptor encephalitis is a severe, potentially treatable, disorder and prognosis depends on early recognition and prompt immunotherapy. We report a case of anti-N-methyl-D-aspartate receptor encephalitis with atypical age and gender, and a characteristic electroencephalographic pattern that supported the diagnosis. A 66-year-old male presented with psychiatric disturbances and focal seizures with alteration of consciousness, and progressed to a state of akinetic mutism. Auxiliary tests were negative or non-specific for anti-NMDAR encephalitis. Electroencephalographic monitoring revealed a unique pattern; the extreme delta brush. The patient improved with immunotherapy and was asymptomatic at six months of follow-up. Ancillary testing was positive for anti-N-methyl-D-aspartate receptor antibodies. Extreme delta brush is a recently described electroencephalographic pattern presenting in only one third of patients with anti-N-methyl-D-aspartate receptor encephalitis. The identification of this pattern, as in our case, may guide early diagnosis and treatment of anti-N-methyl-D-aspartate receptor encephalitis.

Validation of the Spanish version of the Neurological Disorders Depression Inventory for Epilepsy (NDDI-E).

INTRODUCTION: To translate and validate into Spanish (Spain) the screening instrument of major depressive episodes (MDEs), Neurological Disorders Depression Inventory in Epilepsy (NDDI-E), in patients with epilepsy. METHODS: A total of 121 outpatients, aged 18 years and older, with a diagnosis of epilepsy were included. The diagnosis of a current major depressive episode (MDE) was established with the Mini International Neuropsychiatric Interview (MINI). RESULTS: A diagnosis of current MDE was established in 20% of the patients with the MINI. Receiver operator characteristics (ROC) analysis showed an area under the curve of 0.89, with an internal consistency of 0.78. At a cutoff score >13, 22% of patients were considered to suffer from MDE with the NDDI-E (sensitivity: 84%; specificity: 78%; positive predictive value: 64.7%; and negative predictive value: 92.2%). DISCUSSION: The Spanish-Spain version of the NDDI-E appears to be a good screening instrument to identify MDE.

Orthostatic headache and bilateral abducens palsy secondary to spontaneous intracranial hypotension.

Spontaneous intracranial hypotension (SIH) is a well-documented syndrome characterized typically by a benign, self-limited course. Patients typically present with postural or exertional headaches that can be temporarily relieved by lying in a supine or recumbent position. A 35-year-old Caucasian male suffered orthostatic headache that developed to a bilateral abducens palsy. We ordered relative rest and the patient improved and completely recovered after 3 months. Although SIH is considered as a benign and self limited process it could also be associated with disabling complications. We should be aware of the possible complications and inform our patients. SIH can present with headache and bilateral abducens palsy even when the headache is improving.

Reversible manifestations of extraparenchymal neurocysticercosis.

Movement disorders are uncommon manifestations of neurocysticercosis. When present, most are secondary to parenchymal lesions in the basal ganglia. Rarely, movement disorders can occur in racemose/extraparenchymal neurocysticercosis, an aggressive variant frequently associated with cerebrospinal fluid outflow obstruction and hydrocephalus. Appropriate treatment can reverse neurological manifestations.

Benefits versus risks of latest therapies in multiple sclerosis: a perspective review.

Disease-modifying treatments for multiple sclerosis (MS) have now been available for almost 20 years. Interferon ß (IFN-ß) products and glatiramer acetate (GA) were the first available options and are now considered first-line agents for the treatment of MS. These medications have several years of favorable safety data, but are not effective in completely controlling disease activity in all patients. Alternate medications with increased efficacy have been developed and identified; however, these newer medications have known or potential safety concerns which have prompted clinicians to view them as second-line agents. Highly efficacious and safe medications are continuously being searched for and developed; however, time is needed to establish the long-term safety of any new therapeutic agent. MS practitioners are faced with the clinical dilemma of treating patients with very safe modestly effective medications or using more efficacious and potentially riskier agents. The risk-benefit profile of every medication will have to be weighed carefully and clinicians will need to gage the risk tolerance of each patient in order to tailor treatment. This review will summarize benefits and risks of recently approved therapies in MS and will provide a perspective view on the placement of these medications within the MS treatment algorithm in the near future.