[Faciobrachial dystonic seizures. Semiologic diagnosis in limbic encephalitis]. / Crisis distónicas faciobraquiales. Diagnóstico semiológico en la encefalitis límbica.

Limbic encephalitis (LE) associated with positive potassium channel complex antibodies often manifests with faciobrachial dystonic seizures (FBDS). We retrospectively analyze two cases, admitted to our video-EEG unit between 2006 and 2014. Both patients were males, aged 66 and 76 years respectively, presenting with brief, but very frequent uni/bilateral dystonic brachial movements, hand posturing and ipsilateral facial grimacing. Severe hyponatremia was found in both patients who went on to develop cognitive impairment. Immunosuppressive therapy improved both seizures and cognitive dysfunction. Serology testing confirmed anti VGKC antibody presence. FBDS are often the first manifestation of LE associated to positive anti VGKC antibodies, and are refractory to treatment with antiepileptic drugs. Early diagnosis and treatment of FBDS with immunosuppressive therapy is important, not only because of seizure suppression, but also because it may help limit the extent of the cognitive damage.

Cognitive reserve and Aß1-42 in mild cognitive impairment (Argentina-Alzheimer's Disease Neuroimaging Initiative).

BACKGROUND: The purpose of this study was to investigate the relationship between cognitive reserve and concentration of Aß1-42 in the cerebrospinal fluid (CSF) of patients with mild cognitive impairment, those with Alzheimer's disease, and in control subjects. METHODS: Thirty-three participants from the Argentina-Alzheimer's Disease Neuroimaging Initiative database completed a cognitive battery, the Cognitive Reserve Questionnaire (CRQ), and an Argentinian accentuation reading test (TAP-BA) as a measure of premorbid intelligence, and underwent lumbar puncture for CSF biomarker quantification. RESULTS: The CRQ significantly correlated with TAP-BA, education, and Aß1-42. When considering Aß1-42 levels, significant differences were found in CRQ scores; higher levels of CSF Aß1-42 were associated with higher CRQ scores. CONCLUSION: Reduced Aß1-42 in CSF is considered as evidence of amyloid deposition in the brain. Previous results suggest that individuals with higher education, higher occupational attainment, and participation in leisure activities (cognitive reserve) have a reduced risk of developing Alzheimer's disease. Our results support the notion that enhanced neural activity has a protective role in mild cognitive impairment, as evidenced by higher CSF Aß1-42 levels in individuals with more cognitive reserve.

Utility of the Spanish version of the FTLD-modified CDR in the diagnosis and staging in frontotemporal lobar degeneration.

INTRODUCTION: The Clinical Dementia Rating Scale (CDR) is a tool designed to quantify the severity of dementia symptoms and is also useful to assess disease progression, in Alzheimer's disease (AD). A new version of the scale was developed by adding two extra domains that focused on the core aspects of frontotemporal dementia symptomatology, Language and Behavior/Comportment/Personality. OBJECTIVES: In this study, we adapted and validated the modified CDR scale in our setting and language (Rioplatense-Spanish). MATERIALS AND METHODS: 46 patients with probable AD, 27 behavioral variant of Frontotemporal Dementia (bvFTD), 18 Primary Progressive Aphasia (PPA) and 40 healthy participants were included. The adapted version of the scale was administered by a blind rater who interviewed patients together with patient's caregiver. RESULTS: Using ROC curves, the domain language and behavior were superior to the memory domain in accuracy for detecting PPA and bvFTD, respectively, but both of them had equivalent diagnostic accuracies for probable AD. Logistic regression analyses showed that either the LANG or BEHAV domains significantly improved the discrimination between probable AD, bvFTD and PPA. CONCLUSIONS: The Spanish version of the modified CDR adds value for the characterization of the non-amnestic symptoms in patients with neurodegenerative dementias.

Single-dose oral lacosamide in refractory simple partial status epilepticus: case report and review.

OBJECTIVES: This study aimed to present 2 patients with simple partial refractory status epilepticus (RSE) treated with a single oral lacosamide (LCM) dose and to review the literature on this topic. METHOD: A retrospective description of 2 patients with simple partial RSE treated with 300 mg of LCM per os (p.o.) and a literature review were done. RESULTS: Both patients responded to single-dose oral LCM treatment with seizure cessation after 30 minutes. CONCLUSIONS: This is the first report on successful treatment of simple partial RSE with a single-dose of LCM p.o.. Oral LCM might represent an option for treatment of patients presenting with simple partial RSE.