ABSTRACT
Among autoimmune encephalitides, a prevalent group are those associated with antibodies against the N-Methyl-D-aspartate receptor, which present with behavior abnormalities, psychosis, seizures and abnormal movements. A new variant, mediated by antibodies against the GABA-A receptor, was recently described. We report a 66-years-old female with this form of encephalitis whose main manifestation was the presence of severe seizures leading to status epilepticus. The patient had a good response to immunomodulatory therapy with intravenous methylprednisolone, azathioprine and anticonvulsants. The laboratory tests initially detected anti-thyroid peroxidase antibodies which lead to the misdiagnosis of Hashimoto Encephalitis, which was ruled out after the detection of antibodies against GABA-A receptor. No malignancy was detected.
Subject(s)
Humans , Female , Aged , Receptors, GABA/immunology , Encephalitis/immunology , Hashimoto Disease/immunology , Seizures/immunology , Magnetic Resonance Imaging , Encephalitis/diagnostic imaging , Hashimoto Disease/diagnostic imaging , Antibodies/immunologyABSTRACT
Aim. To assess the efficiency of topiramate (TPM), an antiepileptic medication which possesses multiple mechanisms of action, a wide therapeutic spectrum and good pharmacokinetics in the management of status epilepticus (SE), especially in refractory. Several reports have shown that TPM presents different efficacy in the management of different types of refractory status epilepticus, both adults and children. Also present our experience with two patients with SE who responded early without TPM oral complications. Conclusions. TPM oral as drug associated, is an antiepileptic medication that has shown therapeuticefficacy in cases reports and observational studies the management SE of different types and nature. This efficacy has not been contrasted with randomized controlled trials and prospective studies, therefore, future studies with larger numbers of patients are needed to confirm published reports.
Subject(s)
Humans , Male , Middle Aged , Anticonvulsants/administration & dosage , Anticonvulsants/adverse effects , Anticonvulsants/pharmacokinetics , Epilepsy , Status EpilepticusABSTRACT
Frontotemporal dementia is a neurodegenerative condition that presents with a number of distinct behavioral phenotypes. One of them is semantic dementia (SD), where exists a profound impairment for semantic knowledge related to atrophy of temporal poles. Pathologically, in most cases positive intraneuronal ubiquitin and tau negative inclusions are observed. SD is characterized by fluent, effortless, grammatical speech which lacks informational content, with limited and repetitive content, as well as semantic paraphasias. Also, patients may present with associative visual agnosia, surface dyslexia or dysgraphia, behavioral alterations. Both episodic and autobiographical memory are close to normality. Two female patients with fluent progressive aphasia are reported; they failed in a simple test of semantic association (to point to one of four objects with lesser relation to others). Autobiographical memory was fair. SD can be wrongly diagnosed as left-sided variant of Alzheimer's disease; absence of episodic amnesia and parietal defects may be useful for clinical diagnosis.
La demencia semántica es una variante de las atrofias lobares frontotemporales que se caracteriza por la degradación del conocimiento semántico, de los conceptos. En ella existe una atrofia predominante de los polos temporales, a veces asimétrica; la patología generalmente muestra neuronas con inclusiones ubiquitina (+) y tau (-). Se expresa como una afasia progresiva fluente, sin disartria ni agramatismo, con anomia y parafasias verbales o semánticas, poco informativa. Pueden existir agnosia visual asociativa y alteraciones conductuales; a veces existen alexia y/o agrafía de superficie. Hay respeto relativo de la memoria episódica y autobiográfica. Se presentan dos mujeres con afasia progresiva fluente, alteraciones conductuales y falla en una tarea semántica (4º excluido). A pesar de estos defectos la memoria autobiográfica y el desempeño en sus actividades cotidianas estaban dentro de lo normal. Se concluye que estos cuadros deben diferenciarse de los casos de enfermedad de Alzheimer de predominio izquierdo; el respeto de la memoria episódica y la falta de compromiso parietal descartarían clínicamente esta última patología.