ABSTRACT
Dementia is not just a disease of old age. Early-onset dementia affects people younger than 65 and its differential diagnosis is broader than in older people. Nevertheless, although young people are considerably more liable to develop a rare form of dementia, Alzheimer's disease (AD) remains the most common diagnosis. The aim of this article is to report on an early-onset AD patient associated with the rare pathogenic variant PSEN1 (Leu85Pro) presenting as a possible corticobasal syndrome with asymmetric limb apraxia, parkinsonian signs, and myoclonus.
Subject(s)
Alzheimer Disease , Brain , Mental Competency , Myoclonus , Presenilin-1 , Adult , Alzheimer Disease/diagnosis , Alzheimer Disease/genetics , Alzheimer Disease/psychology , Amyloid beta-Peptides/metabolism , Brain/diagnostic imaging , Brain/metabolism , Brain/pathology , Diagnosis, Differential , Disease Progression , Female , Humans , Magnetic Resonance Imaging/methods , Mental Status and Dementia Tests , Mutation , Myoclonus/diagnosis , Myoclonus/etiology , Neurologic Examination/methods , Neuropsychological Tests , Positron Emission Tomography Computed Tomography/methods , Presenilin-1/geneticsABSTRACT
AIM: The aim of this study was to determine the prognosis of 26 consecutive adults with alpha coma (AC), theta coma (TC) or alpha-theta coma (ATC) following CRA and to describe the clinical setting and EEG features of these patients. METHODS: We retrospective analyzed a prospectively collected cohort of adult patients diagnosed as having AC, TC or ATC after CRA between January 2008 and June 2016. None of patients included in this analysis underwent therapeutic hypothermia (TH). Neurological outcome was expressed as the best score 6 months after CRA using the five-point Glasgow-Pisttsburgh Cerebral Performance Categories (CPC) RESULTS: Twenty-six patients were identified with a diagnosis of postanoxic AC, TC or ATC coma. There were 20 (77%) men and 6 (23%) women. The mean age was 63⯱â¯16â¯years. The most frequent EEG pattern was TC (21 patients, 80%), followed by AC (3 patients, 12%) and ATC (2 patients, 8%). The cardiac rhythm as primary origin of the CRA was ventricular fibrillation (VF) in 16 patients (61.5%), asystole in 8 patients (34.6%) and ventricular tachycardia (VT) in one patient (3.8%). The presence of EEG reactivity was present in 8 patients (30%). The mortality rate was 85%. Of the 4 surviving patients, two (3.8%) had moderate disability (CPC 2), one (3.8%) had severe disability (CPC 3) and one (3.8%) reached a good recovery. The age was significantly lower in survivors 46.2⯱â¯10.8 versus nonsurvivors 63.3⯱â¯15.5 (pâ¯=â¯0.04). There was increased association of EEG reactivity with survival (pâ¯=â¯0.07). CONCLUSION: Hypoxic-ischemic AC, TC and ATC are associated with a poor prognosis and a high rate of mortality. In younger patients with AC, TC and ATC and incomplete forms showing reactivity on the EEG, there is a greater probability of clinical recovery.
Subject(s)
Coma/physiopathology , Glasgow Coma Scale , Heart Arrest/complications , Adult , Age Factors , Aged , Coma/classification , Coma/etiology , Electroencephalography , Female , Humans , Male , Middle Aged , Prospective Studies , Recovery of Function , Retrospective Studies , Sensitivity and SpecificityABSTRACT
We describe the case of an elderly woman with an episode of ambulatory de novo absence status epilepticus of late onset (DNASLO) after oral treatment with cefuroxime. A high level of suspicion of DNASLO in cases of unexplained confusion in adults or elderly subjects taking cephalosporins is essential to prompt an emergency EEG and, in turn, rapidly achieve an appropriate diagnosis and enable optimal treatment.
