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Background: Neuropsychological assessments are essential to define the cognitive profile and contribute to the diagnosis of Alzheimer’s disease (AD). The progress in knowledge about the pathophysiological process of the disease has allowed conceptualizing AD through biomarkers as a biological continuum that encompasses different clinical stages. Objective: To explore the association between cerebrospinal fluid (CSF) biomarkers of AD and cognition using the NEURONORMA battery, in a sample of cognitively unimpaired (CU), mild cognitive impaired (MCI), and mild dementia of the Alzheimer type (DAT) subjects, and to characterize the cognitive profiles in MCI subjects classified by A/T/N system. Methods: 42 CU, 35 MCI, and 35 mild DAT were assessed using the NEURONORMA battery. Core AD biomarkers [amyloid-β42 (Aβ42) peptide, total tau (t-tau), and phosphorylated tau 181 (p-tau181)] proteins were measured in CSF. Correlation coefficients, multivariate regression, and effect sizes were calculated. We explored the age- and education-adjusted cognitive profiles by A/T/N variants within the MCI group. Results: Cognitive outcomes were directly associated with CSF Aβ42 and inversely with CSF tau measures. We found differences in both biomarkers and cognitive outcomes comparing all pairs except for CSF measures between cognitively impaired groups. The highest effect size was in memory tasks and biomarkers ratios. Lower performances were in memory and executive domains in MCI subjects with AD pathology (A+T+N±) compared to those with normal levels of AD biomarkers (A– T– N). Conclusion: This study provides further evidence of the validity of Spanish NEURONORMA cognitive battery to characterize cognitive impairment in the AD pathological continuum.

Hypertrophic pachymeningitis is an infrequent disorder that produces focal or diffuse thickening of the dura mater. It can be idiopathic or secondary to infectious, autoimmune or neoplastic processes. The recently described 'IgG4-related disease' could be the cause of many cases considered cryptogenic.A 54-year-old woman, with a history of bronchial asthma, presented with headache, dizziness and hearing loss on her left ear. The brain MRI study with gadolinium showed enhancement and thickening of the dura mater, extending from lateral wall of left cavernous sinus and medial temporal lobe to cerebellopontine angle and ipsilateral tentorium. CSF had 10 leukocytes/µL (90% mononuclear), with 1 g/L protein and without glucose consumption. Pathology showed fibrosis and lymphoplasmacytic infiltrate, with 16 IgG4+ plasma cells per high power field. The rest of analytical and microbiological studies were normal or negative. The plasma IgG4 rate was within normal limits. After treatment with steroids there was clinical improvement accompanied by the virtual disappearance of the alterations detected in neuroimaging.Hypertrophic pachymeningitis as a manifestation of IgG4-related disease can be diagnosed based on MRI findings if plasma IgG4 is elevated. In doubtful cases we must resort to meningeal biopsy. Corticosteroid therapy is usually effective and it is the first line treatment.Paquimeningitis hipertrofica secundaria a enfermedad relacionada con IgG4: descripcion de un caso y revision de la bibliografia.Introduccion. La paquimeningitis hipertrofica es un trastorno infrecuente que produce un engrosamiento focal o difuso de la duramadre. Puede ser idiopatica o secundaria a procesos infecciosos, autoinmunes o neoplasicos. La recientemente descrita 'enfermedad relacionada con IgG4' podria ser la causa de bastantes cuadros considerados criptogenicos. Caso clinico. Mujer de 54 años, con historia de asma bronquial, que consulto por cefalea, vertigo y perdida de audicion por su oido izquierdo. En la resonancia magnetica cerebral con gadolinio se objetivo engrosamiento y realce dural, que se extendia desde la pared lateral del seno cavernoso izquierdo y la parte medial del lobulo temporal al angulo pontocerebeloso y parte del tentorio homolaterales. El liquido cefalorraquideo presentaba 10 leucocitos/µL (90% mononucleares), con 1 g/L de proteinas y sin consumo de glucosa. El estudio anatomopatologico mostro fibrosis y un infiltrado linfoplasmocitario, con 16 celulas plasmaticas IgG4+ por campo de gran aumento. El resto de estudios analiticos y microbiologicos resultaron normales o negativos. La tasa plasmatica de IgG4 estaba dentro de los limites normales. Tratada con esteroides, se produjo mejoria clinica acompañada de la practica desaparicion de las alteraciones detectadas en la neuroimagen. Conclusiones. La paquimeningitis hipertrofica como manifestacion de la enfermedad relacionada con IgG4 puede diagnosticarse basandose en los hallazgos de la resonancia magnetica si la IgG4 plasmatica esta elevada. En casos dudosos, habra que recurrir a la biopsia meningea. La corticoterapia suele ser eficaz y representa la primera linea terapeutica.