title: [Chronic encephalopathy in patients with sex-linked agammaglobulinemia].
abstract: The clinical and neuroradiological findings of two patients with X-linked agammaglobulinemia, who developed a chronic encephalopathy, are presented. The main neurological manifestations in both patients were: progressive spastic tetraparesis, cortico-subcortical type of dementia and seizures. No infectious agent was identified in either patient. A systematic review of the clinical findings of 37 patients reported in the literature with X-linked agammaglobulinemia and chronic encephalopathy allows the distinction of two subgroups of patients according to their form of presentation (acute or insidious). In each subgroup there are significant clinical differences. The clinical-neuroradiological similarities between this complication and the ones derived from the vertically transmitted form of the human immunodeficiency virus are pointed out. Finally, emphasis is made on the need for CSF viral cultures on patients with X-linked agammaglobulinemia as soon as a neurological complication is suspected.
authors.affiliation: Servicio de Pediatría, Hospital Materno-Infantil Vall d'Hebron, Universidad Autónoma de Barcelona.
authors.forename: A
authors.initials: A
authors.lastname: Macaya Ruiz
authors.forename: M
authors.initials: M
authors.lastname: Roig Quilis
authors.forename: C
authors.initials: C
authors.lastname: Rodríguez Galindo
authors.forename: M
authors.initials: M
authors.lastname: Olesti Marco
authors.forename: A
authors.initials: A
authors.lastname: Castellote Alonso
authors.forename: J M
authors.initials: JM
authors.lastname: Bertrán Sanges
authors.forename: T
authors.initials: T
authors.lastname: Español Borén
mesh_terms: D015526:AIDS Dementia Complex / Q000175:diagnosis; D000361:Agammaglobulinemia / Q000150:complications* / Q000175:diagnosis; D001927:Brain Diseases / Q000175:diagnosis / Q000209:etiology*; D002648:Child; D002908:Chronic Disease; D003937:Diagnosis, Differential; D008040:Genetic Linkage; D015497:HIV-1; D006801:Humans; D008297:Male; D014960:X Chromosome
vernacular_title: Encefalopatía crónica en pacientes con agamaglobulinemia ligada al sexo.