[Cognitive disorders in patients with Huntington's disease]. / Trastornos cogitivos en pacientes con enfermedad de Huntington.

INTRODUCTION: Huntington s disease has been clearly recognized since 1872. It is characterized by the appearance of a motor disorder (chorea) accompanied by selective cognitive deterioration which is not interfered with by aging. DEVELOPMENT: Since the genetic defect causing it has been identified (expansion of CAG triplets on the IT 15 gene of chromosome 4) it has been possible to study families from the preclinical stage (asymptomatic persons who carried the mutation) to the stage of full development of the clinical syndrome. The behaviour disorders most often seen in these patients are depression, mania, schizophrenia, paranoia, anxiety, obsessive and obsessive compulsive disorders although other disorders may also occur. Different clinical forms of motor disorders and dementia have been reported in relation to the age of onset of the symptoms. The commonest cognitive symptoms are defects of attention, memory, planning, sequencing and visuo spatial deficits, as occurs in subcortical dementia. Our objective is to present the different clinical forms of the disorder according to the age of presentation, implication of the symptoms, severity of the genetic defect (number of CAG triplets) and to describe the study made of asymptomatic carriers so as to be able to detect incipient cognitive deterioration in these persons.

[Dementia in progressive supranuclear paralysis patients]. / Demencia en pacientes con parálisis supranuclear progresiva.

INTRODUCTION: Progressive supranuclear paralysis is difficult to detect in its initial stages. Its symptoms are not specific and often the patients are mistaken as Parkinson s disease patients, or even Alzheimer s ones. DEVELOPMENT: Initial features in progressive supranuclear paralysis are typically a gait disorder with frequent falls, behavioral disorder and often dysarthria. The gaze disorder, showing voluntary conjugate gase paralysis is characteristic, but it can appear late in the course of disease. Other motor signs are axial rigidity, bradykinesia, stiffness, and rarely chorea, ataxia, muscle weakness or myoclonus. Pseudobulbar paralysis is the more remarkable clinical finding. These motor symptoms are accompanied by cognitive disturbances, the typical ones of subcortical dementia, with mind slowness, apathy, depression, and, more often that in other primary dementias, psychotic features and relatively early behavioral disorders. A useful feature to distinguish the mental changes of progressive supranuclear paralysis with other primary subcortical dementias is a more remarkable impairment in visuospatial function, because of the need of a good gaze function to accomplish it. Progressive supranuclear paralysis patients work better in verbal tests that in visuospatial test. We try to describe clinical data and evolutive profile of this entity, whose cognitive disturbances as described by Albert in 1974 strongly stabilized the concept of subcortical dementia.

[Amusias]. / Las amusias.

INTRODUCTION: We review the definition of the amusias and the cultural components involved in musical experience. We review the characteristics of musical language, perception and performance of it together with reading and writing it. DEVELOPMENT: We analyze the above data and significant clinical cases. We discuss whether in evaluation of the amusias it may be relevant to have had been taught music, since in this condition the functional specialization of the cerebral cortex has different topography of the lesions in persons who have or have not had musical education. CONCLUSIONS: We consider that in persons who have studied musical language, amusia should be evaluated as a second language, and its pathology as aphasia for musical language. In persons without musical education the dysperceptive amusias are a purely agnostic phenomenon with neuropsyschological characteristics which are different to those seen in persons who have had an education in music.

[Toxoplasmosis of the central nervous system in immunodepressed patients. Our experience]. / Toxoplasmosis del sistema nervioso central en enfermos inmunodeprimidos. Nuestra experiencia.

Seven patients carriers of a disease capable of provoking immunodeficiency (six of them suffering AIDS and one case of Hodgkin type lymphoma) are reported. All of them presented neurological complications consisting of toxoplasma Gondii infection of the Central Nervous System. We summarize their evolution and the diagnostic and therapeutical approaches.

[Calcification of the basal ganglia and hypoparathyroidism: Fahr disease. Study of a family]. / Calcificación de los ganglios basales e hipoparatiroidismo: enfermedad de Fahr. Estudio de una familia.

We studied three members of a single family who had basal ganglia calcifications (BGC): two with BGC alone and one associated with parkinsonism and hypoparathyroidism (H). This family links together BGC and H and may help resolve the apparent controversy about relationship of both.