Publicación Oficial de la Sociedad Ecuatoriana de Neurología, de la Liga Ecuatoriana Contra la Epilepsia y de la Sociedad Iberoamericana de Enfermedad Cerebrovascular
 

Artículo original

Progresive Dementia and Behavioral Changes: Pick’s Disease: A rare disease or an underdiagnosed disorder?

Autor: Carlos Valencia-Calderón, Ana Calderón-Valdiviezo

Rev. Ecuat. Neurol. VOL 10 Nº 1-2, 2001
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    Summary

    Pick’s disease is a nosological entity with frontal dementia, early cortical dementia with severe frontal lobe disturbances, absence of apraxia, and absence of gait disturbance at onset. This disorder is underdiagnosed in clinical practice. When specific criteria for the clinic, imaging and neuropathologic diagnosis including the presence of Pick bodies, are used, the diagnosis of Pick’s disease is achieved. However, taking into account that the definitive diagnosis of PD is achieved only with pathological study, in vivo diagnosis requires of the combination of neuroimaging techniques.

    More than 100 years ago, Arnold Pick described several patients who presented with progressive behavioral changes (apragmatism, outbursts of rage, and later stages, mutism) and who, at autopsy, had characteristic frontal or temporal lobar atrophy [1]. Alloys Alzheimer histologically characterized the disorder when he described “argentophylic globes” in the cytoplasm of neurons and the presence of ballooned neurons and spongy cortical wasting in the absence of neurofibrillary tangles or plaques [2]. Pick’s disease is considered a relatively rare neurodegenerative disorder, affecting subjects in their 60s with the progressive development of frontal lobe type features (e.g., difficulty planning, reasoning, abnormal social behavior), language disturbances (decreased fluency followed by echolalia, mutism), later followed by memory and gait abnormalities and occasional parkinsonism [3]. In autopsy studies of progressive dementia, only about 5% are due to PD. The underlying cause is not know, but there does appear to be a hereditary component, with clear autosomal dominant transmission in some families. The disease usually progresses inexorably over 2 to 5 years to death. At the present time there is no specific treatment available [4]. We report our findings in a patient with the purpose of to attract attention about this entity with the aim to avoid unnecessary, expensive and dangerous treatment when this kind of patients received a wrong diagnosis.


     
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