Pick Disease
- Author: Anna M Barrett, MD; Chief Editor: Michael Hoffmann, MBBCh, MD, FCP(SA), FAAN, FAHA more...
Background
Pick disease (named after Arnold Pick) is a progressive dementia defined by clinical and pathologic criteria. Unlike Alzheimer disease, which typically presents with impairment of recent memory associated with entorhinal cortex and hippocampal dysfunction, Pick disease typically affects the frontal and/or anterolateral temporal lobes. (See Etiology.)
First described in 1892, with the defining pathologic characteristics first reported by Alois Alzheimer in 1911, Pick disease is now considered by some to be part of a complex of neurodegenerative disorders with similar or related histopathologic and clinical features. (See Presentation and Workup.)[1, 2]
Frontotemporal dementia
Pick disease is one of the disorders classified under the term frontotemporal dementia. Frontotemporal dementia can be pathologically classified as (1) the microvacuolar type, which is characterized by loss of cortical neurons, with minimal gliosis, and (2) the tau-positive type, which is marked by loss of cortical neurons, with minimal microvacuolation and related gliosis. (See Etiology and Workup.)[3]
Frontal lobe dementia is a term signifying neuropsychological features localizing to the frontal lobes. Clinically, Pick disease may be identical or very similar to frontal lobe degeneration.[4]
Some cases diagnosed premorbidly as Pick disease are shown pathologically to be progressive subcortical gliosis.[5] Other cases may be diagnosed pathologically as dementia lacking distinctive histopathology.[6] A clinical/genetic nosology includes frontotemporal dementia linked to chromosome 17. (See Presentation and Workup.)[7]
Primary progressive aphasia is a focal atrophy syndrome that may be associated with Pick, Alzheimer, or other pathology; clinically the deficit appears restricted to the frontal and/or temporal lobes.[8]
Images of neurodegenerative findings can be viewed online at Internet Pathology Laboratory, University of Utah, CNS Degenerative Diseases.
Cellular characteristics
Pick disease is defined pathologically by severe atrophy, neuronal loss, and gliosis. Classified as a tauopathy, Pick disease is always accompanied by the occurrence of tau-positive inclusions.[9] Swollen (ballooned) neurons (Pick cells) and argentophilic neuronal inclusions, known as Pick bodies,[10] disproportionally affect the frontal and temporal cortical regions. (See Workup.)
Fewer Pick bodies may be present in these regions if the primary symptoms are behavioral (behavioral variant), compared with the primary symptoms of aphasia.[11]
In a clinicopathologic series, only 5% of patients with clinically diagnosed frontotemporal dementia had classic Pick disease with Pick bodies at postmortem evaluation. (See Epidemiology.)[12]
Patient education
For patient education information, see the Brain and Nervous System Center, as well as Pick Disease and Dementia Medication Overview.
Etiology
The specific cause of Pick disease is unknown. As many as 50% of patients with frontotemporal dementia have a positive family history of dementia and inherit frontotemporal dementia as an autosomal dominant trait with high penetrance.[3]
In families with an inherited frontal lobe dementia (some of which have been found to be pathologically or clinically indistinguishable from Pick disease), linkage to markers on band 17q21-22 coding tau protein and chromosomes 3p, 9, 9p, and 17q has been reported, as have presenilin-1 mutations on band 14q21.[7, 3, 13, 14]
These familial disorders are heterogenous in different family members. Some members may present primarily with amyotrophy, and others may present with primary supranuclear gaze palsy, parkinsonism, schizophrenialike thought disorder, or progressive aphasia and/or apraxia.
Goldman and colleagues published a useful algorithm for decision making in the ordering of genetic studies, based on symptom presentation and presence or absence of family history.[15]
Epidemiology
Occurrence in the United States
Frontotemporal dementias as a group are the fourth most common cause of dementia. In most parts of the United States, among patients younger than 60 years, the frontotemporal dementias are the first or second most common cause of dementia. In patients older than 60 years, the incidence and prevalence of Alzheimer disease begins to take off, with Alzheimer disease becoming by far the most prevalent form of dementia.[12]
Pick disease is sometimes used to refer to the clinical phenotype of the frontotemporal dementias. This group of disorders has a variety of pathologic substrates, the most prevalent of which is frontotemporal lobar degeneration with ubiquitin inclusions. In one pathologic series, Pick disease itself (as defined by the presence of tau-positive, silver staining, cytoplasmic inclusions) accounted for only 5% of all cases of frontotemporal dementias.[12]
International occurrence
Familial forms of Pick disease may occur more frequently in Europe (particularly in Scandinavian nations). The estimated frequency ranges from 7-43 cases per 100,000 population.[16] In a study in the Netherlands, the prevalence was 28 per 100,000 persons.[3]
Prognosis
Like most dementias, Pick disease is slowly progressive, leading to increased vocational and personal disability.
Some patients can progress slowly over extremely long periods. Some may develop artistic or other talents during the course of their dementia, a phenomenon that is perhaps related to disinhibition of "creative" brain areas. Musical or artistic tastes also may change (eg, the patient may develop a sudden interest in music intended for much younger listeners).
Some patients may be capable of acquiring new knowledge or skills, such as the use of a computer-assisted, simple communication system.
Race-, sex-, and age-related demographics
Familial forms of Pick-complex dementias, linked to chromosome arm 17q, may be particularly common in people of Scandinavian origin/descent. It may represent as many as 17% of dementias in this population. [#IntroductionAge]
Pick disease occurs in a younger age group than dementia of the Alzheimer type, with peak incidence occurring in individuals aged 55-65 years. More men than women may be affected by Pick disease.[17, 18]
Morbidity and mortality
Pick disease runs a shorter course than Alzheimer disease, on average about 6 years.[17, 19] In some individuals whose main symptoms are a disturbance of speech and language (primary progressive aphasia), the clinical course can be slow. In one small series, these patients survived an average of 5 years longer than patients with behavioral symptoms (behavioral variant).[11] A patient with primary progressive aphasia may preserve the ability to function at home for 10 or more years after onset.
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