Pick's disease has been estimated to occur with a frequency of 2% to 10% of that of AD, in fact, Pick's disease accounted for only approximately 5% of all progressive dementias. Although familial cases have been reported, most cases of Pick's disease occur sporadically.

Pick's disease usually has its onset between the ages of 40 and 60 years, but extreme cases have been reported with onset as early as 21 and as late as 80. In an analysis of 18 cases of Pick's disease average survival was reported to be 6.3 years for men and 8.4 year for women.

* In contrast to most large series studies, which suggest a slight predominance in affected women, recent reports suggests not only a male predominance but also an apparent male predominance in development of progressive degenerative dementia in first and second degree relatives with confirmed Pick's disease.

Clinical Features

Although early descriptions listed aphasia [loss of communication skills] and apraxia [loss of motor skills], modern clinical descriptions list the early and predominant features as changes in personality, insight, and judgment. As the disease progresses, cognitive processes [including memory and language functions] deteriorate. Specific language abnormalities include :the use of "all-purpose" or generic words, and the emergence of the so-called "gramophone syndrome" [repetitious retelling of stories].

MUTISM is frequently observed late in the course of this disease. Such alterations in behavior and cognition likely reflect the frontal lobe involvement in Pick's disease.

Like Alzheimer's, Pick's disease essentially remains a diagnosis of exclusion. Features that are suggestive of the diagnosis of Pick's disease include: behavioral abnormalities, relative absence of generalized intellectual decline, atrophy of the brain[CT or MRI] but a normal EEG.

Neurobiology

The characteristic pathology of Pick's disease is marked by circumscribed atropic changes in the cerebral cortex. Predominantly affecting the frontal lobe. At the microscopic level, the cerebral cortex suffers severe neuronal loss. Structures similar to Pick's disease have been reported in Down Syndrome,

* Swollen neurons are considered by some to be the most characteristic finding in Pick's disease.

Slowly Progressive Aphasia Syndromes

In l982, Dr, Mesulam described the clinical and language characteristics of six patients with a slowly progressive disturbance of language apparently without the additional intellectual and behavioral disturbances of dementia. Although cases of presenile dementia had been described previously before, these cases were apparently unique in that the majority did not experience any impairment in functional capabilities, or behavior, even after 5 to 11 years of follow-up.

Pick's disease although rare and difficult to establish as an antemortem diagnosis, patients often present disinhibited behaviors and language difficulty but with relatively intact memory and construction ability.

In other dementing illnesses such as Parkinson's disease and Pick's disease, the role of genetic factors remains to be determined. Although there are several documented cases of familial Parkinson's disease, studies have not shown a significant genetic risk. In some cases, Pick's disease may be inherited as a dominant trait.

Pick's disease, a neurodegenerative disorder of the frontal lobe causes language impairment. with word- finding difficulty in the early stages giving way later in the disease to mutism and echoing back of words.

Relation To Demographics

Several studies have linked language dysfunctions in AD to specific demographic variables, including age at onset and the rate of progression of the disease, and the presence of a family history of AD. Whether language impairment occurs more often in patients who develop AD before the age of 65 than in late-onset cases is a matter of controversy. with multiple reports of negative and positive findings. The specific language function under study may be important: naming and fluency seem to be impaired regardless of age of onset. One study found that auditory comprehension and writing may be especially vulnerable to disruption in early-onset cases. There is no evidence, however, of more extensive pathologic change in the left cortex in early-onset AD patients. In regard to rate of progression language impairment in the early stages has been associated with a more rapid course of the disease. This relation was found to hold even when dementia severity was assessed with a behavioral rating scale thought to be independent of language functions. Finally, it has been suggested that the familial form of AD is more strongly associated with language dysfunction than is the sporadic form.

From NORD

Next

Back to Picks directory

Back To Other Dementias Directory