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TitleNew Trends in the Diagnosis and Therapy of Non-Alzheimer’s Dementia
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Page 141

Clinical and pathological characteristics of PP A and FLD 139

would be prominent with dominant hemisphere frontotemporal atrophy.
Right frontotemporal atrophy is less frequently described than the left sided
one, but this may be related to a case finding bias due to the dramatic aphasic
symptomatology. Recent studies show the same pathology can be found with
progressive dominant hemisphere temporoparietal atrophy producing the
picture of "semantic aphasia" (Snowden et aI., 1992; Hodges et aI., 1992).
Descriptions of posterior cortical atrophy (Benson et aI., 1988) and right focal
parietal atrophy have less specific pathology and they have not been described
with Pick complex or Pick's disease yet. Alzheimer's disease (Pogacar and
Williams, 1984; Tariska, 1970) and Creutzfeldt-Jakob disease (Mandell et aI.,
1989) has been described as having focal presentation, such as PPA. However,
Pogacar and Williams' case had concomittant early memory change and this
does not fit the strict criteria of PP A. Some of the cases of Creutzfeldt -J akob
disease with PPA may have had Pick variant pathology, similar to one of our
cases of FLD published previously, as a case of familial spongiform enceph-
alopathy (Rice et aI., 1980). However, prion proteins were not found, and a
recent review of the pathology with modern histochemistry in this case also
revealed Pick complex. Other cases reported as AD with PP A had only senile
plaques and no neurofibrillary tangles, and the neuronal loss, gliosis and
superficial spongiosis were suggestive of Pick complex (Benson and Zaias,
1991).

In summary, it appears FLD, PPA, frontotemporal atrophies, circum-
scribed parietal atrophy, CBD, hereditary dysphasic dementia, "dementia
without specific pathology", and dementia with motor neuron disease may
have certain similarities clinically and pathologically with PD and all form a
spectrum that may be justifiably called "Pick's complex" (Kertesz et aI., 1994).
The expanded concept of PD has already gained acceptance in Europe by
some (Constantinidis et aI., 1974), although others are less willing to recognize
the cohesion of the spectrum. New biochemical and genetic evidence of the
relationship may allow less reliance on the pathological variations to provide
a link. The incidence of FLD is considered 15-25 % of dementia in various
series (with classical PD included). Similarly, in our estimation, PPA repre-
sents approximately 10% of patients seen in dementia clinics. Even consider-
ing that in some centres the same patient may be described as FLD and in
others as PPA, the combined incidence of the two presentations (recently
called frontotemporal atrophy) is likely to exceed 20-25 % of all degenerative
dementias. Therefore, Pick complex, including classical and atypical varieties
of PD, may be one of the largest nosological entities after AD to cause
dementia, particularly in the presenile age group. This may have important
implication in the future research of the biology and treatment of dementias.

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Page 282

SpringerN eurology

Peter Riederer, Wolfgang Wesenlann (eds.)

Parkinson's Disease:

Experimental Models and Therapy

1995. 121 figures. XI, 466 pages.

Soft cover DM 240,-, oS 1680,-

Reduced price for subscribers to "Journal of Neural Transmission":

Soft cover DM 216,-, oS 1512,-

ISBN 3-211-82749-8

Journal of Neural Transmission, Supplement 46

Current research on Parkinson's disease is aimed at the goal of determining the under-
lying cause of this terrible disease and of developing adequate treatment strategies to
deal with it. This volume focuses on models that mirror the progression of the symptoms
of Parkinson's disease (iron, MPTP, 6-hydroxydopamine, "TaClo", etc.) while other
topics are the evaluation of oxidative stress, calcium, excitotoxicity, nitric oxide, or nerve
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P,O.Box 89, A-120l \Vien • New York. NY 10010, 175 Fifth Avenue

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Page 283

Springer eurology

Kurt A. J ellinger, Cernol Ladurner~
Manfred ~Tindisc;h (eds.)

New Trends in the Diagnosis

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1994.33 figures. VII, 146 pages .

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Key Topics in Brain Research

Alzheimer's Disease (AD), the most frequent cause of mental decline in the elderly
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