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Other frontotemporal dementias

Association

Pick 's disease (PiD) is a rare type of presenile dementia, neuropathologically confined to the prerolandic frontal convexity, orbitofrontal gyri, anteromesial temporal, and occasionally parietal lobes, despite the frequently documented more diffuse forebrain atrophy with striatal involvement at post-mortem examination. The salient lesions are chromatolytic neurons and Pick bodies (PB).  Ultrastructuraly, Pick bodies consist mostly of bundles of disorganized straight filaments, which may be mixed with coiled fibrils of 157 nm periodicity. Pick bodies are made up of aggregated tau proteins.

Tau component profile is characterized as a main 60 and 64 kDa doublet.

The Pick tau doublet (type III signature) is mainly observed in subcortical nuclei, hippocampus and frontal temporal regions

This biochemical signature results from the specific aggregation of tau isoforms without exon 10 (3R tauopathy)

The characteristic lower doublet (Tau 60 and 64 kDa) is mainly observed in the hippocampus and fronto temporal areas.

THE 3R AND 4R CLASSIFICATION

Tau classification


    Pick 's disease (PiD) is a rare type of presenile dementia, neuropathologically confined to the prerolandic frontal convexity, orbitofrontal gyri, anteromesial temporal, and occasionally parietal lobes, despite the frequently documented more diffuse forebrain atrophy with striatal involvement at post-mortem examination. The salient lesions are chromatolytic neurons and Pick bodies (PB).  Ultrastructuraly, Pick bodies consist mostly of bundles of disorganized straight filaments, which may be mixed with coiled fibrils of 157 nm periodicity. Pick bodies are made up of aggregated tau proteins.

    1.    General outline
    Pick 's disease (PiD) is a neurodegenerative disorder that belongs to the group of "fronto-temporal dementia". This is a rare type of presenile dementia, with sometimes a familial character.


    2.    Synonyms


    3.    Course (Evolution of the disease)
    One of the first and most important warning signs of FLD is insensitivity to other people. This could be linked to difficulty in identifying emotions shown by their relatives. Other clinical signs related to frontotemporal degeneration will occur progressively such as steréeotypies (repetition of words or activities), impaired jugementand abstraction skills, behaviour problems.Attention and langage are particularly affected.

    4.    Caregiver problems
    Those linked to frontotemporal dementia, specially related to behaviour problems.

    5.    Aetiology (Causes and risk factors)

    A specific neurofibrillary degeneration, revealed by the presence of Pick bodies in the hippocampus and frontotemporal cortex and an abnormal processing of tau proteins,  is associated to this pathology.

    6.    Diagnostic procedures

    Clinical with the specific specific features of frontotemporal impairment. Imaging reveals a frontotemporal atrophy and hyperperfusion.

    7.    Treatment and rehabilitation
    See frontotemporal dementia

    8.    Ongoing research
    Like most frontotemporal dementia, tau proteins seem to be involved in the aetiology of the disease. Many laboratories are working on the molecular pathology of tau proteins.

    9.    Available services
    In France, an association is devoted to Frontotemporal dementia (Dr Lebert and Pasquier, Bailleul, France)

    10.    Further reading



Ann Neurol 1998 Feb;43(2):193-204

Vulnerable neuronal subsets in Alzheimer's and Pick's disease are distinguished by their tau isoform distribution and phosphorylation.

Delacourte A, Sergeant N, Wattez A, Gauvreau D, Robitaille Y


DELACOURTE A, ROBITAILLE Y, SERGEANT. N, BUEE L, HOF P, WATTEZ A, LAROCHE-CHOLETTE A, MATHIEU J, CHAGNON P, GAUVREAU D. Specific pathological tau protein variants characterize Pick's disease. J. Neuropathol. Exp. Neurol.1996;55:151-159.)

More information

 

Association "Frontotemporal degeneration"

English version (since avril 1998)

Introduction: « Les degenerescences frontotemporales » is a group from Lille devoted to frontotemporal dementia, and which is regularly publish ing Newsletters in French. Contact: Dr Florence Lebert, Centre Médical des Monts de Flandre, 59270 Bailleul, France.

Bulletin 3:

Difficulty in identifying emotions from the expressions of other people. One of the first and most important warning signs of FLD is insensitivity to other people. This could be linked to difficulty in identifying emotions shown by their relatives. They can see something is different but are unable to link this change of attitude to a specific feeling. Therefore they take refuge in what appears to be insensitivity. Dr I. Lavenu, doctor at the memory Assessment Center at University in Lille is working on this subject and her preliminary findings are:A test was designed for people with FLD. They were shown seven pictures of faces expressing different emotions (happiness, anger, haughtiness, disgust, fear, surprise and sadness). The subjects could detect the presence of an emotion on the face but experienced difficulty in connecting emotion and its meaning.One way to help the sufferers could be to concentrate on the spoken message, the intonation and the body langage of the speaker.

News: The opening of a specific unit for FLD patients at the Centre Medical des Monts de Flandre (Bailleul - France)

Hopefully this spring the Bailleul Center is planning to open a unit for FLD sufferers. After four years experience with Alzheimer's disease, cortical Lewy Body Disease and FLD the need for such a unit has emerged from the specific behaviour and lower age of such sufferers. Their care and social needs are different. They need more stimulation, a direct way of talking, more protection against their own behaviour which may endanger themselves. One must also accept their loss of social abilities and insensitivity to others and not criticize them.


8/01/05
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