Understand what is Primary Progressive Aphasia, a neurodegenerative disease still poorly understood by science.

Understand what is Primary Progressive Aphasia, a neurodegenerative disease still poorly understood by science.

Dementia syndrome that affects language is the subject of a study by IDOR, which is in the process of recruiting patients.

Dementia is a neurodegenerative condition comprising a large number of diseases. Despite being grouped under the same term and having some characteristics in common, there are dozens of diagnoses of dementia, and the recognition of the particularities and correct designation of each clinical case is crucial for adequate therapy and the life quality of the patient and their relatives. 

A diagnosis that covers 5% to 10% of cases of dementia syndromes is frontotemporal dementia (FTD), which is associated with an anomalous protein deposit in the brain, and mainly affects the behavior, personality, and language of individuals, differing from the popular association that these diseases would always be related to memory, as happens with Alzheimer’s disease. 

FTD symptoms begin in most cases between the ages of 45 and 65, but they can also manifest earlier or later, and in some cases, the disease can also affect the muscles of the head and neck. They have segmented into three clinical variants: one behavioral variant and two variants of Primary Progressive Aphasia (PPA), which are the grammatical and semantic variants. However, PPA is a more complex neurological syndrome, which also has two other variants that do not necessarily fit into FTD: the logopenic variant and the mixed variant, which encompasses aspects of more than one clinical subdivision of APP. See the differences below: 

Behavioral Variant FTD: As the name implies, the most notable feature of this subtype is a sudden change in behavior in the patient. “A person who has never had habits such as playing games of chance may suddenly become addicted or other compulsive activities that had never aroused interest before. He can also become a more talkative person and very uninhibited conversations, even reaching a socially inconvenient behavior”, illustrates Dr. Paulo Mattos, psychiatrist researcher at the D’Or Institute for Research and Education (IDOR) and the Center for Applied Neuropsychology (CNA). There are also cases where patients become apathetic and lose interest in their family and social activities. 

PPA Grammatical Variant: All PPA expressions present linguistic problems, but this subdivision is marked by the effort to verbalize words, which often sound incorrect or with unconventional pronunciation, in addition to very evident grammatical errors. 

PPA Semantic Variant: Patients in this division show difficulty in abstraction and in understanding more complex sentences. They often “lose” words, seek to refer to objects and verbs. When asked to draw a turtle, for example, they may forget to represent striking features, such as the animal’s shell, due to the loss of the word’s meaning. 

Logopenic PPA Variant: This variant is commonly associated with Alzheimer’s disease and does not qualify as FTD. The patient has no pronunciation or semantic difficulties. The difficulty in finding words to conclude thoughts is marked, which can make the speech laconic. 

Mixed Variant: Some patients have symptoms of more than one type of PPA, classified as a mixed subdivision. 

The correct designation of the PPA variant is one of the most challenging aspects of diagnosis. In fact, the diagnosis of PPA, itself is very difficult and usually late, because many of the behavioral changes of the disease can be initially interpreted as psychiatric problems, such as bipolar disorder, obsessive-compulsive disorder, and depression. 

A neuroimaging exam is necessary to understand whether or not a neurodegenerative process is taking place in the patient, and if this clinical condition is identified, it is still necessary to perform other neuropsychological exams and evaluations to differentiate other neurodegenerative diseases, such as dementia with Lewy bodies and Alzheimer’s. “The main health professionals who identify that patients may have PPA are neurologists, geriatricians, psychiatrists, and speech therapists. The latter tend to quickly identify the problem, but a hindrance is a fact that many clinics that offer the service do not serve adults or elderly people”, comments Dr. Paulo Mattos. 

At the moment, research focused on the PPA is being carried out at IDOR. One of the main goals of the study is better understand the disease and improve the classification of its variants. The study is recruiting volunteer patients residing in city of Rio de Janeiro or neighborship, who can be referred by their doctors and family members. For registration, it is necessary to send the patient’s name and age, the name of the referring family member, and the contact telephone number to the e-mail: neuropsicologia@idor.org. Participation in the study will involve filling out questionnaires, detailed neuropsychological and speech-language pathology assessments, and carrying out blood tests and magnetic resonance imaging.


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