In the offered article we discussed rare clinical syndrome – Primary progressive aphasia (PPA). PPA or Progressive motor
Primary progressive aphasia (PPA) or progressive motor aphasia is one of the 3 clinical syndromes (progressive
Sometimes, it can develop as the initial symptoms of corticobasal degeneration, Parkinsonism and motor neuron disease.
Description of the PPA as a new nosological entity for the first time was described by M. Mesulam in 1982 in his article named
“Slowly progressive aphasia without generalized dementia” . In 1998, Neary and et al. developed the diagnostic criteria for
clinical syndromes encountered in FTLD .
Epidemiology. By population-epidemiological studies, the epidemiology of FTLD has been studied in several countries. In
Netherlands (Zuid-Holland district), FTLD spread is 2.7 cases per 100,000, and 9.4 cases among people aged 60-69 . In
United Kingdom (Cambridge), this indicator has been 15.1/100,000 among the population over the age of 65 . And this is
in similar level to the prevalence of Alzheimer's disease among the elderly population. Among people aged 45-64
(3.5/100,000), Alzheimer's disease is less common (4.2/100,000) . In the United States (Minnesota, Rochester), both FTLD
and the prevalence of Alzheimer's disease has been the same among people aged 50-59: 3.3/100,000 .
by at least one of the symptoms such as agrammatism, phonemic paraphasia and amnestic aphasia, remains isolated for at
least 2 years, and the main symptom of the disease as it progressing. If, at the beginning of the disease, the speech pathology
shows itself as stammering, falter speech, speech-offs, speech tempo changes, and literal paraphasias, later outrage motor or
sensomotor aphasia develop. After the primary aphasia contraction, for a few years, other neurological symptoms are not
observed, but later disorders of speech are added by behavioral, memory, executive functions and cognitive distortions. In
PPA suffering patients, ideomotor apraxia (usually buccofacial), dyscalculia etc. (Joshi et al. 2003) can be found, in which it is
recommended to use the term “PPA-plus” (Mesulam 2001). The differential diagnosis should be carried out on expected
Alzheimer's disease and the AGD with relative memory preservation.
Neurovisualization. In the PPA, cortical atrophy with involvement of the frontal, temporal, and parietal components of
speech net in the left hemisphere is observed.
controlled study, the effect of dopamine agonist bromocriptine has not been proven. According to some scholars, treatment
with trazodone (Lebert et al. 2004), rivastigmine (Moretti et al. 2004), and selegiline (Moretti et al. 2002) can give positive
results. Finocchiaro et al. (2006) have indicated the positive results of the efficiency of
high frequency repetitive transcranial
observations. When he consulted to doctor for the first time (2008), he had complaints of faltering, troubles to find words
when speaking. The patient considered himself to be ill for more than 1 year. The patient had been diagnosed of cerebral
atherosclerosis and treated accordingly. A year later, the patient re-consulted to a neurologist and worsened speech was
History. For many years, he has worked as a policeman. He was treated with medicamentous therapy with a diagnosis of
chronic cholecystitis. He has never had a surgical operation. According to his wife, his parents lived more than 90 years and
did not suffer from a similar disease, and died from coronary heart disease. The patient has 3 brothers and 2 sisters. A sister
died in 80-year-old of acute myocardial infarction. Any similar pathological process hasn’t been observed in any of them.
Any gross pathology by the circulatory system, urinary system, respiratory system hasn’t been observed. When he was in the
examination of neurologist for the first time, weak accommodation and convergence disorder, agrammatism and amnestic
aphasia were observed.
Examinations. General blood, urine examination has been normal. Gross deviations from the norm haven’t been observed in
biochemical blood examination. Through ECG, left ventricular hypertrophy has been found. Rentgenoscopy of the thorax has
been with no pathology. LP has been with no pathology.
In native, daytime EEG recorded slowing the basic rhythm around Convex, in the left fronto-parietal-temporal region
revealed high-amplitude θ-potentials (Fig. 1).
Figure 1 - Electroencephalography of the patient.
In ultrasound examination of the extracranial cerebrovascular system moderate stenosis was found (Figure 2).
Figure 2 - Ultrasound examination of extracranial arteries, moderate stenosis (<50%).
Through MRI of brain, progressive circumscribed cerebral atrophy was found (Figure 3, 4, 5.).
Figure 4 - MRI examination of the patient with primary progressive aphasia (PPA). Frontal incision. T2 mode.
(0:0:1.25). As the drugs gave no effects, the patient spontaneously stopped taking it.
About a year later (2009), the patient was re-consulted to neurologist with deepened amnestic aphasia and agrammatism.
Gnosis and praxis were kept. The patient was re-prescribed of the previous treatment and galantamine (5mg:0:5mg) was
added. However, the patient did not follow the treatment for long-term.
Nearly 2 years after the start of symptoms of speech disorders for the first time (2010), through repeated examination, rough
sensomotor aphasia, agraphia was observed in the patient.
Course of the disease is compatible with the course of PPA. However, after two years of onset first symptom (speech
problem), other cognitive problems appear – agrammatism, agraphia.
Mesulam MM. Slowly progressive aphasia without generalized dementia. Ann. Neurol. 1982. – №11. – Р.592-598.
Neary D., Snowden J.S., Gustafson L., et al. Frontotemporal lobar degeneration: a consensuson clinical diagnostic criteria.
prevalence estimates from a population-based study. Brain. 2003. - № 126. – Р. 2016-2022.
Ratnavalli E, Brayne C, Dawson K, et al. The prevalence of frontotemporal dementia. Neurology. – 2002. - № 58. – Р.1615-
Kingdom. Neurology.2008, - № 71. – Р.1496-1499.
Knopman DS, Petersen RC, Edland SD, et al. The incidence of frontotemporal lobar degeneration in Rochester, Minnesota,
БІРІНШІЛІК ҮДЕМЕЛІ АФАЗИЯНЫҢ КЛИНИКАЛЫҚ ЖАҒДАЙЫ
Түйін: Мақалада сирек кездесетін синдромдардың бірі берілген – біріншілік үдемелі афазия синдромы (БҮА). БҮА
немесе үдемелі моторлы афазия маңдай-самай бөлімдерінің дегенерациясында кездесетін 3 клиникалық
синдромның (маңдай-самайлық деменция, БҮА, семантикалық деменция) бірі болып табылады. Біздің
бақылауымызда БҮА диагнозымен 76 жасар ер адам болды. Клиникалық ағымы және зерттеу нәтижелері БҮА
ағымына сәйкес келеді.
прогрессирующей афазии (ППА). ППА или прогрессирующая моторная афазия является одним из 3 клинических
синдромов (лобно-височная деменция, ППА, семантическая деменция), встречаемых при дегенерации лобно-
височных долей. Под нашим наблюдением находился больной мужчина 76 лет, с диагнозом ППА. Клиническое