Annals of Clinical and Experimental NeurologyAnnals of Clinical and Experimental NeurologyАнналы клинической и экспериментальной неврологии2075-54732409-2533Eco-Vector58110.25692/ACEN.2019.1.9Clinical analysisКлинический разборUnknownDifficulties of clinical diagnosis in primary progressive aphasia. Clinical observationСложности дифференциальной диагностики первичной прогрессирующей афазии. Клиническое наблюдениеBarantsevichYevgeniy P.БаранцевичЕвгений Робертович
Russian Federation
ejvcons@mail.ruKoval’chukYuri P.КовальчукЮрий Павлович
Russian Federation
ejvcons@mail.ruMel’nikYekaterina V.МельникЕкатерина Валерьевна
Russian Federation
ejvcons@mail.ruEmanuel’Vladimir S.ЭмануэльВладимир Сергеевич
Russian Federation
ejvcons@mail.ruEmanuel’Yulia V.ЭмануэльЮлия Владимировна
Russian Federation
ejvcons@mail.ruAcademician I.P. Pavlov First St. Petersburg State Medical UniversityФГБОУ ВО «Первый Санкт-Петербургский государственный медицинский университет им. акад. И.П. Павлова»15032019131788317032019Copyright ©; 2019, Barantsevich Y.P., Koval’chuk Y.P., Mel’nik Y.V., Emanuel’ V.S., Emanuel’ Y.V.Copyright ©; 2019, Barantsevich Y.P., Koval’chuk Y.P., Mel’nik Y.V., Emanuel’ V.S., Emanuel’ Y.V.2019Barantsevich Y.P., Koval’chuk Y.P., Mel’nik Y.V., Emanuel’ V.S., Emanuel’ Y.V.Barantsevich Y.P., Koval’chuk Y.P., Mel’nik Y.V., Emanuel’ V.S., Emanuel’ Y.V.https://creativecommons.org/licenses/by/4.0https://annaly-nevrologii.com/pathID/article/view/581

Primary progressive aphasia is a syndrome characterized by progressive speech dysfunction. There are three types of this condition. The first — agrammatic — type of primary progressive aphasia is typical for frontotemporal dementia and characterized by progressive worsening of speech during several years without other neurological symptoms. For this type, alexia is typical (can be present before the onset of other clinical symptoms) along with agraphia and oral apraxia.

Second type of primary progressive aphasia, semantic aphasia, may also be present in frontotemporal dementia.

The third type of primary progressive aphasia is a logopenic variant which is a manifestation of the atypical variant of Alzheimer’s disease. In this type, the speech disorders are prevailing over the mnestic manifestations. Differential diagnosis in that case is difficult given lack of specific markers in patients with Alzheimer’s disease.

As a clinical illustration, we present a case of a 50-year-old patient. She has been considering herself ill from the age of 45 years, when her speech impairment emerged and started to progress; she also started to experience difficulties in recalling and correct pronunciation of the words. No significant pathologies in internal organs were identified. Her neurological examination showed pyramidal insufficiency more on the right, slight muscle rigidity in the limbs, acheirokinesis, and mild ataxia. Examination of patient’s higher cerebral functions showed mixed aphasia (amnestic and motor), frequent agrammatisms, paraphasias, anomias, apraxia more in the left arm, simultaneous agnosia, partial hemispatial neglect of the left side, alexia, agraphia, acalculia, and finger agnosia. Frontal Assessment Battery test score was 8 and Mini Mental State Examination score was 16. Vascular, infectious, metabolic, autoimmune, tumorous, and iatrogenic causes of dementia have been ruled out. Speech disturbances were typical for agrammatic variant of primary progressive aphasia, but didn’t contradict with logopenic type of Alzheimer’s disease. The PET scan showed no hemispheric asymmetry. We suggested that the most probable diagnosis could be primary progressive aphasia, which can be a manifestation of both frontotemporal dementia and Alzheimer’s disease. Moreover, the differential diagnosis based on clinical manifestations only, without specific PET investigation or specific CSF study is rather difficult.

Первичная прогрессирующая афазия — синдром, проявляющийся постепенным нарушением речевой функции. Выделяют три формы первичной прогрессирующей афазии. Первая форма характерна для лобно-височной деменции и проявляется нарастанием речевых нарушений при отсутствии другой неврологической симптоматики в течение нескольких лет. Вторая форма — семантическая афазия, когда пациент не может вспомнить функциональную принадлежность предмета, а также развивается неузнавание лиц или голосов. Третья форма — логопеническая, атипичный вариант болезни Альцгеймера, при котором нарушения речи превалируют над мнестическими проявлениями заболевания.

В статье представлено клиническое наблюдение за пациенткой 50 лет с диагнозом первичной прогрессирующей афазии, которая может иметь место и при лобно-височной деменции, и при болезни Альцгеймера. На основании клинической картины трудно проводить дифференцированный диагноз без специфического позитронно-эмиссионного исследования или специфического анализа ликвора. На фоне проводимой терапии улучшения были невелики, но состояние пациентки стабилизировалось.

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