Kenichi Meguro
Department of Geriatric Behavioral Neurology, Tohoku University Faculty of Medicine 2-1, Seiryo-machi,
Aoba-ku, 980-8575 Sendai, Japan. Correspondence mail: k-meg@umin.ac.jp.
ABSTRAK
Seiring dengan meningkatnya populasi usia lanjut, penurunan fungsi kognisi akibat penyakit neuro-
degeneratif atau stroke semakin umum ditemukan dan dianggap tidak dapat dihindari seperti halnya proses
menua. Pada demensia dan stroke, gangguan komunikasi akibat penurunan kualitas berbahasa merupakan
salah satu masalah utama. Neurologi perilaku bertujuan untuk menjelaskan hubungan antara fungsi otak dan
perilaku; penurunan kualitas berbahasa merupakan salah satu target utamanya, dan aplikasi klinisnya sungguh
bermanfaat dalam membantu membangun pemahaman yang lebih baik bagi pasien.
Bahasa merupakan serangkaian suara atau karakter yang membawa makna dalam komunikasi. Berdasarkan
perspektif evolusi, bahasa dapat dianggap berkaitan dengan struktur otak tertentu bagaikan anjing pemburu
yang mampu menunjukkan mangsanya dengan tepat. Struktur otak yang dimaksud adalah ganglia basalis dan
singulata anterior, serta talamus dan korteks serebri yang menjadi latar belakang neurobiologis. Manusia dapat
memanfaatkan bahasa. Area bahasa merupakan area otak yang baru berkembang dalam evolusi dan terutama
terletak di hemisfer otak sebelah kiri. Memori semantik juga telah berkembang pada manusia. Ada dua rute
memori, yakni memori permukaan dan memori dalam. Memori dalam berkaitan dengan arti dan melibatkan
tiga area otak, yakni area peri-silvian, area peri-peri-Silvian dan hemisfer kanan.
Dengan menggunakan prinsip tersebut, gejala bahasa pada demensia yang disertai afasia tak lancar
yang progresifn/(PA), demensia semantik/Semantic Dementia (SD) dan penyakit Alzheimer/Alzheimer Disease
(AD) serta demensia vaskular/vascular dementia (VaD) dapat dimengerti. Gejala PA dapat dipahami dengan
mempelajari disfungsi area bahasa peri-Silvian, sedangkan SD dan AD pada area bahasa peri-peri-Silvian dan
beberapa kasus VaD dan AD pada hemisfer kanan.
Kata kunci: bahasa, afasia progresif tak lancar, demensia semantic, penyakit Alzheimer, demensia vascular.
ABSTRACT
With increasing aging population, cognitive deteriorations due to neuro- degenerative diseases or stroke are
so commonly observed that it is thought to be inevitable with aging. For dementia and stroke, a communication
disorder due to language deterioration is one of the main problems. Behavioral neurology aims to clarify the
relationship between brain function and behavior; language deterioration is one of the main targets, and its
clinical applications are really useful for making better understanding of patients.
Language is sequences of sound or characters that carry meanings for communication. From the evolutionary
perspective, language can be thought about by considering birds and dogs: the basal ganglia and anterior
cingulate, and the thalamus and cerebral cortex are thought to provide the neurobiological background,
respectively. Humans can use language. The language area is a newly developed brain area in evolution and
is mainly localized in the left cerebral hemisphere. Semantic memory has also developed in humans. There are
two routes, the superficial and deep routes, with the latter associated with meaning, and three brain areas are
involved: the peri-Sylvian area, per-peri-Sylvian area, and right hemisphere. Using these principles, language
symptoms of dementia with progressive non-fluent aphasia (PA), semantic dementia (SD), Alzheimers disease
(AD), and vascular dementia (VaD) can be understood. Namely, the symptoms of PA is understood by the
dysfunction of peri-Sylvian language area, those of SD and AD by that of peri-peri-Sylvian language area, and
those of some VaD cases and AD cases by that of right hemisphere.
Key words: language, progressive non-fluent aphasia, semantic dementia, Alzheimers disease, vascular
dementia.
BASIC PRINCIPLES
Evolution
From the evolutionary perspective,
Figure 1. Basic principle 2: Rules of language
language can be thought about by considering
birds, dogs, and humans. Birds can repeat
human words; however; they clearly cannot
understand the language, and can only produce Dual Route
sounds. Neurobiologically, the basal ganglia and Language is a process of auditory or visual
anterior cingulate are thought to be associated input for the affecter, and speech or writing output
with this mechanism.3 Mammals such as dogs from the effecter, as shown in Figure 2. This
can perform attention-related sound learning". process is possible with or without accessing
Thus, a dog can discriminate sounds and show the semantic system. Speaking or reading aloud
body language by shaking its tail. The thalamus the sequence of sounds or written characters
and cerebral cortex are thought to provide the is possible without knowing the meaning. For
neurobiological background. Finally, humans example, German language can be easily read
can use language The language area is a aloud according to the regular pronunciation
newly developed brain area in evolution and is rules; however, it may be difficult to understand.
mainly localized in the left cerebral hemisphere. This is equivalent to repetition of human words
Semantic memory has also developed in humans. by a bird.
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He could not communicate well due to dysarthria to be a poor predictor of underlying histopathology.
and dementia. He was admitted to the hospital for They classified pathological material from
further evaluation and rehabilitation. 79 FTLD brains, blind to clinical diagnosis,
Neurological examination disclosed no according to topography of brain atrophy. There
remarkable findings except for dysarthria. He were highly significant relationships to clinical
had no buccofacial apraxia. Language skills syndrome. Atrophy was predominantly frontal
included relatively good comprehension and and anterior temporal in FTD, frontal markedly
good ability at naming daily items. He could asymmetric peri-sylvian in PNFA, asymmetric
repeat a word, but not a sentence. The Mini- bitemporal in SD. The findings demonstrate
Mental State Examination (MMSE)10 score was 3 predictable relationships between clinical
and the Cognitive Abilities Screening Instrument phenotype and both topographical distribution
(CASI)11 score was 13, with a perfect score of brain atrophy. The findings emphasize the
(10/10) on the Figure copying (double pentagon) importance of refined delineation of both
domain. clinical and pathological phenotype in furthering
An MRI examination disclosed a lacunar understanding of FTLD.
infarction in the right basal ganglia region with We here in present a case of SD and data
white matter changes. The frontal and temporal from patients with Alzheimers disease (AD)
lobes showed atrophic changes. Since the lacunar are discussed as examples of dysfunction of the
infarction was not associated with dysarthria, peri-peri-Sylvian language area.
we evaluated the atrophy in detail. The voxel- SD involves brain regions associated with
based specific regional analysis system for meaning (basic principle 1) and the semantic
AD (VSRAD) 12 images comparing the two system (basic principle 2). Areas 6 (posterior
hemispheres are shown in Figure 5. The left temporal lobe), 7 (anterior temporal lobe), and 9
precentral gyrus had significantly greater atrophic (frontal association area) (Figure 3) are mainly
changes compared with the right side. This area degenerated.
is considered to be associated with speech output. We previously reported a case of SD with
Dysarthria is classified as congenital multilingualism in a 75-year-old right-handed
(functional/organic) or acquired, with the Taiwanese woman.14 She had worked as a tailor
latter class including cerebrovascular diseases, before her illness, and had been able to speak
progressive non-fluent aphasia, frontotemporal Taiwanese, Japanese and Chinese fluently until
lobar degeneration, and amyotrophic lateral 5 years ago. She gradually had symptoms of
sclerosis. The clinical course and the location profound anomia and word-finding difficulty.
of stroke supported the diagnosis of progressive Her mother language was Taiwanese and she
non-fluent aphasia with lacunar infarction. had learned Japanese as her first symbolized
language. Although she had used Chinese for
most of her life, she depended on Japanese for
writing and reading, such as reading a newspaper
and keeping accounts. At the time of examination,
she could speak only very simple Taiwanese
and Japanese, and she could recognize simple
Japanese characters. We speculated that reading
and writing in Japanese seemed to have had a
greater impact on the semantic system in this
case.
It is well known that AD as per the NINCDS-
ADRDA diagnostic criteria15 involves mainly
Figure 5. MRI findings (VSRAD)
posterior brain regions16 associated with meaning
Dysfunction of the Peri-Peri-Sylvian Language (basic principle 2), the semantic system (basic
Area: Semantic Dementia and Alzheimer principle 3), and the pragmatic system (basic
Aphasia principles 2 and 3). Areas 5 (angular gyrus),
A clinicopathologic review on FTLD 13 6 (posterior temporal lobe), and 10 (right
reported that clinical phenotype is often assumed hemisphere) (Figure 3) are mainly degenerated.
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