[Language impairment in amyotrophic lateral sclerosis]. 2010

Hiroo Ichikawa, and Mitsuru Kawamura
Department of Neurology, Showa University School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan.

For a long time, amyotrophic lateral sclerosis (ALS) was thought to be a neuro-degenerative disease with selective involvement of the motor neuron system. However, it has recently been established that ALS is a multisystem disorder that not only involve the motor system but also affects cognition. A typical cognitive impairment in ALS is frontotemporal dementia: a clinical subtype of frontotemporal lobar degeneration (FTLD). Furthermore, language impairment, including progressive non-fluent aphasia (PA) and semantic dementia (SD), which are also FTLD subtypes, have been linked to bulbar-onset ALS. Beside recent studies a Japanese paper published in 1893 gives an account of aphasia in bulbar-onset ALS. Interestingly, this was the first account of aphasia in Japan. Thus, language-related problems in ALS may have been overlooked, because evaluation of aphasic problems in ALS patients is difficult, mainly because of progressive bulbar or pseudo-bulbar palsy that results in and dysarthria. From a clinical point of view, progressing bulbar symptoms and medical interventions, such artificial ventilation make it difficult to evaluate language functions in ALS. However, we do observe frequent omission and paragraphia of kana letters, and syntactic errors in writing. Interestingly, some patients make exhibit errors exclusively in the case of kanji characters. Thus, evaluation of writing samples is important if order to identify language problems in ALS patients. The findings may also provide additional information such as dissociation between errors made in kana and kanji characters. The characteristic features of writing errors are thought to reflect the nature of aphasia including PA and SD, that are easily masked by dysarthria. In addition, writing errors can appear as "isolated agraphia" without aphasia and dementia. Pathologically, writing errors should indicate the brain regions involved by ALS, e.g., hemispheric dominance and frontal or temporal lobe involvement. In addition, selective involvement of Exner's writing center in the frontal lobe may be responsible for "isolated agraphia". Hence, further studies are required to determine the clinical significance of writing errors in ALS patients and their pathological correlation.

UI MeSH Term Description Entries
D007806 Language Disorders Conditions characterized by deficiencies of comprehension or expression of written and spoken forms of language. These include acquired and developmental disorders. Acquired Language Disorders,Language Disorders, Acquired,Acquired Language Disorder,Language Disorder,Language Disorder, Acquired
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000381 Agraphia Loss or impairment of the ability to write (letters, syllables, words, or phrases) due to an injury to a specific cerebral area or occasionally due to emotional factors. This condition rarely occurs in isolation, and often accompanies APHASIA. (From Adams et al., Principles of Neurology, 6th ed, p485; APA, Thesaurus of Psychological Index Terms, 1994) Dysgraphia,Pure Agraphia,Acquired Agraphia,Acquired Dysgraphia,Constructional Agraphia,Developmental Agraphia,Developmental Dysgraphia,Acquired Agraphias,Acquired Dysgraphias,Agraphia, Acquired,Agraphia, Constructional,Agraphia, Developmental,Agraphia, Pure,Agraphias,Agraphias, Acquired,Agraphias, Constructional,Agraphias, Developmental,Agraphias, Pure,Constructional Agraphias,Developmental Agraphias,Developmental Dysgraphias,Dysgraphia, Acquired,Dysgraphia, Developmental,Dysgraphias,Dysgraphias, Acquired,Dysgraphias, Developmental,Pure Agraphias
D000690 Amyotrophic Lateral Sclerosis A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94) ALS - Amyotrophic Lateral Sclerosis,Lou Gehrig Disease,Motor Neuron Disease, Amyotrophic Lateral Sclerosis,Amyotrophic Lateral Sclerosis With Dementia,Amyotrophic Lateral Sclerosis, Guam Form,Amyotrophic Lateral Sclerosis, Parkinsonism-Dementia Complex of Guam,Amyotrophic Lateral Sclerosis-Parkinsonism-Dementia Complex 1,Charcot Disease,Dementia With Amyotrophic Lateral Sclerosis,Gehrig's Disease,Guam Disease,Guam Form of Amyotrophic Lateral Sclerosis,Lou Gehrig's Disease,Lou-Gehrigs Disease,ALS Amyotrophic Lateral Sclerosis,Amyotrophic Lateral Sclerosis Parkinsonism Dementia Complex 1,Amyotrophic Lateral Sclerosis, Parkinsonism Dementia Complex of Guam,Disease, Guam,Disease, Lou-Gehrigs,Gehrig Disease,Gehrigs Disease,Sclerosis, Amyotrophic Lateral
D001039 Aphasia, Broca An aphasia characterized by impairment of expressive LANGUAGE (speech, writing, signs) and relative preservation of receptive language abilities (i.e., comprehension). This condition is caused by lesions of the motor association cortex in the FRONTAL LOBE (BROCA AREA and adjacent cortical and white matter regions). Agrammatism,Aphasia, Motor,Aphasia, Nonfluent,Broca Aphasia,Dysphasia, Broca,Agrammatic Broca Aphasia,Agrammatic Broca's Aphasia,Aphasia, Anterior,Aphasia, Ataxic,Aphasia, Expressive,Aphasia, Frontocortical,Dysphasia, Broca's,Verbal Aphasia Syndrome,Agrammatic Broca Aphasias,Agrammatic Broca's Aphasias,Agrammatic Brocas Aphasia,Anterior Aphasia,Anterior Aphasias,Aphasia Syndrome, Verbal,Aphasia Syndromes, Verbal,Aphasia, Agrammatic Broca,Aphasia, Agrammatic Broca's,Aphasias, Agrammatic Broca,Aphasias, Agrammatic Broca's,Aphasias, Anterior,Aphasias, Ataxic,Aphasias, Broca,Aphasias, Frontocortical,Ataxic Aphasia,Ataxic Aphasias,Broca Aphasia, Agrammatic,Broca Aphasias,Broca Aphasias, Agrammatic,Broca Dysphasia,Broca's Aphasia, Agrammatic,Broca's Aphasias, Agrammatic,Broca's Dysphasia,Dysphasia, Brocas,Expressive Aphasia,Frontocortical Aphasia,Frontocortical Aphasias,Motor Aphasia,Nonfluent Aphasia,Syndrome, Verbal Aphasia,Syndromes, Verbal Aphasia,Verbal Aphasia Syndromes
D014956 Writing The act or practice of literary composition, the occupation of writer, or producing or engaging in literary work as a profession.
D057174 Frontotemporal Lobar Degeneration Heterogeneous group of neurodegenerative disorders characterized by frontal and temporal lobe atrophy associated with neuronal loss, gliosis, and dementia. Patients exhibit progressive changes in social, behavioral, and/or language function. Multiple subtypes or forms are recognized based on presence or absence of TAU PROTEIN inclusions. FTLD includes three clinical syndromes: FRONTOTEMPORAL DEMENTIA, semantic dementia, and PRIMARY PROGRESSIVE NONFLUENT APHASIA. FTLD,Degeneration, Frontotemporal Lobar,Degenerations, Frontotemporal Lobar,FTLDs,Frontotemporal Lobar Degenerations,Lobar Degeneration, Frontotemporal,Lobar Degenerations, Frontotemporal

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