[How to make a differential diagnosis of the aphasic syndromes].
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Biomedical subjects
Publications and source records attributed to Hirotaka Tanabe.
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Patients with frontotemporal lobar degeneration (FTLD) present a profound personality change, social misconduct, overeating, and stereotyped behavior. We examined the hypothesis that many of the behavioral symptoms of FTLD will respond to selective serotonin reuptake inhibitors (SSRIs). Sixteen FTLD patients were treated with an SSRI (fluvoxamine maleate) in an open 12-week trial. Treatment responses for stereotyped behavior and other neurobehavioral symptoms were evaluated by the Stereotypy Rating Inventory and the Neuropsychiatric Inventory. The behavioral symptoms, especially stereotyped behaviors of FTLD, significantly improved after treatment. Randomized, placebo- and other SSRI-controlled trials may improve available treatments.
A few epidemiologic studies have dealt with the prevalence of frontotemporal lobar degeneration (FTLD), including Pick's disease. The aim of this study was to review the epidemiologic studies of FTLD in western countries and to compare them with those in Japan. A community-based study of early-onset dementia in London revealed that 12% of cases with frontotemporal dementia (FTD) fulfilled the Lund-Manchester criteria in contrast to 34% of cases with Alzheimer's disease (AD) in a sample of 185 cases. The Cambridge Group has recently examined the prevalence of early-onset dementia in a community-based study. Of 108 cases, 15.7% had FTLD and 25% had AD. FTLD included 13 FTD cases, and 2 each with semantic dementia (SD) and nonfluent progressive aphasia (PA). Almost one third of cases with FTLD (29%) had a positive family history. Of our consecutive 330 outpatients with dementia (hospital setting without age limitation), 42 (12.7%) had FTLD and 215 (65.1%) had AD. In our series of patients, 22 FTD, 15 SD and 5 PA cases were identified. There was no family history in all subtypes of FTLD. Epidemiologic studies, both community-based and hospital-based, demonstrate that FTLD is a more common cause of early-onset dementia than previously recognized. Regarding the subtypes of FTLD, in Japan, compared with the data from the UK, FTD is less common, SD may be more common and PA is equally common. The reason for this discrepancy is supposed to be mainly based on the role of heredity.
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We have studied the deposition of calcium salts in the autopsied intestines which have not been described previously as far as we were able to determine. In order to clarify the incidence, predisposing conditions, mineral compositions of the deposited materials and clinical significance of this phenomenon, we examined 76 cases of consecutive autopsied small intestines by von Kossa's staining. Deposited calcium salts were further examined by electron microscopically, energy-dispersive X-ray spectroscope and electron diffraction analysis. Deposition of calcium salts was observed in the small intestines of 13 cases. Among them, 10 cases were accompanied by hypercalcemia. Deposition of calcium salts was mainly observed in smooth muscle cells of the proper muscle layers and ganglion cells of the Auerbach's myenteric plexus. Intestinal calcinosis was frequently accompanied by deposition of calcium salts in the proper muscle layers of esophagus and large intestine, renal tubules and cardiac myocardial cells. Electron microscopically, the calcium salts were identified as needle-shaped crystals and located on the surface of the extracellular-collagen bundles, in the cytoplasm, mitochondria and nucleus of the smooth muscles cells. Energy-dispersive X-ray spectroscope and electron diffraction analysis suggested the deposited calcium salts as hydroxyapatite. Two patients among the six cases with moderate to severe calcium deposition showed clinical manifestation of paralytic ileus. In conclusion, intestinal calcinosis was frequently observed mostly associated with hypercalcemia. Calcium salts of hydroxyapatite were deposited to the smooth muscle cells and the Auerbach's myenteric plexus of the muscular layer. Deposition of calcium salts might occasionally causes the paralytic ileus but clinical significance of this lesion requires further examination.
Recently, some standard test batteries such as Boston Diagnostic Aphasia Examination and Western Aphasia Battery are frequently used to assess language abnormalities and to identify individual types of aphasia. Aphasia is, however, a neurologic disorder and differential diagnosis of the type should be made naturally in the neurological examination. Characteristics of verbal output are most important clinically for the classification. The table shows core symptoms of classical aphasic types. The figure presents a systematic approach to aphasia diagnosis based on several different patterns of aphasic output.
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BACKGROUND: Delusions constitute one of the most prominent psychiatric complications in Alzheimer's disease (AD). However, there is little consensus of the prevalence and associated factors for delusions in AD. AIMS: To reveal the characteristics of delusions among Japanese patients with AD. METHOD: 112 consecutive patients with AD were recruited over a one year period and administered the Neuropsychiatric Inventory (NPI). RESULTS: Delusions were present in 53 patients (47.3%). Delusions of theft were the most common type of delusion (75.5% of patients with delusions), followed by misidentification delusions and delusions of suspicion. More hallucination, agitation, and female gender were found in the delusions group. CONCLUSIONS: The authors found a high frequency of delusions, particularly of delusions of theft and suggested that gender was associated with the expression of delusions in Japanese patients with AD.
We studied the reading performance of a Japanese-speaking patient, TI, with bilateral but asymmetrical (left more than right) temporal-lobe atrophy, severe anomia, and poor word comprehension. Most Japanese kanji characters correspond to several different legitimate pronunciations in different contexts, with varying degrees of correspondence consistency. TI made many errors in reading aloud words that violate statistically typical character-sound correspondences, especially for less common words, but had relatively preserved ability to read aloud strings in which the assignment of the typical pronunciation for each component character yields the correct pronunciation for the whole word. The degree of consistency of character-sound correspondences affected his performance on both words and nonwords in a graded manner. One interpretation is that TI's surface dyslexic reading reflects intact direct computation of phonology from orthography, but without the additional constraint from word meaning that is, in this framework, considered critical for correct pronunciation of lower-frequency words with atypical character-sound correspondences. Another interpretation is that TI's performance reflects partially damaged lexical knowledge of whole-word orthography and phonology, coupled with spared sublexcal knowledge of character-sound correspondence rules. Whichever of these interpretations is preferred, this study offers the most detailed information yet available on the characteristics of surface dyslexia in Japanese.
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A many behavioral disturbances, Stereotypic behaviors are among the best discriminators of Frontotemporal Lobar Degeneration (FTLD). A recent preliminary report suggests many of the behavioral symptoms, including stereotypic behaviors in FTLD patients, respond to medication with selective serotonin re-uptake inhibitors. However, there is no scale that evaluates stereotypic behaviors comprehensively. To assess the wide range of stereotypic behaviors encountered in FTLD, we developed a new instrument, the Stereotypy Rating Inventory (SRI). The SRI assesses five distinctive stereotypic behavioral disturbances often seen in patients with FTLD: eating and cooking behaviors, roaming, speaking, movements, and daily rhythm. The SRI uses the same technique as the Neuropsychiatric Inventory (NPI) in that both the frequency and the severity of each behavior are determined. The studies reported here demonstrate the content and concurrent validity, as well as inter-rater and test-retest reliability, of the instrument. Scores of FTLD patients (n=26) on the SRI were much higher than those of patients with Alzheimer's disease (n=46), patients with vascular dementia (n=26), and normal control subjects (n=40). The SRI appears to be a useful instrument for detecting stereotypic behaviors and monitoring of therapies in FTLD patients.
BACKGROUND: Delusions of theft (delusions involving the theft of possessions) are one of the most frequent neuropsychiatric manifestations of Alzheimer's disease (AD). OBJECTIVE: The current study investigated the presence and extent of such delusions before and after drug treatment in a group of AD patients, and the consequent effects on the burden of care on caregivers. METHOD: The study was an open-label cohort design. The delusions studied consisted only of those involving theft of possessions. Sixteen AD patients served as subjects in order to assess the efficacy of Risperidone administration, in the reduction or elimination of these delusions. The caregiver burden was evaluated using the Zarit Caregiver Burden Interview (ZBI) before the administration of Risperidone and 12 weeks after administration, for cases where delusions of theft were eliminated or reduced. RESULTS: The burden of care on caregivers was significantly reduced (p < 0.001) through the elimination or reduction of delusions of theft. CONCLUSION: Delusions of theft are considered to be a major factor in increasing the burden of care, and the treatment of these, through appropriate drug therapy, is therefore of great importance in the continuation of satisfactory care in the home.
Benign adult familial myoclonic epilepsy (BAFME) is an autosomal dominant idiopathic epileptic syndrome characterized by adult-onset tremulous finger movement, myoclonus, epileptic seizures, and nonprogressive course, recognized in Japanese families. We recently assigned the gene locus to chromosome 8q23.3-q24.11 by linkage analysis. In this study, we identified the sequence of human cDNA encoding Kv8.1, a neuronal modulatory alpha-subunit of the voltage-gated potassium channel, mapped to human chromosome 8q22.3-8q24.1. Human Kv8.1 cDNA had a 1,500-nucleotide open reading frame encoding a polypeptide of 500 amino acids, in which six hydrophobic transmembrane segments were well conserved, and its overall amino acids homology as compared with those of rat and golden hamster was 95.0% and 95.0%. Tissue-distribution analysis by reverse transcription-polymerase chain reaction (RT-PCR) showed the presence of the Kv 8.1 transcript exclusively in the brain. The analysis of the genomic organization of the human gene revealed consistency of three exons interrupted by two introns each of 1.2 and 3.5 kb. We analyzed the genomic sequence of the patients with BAFME and found no change in the pathogenesis of the disease.
We report a 71-year-old woman showing rapidly progressive non-fluent aphasia and dementia accompanied by motor neuron disease (MND). There was no family history of dementia or motor neuron disease. There was 10 months history of dysarthria and dysphagia. On examination, she showed profound difficulty in articulation. Her comprehension was impaired in that she was unable to obey three-stage command. Her written language was also impaired with phonological spelling errors, syntactic errors, and perseveration. Neuroradiological investigations showed atrophic changes and hypoperfusion of left temporal and bilateral parietal region revealed by MRI and SPECT, respectively. Her subsequent decline was rapid. It might be likely that aphasia is much more common in dementia with bulbar MND than is currently recognized because bulbar palsy might mask the language disorder.
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