Erratum to: Prognosis of Patients with Behavioral Variant Frontotemporal Dementia Who have Focal Versus Diffuse Frontal Atrophy

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Prognosis of Patients with Behavioral Variant Frontotemporal Dementia Who have Focal Versus Diffuse Frontal Atrophy

BACKGROUND AND PURPOSE: Only a few studies have investigated the relationship between different subtypes and disease progression or prognosis in patients with behavioral variant frontotemporal dementia (bvFTD). Since a localized injury often produces more focal signs than a diffuse injury, we hypothesized that the clinical characteristics differ between patients with bvFTD who show diffuse frontal lobe atrophy (D-type) on axial magnetic resonance imaging (MRI) scans versus those with focal or circumscribed frontal lobe atrophy (F-type). METHODS: In total, 94 MRI scans (74 scans from bvFTD and 20 scans from age-matched normal controls) were classified into 35 D- and 39 F-type bvFTD cases based on an axial MRI visual rating scale. We compared baseline clinical characteristics, progression in motor and cognitive symptoms, and survival times between D- and F-types. Survival analyses were performed for 62 of the 74 patients. RESULTS: While D-type performed better on neuropsychological tests than F-type at baseline, D-type had higher baseline scores on the Unified Parkinson's Disease Rating Scale (UPDRS) Part III. Evaluations of motor progression showed that the disease duration with motor symptoms was shorter in D-type than F-type. Moreover, the survival time was shorter in D-type (6.9 years) than F-type (9.4 years). Cox regression analyses revealed that a high UPDRS Part III score at baseline contributed to an increased risk of mortality, regardless of the pattern of atrophy. CONCLUSIONS: The prognosis is worse for D-type than for those with F-type. Shorter survival in D-type may be associated with the earlier appearance of motor symptoms.

Normative Study of the Literacy Independent Cognitive Assessment in Illiterate and Literate Elderly Koreans

OBJECTIVE: The aim of this study was to provide normative data on the Literacy Independent Cognitive Assessment (LICA) and to explore the effects of age, education/literacy, and gender on the performance of this test. METHODS: Eight hundred and eighty-eight healthy elderly subjects, including 164 healthy illiterate subjects, participated in this study. None of the participants had serious medical, psychiatric, or neurological disorders including dementia. Bivariate linear regression analyses were performed to examine the effects of age, education/literacy, and sex on the score in each of the LICA cognitive tests. The normative scores for each age and education/literacy groups are presented. RESULTS: Bivariate linear regression analyses revealed that total score and all cognitive tests of the LICA were significantly influenced by both age and education/literacy. Younger and more-educated subjects outperformed older and illiterate or less-educated subjects, respectively, in all of the tests. The normative scores of LICA total score and subset score were presented according to age (60-64, 65-69, 70-74, 75-80, and > or =80 years) and educational levels (illiterate, and 0-3, 4-6, and > or =7 years of education). CONCLUSION: These results on demographic variables suggest that age and education should be taken into account when attempting to accurately interpret the results of the LICA cognitive subtests. These normative data will be useful for clinical interpretations of the LICA neuropsychological battery in illiterate and literate elderly Koreans. Similar normative studies and validations of the LICA involving different ethnic groups will help to enhance the dementia diagnosis of illiterate people of different ethnicities.

Cognitive Intervention in a Patient with Carbon Monoxide Intoxication

BACKGROUND: Cognitive intervention (CI) is a nonpharmacological approach used to compensate for cognitive impairment. It is categorized into cognitive training, cognitive stimulation and cognitive rehabilitation. Several studies showed that CI could induce cognitive enhancement and reduction of risk for future cognitive decline in patients with brain injury. We investigated effects of CI on cognitive functions and brain glucose metabolism based on serial cognitive assessments and [18F]-Fluorodexoxyglucose positron emission tomography (FDG-PET) in a patient with carbon monoxide (CO) intoxication. METHODS: A 40-year-old man presented with memory impairment and abnormal behaviors such as apathy, indifference, and perseveration 2-month after CO intoxication. Brain magnetic resonance image (MRI) demonstrated high signal changes in the bilateral basal ganglia, hippocampus and the subcortical white matter on T2 weighted images. FDG-PET also showed glucose hypometabolism in the bilateral hippocampus, basal ganglia, and the subcortical white matter. A detailed neuropsychological evaluation revealed multiple cognitive impairments in memory, language and frontal functions. He received twice a week sessions of 60-minute group-based cognitive intervention for 12 weeks. Several neuropsychological examinations and FDG-PETs were conducted at baseline and after CI. RESULTS: After CI, he showed improvements in memory and frontal functions compared with baseline performances. These cognitive improvements persisted by the 7-month follow-up. The extent of glucose hypometabolism was decreased 1-month after CI, however increased 8-month after CI. CONCLUSIONS: This case study suggested that CI could enhance cognitive functions and improve glucose metabolism in a patient with CO intoxication. Also, the effects of CI on cognitive functions seem to be last at least 7-month after training.

Reliability and Validity of the Short Form of the Literacy-Independent Cognitive Assessment in the Elderly

BACKGROUND AND PURPOSE: The Literacy-Independent Cognitive Assessment (LICA) has been developed for a diagnosis of dementia and is a useful neuropsychological test battery for illiterate populations as well as literate populations. The objective of this study was to develop the short form of the LICA (S-LICA) and to evaluate the reliability and validity of the S-LICA. METHODS: The subtests of the S-LICA were selected based on the factor analysis and validation study results of the LICA. Patients with dementia (n=101) and normal elderly controls (n=185) participated in this study. RESULTS: Cronbach's coefficient alpha of the S-LICA was 0.92 for illiterate subjects and 0.94 for literate subjects, and the item-total correlation ranged from 0.63 to 0.81 (p<.01).The test-retest reliability of the S-LICA total score was high (r=0.94, p<.001), and the subtests had high test-retest reliabilities (r=0.68-0.87, p<.01). The correlation between the K-MMSE and S-LICA total scores were substantial in both the illiterate subjects (r=0.837, p<.001) and the literate subjects(r=0.802, p<.001). The correlation between the S-LICA and LICA was very high (r=0.989, p<.001). The area under the curve of the receiver operating characteristic was 0.999 for the literate subjects and 0.985 for the illiterate subjects. The sensitivity and specificity of the S-LICA for a diagnosis of dementia were 97% and 96% at the cutoff point of 72 for the literate subjects, and 96% and 93% at the cutoff point of 68 for the illiterate subjects, respectively. CONCLUSIONS: Our results indicate that the S-LICA is a reliable and valid instrument for quick evaluation of patients with dementia in both illiterate and literate elderly populations.

Dissociated Automatic-Voluntary Lower Cranial Nerve Palsies and Anarthria After Left Corona Radiata Infarction: Foix-Chavany-Marie Sydrome

Foix-Chavany-Marie Syndrome (FCMS) is characterized by anarthria and bilateral facio-pharyngo-glosso-masticatory paralysis with an automatic-voluntary dissociation, which usually develops in bilateral opercular lesions. We present a case of FCMS caused by unilateral subcortical lesion. A 54-year-old man was admitted due to acute right hemiparesis with anarthria. He had voluntary facial paresis but automatic-involuntary facial movements were preserved. MRI showed an acute left corona radiata infarction and PET revealed decreased glucose metabolism in left basal ganglia and fronto-parietal lobe.