Onset Patterns and Initial Symptoms of Dementia with Lewy Bodies: Possible Pathophysiological Diversities Deduced from a SPECT Study.

BACKGROUND/AIMS: The aim of this study was to investigate onset patterns and initial symptoms in patients with dementia with Lewy bodies (DLB) and their association with SPECT findings. METHODS: We studied 29 probable and 12 possible DLB patients. Onset was defined as 'acute' when symptoms (in various combinations) present within a few weeks or 'chronic' when a few symptoms present and progress during a longer period of time. Initial symptoms were dichotomized into 'cognitive' and 'non-cognitive'. We conducted voxel-to-voxel statistical analyses of SPECT, and uptake deviations from age-matched controls were depicted with Z-scores. RESULTS: In acute patients, decreases were more apparent in the bilateral frontal and temporal lobes, whereas chronic patients showed decreases in the bilateral occipital, posterior cingulate and precuneal regions. Differences in the left frontal and left posterior cingulate were significant and those in the left temporal region showed a tendency toward significance. Patients with cognitive symptoms demonstrated more marked decreases in the bilateral temporal, parietal, occipital, cingulate and precuneal regions. Differences in the left parietal area reached significance and the total decreases were larger in patients with cognitive than non-cognitive symptoms, with a trend toward significance. CONCLUSIONS: The multiplicity of onset patterns and initial symptoms may possibly be based on pathophysiological diversities in DLB.

Regulation of cell motility via high and low affinity autocrine motility factor (AMF) receptor in human oral squamous carcinoma cells.

A tumour-secreted cytokine autocrine motility factor (AMF) induces in vivo invasion and metastasis, and in vitro tumour cell motility by a signal transduction through interaction with its cell surface receptor gp78. In this report, we investigated the characterization of a high-metastatic human oral squamous cell carcinoma (SCC) cell line LMF4 and low-metastatic HSC-3 in comparison with non-metastatic HSC-2 and HSC-4. Morphological and motility analyses revealed LMF4 cells to have the highest motile activity among those cells. However, LMF4 cells shared the similar features with HSC-3: high level secretion of AMF, enhancement of gp78 expression, co-expression of vimentin and cytokeratin, although LMF4 cells showed twice as high motile reactivity as HSC-3. The only difference was that LMF4 had twice as high amount of low-affinity receptor(s) as HSC-3, shown by Scatchard analysis.

Clinicopathological study of carcinomas of the lip and the mucosa of the upper and lower lips.

BACKGROUND: Lip carcinomas are rare oral tumors, and there have been few reports of lip carcinoma in Japan. METHODS: Of 914 patients with oral carcinomas treated between January 1980 and December 1998, 12 (1.3%) had lip carcinoma and 5 (0.5%) had lip mucosal carcinoma. We investigated the clinicopathological features of these 17 patients. RESULTS: Of the 12 patients with carcinoma of the lip, 10 had squamous cell carcinomas (9, external lower lip; 1 commissures) and 2 had mucoepidermoid carcinomas (external upper lip). Of the 5 patients with lip mucosal carcinoma, 3 had squamous cell carcinomas (2, mucosa of the lower lip; 1, mucosa of the upper lip), 1 had mucoepidermoid carcinoma (mucosa of the lower lip), and 1 had acinic cell carcinoma (mucosa of the lower lip). Of the 12 patients with lip carcinoma, 9 were classified as stage I, 2 as stage II, and 1 as stage III; all 5 of the patients with lip mucosal carcinoma were stage I. Five patients with lip carcinoma were treated by resection, 5 by a combination of resection and reconstruction, and 2 by radiotherapy alone. All patients with lip mucosal carcinoma were treated by resection. After the initial therapy, 3 patients without neck dissection had regional recurrences and received delayed neck dissection, and 2 died with neck regional recurrence after dissection. The 5-year cumulative survival rates of the patients with lip carcinoma and those with lip mucosal carcinoma were 82.5% and 80.0%, respectively. CONCLUSION: We suggest that early-stage carcinomas of the lip and of the mucosa of the upper and lower lips are frequent, and we found that the outcome of these patients was excellent. However, an aggressive therapeutic approach to the lip carcinoma patient with cervical metastasis appears warranted, in an attempt to improve locoregional control and ultimate survival.

[A clinical investigation of oro-maxillary sinus-perforation due to tooth extraction].

Oro-maxillary sinus perforation occurs occasionally at the extraction of a maxillary tooth, and it may be a cause of maxillary sinusitis or antro-oral fistula. Our purpose was to investigate the most frequent site of perforation, and to understand the clinical course of patients after perforation. We examined 2,038 maxillary teeth extracted from 1,337 patients (473 males and 864 females) at the First Department of Oral and Maxillofacial Surgery, Tokyo Medical and Dental University, from January 1991 to December 1993. Perforation occurred in 77 of all 2,038 teeth (3.8%). Of these, 38 teeth were from males (38/733; 5.2%), and 39 were from females (39/1,305; 3.0%). The perforation rate was significantly higher in males. Perforation occurred most often with extraction of an upper first molar, and in the third decade of life. The perforation rate gradually decreased with higher age. We classified 38 cases into 3 categories according to panorama X-P: 1) The tip of the root crossed over the bottom line of the maxillary sinus (29 cases), 2) Not crossed over distinctly (4 cases), 3) Just lie on, or whether the tip of the root crossed over the bottom line of the maxillary sinus was not distinct (5 cases). As to treatment, 30 perforations (39.0%) closed spontaneously in the course of observation, irrigation with physiological saline was used in 43 cases (55.8%), radical sinusotomy accompanied by closure of the perforation was performed in 4 cases, and only flap closure was used in 7 cases. There were no cases of relapse.

A case of false median cleft of upper lip with IV-A holoprosencephaly that underwent cheiloplasty at 2.5 years of age.

Holoprosencephaly results from the incomplete development of midline structures within the cerebrum and encompasses a series of abnormalities of mid-facial development. Here, we report a case of male holoprosencephaly associated with false median cleft of upper lip. This patient belonged clinically to the DeMyer's group IV holoprosencephaly, semilobar type. An infant with this type of holoprosencephaly has been thought to die generally within 1 to 2 years after birth and to rarely benefit from an operation. In this case, the patient had cheiloplasty at the age of 2.5 years at the request of his parents and he lives currently, being 3 years and 2 months old.

Non-invasive densitometric and histomorphometric study of the regenerated bone in the distraction gap in rabbits.

The long period of external fixation after the completion of distraction, which is necessary to obtain enough strength for resisting without fixation is a significant disadvantage of distraction osteogenesis. The purpose of this study is to understand the mechanical property of the regenerated bone and try to find an appropriate timing for safely removing the fixation device. An external fixation device was applied to the right tibia in rabbits and transverse osteotomy was performed just below the tibio-fibula junction. The tibiae were lengthened 7.2 mm at 0.72 mm a day for 10 days after surgery. A bone mineral density (BMD) and stress strain index (SSI) analyzed by peripheral quantitative computed tomography showed a significant increase on day 40, but returned to the control level at day 64 after the completion of distraction. Therefore, the newly formed bone between the host cortical bone seemed to be enough to resist the mechanical stress on day 40 during the consolidation period in this study. The present results suggested the possibility of removing the fixation device during the period when the BMD and SSI showed a significantly high level during the consolidation period.

Current trends in cognitive rehabilitation for memory disorders.

Progress in the neuropsychology of memory disorders has provided a foundation for development of cognitive rehabilitation for amnesic patients. Accumulating evidence in the past two decades suggested that certain training techniques could be beneficial to many amnesic patients, such as teaching and acquisition of domain-specific knowledge, motor coding, reality orientation, and meta-cognition improvement. In this article we review and discuss the current trends in cognitive rehabilitation of memory disorders and provide a future direction in this emerging field. In addition, our experience in the successful rehabilitation of Korsakoff syndrome patients is also introduced.

Ellis-van Creveld syndrome: craniofacial morphology and multidisciplinary treatment.

OBJECTIVE: The craniofacial morphology and multidisciplinary treatment of a patient with Ellis-van Creveld Syndrome are presented. PATIENT: The patient presented is female. Her treatment began at the age of 10 years 6 months. She exhibited small stature with acromelic shortening of the extremities, postaxial syndactyly, and dysplastic nails. In the oral region, hyperplastic frena, absent anterior teeth, and small barrel-shaped teeth were noted. SETTING AND INTERVENTION: The patient was treated at the University Hospital, Faculty of Dentistry, of the Tokyo Medical and Dental University. The treatment was divided into three stages: growth observation during which the patient wore acrylic plates with artificial anterior teeth; surgical-orthodontic treatment with sagittal split ramus osteotomy; and prosthodontic treatment with removable partial dentures. MAIN OUTCOME MEASURES: Cephalometric analyses were performed to evaluate the changes in craniofacial morphology with growth during treatment. RESULTS: Characteristic craniofacial morphology included a small posterior cranial base, small maxilla, and a large mandible with an increased gonial angle. Maxillo-mandibular relation was skeletal class III, and skeletal open bite was found. The skeletal class III relationship was progressive with growth. The patient was satisfied by the results of the multidisciplinary treatment. CONCLUSION: The craniofacial morphology of the patient demonstrated disturbed growth of the cartilage bones. An acceptable result was obtained through combined surgical-orthodontic-prosthodontic treatment.

Quantitative evaluation of consistency of normal mucosa, leukoplakia and squamous cell carcinoma of the tongue.

The quantitative differences in consistency of normal mucosa, leukoplakia and squamous cell carcinoma of the tongue were studied. Forty-five patients with squamous cell carcinoma of the tongue, 26 with leukoplakia and 80 with normal tongues were examined using an instrument capable of measuring consistency as a function of the load (in grams) needed to displace tongue mucosa by 2.0 mm using a probe 2.0 mm in diameter. The mean value for consistency in tongue carcinoma, leukoplakia and normal tongue was 22.1 +/- 8.0 g, 8.3 +/- 2.0 g and 4.8 +/- 0.7 g, respectively. Differences in consistency among these entities were significant (P < 0.001). Furthermore, consistency in tongue carcinoma was significantly correlated to tumour thickness (r = 0.954, P < 0.001). It was concluded that the consistency in tongue lesions was objectively and quantitatively evaluated by this method, and this technique could measure the thickness of the tumorous lesions preoperatively.

[Nerve paralysis after third molar extraction].

This paper described the incidence of inferior alveolar nerve paralysis following surgical removal of impacted third molars. The investigated cases were 789 patients with 1,299 teeth that were surgically extracted. Eight (0.6%) patients had the inferior alveolar nerve paralysis and one (0.08%) had lingual nerve paralysis. As for the patients with inferior alveolar nerve paralysis, the incidence was 0.4% in those aged 20 to 29 years, 0.6% in those aged 30 to 39 years, and 3.5% in those aged over 40 years. This study showed that the incidence of inferior alveolar nerve paralysis occurred more frequently in older age cases and in the cases in which the root splitting or the removing the surrounding bone was needed.

Computed tomography in the diagnosis of maxillofacial mass lesions in younger children.

OBJECTIVE: To evaluate the CT findings of maxillofacial mass lesions in younger children and to discuss the effectiveness of CT in their differential diagnosis. PATIENTS AND METHODS: CT findings of 17 patients (age range, 0 years 5 months to 3 years 3 months) with histopathologically proven maxillofacial mass lesions were retrospectively evaluated. About two-thirds (11/17) comprised congenital lesions or more common lesions in infancy or younger children. RESULTS: Hemangiomas, dermoid cysts and ranulas in this age group presented characteristic CT findings in common with those occurring in adults. Two invasive lesions (melanotic neuroectodermal tumor and aggressive fibromatosis) had irregular margins and the latter also showed aggressive bone destruction. Two angiomas had irregular margins. A fibrosarcoma showed expansile bone destruction but had a regular margin. CONCLUSION: CT was useful in demonstrating the extent of maxillofacial mass lesions in children and for surgical treatment planning. Although some invasive lesions could be correctly diagnosed on the basis of CT, its diagnostic value in differentiating malignant from benign maxillofacial lesions in younger children was limited.

Which relapse criteria best predict the mortality risk of treated alcoholics?

We examined which relapse criteria best predict the mortality risk of treated male alcoholics. The subjects were 172 male alcoholics who had previously been hospitalized. Using three criteria which defined relapse as failure to maintain abstinence from alcohol, alcohol abuse, or dependence, the relapse of each subject had been evaluated during a previous 3-year outcome study. Relative mortality risks in the next 3 years classified by the three relapse criteria were compared. The follow-up rate was 93.6% and 31 subjects died. The age-corrected relative mortality risk for subjects failing to maintain abstinence compared with abstainers was 5.32, while the relative mortality risks for the group abusing alcohol and for the group suffering alcohol dependence were 2.23 and 2.56, respectively. These results suggest that relapse defined as failure to maintain abstinence predicts a higher relative mortality risk than do criteria defining in terms of alcohol abuse and alcohol dependence.

Apolipoprotein E epsilon 4 allele distribution in Wernicke-Korsakoff syndrome with or without global intellectual deficits.

Recent genetic studies show that the apolipoprotein E (ApoE) epsilon 4 allele is a risk factor for Alzheimer's disease (AD). Whether this allele is associated with other dementing diseases is the next important question. The information could provide a clue to the pathogenetic role of ApoE. In the present study, patients with Wernicke-Korsakoff syndrome (WKS) of alcoholic etiology were divided into two groups according to the severity of intellectual deficits, i.e., those of "classical" Korsakoff patients with preserved intellectual function other than amnesia and those with global intellectual deficits. Genotyping showed that the frequency of ApoE epsilon 4 allele was significantly higher in the patients with global deficits, suggesting the involvement of this allele in the intellectual decline of WKS. In contrast, distributions of other two markers, alpha 1-antichymotrypsin and presenilin-1, did not differ between the two groups. These results added further support to the notion that the consequence of acute insult to the brain is influenced by the ApoE genotype, and suggested ApoE's role in the development of a certain group of "alcoholic dementia."

Nonconvulsive status epilepticus during antidepressant treatment.

We report 3 patients who experienced nonconvulsive status epilepticus following generalized convulsions during antidepressant treatment. All showed prolonged confusion with some psychiatric symptoms, and their electroencephalograms (EEGs) showed either bilateral synchronous multiple spike wave discharges or continuous diffuse sharp and slow waves. When diazepam was administered intravenously to 1 patient, the clinical symptoms and EEG improved markedly.

[Cognitive dysfunction following anterior communicating artery aneurysm rupture. Comparison with alcoholic Korsakoff syndrome on neuropsychological performance].

The present study aims to compare neuropsychological performance of patients following anterior communicating artery aneurysm rupture (ACoA) with that of patients with alcoholic Korsakoff syndrome (AKS). Fifteen ACoA patients and ten age-and education-matched AKS patients were included in the study. All the patients were tested at least one year post onset of their illness at a stable condition. The WAIS and forward digit span scores of AKS were also matched to ACoA, and simple attention and general intelligence were well preserved both in ACoA and AKS. Frontal function as measured by the Wisconsin card sorting test (Keio version) (KWCST) was equivalently impaired in the two groups. Anterograde memory as measured by Wechsler memory scale subtests, serial seven word learning test, Rey auditory verbal learning test, and logical memorizing test (Luria's paired word-picture association), was more severely impaired in AKS than ACoA in contrast to the comparable attention, intelligence, and frontal function: (1) memory tasks with low correlations to KWCST (serial word learning tasks and paired verbal associates), reflecting primary simple serial memorizing, and (2) memory tasks with high correlations to KWCST (logical memory and logical memorizing), reflecting higher and complicated strategic mnemonic activities. However, the correlations between these anterograde memory subtests and KWCST were substantially equivalent in ACoA and AKS. This suggests that the differences in anterograde amnesia demonstrated in ACoA and AKS may be of quantitative, not of qualitative property. The extent of deficits in semantic encoding as measured by Wickens' release from proactive interference paradigm (PI release) was also milder in ACoA than AKS. Both AKS and ACoA failed to show PI release in contrast to normal PI release demonstrated in age-matched ten healthy subjects. PI release in ACoA, however, was in between AKS and healthy subjects. The results were interpreted in the light of a recently postulated hypothesis that a combination of frontal lobe damage and memory impairment is crucial for causing a deficit in semantic encoding. The extent of damage in the memory circuit in ACoA may be variable, which may result in milder degree of anterograde amnesia and semantic encoding than AKS in the present study.

Basic properties of super-elastic Ni-Ti alloy ligature wires for a new intermaxillary fixation method.

A new continuous wiring method of intermaxillary fixation with super-elastic Ni-Ti alloy ligature wires was developed to avoid the risk in postoperative emergency. Differential scanning calorimetry, three-point bending tests and fastening tests were carried out to investigate basic properties of the wires. Transformation temperatures of the Ni-Ti alloy wires were in the range suitable for exhibiting super-elasticity at body temperature. The Ni-Ti alloy wires possessed enough bending flexibility to be used in the continuous wiring method and showed great recoverable displacement in the fastening test. With use of these properties, easy removal in an emergency and uniform fixation can be attained.