Perivasculitic panencephalitis with relapsing polychondritis: an autopsy case report and review of previous cases.

We herein report an autopsy case of relapsing polychondritis encephalitis coexisting with a Lewy body pathology and also review previous autopsy cases. A 59-year-old man exhibited a tremor of the right hand, small-steppage gait and bradykinesia. Five years later, he presented with relapsing auricular chondritis and scleritis and subsequently showed exacerbation of extrapyramidal symptoms. A histological examination revealed perivascular lymphocytic cuffing and infiltration in the small vessels, as well as loss of nerve cells and gliosis in the basal ganglia, insular gyrus and medial temporal lobe. The present case was characterized by perivasculitic panencephalitis and the coexistence of a Lewy body pathology, which may have augmented the patient's parkinsonism.

Decreased cerebral blood flow and prognosis of Alzheimer's disease: a multicenter HMPAO-SPECT study.

PURPOSE: To determine the usefulness of brain perfusion SPECT for evaluating the severity and progression of Alzheimer's disease (AD). METHODS: Eighty-four AD patients were included. At entry, 99mTc-HMPAO-SPECT, the Mini Mental State Examination (MMSE), Mental Function Impairment Scale (MENFIS), and the Raven Colored Progression Matrix (RCPM) were performed for all 84 patients. During the follow-up periods, two individual MMSE evaluations in 34 patients, two MENFIS evaluations in 30 patients, and two RCPM evaluations in 20 patients were performed. Based on the regions of decreased cerebral blood flow demonstrated on 3D-SSP images of SPECT, the cases were classified as type A (no decrease), type B (decreased blood flow in the parietal or temporal lobe), type C (decreased blood flow in the frontal lobe and parietal or temporal lobe), type Pc (decreased blood flow in posterior cingulate gyrus only), and "other types". The types of decreased blood flow, scores on neuropsychological evaluations, and symptom progression were analyzed. RESULTS: The MENFIS, MMSE, and RCPM scores were poorest in type C patients at entry. The degree of decrease of these scores during the follow-up periods was also greatest in type C. The greatest difference between patients with and without rapid progression in SPECT data of the mild AD patients (MMSE score > or = 24) was in the frontal lobe. CONCLUSION: Decreased blood flow in the frontal lobe of AD patients is correlated not only with reduced cognitive function at the time of the evaluation but with rapid progression in the subsequent clinical course.

Neural correlates of impaired performance on the clock drawing test in Alzheimer's disease.

To identify the neural correlates for impaired performance on the clock drawing test (CDT) in patients with Alzheimer's disease (AD), we examined the relationship between the CDT performances and the regional cerebral blood flow (rCBF) in 100 AD patients. The patients were equally divided into a mildly impaired CDT group, a severely impaired CDT group, and two normal CDT groups, with age and dementia severity matched. Between-group comparisons revealed that rCBF reduction in the posterolateral region of the left temporal lobe was consistently associated with mild to severe impairment of the CDT in AD. Correlation analysis also showed that the rCBF in the left posterolateral temporal cortex was linearly correlated with CDT performance. The CDT scores in AD were significantly improved for the copy condition relative to the drawing-to-command condition. These findings suggest that CDT performance has a close relationship with the left posterior temporal function, and that semantic memory deficit may at least partly contribute to impaired CDT performance in AD.

Factor structure of a modified version of the wisconsin card sorting test: an analysis of executive deficit in Alzheimer's disease and mild cognitive impairment.

In order to explore the factor structure of a modified version of the Wisconsin Card Sorting Test (mWCST) and to identify the dimensions of deficit in patients with Alzheimer's disease (AD) and mild cognitive impairment (MCI), conventional mWCST scores in 55 AD patients, 17 MCI patients, and 22 controls were subjected to factor analysis. Three factors, perseveration, inefficient sorting, and nonperseverative error, were obtained. Perseveration score was significantly poorer in both AD and MCI than in controls. By contrast, the MCI group showed significantly poorer scores on the nonperseverative error factor than did the AD patients, and the controls yielded intermediate values between the two patient groups. The perseveration factor was significantly correlated with the other estimates of executive function. This study suggested that the many mWCST scores could be reduced to three major factors, and that the perseveration score may effectively represent an aspect of executive dysfunction in AD and MCI patients.

Cerebral correlates of the progression rate of the cognitive decline in probable Alzheimer's disease.

OBJECTIVE: To evaluate the possible relation between the rate of cognitive deterioration in patients with probable Alzheimer's disease (AD) and the distribution pattern of neural dysfunction. METHODS: The regional cerebral blood flow (rCBF) was measured in rapidly and slowly progressing groups of AD patients using single-photon emission computed tomography and was compared between the groups. While controlling for demographic and clinical factors that could be associated with the stage and prognosis of the illness, the deterioration rate of the Mini Mental State Examination (MMSE) score was significantly greater in the rapidly progressing group than that in the slowly progressing group. RESULTS: The rCBF in the right posterodorsal, anterior and superior prefrontal cortices and the inferior parietal cortex was significantly lower in the rapidly progressing patients. Moreover, lower perfusion in these regions correlated significantly with rapid deterioration in the MMSE. CONCLUSIONS: These findings suggest that the rCBF values in these cortical regions could be useful in predicting which AD patients will show a relatively rapid cognitive decline.

Regional cerebral blood flow abnormalities in nondemented patients with memory impairment.

BACKGROUND: Patients with objective evidence of memory impairment have been considered to be at risk for developing Alzheimer's disease (AD). However, little is known about patterns of regional cerebral blood flow abnormalities and their prognostic significance in these patients. METHODS: The authors retrospectively studied 28 nondemented subjects with memory loss and investigated patterns of blood flow abnormalities on single photon emission computed tomography (SPECT). RESULTS: The patients were followed up for more than 2 years; during follow-up, 14 patients (50%) developed AD. The onset of memory impairment in patients who progressed to AD was significantly earlier than in those who remained in a nondemented condition. SPECT data from the initial evaluation were analyzed by region of interest analysis and statistical parametric mapping. Interestingly, both groups of patients shared hypoperfusion in the medial temporal regions and the posterior cingulate. In addition to these regions, significant blood flow reduction in the parietal and anterior cingulate cortices was detected in patients who progressed to AD. CONCLUSIONS: These results demonstrate that (1) subjects with an earlier onset of memory loss have an increased risk for developing AD, (2) SPECT can be useful for distinguishing subjects with memory loss who will rapidly progress to AD from those who will not, and (3) perfusion impairment typical of AD was evident even in subjects with memory impairment who remained nondemented.