Primary progressive aphasia: analisys of 16 cases.

Primary progressive aphasia (PPA) is an intriguing syndrome, showing some peculiar aspects that differentiate it from classical aphasic pictures caused by focal cerebral lesions or dementia. The slow and progressive deterioration of language occurring in these cases provides an interesting model to better understand the mechanisms involved in the linguistic process. We describe clinical and neuroimaging aspects found in 16 cases of PPA. Our patients underwent language and neuropsychological evaluation, magnetic resonance imaging (MRI) and single photon emission computerized tomography (SPECT). We observed a clear distinction in oral expression patterns; patients were classified as fluent and nonfluent. Anomia was the earliest and most evident symptom in both groups. Neuroimaging pointed to SPECT as a valuable instrument in guiding the differential diagnosis, as well as in making useful clinical and anatomical correlations. This report and a comparison to literature are an attempt to contribute to a better understanding of PPA.

SPECT in Alzheimer's disease: features associated with bilateral parietotemporal hypoperfusion.

OBJECTIVE: To investigate why bilateral parietotemporal hypoperfusion, the typical SPECT pattern of Alzheimer's disease (AD), occurs in some but not in all patients with probable AD. METHODS: We reviewed the SPECT scans of 220 patients presenting with cognitive impairment. Among them, 104 patients fulfilled NINCDS-ADRDA criteria for probable AD, 48 (32 women) with early onset (before age of 65) and 56 (40 women) with late onset of the symptoms. Dementia severity was assessed by the Mini-Mental State Examination. The SPECT scans were classified by visual inspection blind to clinical diagnoses. RESULTS: Bilateral parietotemporal hypoperfusion was more frequent in patients with severe AD, in those with early onset of the symptoms, and in men. Duration of symptoms, type of gamma-camera or radiopharmaceutical agent used were not associated with this SPECT pattern. CONCLUSION: These findings may be useful in the clinical setting and point to heterogeneity of AD according to age at onset.

Effects of myocardial hypertrophy on neural reflexes controlling cardiovascular function.

There are clinical and experimental evidences that the cardiopulmonary reflex function is impaired in chronic hypertension, but it could be due to myocardial hypertrophy rather than to hypertension itself. To test this hypothesis we evaluated the Bezold-Jarisch reflex in experimental conditions of myocardial hypertrophy and arterial normotension. Adult male Wistar rats were subjected to myocardial hypertrophy (MHR) treating them with the beta-adrenoceptor agonist isoproterenol (0.3 mg/kg/day, s.c.) for 15 days and compared with vehicle injected control rats (CR). No significant changes in body weight (283+/-14 vs. 299+/-9 g), resting mean arterial pressure (104+/-4 vs. 110+3 mm Hg) or heart rate (330+/-11 vs. 358+/-18 bpm) were observed in MHR compared to CR. As expected, MHR showed left and right ventricular and left atrial hypertrophy when compared to CR. The bradycardia and hypotension that characterizes the Bezold-Jarisch reflex, induced by the 5-HT3, agonist phenyldiguanide (1.5-24.0 microg/kg, i.v.), were significantly decreased in MHR compared to CR. Cardiac muscarinic responsiveness, which was assessed by electrical stimulation of the efferent vagus in anesthetized animals or by stimulation of muscarinic receptors in isolated hearts, was unchanged or increased, respectively, in MHR compared to CR. Additional studies showed that the baroreflex and chemoreflex were also attenuated in MHR compared to CR. These data indicate that cardiac hypertrophy impairs the Bezold-Jarisch reflex probably due to changes at central integrative areas of the reflex.

Brain SPECT in dementia. A clinical-scintigraphic correlation.

The aim of this study was to compare the accuracy of computed tomography (CT) and single photon emission computerized tomography (SPECT) in the diagnosis of dementia. Fifty-two patients with clinical diagnosis of dementia and 11 controls were studied. The scans were interpreted by one experienced neuroradiologist and one nuclear radiologist, both blinded to the clinical data. In the diagnosis of dementia, CT and SPECT showed equal sensitivity (82.7%) and statistically similar specificity (63.8 and 81.8%, respectively). The specificity of SPECT in diagnosing Alzheimer's disease (100%) was statistically superior to CT (69%). However, both methods showed similar sensitivity in detecting Alzheimer's disease. In conclusion, SPECT and CT showed similar accuracy in the diagnosis of dementia. The quite high specificity of SPECT in Alzheimer's disease may be useful for confirming that diagnosis, particularly for patients with presenile onset of the disease.

MRI of congenital pituitary insufficiency.

We compared 1.5 T magnetic resonance (MR) image findings in 193 patients with congenital pituitary insufficiency. One hundred and thirty nine of the MR studies were obtained in patients who had isolated growth hormone deficiency (GHD). Other fifty-four patients had multiple pituitary hormone deficiency (MPHD). On MR images, normal anterior and posterior lobes of the pituitary gland can be clearly differentiated because the posterior lobe has a characteristic high intensity on T1-weighted images. In fifty-four patients, the high-intensity of the posterior lobe was not seen, but a similar high signal intensity was observed at the proximal stump in fifty-one patients. This high-intensity area is the newly formed ectopic posterior lobe, which also secrets anti-diuretic hormone just as the posterior lobe would. MR imaging can demonstrate the transection of the pituitary stalk and the formation of the ectopic lobe, revealing to be a usefull diagnostic tool in the definition of the type of alteration in growth defects of endocrine origin.

Evaluation of 100 patients with dementia in São Paulo, Brazil: correlation with socioeconomic status and education.

One hundred consecutive outpatients with dementia were prospectively studied to investigate the diagnoses of dementing diseases and to correlate these diagnoses with socioeconomic status and with education. Alzheimer disease was the most common cause of dementia (54%), followed by vascular dementia (20%). Eight patients presented with potentially reversible causes of dementia. These frequencies are similar to those reported by case register studies from Western Europe and the United States. We did not find differences in the frequencies of the dementing diseases according to socioeconomic status or education. Alzheimer disease was the most common cause of dementia in all socioeconomic classes. Potentially reversible dementias, vascular dementias, and other secondary dementias were not more frequent in the lower socioeconomic strata. There was a trend to a higher frequency of vascular dementia among patients with less education, but this was not statistically significant.

[Neuropsychological tests of simple application for diagnosing dementia]. / Testes neuropsicológicos de aplicação simples para o diagnóstico de demência.

Thirty patients with dementia defined by DSM-III-R criteria (Alzheimer's disease (22), vascular dementia (3), Parkinson's disease, frontal lobe dementia, possible diffuse Lewy body dementia, normal pressure hydrocephalus and uncertain diagnosis), with scores below 24 points in the Mini-Mental Status Examination and more than 4 years of education were submitted to a neuropsychological evaluation. The scores in the neuropsychological tests were compared to those obtained by thirty normal volunteers paired for age, sex and education. Sensitivity, specificity and accuracy of the tests in the distinction of demented and normal volunteers were determined. The accuracies were calculated using ROC curves. Blessed's information-memory-concentration test showed greatest accuracy, followed by copy of simple figures, delayed memory of 10 figures (after 5 minutes), recognition of 10 figures and verbal fluency test (animals). A linear discriminant function, composed by 6 tests: visual perception, incidental memory, delayed memory (after 5 minutes), drawing of a clock, verbal fluency (animals) and calculation tests, was able to discriminate all controls from patients and only one patient was wrongly classified as normal control. These tests were chosen because they can be applied in less than 10 minutes and are very easy to interpret. This discriminant function must be applied in another group of patients and controls in order to demonstrate its value. When associated to the MMSE it may be useful to discriminate patients with dementia from normal people in epidemiological studies.