Ubiquitin-positive neuronal and tau 2-positive glial inclusions in frontotemporal dementia of motor neuron type.

Attempts at classification of fronto-temporal dementias have not yet been completely successful. We report ten cases of sporadic fronto-temporal dementia (FTD) with ubiquitin-positive neuronal inclusions in cortex or in motor neurons in brain stem or spinal cord, which may contribute to the classification of FTD. Marked variation in clinical presentation as well as in pathological findings was the rule in all cases. Dementia was a prominent feature. Only one case had clinical features suggestive of motor neuron disease. Three of four younger onset cases displayed an especially severe atrophy of the temporal lobes, the basal ganglia and the substantia nigra. This contrasted with the other seven cases in which the fronto-temporal atrophy and changes in basal ganglia and substantia nigra were variable and sometimes mild. In addition to the presence of ubiquitin-reactive, but tau-and silver impregnation-negative neuronal inclusions, all cases demonstrated tau 2-positive glial inclusions, similar to those recently reported in three motor neuron disease cases with dementia. The glial inclusions were not visible with antibody to tau 1. Reaction with antibody to alpha-synuclein was invariably negative. If the combination of ubiquitin-positive neuronal and tau 2-positive glial inclusions is found to be consistently present in FTD of motor neuron type, this feature will provide a firmer basis for this diagnosis than previously available.

Suprasellar adrenocorticotropic hormone-secreting ectopic pituitary adenoma: case report and literature Review.

OBJECTIVE AND IMPORTANCE: Functional ectopic pituitary adenomas are rare and can be misdiagnosed as extensions of pituitary adenomas when they are located in the vicinity of the normal gland. In this report, we present a case of an ectopic adrenocorticotropic hormone-secreting suprasellar pituitary adenoma that caused Cushing's disease. A literature review of previously reported ectopic pituitary adenomas is included to illustrate the diverse clinical manifestations of this disease entity. CLINICAL PRESENTATION: An 11-year-old boy was noted to have hirsutism, a buffalo hump, and unexplained weight gain consistent with Cushing's syndrome. Laboratory investigations revealed that the boy had elevated adrenocorticotropic hormone and serum cortisol levels unsuppressed by dexamethasone. Magnetic resonance imaging scans were suggestive of a pituitary adenoma with suprasellar extension. INTERVENTION: The initial transsphenoidal approach failed to achieve complete surgical resection. A repeat operation in which the pterional approach was used revealed a suprasellar pituitary adenoma without association with intrasellar contents. The patient's cushingoid symptoms improved significantly 3 months after surgery. CONCLUSION: Ectopic pituitary adenomas should be considered in the differential diagnosis for all patients with Cushing's syndrome. Furthermore, surgical approaches should be chosen carefully once the diagnosis of ectopic pituitary adenoma is made.

Common carotid artery wall properties in Takayasu's arteritis.

The vessel wall properties of the common carotid artery have been noninvasively and quantitatively assessed with an ultrasonic instrument. Stiffness parameter beta, which represents the mechanical properties of the vessel, was calculated from the relationship between blood pressure and the diameter of the artery. There are no reports that quantitatively assess wall properties in Takayasu's arteritis. The authors compared, in vivo, the vessel wall properties of the common carotid artery in 14 patients with Takayasu's arteritis versus those in 60 normal subjects (controls). They measured changes in the inner diameter of the artery between systole and diastole with an ultrasonic, phase-locked, echo-tracking system. Beta was significantly higher in Takayasu's arteritis than in normal subjects (better than a 99% confidence interval). The findings for each decade were as follows: 3rd decade (20s): 35.7 +/- 28.9 vs 5.01-6.46, P=0.0001; 4th decade (30s): 19.5 +/- 9.71 vs 6.09-7.80, P=0.02; 5th decade (40s): 26.2 +/- 11.3 vs 7.26-9.28, P = 0.0001; 6th decade (50s): 19.1 +/- 4.27 vs 8.66-11.25, P = 0.0001. Takayasu's arteritis significantly impaired the mechanical performance of the common carotid artery. Thus, beta shows promise as a useful diagnostic indicator of Takayasu's arteritis.

Reliability of inferred age, and coincidence between inferred age and chronological age.

Outdoor research is restricted by many factors. The age inference was one of the biggest problems for the outdoor researchers. We have investigated the reliability of inferred age for the Japanese people, and took out the estimation formula for the age, even if it was based on the inferred age. The age classification was the most popular method for this purpose, and there were many classifications. We took the classification of young, middle aged, and elderly groups, in which classification of the SDs were rather small, that is, 4, 5, and 7 years for the young, middle aged, and elderly age groups, respectively.

Correlation of ultrasound-measured common carotid artery stiffness with pathological findings.

To quantitatively and noninvasively evaluate common carotid atherosclerosis in a series of patients, we measured the stiffness parameter beta, which represents the mechanical properties of the vessel. beta was calculated from the relationship between blood pressure and the diameter of the artery as measured by an ultrasonic, phase-locked, echotracking system. Increases in the severity grade of atherosclerosis as subsequently determined at autopsy were correlated with increased beta values in 60 common carotid arteries (r = .68). Patients with beta values greater than 13 had a pathological diagnosis of atherosclerosis in the common carotid artery. The sensitivity of this discrimination ratio was 80%, and the specificity was 80% as well. Thus, beta shows promise as a useful diagnostic indicator for detecting asymptomatic common carotid atherosclerosis.

A study on cerebral nicotine receptor distribution, blood flow, oxygen consumption, and other metabolic activities--a study on the effects of smoking on carotid and cerebral artery blood flow.

We investigated middle cerebral artery flow velocity (MCA-FV) by a noninvasive method to determine whether or not smoking causes an increase in cerebral blood flow (CBF). Furthermore we determined sequentially the changes in CBF caused by smoking in order to evaluate changes in responses at different times in daily activities and the effect from meals. The subjects were 25 healthy individuals ranging in age from 20 to 36 yr. MCA-FV was measured by a transcranial Doppler system. They smoked a filtered cigarette for 5 min at 1 P.M., 3 P.M., 6 P.M., 8 P.M., 10 P.M., 8 A.M., and 11 A.M. Results (1) Smoking caused increases in both common carotid artery flow volume and MCA-FV, and the percentage increase of these parameters showed a good correlation (r = 0.809). (2) MCA-FV increased significantly during the first (by 6.6%) and second halves (by 5.4%) of the smoking period. (3) The change in MCA-FV after meals was slight. (4) Smoking tended to increase MCA-FV during each smoking session but the changes were not significant. The pulsatility index reduced significantly during almost every smoking session. These results lead to the conclusion that smoking reduces vascular resistance in cerebral arteries and increases CBF.

[Cardiac function noninvasively evaluated by phasic pattern analysis of ventricular power during ejection].

To evaluate cardiac function, ventricular power during ejection (power) and the rate of change of power (dPower/dt) were assessed noninvasively. Power was determined from the product of aortic flow (Fa(t] and brachial arterial pressure (Pa(t]. Fa(t) was measured at the suprasternal notch using an ultrasonic pulsed Doppler flowmeter with a 2 MHz carrier frequency and 10 KHz repetition frequency. The maximum detectable blood velocity was 380 cm/sec. Pa(t) was measured using a newly-developed method based on indirect unloading techniques and an air pressure system. There were 21 normal subjects and seven patients with coronary artery disease in this study. The following results were obtained. There was no significant difference between power patterns calculated by Pa(t) and Pao(t) (aortic pressure measured by catheter). The average peak dPower/dt was 160.2 J/sec2 in normal subjects, 145.2 L/sec2 in patients with ejection fractions greater than 50%, and 93.5 J/sec2 in patients with ejection fraction less than 50%. Peak dPower/dt was significantly decreased in patients with the lower ejection fraction (p less than 0.005). The results indicated that this index is clinically useful in evaluating cardiac contractility.