Admission hyperglycemia causes infarct volume expansion in patients with ICA or MCA occlusion: association of collateral grade on conventional angiography.

BACKGROUND AND PURPOSE: Hyperglycemia (HG) is associated with infarct volume expansion in acute ischaemic stroke patients. However, collateral circulation can sustain the ischaemic penumbra and limit the growth of infarct volume. The aim of this study was to determine whether the association between HG and infarct volume expansion is dependent on collateral circulation. METHODS: We performed a retrospective analysis of 93 acute ischaemic stroke patients with internal carotid artery or middle cerebral artery occlusion within 24 h of onset were retrospectively studied. HG was diagnosed in patients with an admitting blood glucose value ≥140 mg/dl. Angiographic collateral grade 0-1 was designated as poor collateral circulation and grade 2-4 as good collateral circulation. Infarct volume was measured at admission and at again within 7 days using diffusion-weighted magnetic resonance images. RESULTS: Among 34 patients with poor collateral grade, the change in infarct volume was significantly greater in the HG group than in the non-HG group (106.0 ml vs. 22.7 ml, P = 0.002). Among the 59 patients with good collateral circulation, the change in infarct volume was greater in the HG group than in the non-HG group (53.3 ml vs. 10.9 ml, P = 0.047). Multiple regression analysis indicated that admission HG (P = 0.004), baseline National Institutes of Health Stroke Scale score (P = 0.018), and poor collateral circulation (P = 0.040) were independently associated with infarct volume expansion. CONCLUSIONS: Infarct volume expansion was greater in individuals with HG on admission regardless of collateral circulation status.

Cardiovascular dysautonomia in de novo Parkinson's disease without orthostatic hypotension.

BACKGROUND: Clinical symptoms of Parkinson's disease (PD) include not only motor distress, but also autonomic dysfunction. OBJECTIVE: To study the characteristics of subclinical autonomic nervous dysfunction in de novo PD without orthostatic hypotension (OH). METHODS: Autonomic nervous function including cardiac sympathetic gain was evaluated on the basis of cardiac radioiodinated metaiodobenzylguanidine (MIBG) uptake, the response to the Valsalva maneuver, and spectral analyses of the RR interval and blood pressure in 20 patients with de novo PD without OH. RESULTS: Decreased cardiac MIBG uptake was found even in patients with PD without OH. Hemodynamic studies using the Valsalva maneuver revealed that patients with PD without OH had preserved baroreceptor reflex sensitivity in phase II and phase IV. Blood pressures normally responded in early and late phase II, but not in phase IV. Blood pressure recovery time was slightly reduced in patients with PD without OH when compared with the value in controls. The low frequency component of the RR interval and systolic blood pressure and the ratio of RR-LF to RR-HF in de novo PD without OH were significantly reduced when compared with the control values, whereas the high frequency component of the RR interval did not differ significantly. CONCLUSION: These results show that latent cardiac and vasomotor sympathetic dysfunction but not parasympathetic dysfunction is already present in early stage de novo PD, even without orthostatic hypotension.

Actions anchored by concepts: defective action comprehension in semantic dementia.

OBJECTIVE: To study the ability of patients with semantic dementia to understand actions, in order to examine the contribution of semantic memory to action comprehension. METHODS: The ability to comprehend symbolic and instrumental actions was assessed in 6 patients with semantic dementia and 10 healthy controls. The patients were also given the imitation test of meaningful and meaningless actions. RESULTS: In all patients with semantic dementia, comprehension of both symbolic and instrumental actions was defective. The comprehension of symbolic actions was more impaired than that of instrumental actions. Their ability to imitate other's actions was well preserved. CONCLUSION: This study showed that comprehension of action was impaired in semantic dementia, suggesting that semantic memory has an important role in comprehension of human action.

Neurophysiological evidence for altered higher brain functions in NIDDM.

OBJECTIVE: To investigate neurophysiological alterations of higher brain function in patients with NIDDM. RESEARCH DESIGN AND METHODS: Auditory P300 event-related potentials were recorded in 60 NIDDM patients who had no evidence of stroke, dementia, or any other neurological illnesses. The P300 wave latencies in diabetic patients were compared with those in neurologically healthy control subjects, with consideration of clinical parameters and diabetic complications. RESULTS: Diabetic patients had significantly longer P300 latencies than control subjects. Seven diabetic patients had pathologically prolonged P300 values. There was a trend toward longer P300 latencies in diabetic patients with retinopathy and in those with HbA1 of > or = 10%; the differences between these diabetic subgroups and control subjects were both statistically significant. However, three of seven diabetic patients with abnormal P300 latencies had no retinal lesions, and no significant correlation was observed among diabetic patients between P300 latencies and HbA1 levels. Peripheral neuropathy, nephropathy, blood glucose levels, and disease duration appeared not to correlate with P300 alterations. CONCLUSIONS: These findings suggest the presence of central nervous pathological processes in NIDDM that affect higher brain functions, as assessed by P300 latencies. Our findings also suggest that microangiopathy and metabolic derangement during the preceding 4- to 8-week period may contribute in small part to the pathophysiology of this central nervous involvement.

Development of diabetic complications in a new diabetic strain of rat (WBN/Kob).

The development of ocular, renal, and neural lesions was examined in male diabetic WBN/Kob rats with endoexocrine pancreatic insufficiency. As for the ocular lesions, around 15 months of age, opacity of the lens began to appear. Opacity was first observed in the periphery of the lens, and then increased rapidly in severity, extending concentrically and centripetally, until total cataracts developed. The incidence of cataracts in male rats was gradually increased and reached almost 100% at 24 months of age. As for renal lesions, the 24-h urinary total protein began to increase at about 13 months of age and reached 50-300 mg/24 h at 13-28 month of age, which was significantly higher than in age-matched male Wistar rats (15-25 mg/24 h). Electrophoretic analysis revealed that the urinary protein was almost all albumin. Morphologically, an increased GBM thickness and glomeruli with segmental or global enlargement of mesangial areas were observed. As for neural lesions, a reduction in motor nerve conduction velocity was demonstrated electrophysiologically, and a marked decrease in density and diameter of myelinated fibers in the sciatic nerves were observed morphometrically. In conclusion, the WBN/Kob rat strain with slowly developing but severe lesions associated with pancreatopathy presents a suitable model for human diabetic complications.

Prolongation of QTc interval and autonomic nervous dysfunction in diabetic patients.

We have attempted to clarify the relationships of the QTc interval to alpha and beta sympathetic, as well as parasympathetic function tests including spectral analyses of the R-R interval and systolic blood pressure. The QTc intervals were estimated in 76 diabetic patients and 76 age-matched healthy controls whose R-R intervals were comparable to those of the diabetic patients. We also investigated the relationships of the QTc interval to various clinical features of diabetes mellitus and to autonomic function tests. The QTc interval in diabetic patients was significantly greater than that in healthy controls. The QTc intervals were unrelated to diabetic control, retinopathy and nephropathy, but were prolonged in patients with a long duration of diabetes as compared to those with a short disease duration. There were significant correlations between the QTc interval and orthostatic hypotension, R-R variation and the Valsalva maneuver. Significant correlations were observed between the QTc interval and both the low and the high frequency component of spectral analysis of the R-R interval, whereas no correlations were found with spectral analysis of systolic blood pressure. An abnormal QTc interval is an indicator of cardiac sympathetic and parasympathetic nervous dysfunction, but not vasomotor dysfunction.

A young case with multi-infarct dementia associated with lupus anticoagulant.

A 39-year-old man was admitted because of an abrupt onset of right-side weakness and dysarthria. During the 2 years before admission, he had suffered from insomnia, depressed mood and progressive memory disturbance. Neurological and psychiatric examination revealed severe intellectual impairment in addition to the neurological deficits. Neuroradiological examinations revealed multiple brain infarcts. He had no risk factor for stroke except for lupus anticoagulant. He was diagnosed as having multi-infarct dementia associated with antiphospholipid antibodies. This case suggests that it is necessary to investigate antiphospholipid antibodies in addition to neuroradiological examination when relatively young patients present with unexplained cognitive or behavioral symptoms.

Comparison of color flow and 3D image by computer graphics for the evaluation of carotid disease.

Morphologic characteristics and hemodynamics in the carotid artery were investigated in patients with stroke by use of Doppler color-flow analysis and three-dimensional (3D) analysis by computer graphics. The 3D imaging of the carotid bifurcation was reconstructed from a series of transverse B-mode images. Comparison of color-flow and 3D images demonstrated that wall configuration in vivo, dilation, and curvature play an important role in reversal of flow and stagnation at the carotid bifurcation. Furthermore, 3D analysis is very useful in mapping the distribution of the atheromatous plaques. The analyses suggest that it is important to investigate the wall configuration of the bifurcation in vivo on flow patterns, especially flow separation and stagnation, in relation to the development of atheromatous plaques.

[The effects of nicotine on a patient with spinocerebellar degeneration whose symptoms temporarily exacerbated by cigarette smoking].

The purpose of the present study was to elucidate the influences of nicotine on a patient with spinocerebellar degeneration whose symptoms temporarily exacerbated by cigarette smoking. We have examined the influences of nicotine gum on cerebellar ataxia. Limb ataxia, dysarthria and truncal titubation were maximally aggravated 15 min after nicotine gum chewing, which paralleled blood nicotine level. Specific increased accumulation of 123I-IMP was observed in the cerebellum 15 min after nicotine gum load by 123I-IMP-SPECT. On the other hand, in a control with spinocerebellar degeneration whose symptoms not changed by nicotine gum load, the specific finding on 123I-IMP-SPECT was not detected. These findings suggested a direct effect of nicotine on the cerebellum.

[Relation between autonomic dysfunction and progression of Parkinson's disease].

It has been claimed that cardiovascular dysfunction in patients with Parkinson's disease is less severe than multiple system atrophy. Autonomic dysfunction, however, increases with progression of Parkinson's disease. We studied the relation between autonomic dysfunction and disease severity by cardiovascular function testing with the Valsalva maneuver. The study group comprised 20 healthy controls (age, 52.7 +/- 13.6 years) and 31 patients with Parkinson's disease (59.0 +/- 7.2 years), including 13 who were previously untreated (55.0 +/- 6.2 years) and 18 who were receiving levodopa (61.8 +/- 7.1 years). The Valsalva maneuver was done having the subject exhale into a mouthpiece with an expiratory pressure of 40 mmHg for 15 seconds. Blood pressure and RR interval were measured during the Valsalva maneuver by tonometry, using a non-invasive blood pressure monitoring system (ANS 508, Nihon Colin Co., Ltd). Baroreceptor reflex sensitivities (BRS) of the second phase (BRS II) and fourth phase (BRS IV) of the Valsalva maneuver were calculated, and blood pressure elevations during the late second phase (IIp) and fourth phase (IVp) were measured. BRS II, BRS IV, and IVp in the patients of Parkinson's disease were significantly lower than those of healthy controls. BRS II, BRS IV, and IIp, however, did not significantly differ between the previously untreated patients and healthy controls. IVp of the previously untreated patients was significant lower than that of healthy controls. BRS II and BRS IV of patients with Parkinson's disease who were receiving levodopa for less than 5 years were significantly lower than those of the healthy controls. BRS II, BRS IV, and IIp decreased as disease duration increased, while IVp was unrelated to disease duration. These results suggest that patients with early Parkinson's disease have cardiac sympathetic autonomic dysfunction with maintained baroreceptor reflex sensitivity. Reduced baroreceptor reflex sensitivity was associated with levodopa treatment in patients with Parkinson's disease. Baroreceptor reflex sensitivity decreased and vasomotor sympathetic autonomic dysfunction developed as duration of disease increased.

[Central pontine myelinolysis followed by frequent hyperglycemia and hypoglycemia--report of an autopsy case].

A case of central pontine myelinolysis (CPM) followed by hyperglycemia and hypoglycemia was reported. The case was 53-year-old female. Diabetes mellitus was found when she was 32 years old, insulin therapy was started at 37 years of age. Since she was 50 years old, proteinuria and ankle edema had developed and she was admitted to The Keihin Hospital. The peritoneal dialysis (PD) was performed next year, followed by the hemodialysis (HD). In January 1978, strange movements and the disturbance of her consciousness were occurred during PD, then blood glucose level showed over 1,800 mg/dl and serum osmolarity was over 390 mosm/KgH2O. Then she was diagnosed as non-ketotic hyperosmolar coma. After that, during HD and PD, hyperglycemia (approximately 1,200 mg/dl) and hypoglycemia (approximately 40 mg/dl) developed frequently. She died soon after HD on 19th December 1979. The autopsy disclosed bilateral atrophic kidneys due to diabetic changes and atrophic pancreas. Gross neuropathological findings revealed a few small infarcts at the putamen and the globus pallidus, however, other area were observed to be normal. The most remarkable change in microscopical finding was nearly symmetrical demyelinative lesion in the center of the basis pontis. The nerve cells and axon cylinders were relatively well preserved in the demyelinative lesion. The hyaline degeneration was observed in the arterial wall, however, any arterial obstruction was not found. Recent studies would suggest that the electrolyte disturbance, such as hyponatremia, may lead to CPM, particularly when this disturbance was rapidly corrected. On the other hand, CPM induced by diabetic coma has been reported, however, its pathogenesis has been unclear.(ABSTRACT TRUNCATED AT 250 WORDS)

[An autopsy case of Prader-Labhart-Willi syndrome].

An autopsy case of 23-years-old man with Prader-Labhart-Willi syndrome (P-L-W syndrome), who had died by acute renal failure due to burn injury, was reported. P-L-W syndrome was constituted by hypotonia, hypogonadism, hypomentia, obesity and other minor anomalies, however, CNS anomaly had not been reported. The patient sat at 3 years of age, walked at 4 years old, began to utter single words at 3-4 years, and he began to obese at 4 years of age. He fulfilled the condition of P-L-W syndrome mentioned above. On his age 15, laboratory findings on admission revealed remarkable diabetic pattern by oral glucose tolerance test and intelligence quotient was 28, and the other laboratory findings were within normal limit. During his clinical history, complications of diabetes mellitus, such as diabetic retinopathy and neuropathy, were aggrevated, and upstanding and gait were impossible at 20 years of age. On his age 23, he suffered from burn injury at left lower extremity and he fell in acute renal insufficiency. Five autopsy cases of P-L-W syndrome have been reported so far, however, CNS anomaly has not been observed. Following anomalies in our case was recognized, such as shortness of the frontal lobe, partial micropolygyria of the dentate nucleus, heterotopia of the inferior olivary nucleus, ectopia of Purkinje cell in the molecular layer, heterotopia of middle sized neuron in the deep white matter of the cerebellum and large number of residual nerve cells in the cerebral subcortical white matter.(ABSTRACT TRUNCATED AT 250 WORDS)

[Clinicophysiological study of tremor in Parkinson's disease: quantitative tremor-based assessment of motor count using actigraphy].

In Parkinson's disease, resting tremor is often the initial symptom. This report focuses on the mechanism underlying tremor in Parkinson's disease and quantitative assessment of tremor. Central factors including Vim (nucleus ventralis intermedius) in the thalamus, and peripheral factors, such as acceleration of input pathways from muscle spindles via muscle tonus, are important aspects of the tremor mechanism in Parkinson's disease. It has also been suggested that tremor in Parkinson's disease is associated with parasympathetic and sympathetic dysfunctions. Objective assessments of tremor, such as the application of surface electromyography, are useful in the diagnosis and treatment of Parkinson's disease. Actigraph, as introduced herein, is a three dimensional motor sensing apparatus. Therefore, motor counts over 0.01 G can be detected by actigraphy. To date, this device has been used for evaluating akinesia in Parkinson's disease and insomnia. In this study, actigraphy was used in Parkinson patients with tremor, and it reflected motor activity in the wrist and was associated with the severity of hand tremor. Activities of daily living (ADL) are disturbed by hyperkinesia of the hand for Parkinson patients with hand tremor. We demonstrated that actigraphy is a simple and quantitative method of assessing motor activity in Parkinson patients with tremor.

[Central motor conduction time using magnetic and vibratory stimulation in Parkinson's disease, especially in patients with rigidity].

Rigidity, tremor, akinesia and disorder of postural reflex are the main clinical features of Parkinson's disease. We presented the mechanism underlying rigidity and assessed central motor conduction time (CMCT) using magnetic, with or without vibratory, stimulations. Basal ganglia, especially, the internal pallidum, and the thalamus play major roles in the mechanism of rigidity in Parkinson's disease. Hyperexcitability of the spinal motor nucleus due to low threshold has been recognized. Magnetic stimulation is painless and is simpler than electric stimulation. Therefore, this method is used clinically for evaluating conduction disturbance of the upper motor neurons in multiple sclerosis, cerebrovascular disease and so on. CMCT measured by magnetic and/or electric stimulation may be abbreviated or normal in Parkinson's disease, according to the literature, though controversy persists in this regard. In our study, CMCT was normal in Parkinson patients. However, CMCT was reduced in patients with rigidity and tremor. Furthermore, in a portion of the patients, CMCT was further abbreviated by also applying vibratory stimulation. These observations support the hypothesis that cells in the thalamus, cortex and spinal cord and/or pathways in these portions of the central nervous system are excitable or activated in Parkinson patients with rigidity and tremor. However, elucidation of the mechanisms underlying rigidity and tremor awaits further investigation.

[Chronic fatigue syndrome--51 cases in the Jikei University School of Medicine].

Between April 1991 and August 1992, we diagnosed 51 cases of CFS who met definition of CFS designated by CDC, 1988. They are 41 female and 11 male, and 78% are women. At first visit, their ages are ranged from 16 to 64 years old, and approximately 45% is 20 to 30 years old. In periods of illness from onset, 39.2% of the patients are in period of 6 month to 1 year, 19.6% within 2 years, and 15.6% within 3 years, respectively. The sufferer who have symptoms of CFS over 10 years long are in 6 cases. Most of patients have already been examined by many other clinics and hospitals. They have been told as no abnormal medical condition, or often as neurosis, depressive state and autonomous imbalance etc. Interesting things are trigger of CFS. 77.5% of patients have onset of flu-like symptom, including 5 cases of acute infectious mononucleosis. In many female patients, symptoms of CFS begun after hand work in addition to psychological factors. Specific laboratory results are not shown in CBC, urinalysis, biochemical studies and inflammatory marhers. 6 cases hare positive Rheumatoid factor and positive ANF are shown in 16 cases (31.3%). Specific patterns of anti EBV antibodies are not shown. Lymphocyte subsets used by monoclonal antibodies are not specific. At the present, prognosis is good and 56.8% of CFS patients are generally improved. For severe cases, NSAID, Sulpiride, Amitryptiline and minor tranquilizer are used.

Reduced cardiac 123I-MIBG uptake reflects cardiac sympathetic dysfunction in Lewy body disease.

OBJECTIVE: To examine the relation between the results of cardiac (123)I-meta-iodobenzylguanidine (MIBG) scintigraphy and cardiovascular autonomic function in Lewy body disease (LBD). METHODS: The subjects were 66 patients with LBD, 44 of whom had Parkinson disease (PD), 10 PD with dementia (PDD), and 12 dementia with Lewy bodies (DLB); 20 age-matched healthy subjects were studied as controls. Cardiovascular autonomic function was evaluated on the basis of cardiac (123)I-MIBG uptake, cardiovascular autonomic response on the Valsalva maneuver (VM), and systolic blood pressure (SBP) response on head-up tilt table (HUT) testing. RESULTS: Patients with LBD had reduced cardiac (123)I-MIBG uptake, cardiovascular autonomic response on the VM, and SBP response on HUT testing as compared with controls. Cardiac (123)I-MIBG uptake and cardiovascular autonomic function in PDD and DLB were severely impaired as compared with those in PD. Cardiac (123)I-MIBG uptake in LDB was not significantly related to vasomotor sympathetic function, baroreceptor reflex gain, cardiac parasympathetic function, or the changes in SBP on HUT testing. Cardiac (123)I-MIBG uptake was, however, significantly related to the blood pressure overshoot in phase IV of the VM. CONCLUSION: Cardiac (123)I-meta-iodobenzylguanidine uptake clinically reflects cardiac sympathetic dysfunction in Lewy body disease.