A 52-year-old woman with dyskinesia, epilepsy and gait disturbance]

We report a 52-year-old Japanese woman who developed dyskinesia, epilepsy, and gait disturbance. She was well until 35 years of age, when she noted the onset of gait disturbance. She also noted abnormal involuntary movements in her limbs. She also noted dysarthria at age 38. A neurologist examined her at age 41. The neurologist found cerebellar ataxia and dyskinesia. The atrophy of the brain stem and the cerebellum was on CT. She started to have generalized convulsion with loss of consciousness. Dementia became apparent at age 40. In October, 1993, she became psychotic in which she behaved violently taking off her clothes shouting as "Fire". She was treated with major tranquilizers and became quiet. However, choreic movements became prominent. Her subsequent course was complicated with dysphagia, dementia, convulsion, and frequent bouts of pneumonia. She expired on January 24, 2000 after developing pneumonia. Her father and one sibling had similar motor disturbances. She was discussed in a neurological CPC. The chief discussant arrived at conclusion that the patient had dentatorubral-pallidoluysian atrophy. Most of the participants agreed with this diagnosis. Postmortem examination revealed that entire brain looked smaller than normal including the brain stem and the cerebellum. The cerebellar dentate nucleus showed loss of neurons and gliosis; glumose degenerations were also seen. The external segment of the pallidum showed neuronal loss and gliosis. The subthalamic nucleus showed gliosis without neuronal loss. A demyelinated focus was found in the pons; the lesion looked similar to central pontine myelinolysis. The cerebral white matters were unremarkable. Other areas were unremarkable. The pathological diagnosis was dentatorubral-pallidoluysian atrophy. The pathologic lesion which might explain her dementia was not apparent.     

Activation of hypothalamic angiotensin receptors produces pressor responses via cholinergic inputs to the rostral ventrolateral medulla in normotensive and hypertensive rats

We have previously reported that the angiotensin system in the anterior hypothalamic area (AHA) is enhanced in spontaneously hypertensive rats (SHR) and that this enhancement is involved in hypertension in SHR. In addition, acetylcholine (ACh) release is increased in the rostral ventrolateral medulla (RVLM) of SHR, which has also been shown to be involved in hypertension in SHR. In this study, we examined whether the enhanced angiotensin system in the AHA of SHR is related to the increase in cholinergic inputs to the RVLM. Electrical stimulation in the AHA produced a pressor response and an increase in firing rate of RVLM barosensitive neurons. These responses were inhibited and enhanced by RVLM application of the muscarinic receptor antagonist scopolamine and the cholinesterase inhibitor physostigmine, respectively. AHA stimulation also produced release of ACh in the RVLM. Microinjections of angiotensin II and carbachol into the AHA produced pressor responses. The pressor response to angiotensin II was inhibited by scopolamine microinjected into the RVLM, although this produced no effect on the response to carbachol. In SHR, although not in Wistar-Kyoto rats, microinjection of losartan into the AHA inhibited pressor responses to physostigmine. However inhibition was not observed in response to the directly acting muscarinic receptor agonist carbachol, injected into the RVLM. These findings demonstrate that angiotensin receptor activation or electrical stimulation in the AHA produce a pressor response via an increase in ACh release in the RVLM. In addition, the present study suggests that the enhanced angiotensin system in the AHA of SHR increases cholinergic inputs to the RVLM, which leads to increases in blood pressure.     

Different blood acetaldehyde concentration following ethanol administration in a newly developed high alcohol preference and low alcohol preference rat model system

A significant difference in blood-acetaldehyde concentration was observed between high alcohol-preference (HAP) rats and low alcohol-preference (LAP) rats, newly developed different alcohol preference lines. This difference of acetaldehyde accumulation may be due to cytosolic aldehyde dehydrogenase (ALDH1) polymorphism, which has been reported previously. As the doses of ethanol we employed are higher than that of voluntary drinking, there may be little direct relationship between acetaldehyde accumulation and alcohol preference. We suggest therefore that the ALDH1 polymorphism is associated with alcohol preference in HAP/LAP lines through some other unidentified mechanism.     

Purification and partial characterization of acetyl-coA synthetase in rat liver mitochondria

Acetyl-CoA synthetase (AceCS), which catalyzes the activation of acetate to produce acetyl-CoA, was found to have a much greater Km value for acetate in liver mitochondria than that in the heart mitochondria of rats, indicating that two different types of AceCS are located in the liver and heart mitochodria. Recently, Fujino et al. reported that mouse heart mitochondrial AceCS, designated AceCS2, was expressed in a wide range of tissues, however, it was apparently absent from the liver. In this study, liver mitochondrial AceCS activity, but not heart AceCS2, was greatly induced in di(2-ethylhexyl)phthalate (DEHP)-treated rats. We purified and characterized the rat liver mitochondrial AceCS. The molecular mass of the enzyme estimated by SDS-PAGE was -58 kDa, which was quite different from that of the heart mitochondrial enzyme, AceCS2. The calculated Km value for the acetate of the partially purified liver enzyme was much greater, being about 100 times that of heart enzyme, AceCS2.     

Effects of local administration of interferon-beta on proliferation of retinal pigment epithelium in rabbit after laser photocoagulation

PURPOSE: To investigate whether local administration of interferon (IFN)-beta promotes proliferation of the retinal pigment epithelium (RPE) in vivo. METHODS: Following local injection of IFN-beta into the sub-Tenon space of rabbit eyes, the penetration of IFN-beta into various intraocular areas was determined by means of enzyme-linked immuno-adsorbent assay. Retinal lesions were produced by laser photocoagulation (PC), and IFN-beta (1 x 10(6) IU, 1 x 10(5) IU, or 1 x 10(4) IU) was administered into the sub-Tenon space. Physiological saline was substituted for IFN-beta in controls. The proliferation of RPE cells was inspected histopathologically. RESULTS: After IFN-beta administration, IFN-beta was found in all intraocular areas examined, with the highest concentration detected in the choroid. After PC, profuse proliferation of RPE cells began earlier in the rabbits that received the highest dose of IFN-beta than in the control rabbits; repair of the central part of the coagulated lesion in those rabbits was complete within 7 days after PC. In control rabbits, the histopathologic wound repair process proceeded more slowly and to a limited extent. Proliferation of RPE cells in the low and medium dose IFN-beta-treated rabbits was similar to that in the control rabbits. CONCLUSION: The present study demonstrates that repair of the PC-induced retinal lesions, particularly the proliferation of RPE cells, is promoted in vivo by local administration of IFN-beta.     

Epidemiologic Studies on mumps. II. Prospective, Periodic Surveillance on Mumps in Three Different Areas

Prospective, periodical sero-epidemiological surveillance on mumps was carried out in three different populations in Aichi prefecture. 1) In a newly developed residental quarter full of big apartment buildings, where heavy population density burst has been occurring, mumps has been observed as an endemic disease. Serologically, almost all the children under six years old has experienced mumps infection in such an area. 2) In an old city with stable population dynamics, mumps outbreaks have been taken place pediodically. The results of serosurvey suggested that the periodic outbreaks occurred when more than 60–70% of the children became susceptible, regardless of seasons. 3) In a relatively isolated small town, mumps epidemics has not been observed for many years and when the disease is introduced into the community however, large outbreak develops in the town.     

Impact of previous multiple portoenterostomies on living donor liver transplantation for biliary atresia

BACKGROUND/AIMS: The present study examined the impact of portoenterostomy on the morbidity and mortality of patients who later underwent living donor liver transplantation for biliary atresia. METHODOLOGY: Sixty-one consecutive patients from January 1996 to May 2001 were analyzed. They were divided into two groups according to the number of previous portoenterostomies: once (group A, n=26) and twice or more (group B, n=35). Preoperative status, mortality, morbidity, hospital duration and survival were examined and compared between the groups. RESULTS: Preoperative parameters regarding liver function and urgency status were comparable between the groups. The operation duration tended to be longer in group B than in group A (p=0.07). The blood loss and transfusion volumes in group B were greater than those in group A (p=0.03 for both comparisons). Vascular complications tended to be more frequent in group B patients. However, this difference was not significant (12% vs. 29%, p=0.06). The duration of hospitalization was longer in group B (p=0.04). Survival rates were comparable between the groups. CONCLUSIONS: Our surgical results suggest that multiple previous portoenterostomies might have negative short-term effects in patients who undergo living donor liver transplantation for biliary atresia.     

Renal actinomycosis mimicking renal tumor: case report

A 68-year-old man was admitted after fever and general fatigue with severe inflammatory signs and anemia. T1- and T2-weighted magnetic resonance imaging showed low- to isointensity and low-intensity tumor in the right kidney, respectively, suggesting renal actinomycosis. However, the right kidney was explored transabdominally because the possibility of renal malignant lymphoma could not be excluded. After nephrectomy, characteristic colonies of Actinomyces were seen microscopically, and the histologic diagnosis was renal actinomycosis. The patient was treated with antibiotics and made good progress after operation. This case highlights the importance of magnetic resonance imaging for the diagnosis of renal actinomycosis.