Performance of a rapid human metapneumovirus antigen test during an outbreak in a long-term care facility.

Using a newly developed rapid test, an outbreak of human metapneumovirus (HMPV) infection in a long-term care facility was detected within only 2 days after the onset of symptoms in a putative index case. The outbreak was almost under control within 8 days mainly by zoning patients, with the exception of two cases of HMPV that were diagnosed 16 and 17 days after the onset of the outbreak. According to an immunological diagnosis as well as the rapid test, it was eventually proven that 18 patients had HMPV infections. We suspected that even asymptomatic residents, who had not been completely separated from the facility population, were a source of infection. That suggested that all asymptomatic residents should be tested and that the separation of the infected patients should be absolute, if an outbreak of HMPV infection is suspected in such a facility.

Saccular aneurysm of pulmonary autograft after the Ross procedure.

The Ross procedure, which includes removal of the malformed aortic valve and replacement of a pulmonary autograft in the aortic position, has increased the number of available treatment options. Recently, dilatation of the autograft pulmonary root after the Ross procedure has been reported as a complication. We report a patient with bicuspid aortic valve malformations and aortic annulus dilatation, who had a saccular-form, true-type, aneurysm in the pulmonary autograft seven months after the Ross procedure. These changes have not been described so far as complication. Pathologically, marked mucoid degeneration was noted in the tunica media of the aneurysm, as well as in the original aortic root. These findings may suggest similar pathological characteristics between the aorta and pulmonary arteries. Hence, the surgical risks accompanying vascular characteristics in patients with congenital aortic valve malformations should be considered.

Successful three-dimensional reconstruction using transesophageal echocardiography in a patient with a left atrial myxoma.

Two-dimensional echocardiography can provide intracardiac images. However, the cross-sectional images require mental reconstruction to understand a three-dimensional intracardiac structure. It is sometimes hard for inexperienced echocardiographers to engage in reconstruction. Thus, three-dimensional echocardiography is potentially beneficial because these images can provide extra information without mental reconstruction. Herein we demonstrate three-dimensional reconstruction using transesophageal echocardiography in a patient with a left atrial myxoma. It contributed to clarifying the surgical considerations, including whether the tumor was adhering to the left atrium or the mitral valve.

Decreased cerebrospinal fluid levels of beta-phenylethylamine in patients with Rett syndrome.

To clarify the mechanism of brain impairment in Rett syndrome, we measured the cerebrospinal fluid levels of beta-phenylethylamine (PEA) in 17 patients with Rett syndrome. Findings were compared with those obtained in age-matched controls and diseased controls. The cerebrospinal fluid level of PEA was significantly lower in patients with Rett syndrome than in the controls (31% of control values). The alteration in the cerebrospinal fluid level of PEA may reflect dopamine system impairment in Rett syndrome.

[Pitfalls in echocardiographic diagnosis of mitral bileaflet prolapse].

Preoperative echocardiography provides good planning information for successful repair of mitral valve regurgitation, but identifying the prolapse of both the anterior and posterior leaflets is sometimes difficult. To clarify the cause of this problem, preoperative echocardiographic findings and intraoperative observations of the prolapse were analyzed in 124 patients with non-rheumatic pure mitral regurgitation. In 48 patients with final diagnoses of bileaflet prolapse, 16 (33%) were considered to have only single leaflet prolapse before the operation. Anterior leaflet prolapse was overlooked in 14, and prolapse of either of its commissural segments was the least detectable by echocardiography. Chordal rupture was seen more in the posterior leaflet than in the anterior leaflet. Movement of the anterior leaflet may be influenced by a prolapsed and hypermobile posterior leaflet and/or regurgitant jet flow caused by the posterior leaflet prolapse.

CSF beta-endorphin levels in patients with infantile autism.

We measured CSF levels of beta-endorphin, an opioid hormone, in 19 patients with infantile autism and in 3 patients with Rett syndrome, and compared them with control values. In infantile autism, CSF levels of beta-endorphin did not differ significantly from those of age-matched controls. There was no significant correlation between CSF levels and clinical symptoms, including self-injurious behavior, pain insensitivity, and stereotyped movement. However, CSF levels of beta-endorphin were significantly higher in the patients with Rett syndrome than in the control (p < .05). Data suggest that neurons containing beta-endorphin may not be involved in patients with infantile autism. Thus, there is no relationship between dysfunction of brain opioid and autism.

Idiopathic restrictive cardiomyopathy with diffuse perimyocytic fibrosis--a rare observation.

Idiopathic restrictive cardiomyopathy (RCM) is rare. Even today little is understood of its etiology or underlying mechanisms, and definitive diagnostic criteria are lacking. In this report, we describe a case of idiopathic RCM in a young Japanese woman who died while awaiting cardiac transplantation during the 5-year course of the disease. Rare pathologic findings of diffuse perimyocytic fibrosis were revealed at autopsy.

Serial magnetic resonance images in a patient with congenital sensory neuropathy with anhidrosis and complications resembling heat stroke.

We report the results of serial computerized tomography (CT) and magnetic resonance imaging (MRI) in a 9-month-old Japanese girl with the rare disorder, congenital sensory neuropathy with anhidrosis (CSNA). She developed a prolonged high fever, anorexia, and weight loss with laboratory findings of hemoconcentration and elevated levels of GOT, LDH and creatine phosphokinase (CK) in May 1995, and was hospitalized. The cerebrospinal fluid (CSF) was normal on admission. Elevation of CSF myelin basic protein on the 16th hospital day suggested a destruction of the myelin sheath. The first MRI performed on the 16th hospital day revealed no marked abnormalities when the patient exhibited a high fever, generalized tonic-clonic convulsions, and impaired consciousness. The patient had a persistent high fever, and developed a second generalized tonic clonic convulsion and became comatose. A second MRI on the 20th hospital day showed a bilateral symmetrical paracentral hypo-intensity of the white matter with occipital hypo-intensity on T2-weighted images. MRI findings were considered to represent the complications of the high fever with a loss of water from the cerebral cortices and deep white matter. MRI and CSF findings indicated the presence of brain damage due to the high fever.

Acute disseminated encephalomyelitis after treatment with Japanese B encephalitis vaccine (Nakayama-Yoken and Beijing strains).

Seven children with acute disseminated encephalomyelitis (ADEM) after treatment with Japanese B encephalitis vaccine (JBEV) (Nakayama-Yoken strain 1968-88 and Beijing strain 1989-93) were identified by mailed questionnaires and by compilation of previously published case reports. It was considered that encephalomyelitis might have been related to vaccine treatment as the vaccine is derived from mouse brain tissue infected with Japanese B encephalitis virus, a potentially cross reactive antigen. The incidence of severe neurological complications associated with the newer Japanese B encephalitis Beijing strain vaccine seemed to be less than one case per 1,000,000, which is similar to the incidence of neurological complications associated with the older Nakayama-Yoken strain vaccine.

A novel immunoassay of smooth muscle myosin heavy chain in serum.

We have developed a double monoclonal sandwich enzyme immunoassay to measure smooth muscle myosin heavy chain (MHC). Analytical performance of the assay showed reliable detection of smooth muscle MHC in human sera. The mean of the smooth muscle MHC level in normal human sera was 0.9 +/- 0.9 ng/ml. In sera of patients with aortic dissection, the smooth muscle MHC level sharply elevated at the onset and rapidly decreased to normal levels. Immunoassay of smooth muscle MHC in serum is a promising method for biochemical diagnosis of smooth muscle disorders.

Serial transesophageal echocardiography imaging of a postoperative aortic ring abscess: a case report.

Transesophageal echocardiography (TEE) was used for the serial observation of a postoperative perivalvular abscess of a prosthetic aortic valve in a 65-year-old man who underwent aortic valve replacement for aortic regurgitation. Five months later, TEE revealed an aortic ring abscess. Ascending aortic grafting (Bentall method) and translocation of the aortic prosthetic valve were conducted for progressive mechanical valve dysfunction. Postoperative TEE showed flow between the left ventricular outflow tract and the abscess, which was unrestricted 1 month later. Associated clinical improvement suggested that washout of the abscess was a critical factor. This case illustrates the usefulness of TEE for examining postoperative aortic ring abscess.

Cerebrospinal fluid biopterin and biogenic amine metabolites during oral R-THBP therapy for infantile autism.

Treatment with 6R-L-erythro-5,6,7,8-tetrahydrobiopterin (R-THBP) has been suggested to improve autistic behavior. Cerebrospinal fluid (CSF) levels of total biopterin, oxidized and reduced forms of biopterin, homovanillic acid, and 5-hydroxyindoleacetic acid were measured in 14 autistic children and 18 controls to clarify the mechanism of action of R-THBP. The 14 autistic children received R-THBP orally at 1 mg/kg per day; 7 children showed clinical improvement (responders) and the other 7 patients did not (nonresponders). There were no significant differences between responders, nonresponders, and controls in the CSF levels of the metabolites before R-THBP administration. When lumbar puncture was repeated in 6 autistic children in the 24th week of R-THBP therapy, there was no significant change in the CSF levels of any metabolites.

Turner syndrome and occlusion of the internal carotid artery.

A 2-year-old girl with Turner syndrome was admitted with left hemiplegia and left facial palsy. Serial cranial computed tomographic scan demonstrated multiple cerebral infarctions in the right putamen and right medial cortical areas. Single photon emission computed tomographic scan revealed hypoperfusion from the right frontal to the right temporal area. Right carotid angiography showed narrowing and occlusion of the right internal carotid artery at the sphenoidal portion. Collateral circulation was not detected between the external and internal carotid arteries. Left carotid angiography revealed that the left anterior artery was narrow, and that the left internal carotid artery provided blood to the right internal carotid artery through the anterior communicating artery. These findings suggested that the cerebrovascular abnormality might be due to congenital hypoplasia of arteries in this patient. The unusual combination of cerebral infarction and Turner syndrome was reported.