Case of Heerfordt's syndrome with prolonged peripheral nerve involvement]

A 28-year-old man complaining of myiodesopsia was given a diagnosis of uveitis. Subsequently he complained facial nerve palsy and enlargement of parotid gland. Heerfordt's syndrome was diagnosed based on the results of several examinations. Facial nerve palsy, enlargement of the parotid gland and uveitis were improved by systemic corticosteroid therapy. At present he is receiving systemic corticosteroid therapy, but numbness in the mouth, thought to be the involvement of the trigeminal nerve, remains. Systemic corticosteroid therapy is usually effective for most cases with Heerford's syndrome. On the other hand, there are some cases with the prolonged peripheral nerve involvement despite systemic corticosteroid therapy, as seen in this case. If peripheral nerve involvement is prolonged, it is necessary to consider small-fibre neuropathy as one possible cause.     

Crossed cerebellar hypoperfusion in unilateral major cerebral artery occlusive disorders.

We evaluated regional blood flow and oxygen metabolism in the cerebral and cerebellar cortices of 15 patients with unilateral major cerebral artery occlusive disorders with PET. These patients showed a cortical blood flow asymmetry in middle cerebral artery distribution. Only subcortical abnormalities were detected on computed tomography. Nine patients showed crossed cerebellar hypoperfusion, a reduction in contralateral cerebellar blood flow, while six did not. No difference in the degree of cerebral blood flow asymmetry existed between the two patient groups. However, oxygen metabolism asymmetry was more pronounced and was more closely matched to blood flow asymmetry in patients with crossed cerebellar hypoperfusion. These findings suggest that a major cause of cerebral cortical blood flow reduction is reduced metabolic demand in patients with crossed cerebellar hypoperfusion. Crossed cerebellar hypoperfusion may have clinical significance as a reflection of the cerebral metabolic state on blood flow images.     

Operative results with total composite replacement of the aortic valve and ascending aorta]

From December 1980 to December 1990, ten patients, 9 male and 1 female, ranging in age from 21 to 68 years, were operated on for aortic valve insufficiency associated with an aneurysm of the ascending aorta. The surgical treatment in all cases consisted of total replacement of the ascending aorta with Bentall's procedure (n = 4), or Cabrol's procedure (n = 6). In 5 patients an uncomplicated annulo-aortic ectasia existed. Three of them had annulo-aortic ectasia with an aortic dissection. One had aortitis syndrome, and one had syphilitic aortitis. The operative mortality for the entire group was 0% (0 death). Hospital survivors revealed satisfactory clinical improvement in NYHA class (mean value: 3.2 to 1.0). Late complications developed in 2 of the 10 patients. They had a picture of pseudoaneurysm formation at the anastomoses between the graft and the right coronary 46 months and 15 months, respectively, after the initial operation. Despite the reoperation, one died of hepatic failure 30 days after the operation, and the other died of postoperative bleeding at the anastomosis sites. We, furthermore, considered the difference in aortic cross clamp time and cardiopulmonary bypass time between Bentall's procedure and Cabrol's procedure. Aortic cross clamp time and cardiopulmonary bypass time were significantly shorter in Cabrol's procedure than in Bentall's procedure, if a probability value less than 0.20 was considered to be of statistical significance. We were able to conclude that the treatment of aortic valve regurgitation associated with an aneurysm of the ascending aorta by insertion of a composite graft is a reliable method with low operative mortality and excellent long term results, especially in Cabrol's procedure.     

A suspicious case of central neurosarcoidosis, manifesting as a progressive gait disturbance with a lesion in the central gray matter of the midbrain, accompanying an elevated level of angiotensin converting enzyme in the cerebrospinal fluid]

We present a suspicious case of central neurosarcoidosis that presented with progressive gait disturbance probably caused by central vestibular dysfunction. And this case showed elevated level of angiotensin converting enzyme (ACE) in the cerebrospinal fluid, compared with the average level of two cases with acute inflammatory demyelinating neuropathy syndrome and four cases of multiple sclerosis. A 33-year-old man was admitted to our hospital with chief complaint of a gait disturbance that had appeared 3 years prior to the admission. And the symptom had exacerbated in these 3 months. Except for the gait disturbance and positive Romberg's sign, no neurological abnormality was detected. The findings of the cerebrospinal fluid test supported the diagnosis of meningitis. An increased level of angiotensine converting enzyme was detected when compared with our previous samplings from two cases of Guillain Barré syndrome and four cases of multiple sclerosis. With T1 weighted imaging of brain MRI, a high intensity lesion with gadolinium enhancement was identified in the central gray matter of the midbrain. Scan of the chest confirmed bilateral hilar lymphadenopathy. Based on these findings and the patient's clinical course, central neurosarcoidosis was suspected. The patient's symptoms improved dramatically after the administration of corticosteroid. The enhancement of the central gray matter ameliorated, and the ACE level of the CSF was decreased to the level of the demyelinating disorders.     

Combined aortic and mitral valve repair in active infective endocarditis

We describe a case of successful combined repair of the aortic and mitral valves for an indication of active infective endocarditis involving both valves. Mitral valve repair was achieved by vegetation debridement, fixation of the anterior mitral commissure, resection and suturing of the posterior mitral leaflet, and posterior annuloplasty with autologous pericardium. Aortic valve repair was achieved by vegetectomy and commissural plication. Postoperative clinical course was without signs of recurrent infection, and echocardiogram demonstrated mitral valve competence with trivial aortic regurgitation. We concluded that simultaneous valve repair is a viable option in the context of active endocarditis.     

Dental management of a patient with takotsubo cardiomyopathy: a case report.

Takotsubo cardiomyopathy (TCM) is a relatively new concept in cardiovascular disease. The clinical symptoms of TCM are similar to those of a myocardial infarction, but both the mechanism and the management of TCM are different from those of myocardial infarction. The cause of TCM is unclear, but it is suggested to occur in conjunction with excessive circulating catecholamines due to stress. Thus, control of the stress reaction and restriction of catecholamine levels are considered important for prevent of TCM onset. We report the dental management of a patient with intellectual disability who had anamnesis of TCM and cardiopulmonary arrest under restraint during a previous dental appointment in another dental clinic. We used intravenous sedation with both midazolam and propofol, by which the excessive hormonal reaction that caused TCM onset and cardiopulmonary arrest was controlled, for dental treatment in our facility. All planned dental treatment was then performed without any problems.     

Validity of a newly developed ultraminiature cordless EMG measurement system.

To verify validity of a newly developed ultraminiature EMG measurement system (BMS), the ability of BMS to record masseteric EMG was compared with that of a conventional polygraph system (PG) in the daytime. Effective distance between the transmitter unit and receiver unit of BMS was also examined. Subjects were 12 healthy volunteers. During tapping, maximum clenching, and gum chewing of all subjects, distinct bursts were observed in EMG recorded by BMS as well as PG. RMS values of maximum clenching measured by BMS and PG showed a linear and significant correlation, and there was no significant difference between the data of BMS and PG. When distance between the transmitter unit and receiver unit of BMS was 100 cm or less, no artifact signal was observed. Having obtained these findings suggesting ability for precise measurement in the daytime, we are planning to use BMS in home sleep studies in the next step.     

HTLV-I, HIV-I, and Hepatitis B and C Viruses in Western Province, Papua New Guinea: A Serological Survey

Seven hundred and twenty-three serum samples from individuals in 13 Gidra-speaking villages in Western Province, Papua New Guinea were tested for evidence of infection with human T- lymphotropic virus type I (HTLV-I), human immunodeficiency virus type I (HIV-I), hepatitis B virus (HBV) and hepatitis C virus (HCV). No samples were positive for antibodies to HIV-I. Antibodies to HTLV-I were found in 13 samples (1.8%), HBV surface antigens (HBsAg) were found in 86 samples (11.9%), and antibodies to HCV were found in 30 samples (4.1%). Six (46.2%) of 13 HTLV-I positive samples were positive for HCV or HBsAg. The seropositive rate varied in different villages and the incidence of HTLV-I and HCV was higher in coastal and riverine areas than inland.     

Expression of leukocyte adhesion molecules (ICAM-1/LFA-1) related to clinical behaviour in B cell lymphomas.

We investigated the expression of adhesion molecules of lymphocyte function-associated antigen-1 alpha (LFA-1 alpha) and its ligand intercellular adhesion molecule-1 (ICAM-1) on 74 well-characterized B cell lymphomas. The LFA-1 was expressed on B cell lymphomas (21/74; 28 per cent), but to a lesser degree than ICAM-1 which was highly expressed (48/74 cases; 64 per cent). From the results of bone marrow examination of 39 cases with B cell lymphomas, 13 of 16 cases with a lack of ICAM-1 molecule showed a higher incidence of marrow involvement, but nine of 23 cases with the expression of ICAM-1 molecule showed a lower incidence. These findings suggest that the lack of expression of the ICAM-1 molecule by B cell lymphomas correlates with bone marrow involvement by lymphoma cells (p < 0.05). Expression of the LFA-1 molecule appears not to correlate with marrow involvement (p < 0.05).