Muscle CT scan findings in McLeod syndrome and chorea-acanthocytosis.

Computed tomography (CT) scans of lower leg muscles reveal a selective pattern of fat infiltration in the posterior compartment with spared gracilis, semitendinosus, and the lateral head of the gastrocnemius in both McLeod syndrome and chorea-acanthocytosis, which are disorders characterized by the presence of circulating acanthocytes. The selectivity of affected muscles indicates that late onset and slowly progressive muscular atrophy in both diseases could be a consequence of primary myopathy. Asymmetrical muscle involvement may be seen during the process of degeneration only in McLeod syndrome, however, and may be helpful in distinguishing this disease from chorea-acanthocytosis.

[Effects of L-threo-Dops on orthostatic hypotension in Parkinson's disease].

Effects of L-threo-Dops (Dops) administration on orthostatic hypotention were evaluated with changes in blood pressure by postural change (lying to standing position) and subjective symptoms in 15 patients of Parkinson's disease having symptoms of orthostatic hypotention. Orthostatic syncope had improved significantly (p < 0.01) after 2 and 4 weeks of administration with maintenance dose of 460 mg/day of Dops in average. In the standing-up (Schellong) test, decrease in blood pressure levels by a postural change, both with systolic and diastolic blood pressure, was significantly smaller at 3, 5 and 10 minutes after standing after 4 weeks of drug administration. Decrease in the blood pressure level immediately after standing-up improved by 10.2 +/- 4.0 for systolic and 6.5 +/- 1.8 for diastolic (mmHg, mean +/- SE) (p < 0.01). The group that showed improvement in orthostatic syncope had a significant improvement in decline in blood pressure by standing after administration of Dops, while the group without any change in severity of syncope did not show significant improvement in orthostatic hypotention.

[Prospective study of the etiology of community-acquired pneumonia among patients in a general hospital].

We prospectively studied the etiology of community-acquired pneumonia among all patients who were admitted to our hospital from July 1994 to June 1995. Tests for microbial pathogens including Chlamydia spp. and Legionella spp. were performed and diagnoses were made with strict criteria. A total 110 patients with 111 episodes of pneumonia were evaluated, and a pathogen was identified in 61 episodes (55%). The most common pathogen was Streptococcus pneumoniae (18%), followed by Haemophilus influenzae, Klebsiella pneumoniae, Pseudomonas aeruginosa, Mycoplasma pneumoniae, and Chlamydia spp. Infection with Legionella pneumophila was not found. Dual pathogens were identified in five episodes. Few prospective studies of the etiology of community-acquired pneumonia have been done in Japan. To prepare guidelines for the management of community-acquired pneumonia in Japan, a national study of the etiology of pneumonia is necessary.

[Diffuse alveolar hemorrhage after allogeneic bone marrow transplantation].

A 44-year-old woman with malignant T-cell lymphoma and secondary leukemia received an allogeneic bone marrow transplant (BMT). She had received BMT conditioning treatment with total body irradiation and chemotherapy. Hemoptysis and progressive dyspnea developed 11 days after the transplant. A chest roentgenogram showed bilateral diffuse infiltrates. Bronchoalveolar lavage fluid was bloody, and diffuse alveolar hemorrhage (DAH) was diagnosed. Respiratory failure progressed despite mechanical ventilation and administration of corticosteroids. The patient died 58 days after the transplant DAH after BMT has been recognized in western countries as a syndrome with high mortality. We draw attention to the fact that DAH is a serious early pulmonary complication of BMT also in Japan.

Bronchoalveolar mast cells in summer-type hypersensitivity pneumonitis: increase in numbers and ultrastructural evidence of degranulation.

To investigate the role of mast cells in the development of summer-type hypersensitivity pneumonitis (SHP), which is the most common type of hypersensitivity pneumonitis in Japan, bronchoalveolar lavage fluid (BALF) cells from 13 patients with this disease was studied by light and electron microscopy. The proportion and number of BALF mast cells recovered from SHP patients were significantly greater than those from patients with sarcoidosis and from control subjects. The BALF mast cells from the SHP patients showed various ultrastructural changes such as the transformation of granules, cytoplasmic vacuolation, and an increase of lipid droplets. These findings appeared to reflect activation and degranulation of bronchoalveolar mast cells, suggesting that mast cells play a role as inflammatory cells in the development of SHP.

[Familial idiopathic vitamin E deficiency associated with cerebellar atrophy].

Sibling cases of familial vitamin E deficiency accompanied by ataxia, polyneuropathy and mental retardation were reported. Case 1 was a 37-year-old male who developed progressive gait disturbance, deformity of the feet and head tremor from childhood, after normal delivery and development of early childhood. On physical examination, he had cataract, high arched palate and pes cavus. Neurological examination revealed mental retardation (WAIS 68), scanning speech, muscular atrophy of the face and extremities with predominance in the lower limbs, absent Achilles tendon reflex, disturbance of superficial and deep sensation predominant in distal limbs, and marked gait ataxia. Ataxia was both cerebellar and sensory in nature. Laboratory data of the blood showed no significant abnormalities including blood glucose and vitamin B12 except a markedly low level of serum vitamin E. The brain CT scan revealed severe cerebellar atrophy and marked dilatation of the cisterna magna and the subarachnoid space around the cerebellum. Motor nerve conduction velocity in the leg was decreased. Biopsy specimen from the quadriceps muscle showed neurogenic atrophy. Sural nerve biopsy revealed decrease in large myelinated fibers with axonal degeneration and regeneration. Oral administration of alpha-tocopherol acetate, 600 mg per day, diminished ataxia significantly. Based on lysosomal enzyme activity in leukocytes, clinical and laboratory examination, lipidosis or spinocerebellar degeneration was excluded. Chronic lipid malabsorption or beta lipoprotein deficiency which can cause decrease in vitamin E absorption, was not recognized. On oral loading with 2 g of alpha-tocopherol acetate, the decrease rate of serum vitamin E was normal. Consequently the low vitamin E was considered to have resulted from selective impairment of vitamin E absorption.(ABSTRACT TRUNCATED AT 250 WORDS)

Neoplastic angioendotheliomatosis. Report of two autopsy cases with special reference to the origin of atypical cells.

Two autopsy cases of neoplastic angioendotheliomatosis (NAE) were presented. Both patients were elderly woman, characterized by an ascending progression of transverse myelopathy and abnormal brain shadows similar to metastatic tumor in computer tomographic examination in Case 1, and by typical clinical features of cerebral infarction in Case 2. Postmortem examinations of both cases revealed an exclusive intravascular distribution of large atypical cells in generalized organs, especially in the central nervous system, and associated multiple cerebral infarcts and, in Case 1, widespread demyelination of the spinal cord. There were no distinct lesions suggestive of a primary focus. In both cases only a few atypical cells were immunohistochemically positive for factor VIII-related antigen, likely due to non-specific absorption of serum factor VIII into the cells. On the other hand, almost all of the atypical cells were immunoreactive for LN-1, LN-2, and leukocyte common antigen, suggestive of lymphocytic (B cell) origin.