Hideo Tamari

Longitudinal changes in selenium content of breast milk.

ABSTRACT. The selenium (Se) contents of human milk, serum and hair obtained from 22 lactating mothers were measured by fluorometric analysis. The Se contents in 41 milk samples from different stages of lactation were obtained longitudinally from 10 mothers. They showed a large variance of individual samples at any stage of studies. The highest Se level was found in colostrum (median 80 ng/ml); subsequently, Se content declined significantly during the first month of lactation and then came to a plateau level (median 17–18 ng/ml). No positive correlation of Se content was found between the serum and the milk samples at three months of lactation. No positive correlation of Se content was found between the hair and the milk samples obtained from lactating mothers.

Muscle fiber type transformation in nemaline myopathy and congenital fiber type disproportion

In a morphometric study on biopsied muscles from 5 patients with nemaline myopathy (NM) and 5 with congenital fiber type disproportion (CFTD), the common findings were relative type 1 fiber smallness, type 1 fiber predominance and occasional hypertrophic type 2 fibers. In NM, the relatively larger type 1 fibers increased in number with age in parallel with a decrease in the number of normal to hypertrophic type 2 fibers, reflecting active fiber type transformation from type 2 to type 1, which resulted in striking type 1 fiber predominance. The presence of scattered non-atrophic type 2C fibers also reflected active fiber type transformation because the fibers during the maturational or degenerating process are known to show the type 2C reaction on ATPase staining. On the other hand, the type 1 fibers in CFTD were small in caliber and showed minimal variation in size, suggesting practically no fiber type transformation from hypertrophic type 2 to type 1.

A fluorescent microscopy study of biopsied muscles from infantile neuromuscular disorders.

SummaryThe Acridine Orange (AO) stain for muscle biopsies is particularly useful to identify regenerating and ongoing hypertrophic muscle fibers under fluorescent microscopy. This method was applied to muscle biopsies from 65 patients who suffered from various childhood neuromuscular disorders. While normal fibers showed dull green cytoplasm with small green-yellow nuclei, striking fluorescent fibers were observed in eight cases of congenital muscular dystrophy (CMD) and 12 cases of Duchenne muscular dystrophy (DMD); these fibers were characterized as follows: (1) small fibers with big oval or spherical nucleic which fluoresced strongly with a bright orange color; (2) fibers of various sizes and different degrees of orange fluorescence; and (3) opaque fibers with bright yellow cytoplasm. The small diameter fibers in Werdnig-Hoffmann (WH) disease, nemaline myopathy, and congenital fiber type dysproportion failed to show apparent AO-RNA fluorescence. Although all the atrophic fibers in Kugelberg-Welander (KW) disease showed a vague orange fluorescent color, this was obviously different from that of regenerating fibers seen in CMD and DMD. In addition to these findings, the hypertrophic fibers in a case of unclassified myopathy also showed moderate organe fluorescence around the entire periphery of the cytoplasm.

Behavior of sarcotubular system formation in experimentally induced regeneration of muscle fibers

To examine the behavior of transverse (T)-tubule formation in experimentally-induced regenerating fibers, a local anesthetic, bupivacaine hydrochloride, was injected directly into the rat soleus muscle to cause myonecrosis. The regenerating fibers following necrosis were then examined by electron microscopy using lanthanum nitrate which clearly demonstrated the sarcotubular system. In the early stage of regeneration within 7 days after muscle necrosis, the T-tubules seemed to be composed of markedly proliferated subsarcolemmal caveolae with occasional honeycomb structure formation. Around 10 days, the T-tubules in regenerating fibers were tortuously and irregularly arranged with focal dilatation in diameter, and extended longitudinally along the axis of well organized myofibrils. As the regenerating fibers matured, the sarcotubular system, irregular in course and in shape, gradually became organized into a regular transverse position against the myofibrils, along with a marked decrease in longitudinally arranged tubular components. These morphological findings of the early T-tubule formation seen in the present study were similar to those found in early myogenesis, and in biopsied muscles from cases of polymyositis and progressive muscular dystrophy.

An acute variant of subacute sclerosing panencephalitis: an autopsy case report.

An acute variant of subacute sclerosing panencephalitis (SSPE) was described in a 5-year-old boy who showed rapid progression of coma within 14 days of right hemiplegia. He had measles at 3 years of age. The diagnosis of SSPE was based on the following findings: high anti-measles antibody titer in the serum and in the spinal fluid, periodic complex of EEG, and typical pathological changes of the brain. Treatment with transfer factor failed to improve the worsening clinical course. It is suggested that SSPE should be considered in the differential diagnosis of acute fulminating encephalitides or intracranial vascular lesions.

SUBACUTE SCLEROSING PANENCEPHALITIS

An autopsy case of subacute sclerosing panencephalitis (SSPE) in a 5‐year‐old boy, with rapid progression to a comatose state in 2 weeks after the onset of right hemiplegia, is described. The levels of antibody to measles virus in the serum and the cerebrospinal fluid were increased, and high levels of IgG in the latter were found. A characteristic pattern of electroencephalogram (EEG) showing periodic suppression of high voltage complexs was also found during the course of the disease. Microscopical examination revealed perivascular cuffing, numerous hypertrophied astrocytes with a diffuse gliosis and sporadic intranuclear inclusions in the brain. In addition to these typical findings of SSPE, impaired cellular immunity was recognized by delayed skin test in vivo, and pathologically severe atrophy of thymus, and follicular atrophy of spleen with amyloid deposition in the wall of the sheathed arteries were found.