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Biomedical subjects

R Minami

Publications and source records attributed to R Minami.

171 records · Page 10Linked to original sources

Attenuated SEPs with no latency shifts in a family with hereditary spastic paraplegia.

Five siblings with hereditary spastic paraplegia of autosomal-dominant inheritance were studied with somatosensory evoked potentials. Somatosensory evoked potentials were recorded from Cz', T12, and the left popliteal fossa following left posterior tibial nerve stimulation. The latency and amplitude of the corresponding potentials (i.e., P37, N20, and N7) were compared with normal values obtained from age- and height-matched controls. There was no significant difference in the values of N7, suggesting an intact afferent peripheral pathway; in contrast, the amplitudes of P37 and N20 were decreased with normal latencies. The degree of amplitude decrease correlated with the severity of vibration sense impairment in the lower limbs. These results appear to support selective axonopathy of the centrally directed axons of the dorsal root ganglion cells. Furthermore, our results suggest that different degrees of dorsal column involvement in hereditary spastic paraplegia can cause different types of somatosensory evoked potential abnormalities, namely, attenuated amplitudes with no latency shifts, as recorded in this family, and the prolonged latencies, as reported previously.

Adolescent↗

Infantile neuroaxonal dystrophy: axonal changes in biopsied muscle tissue.

Dystrophic axons were found in the biopsied muscle tissue of a 17-month-old hypotonic infant after confirmation of a diagnosis of infantile neuroaxonal dystrophy (INAD) by subsequent peripheral nerve biopsy. He manifested gradual deterioration and had brief tonic seizures. The sural nerve biopsy, performed at age 42 months, confirmed INAD. Intensive ultrastructural investigation of the preceding muscle biopsy specimens demonstrated infrequent but definite findings of axonal degeneration in the musculature. Ultrastructural investigation of muscle biopsy should include a careful search for neural tissue because in INAD axonal changes occur primarily in the presynaptic region.

Axons↗

Correlation between progression of spinal deformity and pulmonary function in Duchenne muscular dystrophy.

The purpose of this study was to investigate how age at and value of the plateau of vital capacity (VC plateau) correlate with the severity of the progression of spinal deformity in patients with Duchenne muscular dystrophy (DMD). Changes in spinal deformity and pulmonary function in 36 DMD patients were examined in a retrospective longitudinal study. Patterns of progression of spinal deformity were classified into three types according to Oda's classification. Of the 32 patients who showed spinal deformity, six were classified as type 1, 19 as type 2, and seven as type 3. The correlation between the patterns of progression of spinal deformity and the VC plateau was examined using a discriminant analysis. Rapid and severe progression of spinal deformity could be expected in patients whose VC plateau was less than 1,900 mL and in those in which it occurred before age 14 years. Thus VC plateau may be an indicator of the severity of the progression of spinal deformity in DMD patients.

Adolescent↗

Ictal video-EEG analysis of infantile neuroaxonal dystrophy.

A 4-year-old boy with infantile neuroaxonal dystrophy (INAD) showed gradual deterioration from age 9 months with seizure development at age approximately 36 months. Sural nerve biopsy performed at age 42 months confirmed INAD. The seizure, recorded by video-EEG, consisted of a series of symmetrical tonic spasms of both upper extremities after a prodrome period of staring and akinesis. Each spasm had phonation, and episodic autonomic symptoms such as hypertension and flushing of the face occurred throughout the seizure. Ictal EEG with each tonic spasm, showed diffuse 1-s, irregular sharp and high-voltage slow wave complexes followed by desynchronization.

Axons↗

Fine needle aspiration cytology of the malignant variant of ossifying fibromyxoid tumor of soft parts: a case report.

BACKGROUND: Ossifying fibromyxoid tumor (OFMT) of soft parts is a rare, recently defined, fibroosseous neoplasm, generally regarded as clinically benign; however, one-third of cases recur locally, and several malignant examples have been reported. Fine needle aspiration (FNA) cytology of the tumor is rarely described in the literature. We provide the first cytomorphologic study of the malignant variant. CASE: A 70-year-old man presented with an intramuscular mass in the right buttock. Computed tomography revealed ossification within the mass and multiple pulmonary nodules. FNA biopsy showed round and polygonal to spindled tumor cells, arrayed singly, cordlike or in small aggregates, with scattered dense stromal fragments and a slightly myxoid background. The nuclei showed significant pleomorphism accompanied by coarse chromatin with clumping, irregular contours, and one to two distinct nucleoli. The tumor cells were recognizable as sarcoma, with no evidence of high grade malignancy. The tumor was totally excised, histopathologically confirmed as the malignant variant of OFMT of soft parts, and immunohistochemically and ultrastracturally analyzed as of neural origin. CONCLUSION: The FNA specimen revealed that the cytomorphology was consistent with the histologic features of the malignant variant of OFMT, but several characteristic histologic parameters, such as multilobular proliferation and peripherally placed mature, bony trabeculae, were not reflected in the aspirates. Although FNA cytologic findings may be of limited diagnostic utility in OFMT, radiographic evidence of calcification/ossification suggests that OFMT should be subjected to differential diagnosis with fine needle aspiration biopsy of soft tissue tumors. Additional studies will be required for further clarification.

Aged↗

Outbreaks of paralytic poliomyelitis and polio surveillance in Shandong province of China.

Widespread outbreaks of paralytic poliomyelitis occurred in Shandong province, China, starting from 1988. In 1989, 484 cases were recorded, which was the peak during the past 4 years. Although emergency immunization with trivalent oral poliovirus vaccine (OPV) was carried out in selected counties in 1989 and 1990, control of the outbreak was not satisfactory. OPV mass immunization campaigns were introduced to cover the whole province in early 1991, and the number of patients with paralytic poliomyelitis decreased to 95. In addition to this new immunization strategy, we began to construct new polio surveillance systems. These were a network for case-negative reporting and an immediate reporting system of acute flaccid paralysis (AFP). As for the case-negative reporting, presently more than 90% of counties have been reporting presence or absence of new AFP cases. Monitoring of AFP immediate reporting has also shown a gradual improvement in several aspects. These polio surveillance activities are crucial to polio eradication programme management.

Child↗

Gradient separation of normal and malignant cells. II. Application to in vivo tumor diagnosis.

Data were obtained concerning the usefulness of discontinuous Ficoll and Isopaque-Ficoll gradients in separating malignant from non-malignant cells from a variety of sources. Most of the material was from effusions, but there is no theoretical restriction of the methods to effusions. Animal and human material were studied, utilizing non-malignant as well as malignant material, the former acting as controls. In the discontinuous Ficoll gradient, concentration of malignant cells at a specific interface was mainly a function of cell density. Thus, while most malignant cells banded in the last two fractions, adenocarcinomas, especially those high in mucus (which decreases total cell density) banded higher in the gradient. The Isopaque-Ficoll technique also proved useful in concentrating malignant cells. The advantage of the discontinuous Ficoll gradient was its ability to separate cells from small specimens (1-10 X 10(6) cells). Contrarily, the Isopaque-Ficoll gradient was preferable for processing specimens of larger volumes.

Adenocarcinoma↗

A management trial for Duchenne cardiomyopathy.

The physiatrist can now be instrumental in prolonging the survival of individuals with neuromuscular disease by using respiratory muscle aids. As a result, morbidity and mortality from cardiomyopathy are likely to increase for patients with generalized myopathies. One hundred consecutive patients with dystrophin-deficient muscular dystrophy and a mean age of 17.2 yr (range, 5-41) satisfied criteria for having dilated cardiomyopathy (DCM) and received digitalis and diuretics. Nine of the 14 patients were symptom-free, despite left ventricular ejection fractions (LVEFs) of 25-40%. The five patients with symptomatic heart failure had severe ventricular dilatation, with LVEFs < 25%. Two of the five patients died of heart failure within 1 yr. For the remaining three patients, we evaluated the addition of the angiotensin-converting enzyme (ACE) inhibitor enalapril and, subsequently, the use of beta-blockers to the therapeutic regimen. Addition of these medications, never before attempted in the management of cardiomyopathy associated with generalized myopathic disease, complemented each other in relieving symptoms and reversing signs of congestive heart failure and DCM. We conclude that the combination of ACE inhibitor and beta-blocker deserves further exploration for inclusion in any management regimen for the treatment of muscular dystrophy-associated cardiomyopathy.

Adolescent↗