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Biomedical subjects

T Mannen

Publications and source records attributed to T Mannen.

At least 91 records · Page 5Linked to original sources
PubMed1988

Increased dermal collagen density in amyotrophic lateral sclerosis.

We examined specimens of skin overlying the sacral region, among the most common sites of bedsores, from patients with amyotrophic lateral sclerosis (ALS) and controls, and found that in ALS patients, collagen fibrils had a greater density and became more tightly packed with the duration of illness. Our results suggest that the increased density of collagen fibrils may protect the skin of ALS patients from pressure ischemia, a major cause of bedsore formation.

Amyotrophic Lateral Sclerosis↗
PubMed1988

Combined features of myasthenia gravis and Eaton-Lambert syndrome: anti-ganglioside antibodies in serum.

We studied 2 patients with Eaton-Lambert syndrome showing some features of myasthenia gravis. Among 9 different glycolipid antigens examined, serum titers of IgG antibodies to GT1b, GD1a, and sialylparagloboside were increased. Those antibodies may be involved in the pathogenesis of the presynaptic neuromuscular block that was presumed to be present in these patients.

Autoantibodies↗
PubMed1988

Familial bulbo-spinal muscular atrophy associated with testicular atrophy and sensory neuropathy (Kennedy-Alter-Sung syndrome). Autopsy case report of two brothers.

Autopsy cases of two brothers with bulbo-spinal muscular atrophy associated with gynecomastia, testicular atrophy and sensory neuropathy are reported. The disease started with finger tremor, proximal muscle weakness and facial muscle twitching at the second and fourth decades, accompanied by bulbar signs and glove-stocking type sensory disturbance. Systemic neurogenic patterns and diminished sensory nerve action potential amplitudes were recorded by electrophysiological studies. A marked loss of myelinated fibers was noticed upon sural nerve biopsy. Gonadal hormone values were normal, except for elevated urinary estrogen. Postmortem examinations revealed a remarkable degeneration of the facial and hypoglossal nuclei, and the spinal cord motoneurons. The skeletal muscles and the tongue showed neurogenic muscular atrophy with fatty replacement. Testicular atrophy was prominent showing hyalinized seminiferous tubuli with nodular and diffuse Leydig cell hyperplasia, containing estrogen immunoreactive substance. These clinical and histological features seemed to be highly compatible with those of Kennedy-Alter-Sung type bulbo-spinal muscular atrophy. The involvement of sensory peripheral nerves, however, was a distinct feature of this family.

Aged↗
PubMed1988

Central motor conduction in cerebrovascular disease and motor neuron disease.

Conduction in the central motor pathways was studied in 9 patients with cerebrovascular disease (CVD), 13 with amyotrophic lateral sclerosis (ALS) and 3 with spinal progressive muscular atrophy (SPMA). Motor responses evoked in the limb by cortical, cervical and lumbar stimulations were recorded. The central conduction time (CCT) was calculated for each muscle. In patients with CVD, responses to cortical stimulation were unobtainable or delayed in the paretic limb muscles. In patients with ALS the abnormality of central motor conduction had significant correlation with the extensor plantar response. The CCTs were normal in patients with SPMA. This technique demonstrated a subclinical lesion in some patients. We conclude that the new technique of examining motor conduction along the corticospinal tract may be useful to detect a subclinical lesion in the corticospinal tract.

Amyotrophic Lateral Sclerosis↗
PubMed1988

"Delayed return phenomenon" in amyotrophic lateral sclerosis.

When the skin of patients with amyotrophic lateral sclerosis (ALS) is stretched, it returns only sluggishly to its original position. We term this property of skin the "delayed return phenomenon" (DRP). The reticular dermis was examined in patients with ALS and controls with or without DRP, respectively. On light microscopy, ALS patients with DRP showed collagen bundles which were reduced in amount, more loosely woven, and separated by wide space, while controls with DRP revealed decrease of dermal thickness, without abnormalities of collagen bundles. Electron microscopy disclosed a markedly large amount of amorphous material positive for ruthenium red, separating collagen fibrils and bundles in ALS patients with DRP. These observations suggest that DRP in ALS is a specific feature not previously reported in cases of ALS.

Actin Cytoskeleton↗
PubMed1988

Central motor and sensory conduction in adrenoleukomyeloneuropathy, cerebrotendinous xanthomatosis, HTLV-1-associated myelopathy and tabes dorsalis.

Central motor and sensory conduction was studied by percutaneous electrical stimulation of brain and spinal cord and by somatosensory evoked potential techniques respectively, in patients with adrenoleukomyeloneuropathy, cerebrotendinous xanthomatosis, human T-cell lymphotropic virus-1-associated myelopathy and tabes dorsalis. The results were all consistent with clinical and neuropathological findings in these disorders. Conductions in the corticospinal tract and posterior column could be evaluated separately with these two techniques. Percutaneous electrical stimulation technique would be useful for investigating conduction in the corticospinal tract in patients with spinal cord disorders.

Adrenoleukodystrophy↗
PubMed1988

A nation-wide collaborative study on the long-term effects of bromocriptine in patients with Parkinson's disease. First interim report in Japan.

Two prospective projects were started in May 1985 to evaluate the long-term effects of bromocriptine in patients with Parkinson's disease. One of the projects is to see if combination therapy with levodopa and bromocriptine is superior to levodopa alone with regard to the prevention of late side effects of levodopa therapy. The other is to see the long-term effects of bromocriptine monotherapy. Patients with Parkinson's disease were allocated randomly to either combination or levodopa group in the first project. Parkinsonian symptoms, disabilities of daily life and severity of late side effects of long-term levodopa therapy were evaluated by a semiquantitative rating scale. This communication represents the first interim report. Methods of the studies and synopsis of the results at the end of the 12th month are described. A total of 702 patients was enrolled in the study (combination therapy, n = 216; levodopa therapy, n = 200, bromocriptine monotherapy, n = 286). At the end of the 12th month, the numbers of patients who dropped out from the study were 19, 16 and 55 in the three groups, respectively. It appears to be too early to make any definite conclusion with regard to which mode of treatment is superior, however, some evidence suggesting a superiority of the combination therapy over levodopa alone was noted in managing wearing-off phenomenon and dyskinesia. As many patients are sticking to the originally intended mode of treatment, it appears to be possible to obtain more meaningful data in several years.

Adult↗
PubMed1987

Rimmed vacuolar distal myopathy: a clinical, electrophysiological, histopathological and computed tomographic study of seven cases.

The following report describes the clinical, laboratory, electrophysiological, histopathological and computed tomographic studies of seven cases of distal myopathy with rimmed vacuoles in the muscle fibers. Each displayed several characteristic features. First, the onset was in early adulthood. Second, there was a unique distribution of muscle involvement: tibialis anterior and extensor digitorum and hallucis muscles were initially and most severely affected. The hamstrings and adductors of the thigh were also markedly involved. The gluteus medius and minimus muscles and the neck flexors were mildly affected in the relatively early stages. In contrast, the gastrocnemius, soleus, quadriceps femoris, and gluteus maximus muscles were well preserved until an advanced stage. Third, serum creatine kinase activity was normal or only mildly elevated; fourth, EMG were mainly myopathic, with certain neuropathic features; and fifth, histopathologically rimmed vacuoles in muscle fibers were found associated with certain "neuropathic" features, such as angular fibers, clustering of atrophic fibers, pyknotic nuclear clumps, and fiber-type predominance. The characteristic distribution of skeletal muscle involvement was particularly noticeable, together with certain "neuropathic" features of the EMG and muscle biopsy in rimmed vacuolar distal myopathy.

Adult↗
PubMed1987

Rimmed vacuolar distal myopathy. An ultrastructural study.

An ultrastructural study of biopsied muscles was performed in seven patients with rimmed vacuolar distal myopathy, which was characterized by prominent rimmed vacuoles in the muscle fibers. The earliest changes noted were focal proliferation of the Golgi's apparatus and mitochondrial degeneration with myofibrillar loss. A proliferation of the T-system appeared later. Secondary lysosomes (autophagosomes) could be noted much later and gradually increased in number. Autophagosomes tended to coalesce and became larger autophagic vacuoles, which were surrounded in part by relatively preserved myofibrils and partly by a single membrane. Gently curved laminated structures (tubulomembranous structures) were seen in the degenerating muscle fibers and also in relatively intact fibers, satellite cells, and interstitial cells in all cases. They were closely associated with lipofuscin-like material. These findings suggest that an abnormality of the lysosomal system might be essential in the pathogenesis of rimmed vacuolar distal myopathy.

Adult↗
PubMed1987

Myoclonus in Alzheimer's disease.

Myoclonus was studied electrophysiologically in seven patients with clinically diagnosed Alzheimer's disease. There seem to be at least two physiological types of myoclonus in Alzheimer's disease. Cerebral cortical structures might participate in the generation of myoclonus in one type, while the other type is probably generated by subcortical structures.

Aged↗