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At least 109 records · Page 6Linked to original sources
PubMed2004

Castration restores function and neurofilament alterations of aged symptomatic males in a transgenic mouse model of spinal and bulbar muscular atrophy.

Transgenic models of neurodegenerative disease have proved uniquely powerful for delineating pathways of neuronal dysfunction and cell death. We have developed a transgenic model of the polyglutamine disease spinal and bulbar muscular atrophy (SBMA), an adult-onset, slowly progressive motor neuron disease caused by polyglutamine expansion in the androgen receptor (AR). Mice bearing a human AR with 112 glutamines reproduce many aspects of SBMA, including slowly progressive, gender-specific motor deficits, and neuronal intranuclear inclusions. Despite substantial motor deficits in male AR112Q mice, no motor neuron loss was observed, indicating that neuronal dysfunction, rather than neuronal death, is central to disease. Moreover, reduced levels of unphosphorylated neurofilament heavy chain (NF-H) were observed in motor neurons, suggesting a role for NF-H in SBMA neuronal dysfunction. The elimination of androgens by surgical castration of severely affected, aged 112Q male mice partially restored motor function as well as NF-H levels. These data suggest that hormone-based therapies designed to treat SBMA patients, even with advanced disease, are likely to be effective.

Animals↗
PubMed2002

A new model for the immobilization of the rat hind limb.

An alternative device for the immobilization of the hind limb of the rat was developed to study the effects of chronic disuse on the soleus and tibialis anterior muscles, maintained for 3 weeks in the shortening and the stretching positions, respectively. The proposed device is made of steel mesh and cotton materials, and has some advantages when compared to cast or plaster cast: it is cheaper, lighter (12 g or 4% of the body weight of the rat) and the same unit can be easily adjusted and used several times in the same animal or in animals of similar size. Immobilization is also useful to restrain the movements of the hip, knee, and ankle joints. Male rats (291 +/- 35 g and aged 14 +/- 2 weeks) were used to develop and test the model. The soleus muscle of 18 rats was maintained in a shortened position for 21 consecutive days and lost 19 +/- 7% of its length (P = 0.008) and 44 +/- 6% of its weight (P = 0.002) compared to the contralateral intact muscle. No difference (P = 0.67) was found in the stretched tibialis anterior of the same hind limb when compared to the contralateral muscle. No ulcer, sore or foot swelling was observed in the animals. Immobilization was effective in producing chronic muscle disuse in the hind limbs of rats and is an acceptable alternative to the traditional methods of immobilization such as cast or plaster cast.

Adaptation, Physiological↗
PubMed1999

[A family with probable autosomal dominant bulbospinal muscular atrophy with gynecomastia].

We reported a 52-year-old man and his family with bulbospinal muscle atrophy (BSMA) and gynecomastia. The propositus presented with the clinical picture of late onset progressive bulbospinal muscular atrophy including postural tremor, general hyporeflexia, mild maturity onset diabetes, gynecomastia and sexual impotence. One of his brother and his two sons had gynecomastia. His elder son had ocular movement abnormality, associated movement of facial muscle and finger tremor. One of his brothers showed tongue fasciculation without gynecomastia. None of members examined had abnormal expansion of CAG repeats in the androgen receptor gene. We speculate that this family has a new clinical entity characterized by bulbospinal muscular atrophy with an autosomal dominant inheritance.

Aged↗
PubMed2000

[Lewis-Sumner syndrome presenting unilateral quadriceps amyotrophy as an initial symptom].

We report a 55-year-old man with a chief complaint of wasting and weakness of the left quadriceps muscle. At age 54, he noticed difficulty in running and weakness in the left thigh, which gradually progressed. On the first admission to our hospital, based on the nerve conduction studies (NCS), the muscle biopsy findings showing neurologenic changes, and no abnormality of spinal MRI, we diagnosed as unilateral quadriceps amyotrophy, which resulted from an atypical form of spinal progressive muscular atrophy. One year later, he showed the bilateral hand weakness, conduction blocks on the right median and ulnar nerves by NCS, and the presence of serum anti-GM 1 antibody. From these findings, Lewis-Sumner syndrome was diagnosed. The therapy of high-dose intravenous immunoglobulin moderately improved his symptoms. The clinical symptoms of quadriceps amyotrophy is produced by various disorders including spinal progressive muscular atrophy, spinal extradural arachnoid cyst, rimmed vacuole myopathy, Becker dystrophy, limb-girdle dystrophy, and focal myositis. However, there have been no reports of a case of Lewis-Sumner syndrome. It is important to consider Lewis-Sumner syndrome in the differential diagnosis of quadriceps amyotrophy.

Demyelinating Diseases↗
PubMed2000

[Triplet repeat disease, with particular emphasis of spinal and bulbar muscular atrophy (SBMA)].

Spinal and bulbar muscular atrophy (SBMA) is an X-linked neurodegenerative disease caused by the expansion of a CAG repeat in the first exon of the androgen receptor (AR) gene. To date, eight CAG-repeat diseases have been identified, including spinal and bulbar muscular atrophy (SBMA). Huntington's disease (HD), dentatorubralpallidoluysian atrophy (DRPLA) and five spinocerebellar ataxias (SCAs 1, 2, 3, 6, 7). These disorders likely share a common pathogenesis caused by the gain of a toxic function associated with the expanded polyglutamine tract. Several mechanisms have been postulated as a pathogenic process for neurodegeneration caused by the expanded polyglutamine tract. Processing of the polyglutamine containing proteins by proteases liberate truncated polyglutamine tract, which may cause neurodegeneration as demonstrated in transgenic mice and transfected cells. In addition to cellular toxicity, truncated and expanded polyglutamine tracts have been shown to form intranuclear inclusions (NI). The NIs formed by the disease protein are a common pathological feature of these diseases. In SBMA, NIs containing AR protein have been observed in regions of SBMA central nervous system susceptible to degenerations. Transcriptional factors or their cofactors, such as cerb or creb-binding protein (CBP) sequestrated in the NI may alter the major intracellular transcriptional signal transduction, and ultimately may result in neuronal degeneration. The ubiquitin-proteasome pathway may also contribute to the pathogenesis of CAG-repeat diseases. As for the therapeutic strategies, many possibilities have been demonstrated. Overexpression of Hsp70 and Hsp40 chaperones act together to protect a cultured neuronal cell model of SBMA from a cellular toxicity of expanded polyglutamine tract.

Animals↗
PubMed2002

[Pathogenesis of "Leningrad" (blockade) hypertension (60-th anniversary of the Leningrad blockade)].

Leningrad (blockade) hypertension observed in survivors of the Leningrad blockade during the World War II is a unique form of hypertension initiated and maintained pathogenetically by disturbed neuroregulation resultant from a severe psychoemotional stress. Pathogenesis of Leningrad hypertension involves mechanisms playing a key role in pathogenesis of other forms of essential hypertension. A characteristic feature of this pathogenesis is interaction of the initial and key neurogenic factor with such hypertensive factors of alimentary-dystrophic genesis as lesions of the vascular wall secondary to marked hypoproteinemia and hypovolemia. Later, in addition to hypertension the patients developed atherosclerosis, nephrosclerosis and other diseases.

Adult↗
PubMed2003

Electrical stimulation prevents deterioration of the oxidative capacity of disuse-atrophied muscles in rats.

INTRODUCTION: The purpose of this study was to evaluate the effects of electrical stimulation on disuse-atrophied muscles. METHODS: Sprague-Dawley rats were used and divided into three groups: control (C), hind-limb suspended for 7 d (HS), and HS plus transcutaneous electrical stimulation for 7 d (ES). In the ES group, transcutaneous electrical stimulation was induced at 1 Hz for 1 h every day to condition the gastrocnemius-plantaris-soleus muscles. Muscle oxidative capacity was evaluated by 31P-MRS in vivo. Maximum tension and muscle wet mass were also measured. RESULTS: Muscle oxidative capacity decreased within 1 wk in HS; however, it was maintained when electric stimulation was applied to the suspended limb. The maximum twitch tension in HS was significantly smaller than that in C (p < 0.05), while in ES it did not differ from that in C. The muscle mass was significantly smaller in the HS and ES groups compared to C (p < 0.05). CONCLUSION: These data indicated that twitch electrical stimulation was effective in preventing deterioration of muscle functions, such as maximum tension and oxidative capacity, induced by 1 wk of disuse.

Animals↗
PubMedDate not supplied

Disuse atrophy alterations in normal and low temperature environments during hindlimb unloading in Syrian hamsters.

This study examined whether a hypothermic environment reduces experimentally-induced atrophy of skeletal muscle, as judged by histochemical findings. The hind limbs of hamsters in a hypothermic group were suspended and flexed into plantar positions at the ankle joint, and housed for one week at 8 to 12 degree celsius in a temperature-controlled room, while the normothermic group was housed at 23 to 25 degree celsius. Hypothermia did not significantly alter the average caloric intake, and the animals from the hypothermic group lost a significant amount of body weight when compared with the normothermic group. The hypothermic group retained more muscle wet-weight and myofibers cross-sectional area in the soleus and gastrocnemius muscles compared with the normothermic group. Our results indicate that a hypothermic environment inhibits short-term muscle atrophy. This inhibition may be caused by the increased caloric intake combined with a state similar to hibernation in low-temperature environments.

Animals↗
PubMed1958

Nerve lesions in industry; atrophy of disuse as a confusing element in diagnosis; the value of electromyogrphy.

Traumatic peripheral nerve lesions characteristically result in denervation muscular atrophy. Atrophy of disuse may take place concomitantly, either proximal, adjacent to or distal to the denervation muscular atrophy. The degree of atrophy of disuse depends upon the severity of the nerve lesion. Clinically, it is difficult to determine where true denervation muscular atrophy ends and accompanying atrophy of disuse begins. In such circumstances a clinician may be misled into belief that the cause of so apparently extensive a lesion is elsewhere. The patient then is often submitted to other complex diagnostic procedures and treatments. This difficulty can usually be dissipated by the use of electromyography, for each specific type of muscular atrophy produces its own characteristic electromyographic changes. Disuse atrophy produces no changes in electrical activity, whereas denervation atrophy manifests itself by typical denervation activity. Moreover it is possible to determine what part of muscular atrophy in a given area is owing to damage to a nerve and what part is owing only to disuse without denervation.

Accidents↗