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PubMed1989

Focal spinal muscular atrophy in two German shepherd pups.

Focal spinal muscular atrophy evolved rapidly in two German Shepherd pups 2 weeks after birth. By 1 month, the male and female littermates had developed a valgus deformity of the right forelimb, a flexed carpus, and arm and forearm muscle weakness and atrophy. In the male, less severe left forelimb weakness and wasting also occurred, and bilateral forelimb denervation potentials were recorded. Postmortem study of the male revealed asymmetric loss and degeneration of motoneurons in the cervical spinal cord intumescence. Degenerating neurons appeared vacuolated or chromatolytic. Chromatolysis was often peripheral and resulted from dispersion and loss of the free and attached ribosomes which normally from Nissl bodies. Although this focal neuronopathy was unlike motoneuron diseases described previously in animals, it resembled the asymmetric and unilateral, benign spinal muscular atrophies found in humans.

Animals↗

PubMed1979

Cerebral atrophy and long-term response to levodopa in Parkinson's disease.

In 92 parkinsonian patients (42 men, 50 women) aged from 37-79 years (mean 62.8) the impact of cerebral atrophy as assessed by computed tomography on the course of the clinical symptomatology under levodopa during a period of 1 to 5 years was investigated. It could be shown that patients suffering from medium to severe degrees of atrophy-independent of its special location--have a less favorable response than those with normal CT findings. The data do not allow a definite decision whether cortical artrophy or ventricular enlargement are of major importance. At any rate, the width of the third ventricle seems to have no influence on the course of the clinical symptoms. After 3 years of levodopa treatment a dissociation between cerebral atrophy and therapeutic effectiveness can be observed.

Adult↗

PubMed1976

Clinical observations of juvenile nonprogressive muscular atrophy localized in hand and forearm.

Twenty-seven patients with juvenile nonprogressive muscular atrophy localized in the hand and forearm were analyzed. The clinical characteristics were juvenile male occurrence, insidious onset, specific distribution of localized muscular atrophy and a stationary course. On electromyography, denervation voltage (or giant NMU) is found in the atrophied muscles and sometimes in contralateral nonatrophied ones. Sensory disturbance was not remarkable. Although the etiological factor was not known, strenuous exercise of arms in sports was noted frequently in the history.

Adolescent↗

PubMed1976

Dynamic aspects of peripheral nerve changes in progressive neural muscular atrophy: light- and electronmicroscopic studies of serial nerve biopsies.

Serial nerve biopsies were performed at an early, and at an advanced stage of the disease in 2 patients with progressive neural muscular atrophy. The early biopsy showed a complete loss of the large diameter and thickly myelinated fibres, as well as an expansion of the endoneurial interstitium in both cases. Myelinated and unmyelinated fibres exhibited axonal degeneration in all biopsies occasionally. "Onion bulb" formation, a typical feature of peripheral neuropathy in neural muscular atrophy, was found to be prominent only in the latter biopsies. As regards the formal pathogenesis of hypertrophic neuropathy in neural muscular atrophy, axonal dystrophy and interstitial changes of the endoneurium were regarded as primary phenomena, demyelination and "onion bulb" formation as secondary. A possible causal relation between axonal dystrophy and interstitial changes, observed in these cases, is discussed in the light of the present literature.

Adolescent↗

PubMed1984

Carnitine and acyltransferase in experimental neurogenic atrophies: changes with treatment.

Carnitine level and carnitine palmityl transferase (CPT) activity were investigated in muscles of patients with infantile and juvenile spinal muscular atrophy and polyneuropathies. A significant decrease of both carnitine and CPT was found in the infantile spinal muscular atrophy, but not in the other neurogenic muscle atrophies. These findings were compared with the experimental effect of denervation and reinnervation upon the lipid metabolism in soleus and extensor digitorum longus (EDL) of adult and newborn rats. Twenty-one days after denervation free and total carnitine decreased significantly in both EDL (P less than 0.001) and soleus (P less than 0.05) of adult animals. CPT activity was significantly decreased in the soleus 50 days after denervation (P less than 0.005). Long-term reinnervation restored the level of carnitine fraction and CPT activity. L-carnitine treatment for 21 days restored the level of free carnitine to normal in the soleus of denervated adult animals. Denervation in newborn rats influenced carnitine concentration in soleus and EDL to a lesser extent; the treatment with L-carnitine raised short-chain acylcarnitines in denervated muscles, while reinnervation restored carnitine level within 50 days.

Acyltransferases↗

PubMed1990

Axonal atrophy in the painful peripheral neuropathy in AIDS.

We compared the sural nerves from patients with AIDS; four with the painful peripheral neuropathy (PPN), three patients with non-painful distal symmetrical peripheral neuropathy (DSPN), one without clinical peripheral nerve involvement and two human immunodeficiency virus negative controls. Fibre diameter distributions showed a slight reduction in larger fibres in all patients with AIDS compared with controls. No significant difference was found in the relative frequency distribution of the lamellae counts between any of the groups. The relative frequency distribution of axonal area of myelinated fibres did not differ between controls, non-painful DSPN or the patient without peripheral nerve involvement; however, all patients with PPN showed marked reduction in the number of axons of myelinated fibres of larger area which was significant when compared to each patient from the other groups (P less than 0.0001). This indicates that the reduction in larger fibres in PPN is mostly due to axonal atrophy rather than selective fibre loss. Axonal atrophy is associated with painful peripheral neuropathy in AIDS but not with those without pain. The possible role of axonal atrophy as a pathological substrate for pain is discussed.

Acquired Immunodeficiency Syndrome↗

PubMed1989

Significance of MRI-confirmed atrophy of the cranial spinal cord in Friedreich's ataxia.

The severity of Friedreich's ataxia was graded in ten patients by clinical examination and in five by use of posturography. These data were compared with neuroradiology findings. CT-confirmed infratentorial atrophy occured only in advanced cases of Friedreich's ataxia; the correlation with the clinical score was poor. On mid-sagittal MRI planes the diameters of fourth ventricle, brain stem at the level of the inferior olive and spinal cord at the levels of the foramen magnum and C3 were measured. Patients with Friedreich's ataxia had significant MRI-confirmed atrophy of the cranial spinal cord as compared with a normal, age-matched control group. This was also observed in patients with Friedreich's ataxia in the early stages. A reliable correlation between atrophy of the cranial spinal cord and the clinical score, however, could again not be found. MRI exploration of the cranial spinal cord may be recommended as an additional diagnostic marker in Friedreich's ataxia.

Adult↗

PubMed1978

Basiparallel cut by pneumoencephalotomography and cerebellar atrophy.

The basiparallel cut is a nearly horizontal slice of the posterior fossa used in air study and the structures are observed on a plane parallel to the clivus. Total shrinkage of the cerebellum, with or without fourth ventricle dilatation, was found in cases of olivopontocerebellar atrophy, some intoxications, and a few senile subjects. Some atrophy of paleocerebellar portions was seen in Holmes-type degeneration. Another pattern was symmetric shrinkage of neocerebellar lobules and relatively specific for the cases of 'multiple system atrophy' such as progressive supranuclear palsy, striatonigral degeneration, Marie's ataxia, etc. Loss of the superior cerebellar peduncle was clearly demonstrated in this view.

Adult↗

PubMed1987

Atrophy of the thumb web space in rheumatoid arthritis: clinical and electrodiagnostic studies.

One hundred and twenty definite or classical rheumatoid arthritis (RA) patients with an average duration of the disease of 12.1 years were studied. Sixty-two patients had distinct atrophy of the first dorsal interosseous of the hand without definite signs of carpal, cubital, or ulnar tunnel syndrome (group A); 43 patients showed neither distinct atrophy nor sensory disturbance of either hand (group B). Other patients had sensory and/or motor disturbances due to carpal, cubital, or ulnar tunnel syndrome and other neuropathies. Electrodiagnostic examinations revealed that there were differences in the distal latency to the first dorsal interosseous muscle from the wrist between 24 group-A patients and 14 normal controls (P less than 0.05), and between the group-A patients and 12 group-B patients (P less than 0.1). The results of this study indicate that some RA patients with atrophy of the thumb web space may have compression neuropathy of the most distal branches of the ulnar nerve.

Arthritis, Rheumatoid↗

PubMed1979

The course of brain atrophy in Parkinson's disease.

In 110 parkinsonian patients (53 men, 57 woman) aged 38--81 years, computer-tomographic follow-up investigations were done to assess the development of brain atrophy. The control examinations were done after an average of 28 months. At that time an increase in brain atrophic changes of different localization could be observed in 23% of the patients. In addition, it could be demonstrated that the increase in pathologic CT findings is to be observed especially in patients with higher age, a more marked impairment in psycho-organic capacity, more pronounced handicaps in the fine-motorial performances at the beginning of the study. From the neuroradiological point of view, patients with more marked pathologic CT findings upon the first examination, be these ventricular enlargement and/or cortical atrophy, more often showed a progression of brain atrophy.

Adult↗

PubMed1980

Effects of a mild weight-lifting program on the progress of glucocorticoid-induced atrophy in rat hindlimb muscles.

The effect of a mild weight-lifting program on the progress of glucocorticoid-induced atrophy was investigated. Groups of rats were either injected daily for 6 weeks with triamcinolone acetonide, 1 mg/kg (group S), subjected to a weight-lifting program 4 times per week (group T), or subjected to the injection and weight-lifting programs concurrently (group ST). The training program, besides decreasing the normal body weight gain of control (group C) rats, had no effects on gastrocnemius and soleus muscles. In group ST, the extent of gastrocnemius atrophy was less severe than in group S, and the mean areas of all fiber types were greater in similar proportion. Gastrocnemius muscles were also tetanically stronger (g, g/g body weight) in group ST than group S. The soleus muscles of groups ST and S were similar in all indices of size and strength, except for a significantly decreased fast-twitsch-oxidative-glycolytic (FOG) mean fiber area in the ST seleus muscles. The extent of fast-twitsch muscle atrophy resulting from chronic glucocorticoid treatment can be lessened by mild weight-lifting exercise. Differences in fiber area responses between soleus and gastrocnemius may reflect recruitment and/or metabolic differences of similar fiber types in the two muscles.

Animals↗

PubMed1983

Morphological feedback effect on neurons of the nucl. arcuatus (sive infundibularis) and nucl. subventricularis hypothalami due to gonadal atrophy.

We have correlated the light-microscopic features in the unmyelinated hypothalamus with gonadal atrophy in 15 women of 30-111 years of age and in 7 men between 29 and 82 years. In the postmenstrual cases there is a distinct concordance of gonadal atrophy and the manifestation of nucleolar changes (augmentation, multiplication and vacuolization) in many nerve cells of the arcuate and subventricular nuclei. In younger, still fertile women this nucleolar finding was seen only rarely and sporadically, was limited to the arcuate nucleus and was absent in the subventricular nucleus. We interpret this nucleolar finding as a feedback effect. In man, too, this coincidence is obvious with age and in gonadal atrophy, with fewer nucleolar changes in old age than are seen in women. This difference is probably caused by a more rapid drop of the estradiol than of the testosterone level. - The hypertrophy of the subventricular nucleus (Sheehan and Kovacs) was also observed in our postmenstrual cases.

Adult↗

PubMed1983

Intrauterine multisystem atrophy in siblings: a new genetic syndrome?

A condition is described in two siblings, dying in early infancy, characterized by an extreme degree of cerebellar hypoplasia, hypoplasia or atrophy of the brain stem with partial preservation of cranial and spinal nerve nuclei, total degeneration of basal ganglia and thalamus, laminar atrophy of the cerebral cortex, and accumulation of sudanophil lipid in astrocytes and macrophages of the hemispheric white matter. It is suggested that this condition, possibly inherited as an autosomal recessive, may represent a progressive multisystem atrophy occurring in utero, affecting various parts of the neuraxis in different stages in their development.

Abnormalities, Multiple↗

PubMed1981

Pallido-nigro-luysial atrophy with massive appearance of corpora amylacea in the CNS.

A case of pallido-nigro-luysial atrophy, characterized by symptoms typical of progressive supranuclear palsy, presented (1) severe symmetrical abiotrophic degeneration of the globus pallidus, substantia nigra, and subthalamic nucleus and (2) widespread accumulations of corpora amylacea in the CNS. The former is thought to be consistent with pallido-nigro-luysial atrophy and we regard it as a combined system degeneration of both the pallido-nigral and the pallido-luysial systems. The significance of the latter is quite obscure. As far as we know, only three cases of pallido-nigro-luysial atrophy have been reported. However, there is no reported case of this disorder with such widespread and massive accumulations of corpora amylacea as in our case.

Aged↗

PubMed1982

Disuse atrophy of human skeletal muscle. An enzyme histochemical study.

The effect of inactivity was examined in muscle biopsies from ten patients who were operated upon for unilateral knee joint lesions of long duration. Biopsies were taken from the vastus medialis on both sides, and transverse frozen sections were stained for myofibrillar adenosine triphosphatase (ATPase). Fibre type counting and exact measurements of muscle fibre size were performed and compared on the two sides. The muscle fibres on the affected side showed a definite reduction in size and, on the average, both types of fibres were about equally affected (27.6% vs. 25.0% reduction for types 1 and 2). However, there were considerable and unpredictable variations from case to case with predominant affection of type 1 in some and type 2 in others. The numerical distribution of fibre types did not change. It is concluded that both fibre types are affected in disuse atrophy, but it seems impossible to predict which of the fibre types will show the most severe atrophy. Thus, enzyme histochemical staining methods are of little help in the diagnosis of disuse atrophy.

Adenosine Triphosphatases↗

PubMed1989

Brain atrophy in Huntington's disease. A CT-scan study.

CT-scan measurements of cortical and subcortical atrophy were carried out in 34 patients with Huntington's disease (HD). While a significant correlation was observed between parameters of subcortical atrophy (bicaudate ratio, bifrontal ratio and third ventricular ratio) and duration of the disease, there was no significant correlation between these parameters and age. On the other hand, measurements of cortical atrophy (frontal fissure ratio and cortical sulci ratio) correlated significantly with age but not with duration of the disease. When a group of 24 HD patients were compared on CT-scan measurements with a group of 24 age-matched normal controls, significant differences were obtained for all the variables examined, but the bicaudate ratio showed the highest sensitivity and specificity. Even mildly affected patients, with duration of motor symptoms less than 3 years had higher bicaudate ratios than age-matched controls.

Adult↗

PubMed1990

Dark adaptation in glaucomatous and nonglaucomatous optic nerve atrophy.

Optic nerve damage is associated with impairment of psychophysical functions. We measured dark adaptation in 21 eyes of 14 normal subjects, 35 eyes of 19 patients with primary open-angle glaucoma, and 7 eyes of 4 patients with nonglaucomatous descending optic nerve atrophy. In the normal subjects light thresholds and time of the shoulder in the dark adaptation curve increased significantly with age. In eyes with glaucomatous or nonglaucomatous optic nerve damage light sensitivity was lower than in normal eyes of age-matched control groups. Rod light sensitivity was significantly (P less than 0.05) correlated with neuroretinal rim loss, parapapillary chorioretinal atrophy, and relative afferent pupillary defects. We conclude that velocity and degree of dark adaptation decrease with increasing age. Patients with glaucomatous and nonglaucomatous optic nerve atrophy show decreased light sensitivity especially in the rod part of dark adaptation worsening with advancing optic nerve damage.

Adult↗

PubMed1993

Peripapillary atrophy in unilateral capsular glaucoma.

Pairwise comparisons of peripapillary crescents and haloes were performed for 56 eyes of 28 patients with early or moderate unilateral capsular glaucoma and no signs of exfoliation in the contralateral eye. The eyes differed highly significantly in rim area, rim/disc area ratio, cup area, and cup volume. They did not differ in disc areas, scleral ring areas, or size of peripapillary crescents. In the nonglaucomatous and glaucomatous eyes a significant correlation existed between the intraocular pressure (IOP) and the area of peripapillary atrophy. The area of peripapillary atrophy was significantly correlated with the damage to the glaucomatous optic nerve head. We concluded that the area of peripapillary atrophy is largely an inborn feature, and that nerve fiber loss can occur without differences in the peripapillary tissues. Nevertheless, an IOP-induced change in the retinal pigment epithelium cannot be ruled out.

Aged↗