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PubMed1992

[Dentatorubropallidoluysian atrophy (DRPLA): comparative pathological study on clinical groups classified into juvenile, early adult and late adult types].

We performed a clinicopathological study on 12 autopsied cases of dentatorubropallidoluysian atrophy. They were divided into 3 groups according to the age at onset: juvenile type (6 cases), early adult type (4 cases) and late adult type (2 cases). In juvenile type showing progressive myoclonus epilepsy (PME) syndrome, degeneration of the globus pallidus was more marked than that of the dentate nucleus. Mild to moderate atrophy was seen in the brain stem and spinal cord. In early adult type showing milder symptom of myoclonus and epilepsy, the globus pallidus and dentate nucleus were equally degenerated to various extents in most cases. Atrophy of the brain stem and spinal cord was mild to moderate in degree. In late adult type without PME syndrome, degeneration of the dentate nucleus was more marked than that of the globus pallidus. The brain stem and spinal cord were severely atrophic. On the other hand, the cases showing severe dentate lesion had a tendency to show severe atrophy of the brain stem and spinal cord. We consider that development of myoclonus, epilepsy and choreoathetoid movement in DRPLA patients has close relation to the extent of not only degeneration of the globus pallidus and dentate nucleus, but also atrophy of the brain stem and spinal cord.

Adolescent↗

PubMed1992

[Differential diagnosis in early muscular hypotonia: spinal atrophy].

Changes in type, size and structure of muscle fibres were analyzed in 68 biopsies with clinical and morphological signs of spinal atrophy. Reliability of clinical prebioptic diagnosis was evaluated in comparison with bioptic finding and final diagnosis. Muscle biopsy is often to be performed in spite of apparently unambiguous clinical and electromyographical finding. Contribution of biopsy to the diagnosis of spinal atrophy with characteristical grouping of atrophic fibres is undisputable in most cases. However, unproper sampling in excessively atrophic areas and structural "myogenic" changes can made the bioptic diagnosis difficult or impossible. Size analysis of muscle fibres proved participation of both the basic fibre types in atrophy with increase in number of immature type 2c fibres, and prevalence of type 1 fibres in hypertrophic areas (compensatory hypertrophy or re-innervation respectively) where also hybrid fibres can often be found during transformation of their type. Angular atrophic fibres occurring in hypertrophic fibre groups gave evidence of continuous or secondary denervation in benign forms of spinal atrophy. The author found regressive structural changes in benign form and in malignant Werdnig-Hoffman atrophy either--though less extensive.

Diagnosis, Differential↗

PubMedDate not supplied

Progressive dysphasic dementia with localized cerebral atrophy: report of an autopsy.

A 64-year-old Japanese woman showed an initially aphasic disturbance followed by complete mutism, progressive dementia, parkinsonism and muscular atrophy. Autopsy revealed localized cortical atrophy confined to the pars triangularis, pars opercularis of the inferior frontal gyrus, supramarginal and angular gyri of the inferior parietal lobe, precuneus and posterior half of the middle and inferior temporal gyrus predominantly on the left hemisphere. The right cerebellar hemisphere showed crossed cerebellar atrophy with shrinkage of the right middle cerebellar peduncle. In the atrophied cerebral areas there were diffuse outfall of neuronal cells in all cortical layers and remaining neurons generally showed simple atrophy, and there were a few swollen neurons. Gliosis of the subcortical white matter was confined to the affected gyri and GFAP positive astrocytes were observed in the 1st, 2nd, 5th and 6th layers of the cortex. In addition, the degenerative changes of the substantia nigra, gliosis of the amygdaloid complex and inferior olivary nucleus were bilaterally observed. The distribution and characteristics of the cortical and white matter degeneration are different from those of Pick's disease, and it is likely that this case belongs to a group of so-called degenerative dysphasic dementias.

Aphasia↗

PubMed2006

[Two boys with non-progressive unilateral atrophy of the calf muscles].

We report here two boys who presented with atrophy of the right calf muscle. The onset was insidious and the symptom was found in infancy in case 2. They were followed for more than 5 years and no progression was seen. No sensory disturbances or autonomic nervous system symptoms were observed. Although needle electric myograph and muscle biopsy findings showed a neurogenic pattern, no cause was confirmed. Recently, a new disease concept of "benign monomelic amyotrophy of lower limb" was established and this could be applied to these patients. This disorder is rare and it is seen in young adults. It is characterized by non-progressive unilateral calf muscle atrophy. There is almost no possibility that this disorder is a variant of spinal muscular atrophy. When a patient presents with unilateral calf muscle atrophy, the treatable causes of the atrophy, including spinal cord disorders or peripheral nerve disorders, should be excluded first. If a confirmed cause cannot be found, then there should be a careful follow-up of the patient, even if the symptoms are stable.

Adolescent↗

PubMed1990

[The dependence of the diaphragmatic properties of the iris on the degree of its atrophy in the pseudoexfoliative syndrome in cataract patients].

A study of the dependence of diaphragmatic properties of the iris on the degree of its atrophy carried out in 76 patients (78 eyes) with a pseudoexfoliative syndrome has shown that under the action of mezatone injected into the anterior chamber the maximum diameter of the pupil and the degree of its widening are in direct dependence on the degree of iris atrophy. The results obtained are of practical importance, allow to plan the course of cataract extraction and foresee complications. In iris atrophy of degree I, the maximum diameter of the pupil is sufficient to be that needed for cataract extraction made by a common method. In atrophy of degree II, it may become necessary during the operation to make sphincterotomy for a free withdrawal of the lens, and in atrophy of degree III, sphincterotomy should be planned, because maximum widening of the pupil in such patients is 3.88 +/- 0.16 only.

Adult↗

PubMedDate not supplied

[Brain atrophy: radiological-neurological correlations].

Computerized tomography of the head was done in 5021 patients aged 21 to 81 years from various neurological, neurosurgical and neurotraumatological indications. Brain atrophy as an only finding (primary) was noted in 11.5%- and in 5.4% of cases it was associated with other changes. The neurological-radiological correlations were established in 200 cases of primary strophy. History data included: headaches in 54.5%, dizziness in 15.0%, epilepsy in 24.5%. Objective examination showed: slight hemiparesis in 37.5%, spastic-atactic gait disturbances in 31.5%, isolated damage to the corticospinal tracts with signs limited to one side of the body was more frequent in cortical atrophy, and these signs associated with ataxia were more frequent in subcortional atrophy. The authors explain this as a loss of cortical cells or damage to the paraventricularly coursing nerve fibres. A probable aetiology of "primary" atrophy was established in 405% of cases (hypertension, atherosclerosis, minor craniocerebral trauma). No signs or neurological syndromes were observed which could be regarded as more or less characteristic of brain atrophy.

Adult↗

PubMed1989

[Two cases of post-poliomyelitis muscular atrophy].

We report two males with progressive post-poliomyelitis muscular atrophy (PPMA) who developed slowly progressive weakness and atrophy in the previously unaffected muscles 50 years after acute poliomyelitis. Case 1 is a 61-year-old male who had suffered from poliomyelitis at the age of 3 years. After recovery from acute symptoms, paralysis remained in the right face, left upper extremity and right lower extremity. Paralysis had remained unchanged until the age of 55 years, when he felt increased fatigue and progressive muscle weakness of all extremities. Neurological examination revealed muscle atrophy of the left upper and right lower extremities, and muscle hypertrophy of the left lower extremity. Deep tendon reflexes were decreased or absent. Babinski sign was negative. Reduction of his physical level and administration of anticholinesterase agents improved his feeling of fatigue. Case 2 is a 66-year-old male who suffered from poliomyelitis in his infancy, and paralysis remained in the left hand and right lower extremity. At the age of 50 years, he felt fatigue, muscle weakness of the previously unaffected legs and joint pain. Neurological examination revealed proximal muscle atrophy and weakness of lower extremities. Deep tendon reflexes were decreased and Babinski sign was negative. Needle electromyography of both cases showed typical neurogenic pattern or high amplitude and polyphasic long duration MMUs. Muscle biopsy of them from quadriceps femoris showed variability in fiber size, moderate increase of internal nuclei, fiber splitting, type grouping, grouped atrophy and scattered small angulated fibers, indicating chronic denervation and reinnervation. The clinical features and laboratory findings of the present two cases are consistent with those of PPMA.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

PubMed1985

[Animal experiment and biopsy studies on the cause, manifestations and diagnostic relevance of perifascicular muscle fiber atrophy].

On the basis of previous (n = 23) and current muscle biopsies (n = 25) as well as five animal-experimental investigations, the paper deals with the cause and the manifestation of perifascicular muscle-fiber atrophy. A deteriorated trophic situation of the subfascial or marginal muscle fibers in patients suffering from immunocomplex vasculopathies in connection with a reduced supply of blood to the muscles is seen as the cause of the areactive weight loss (atrophy). This is accompanied by a considerably reduced capillary supply of blood to the muscle fibers. Even in normal conditions, the portion of collateral vessels and capillaries is lower in the subfascial region than in the center of the fascicle. When the blood supply is disturbed, the marginal fibers are in a trophic situation worse than that of the central muscle fibers. They become atrophied. In responsive patients, the muscle fibers regenerate and recapillarize during the convalescence period. This can be shown histochemically by means of the alkaline phosphatase reaction. The extent of perifascicular musclefiber atrophy can be fixed in quantitative terms by the method of Baumli and Mumenthaler. Where a second biopsy is indicated, results can be obtained regarding the changes in the perifascicular atrophy as a consequence of the therapy provided.

Acid Phosphatase↗

PubMed1985

Relationship between Ah receptor-mediated polychlorinated biphenyl (PCB)-induced humoral immunosuppression and thymic atrophy.

Thymic atrophy and humoral immunosuppression by certain polychlorinated biphenyls is associated with the aromatic hydrocarbon (Ah) receptor in mice. We examined the relationship between these two toxic effects. 3,3',4,4'-Tetrachlorobiphenyl (TCB), which causes immunosuppression and thymic atrophy, and 2,3,3',4,4',5-hexachlorobiphenyl, which causes immunosuppression without thymic atrophy, were administered i.p. to C57BL/6 mice at 0, 35 and 350 mumol/kg b.wt. 2 days before i.v. immunization with 10 micrograms of Escherichia coli lipopolysaccharide. Both congeners caused significant suppression of the day 4 anti-lipopolysaccharide plaque-forming cell response/spleen (less than or equal to 46% of control). TCB (350 mumol/kg) was also administered 2 days before either a primary or secondary i.p. immunization with sheep erythrocytes. TCB treatment before primary immunization had no effects on the day 5 secondary response, whereas treatment before the secondary immunization significantly inhibited both day 5 immunoglobulin M and immunoglobulin G plaque-forming cells (less than 10 and less than 2% of control, respectively) and decreased serum antibody. TCB administered either 8 or 2 days before or 2 or 4 days after immunization with sheep erythrocytes demonstrated that significant suppression of both plaque-forming cells and serum antibody could occur without thymic atrophy. Immunity was most impaired when TCB was given 2 days before immunization. These results demonstrate that thymic atrophy does not always accompany the severe immunosuppression caused by Ah receptor ligands and suggests that it may not be a sensitive measure of Ah receptor-mediated immunosuppression. The data also suggests that differentiation of B lymphocytes into antibody producing cells is impaired during Ah receptor-mediated gene activation.

Animals↗

PubMed1988

Thigh muscle atrophy and postoperative osteoarthrosis after knee ligament tears.

The aim of the study was to clarify if atrophy of the thigh muscles was related to the radiological and clinical long-term healing results of primary knee ligament surgery. At the Tampere University Central Hospital a radiological and clinical reexamination was performed in 61 patients on an average 40 months after primary operation of acute knee ligament injury. The study group consisted of 26 patients having clear quadriceps muscle atrophy in the injured knee at the follow-up. Thirty-four patients with equal circumference of the thighs, served as controls. One patient was excluded. The groups did not differ significantly from each other in respect to age, sex, physical activity, type of ligament injury, meniscectomy, and follow-up time. In radiological evaluation the previously developed knee scoring scale was used taking into account only changes caused by the studied injury. The mean score of the study group was 93.7 +/- 6.2 (range 73-100) and the controls 96.2 +/- 4.0 (87-100); p less than 0.05. In both the most common changes of post-traumatic osteoarthrosis were osteophytes, ligament calcification and joint space narrowing. In clinical examination the patients of the study group were placed significantly more often in the groups of fair or poor healing in subjective, objective, overall (in each p less than 0.001) and functional (p less than 0.05) evaluation. According to this study, it seems that thigh muscle atrophy plays an important role as a poor prognostic factor of knee ligament injuries being thus a good target for efforts to stop the continuous deterioration of the injured knee. Obviously the post-traumatic osteoarthrotic process is a vicious circle: thigh atrophy increases dynamic knee instability, which in turn not only starts or accelerates the arthrotic process but also increases--by discomfort and disuse of the knee--the atrophy itself.

Adolescent↗

PubMed1988

[Studies of the factors affecting the reduction of chronic subdural hematoma: effect of preoperative factors with special reference to cerebral atrophy].

The authors examined the preoperative factors affecting the reduction of chronic subdural hematoma (CSDH). A new method was developed to calculate the reduction rate of CSDH using CT volumetry technique. Postoperative serial CT films were examined and each hematoma volume was calculated by microcomputer. A postoperative exponential decrease of hematoma volume was observed, and the log-transformation of the hematoma volume turned the curve to a linear decrease. The least square method was used to fit an exponential curve, and the half reduction date (HRD) was calculated from the fitting curve. This HRD represents a mathematical indicator of the reduction rate of CSDH. Using this technique, the relationships between this HRD and the following parameters were examined in 61 patients. The preoperative factors examined are as follows; 1) patient's age, 2) preoperative hematoma volume, 3) CT number of the hematoma, 4) time interval between the head injury and the onset of symptom, and 5) the degree of cerebral atrophy. The degree of cerebral atrophy was calculated by means of a simple approximate formula as described by one of the authors (K.N.). Patient's age and the hematoma volume were correlated with HRD, and the correlation coefficients were 0.346 (p less than 0.01) and 0.298 (p less than 0.05) respectively. On the other hand, preoperative CT findings and the duration from the trauma had no correlation with HRD. Cerebral atrophy has hitherto been thought to affect the reduction of hematoma. However, our quantitative study revealed no correlation between cerebral atrophy and the reduction of hematoma rate. This unexpected result may be attributed to the difference between cerebral atrophy and cerebral elasticity.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1987

Muscle atrophy in rheumatoid arthritis.

The purpose of this paper is to describe some research opportunities in the area of muscle atrophy secondary to arthritis. Future research should utilize quantitative methods of evaluating muscle atrophy such as computerized tomography or ultrasonography. The gross muscle atrophy should be further characterized by muscle fiber type measurements. These techniques as well as biochemical determinations should be utilized to identify mechanisms of muscle atrophy as well as determine ideal methods for reversing muscle atrophy and weakness.

Arthritis, Rheumatoid↗

PubMed1986

[CT study of brain atrophy--the value of various study methods].

55 rather young patients suspected of dementia were examined via CT to assess the comparative value of various CT examination methods to determine cerebral atrophy. The patients were assessed clinically and classified as normal (24), slightly demented (21) and moderately demented (10). There were no severely demented patients. The parameters measured consisted of several diameters and indexes as well as the volume of the ventricular system and of the subarachnoidal space. The atrophy level was also determined visually. With the exception of the breadth of the third ventricle there was no correlation between internal cerebral atrophy and the level of dementia. Neither the mean breadths of four external sulci nor the volume of the subarachnoidal space (both being representative of external atrophy) showed any significant correlation with the degree of dementia of the patients on the levels 0.005 or 0.025, respectively. However, the results were not better than those obtained by visual assessment of external atrophy. Reasons are discussed why objective measurements do not yield reliable results.

Atrophy↗

PubMedDate not supplied

[Cryptogenic cerebral atrophy in late-onset epilepsy].

174 subjects suffering from late onset epilepsy were examined. The incidence of cerebral atrophy, defined with objective criteria on CT scans by means of a comparative analysis with a matched for age control group was 37.9%. Head traumas, cerebrovascular disorders and alcoholism were the presumed etiological factors in most of the cases while in 25 subjects (20%) the etiology of cerebral atrophy remained unknown ("cryptogenous atrophy"). Clinical profil, psychometric performance and EEG characteristics of these subjects were investigated and compared with the remainder groups of late-onset epileptics. The results obtained show that the subjects with cryptogenous atrophy behave, as regard the features considered, as the late-onset epileptics with normal CT but present an higher familial prevalence for epilepsy. On the contrary they differs in various ways from late-onset epileptics with atrophy of known origin and from epileptics with focal lesions.

Alcoholism↗

PubMedDate not supplied

[Cerebral atrophy, diagnosis using tomodensitometry compared with the new Wechsler intelligence scale in a psychiatric population].

In a psychiatric service, the present compares results of a simple psychometric test (in this case the new Wechsler Adult Intelligence Scale - WAIS) with those obtained from a brain CT Scan in two groups of 20 patients showing cerebral atrophy vs. twenty patients with normal CT Scan. Results show there exists a rather complex relationship between the cerebral atrophy scan and the intellectual deterioration. The performance to WAIS appears as globally lessened in cerebral atrophy cases. As for the cerebral rating scale, lower scores are registered only in vocabulary and similitudes in cerebral atrophy cases. Psychometric deterioration, according to scales and to formulation of "applicable" or "no-applicable" to age does not apply to the tests given in the course of the present study of cerebral atrophy.

Adult↗

PubMedDate not supplied

Vaginal atrophy in the postmenopausal woman. The importance of sexual activity and hormones.

The effect of sexual activity on vaginal atrophy was investigated in a group of 52 postmenopausal women (mean age, 57 years). Subjects were divided into two groups: sexually active (intercourse frequency, three or more times monthly) and sexually inactive (intercourse frequency, less than ten times yearly). Two gynecologists examined all subjects and completed an index of vaginal atrophy that assessed six genital dimensions. Blood samples were also analyzed by radioimmunoassay for levels of circulating estrone, estradiol, androstenedione, testosterone, follicle-stimulating hormone, and luteinizing hormone (LH). As predicted, less vaginal atrophy was apparent in the sexually active women as opposed to the sexually inactive women. Further, women with less vaginal atrophy had significantly higher mean levels of androgens (androstenedione and testosterone) and gonadotropins (particularly LH). We discuss herein the implications of this study, particularly the importance of androgens in reducing atrophy and maintaining sexual interest.

Aged↗

PubMed1982

Transcapillary and interstitial diffusion of Na-fluorescein in chronic venous insufficiency with white atrophy.

White atrophy (atrophie blanche) in the medial ankle region due to chronic venous insufficiency (stasis syndrome) was studied in 12 patients by fluorescence video microscopy [1,8]. After intravenous bolus injection of 1 ml of 20% Na-fluorescein the dynamic phenomena of transcapillary and interstitial diffusion of the dye were analyzed by a videodensitometer which has moved on single frames of the TV-recordings across white atrophy at different times after dye appearance. White atrophy is characterized by 3 main areas: 1) the avascular field sensu strictu, 2) the border region with enlarged and tortuous capillary loops, and 3) the more remote capillaries showing less altered morphology. The dye reached the ankle skin after 39.2 +/- 13.3 S and leaved rapidly the intravascular compartment. In the region of the border capillaries, the maximal fluorescent light intensity was reached after 5 min, in the centre of the avascular field only after 40 min. Initially, the densitometer curves show a 'valley' which is slowly filled up by the dye. At 40 min and later on the highest light intensities were measured in the central parts of the avascular field ('mountain'), where the clearance was just beginning. The slow diffusion of the small tracer into the avascular field explains that white atrophy is a predilection site for venous ulcer formation.

Adult↗

PubMed1980

[Ultrastructural stereologic study of cardiac muscle cells during myocardial atrophy].

Wistar rats were exposed to complete starvation over 2, 5 and 10 days. In the course of the experiment the animals developed general atrophy associated with myocardial atrophy. They showed also diminution of the absolute weight of the heart and the diameter of cardiomyocytes. The volumetric and surface density of cell structure were detected stereologically by electron microscopy to evaluate the volumetric and surface-volumetric ratio of the main organellas. It was demonstrated that the volumetric density of mitochondria diminishes in myocardial atrophy while the relative amount of myofibrils increases. The surface density of mitochondria is maintained at the same level as that seen in the control and declines by the end of the experiment. The myocardial atrophy was stereologically ascertained by the volumetric ratio of mitochondria to myofibrils and by that of the T system to myofibrils. This ratio becomes the lesser the higher the degree of atrophy.

Animals↗