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Results for “Multiple System Atrophy”

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PubMed1993

[Magnetic resonance tomography of the brain in Parkinson's disease].

Because cerebral atrophy and white substance of the cerebral hemispheres became typical findings at NMR-tomography of the brain in Parkinson's disease, the authors initiated a study to quantify the observed defects. A combination of neurological, neuropsychological and NMR-tomography methods with addition of formalized scales has been utilized. The severity of cerebral atrophy and that of neurological and psychic disorders were related. Internal atrophy was more frequently observed in older patients, the external one was more characteristic for patients with systemic atherosclerosis. Multiple focal lesions of the white substance were encountered in associated arterial hypertension and was present in all the patients with distinct cerebral atrophy. Diffuse lesions of the white substance (leukareosis) showed no relationship to any of the factors studied.

Adult↗
PubMed2003

The role of nonconventional magnetic resonance imaging techniques in demyelinating disorders.

The use of nonconventional magnetic resonance imaging techniques (eg, magnetization transfer, magnetic resonance spectroscopy, and diffusion weighted imaging) allows for an accurate characterization of lesions as compared with conventional or standard approaches in demyelinating diseases. Magnetization transfer, magnetic resonance spectroscopy, and diffusion weighted imaging have revolutionized our understanding of demyelinating diseases because these techniques have been used to identify pathologic changes of normal-appearing brain tissue and characterize the differences in lesions. Metrics derived from these methods correlate with clinical disability and provide more accurate tools for monitoring disease activity and treatment effect over time. Quantitative T1 and T2 relaxation time maps provide additional information on demyelinating diseases, allowing for the evaluation of myelin water and distribution of water within tissues. Finally, the measurement of central nervous system atrophy has become a valuable element in determining the course of multiple sclerosis.

Atrophy↗
PubMed2005

Systemic AL amyloidosis associated with multiple myeloma in a horse.

AL amyloidosis is the most common type of systemic amyloidosis in humans, and it is frequently associated with multiple myeloma. But, AL amyloidosis is very rare in domestic animals. A 16-year-old Quarter horse gelding was diagnosed with systemic AL amyloidosis associated with multiple myeloma. Clinical problems were rapid weight loss, muscle atrophy, soft unformed stool, and ventral edema. Grossly, diffuse gastrointestinal hemorrhage, markedly thickened jejunal mucosa, and splenomegaly were present. Microscopically, diffuse severe amyloid deposits were present in the lamina propria of glandular stomach, duodenum, and jejunum. Much of the spleen and sternal bone marrow was replaced by neoplastic round cells, and multiple foci of amyloid were also present in the spleen and bone marrow. Electron microscopy revealed the neoplastic round cells to be of plasma cell origin, and the amyloid showed a strongly positive immunoreactivity with polyclonal anti-human immunoglobin lambda light-chain antisera. To our knowledge, this is the second report describing systemic AL amyloidosis in domestic animals-associated plasma cell neoplasia and the first associated with multiple myeloma, as is common in humans.

Amyloidosis↗
PubMed1993

Low vision rehabilitation of multiple sclerosis: a case report.

A 34-year-old white male presented with a previous diagnosis of optic atrophy secondary to multiple sclerosis, seeking low vision rehabilitation. This case report is presented to illustrate the importance of prescribing both conventional optical low vision devices, as well as computer low vision aids in order to provide comprehensive low vision rehabilitation for a young visually impaired adult.

Adult↗
PubMed1990

Multiple system degeneration with glutamate dehydrogenase deficiency: pathology and biochemistry.

The neuropathological findings in a patient with antemortem diagnosis of olivopontocerebellar atrophy (OPCA) and reduced leucocytic glutamate dehydrogenase (GDH) activity included cerebellar cortical degeneration, most marked in the superior vermis, mild atrophy of the pons and the inferior olivary nucleus, marked reduction of anterior horn cells at all levels and gliosis in both lateral columns. GDH activities and their thermolability in "soluble" and "particulate" fractions in the cerebral cortex, cerebellar hemisphere and vermis were not significantly different from the values in two control brains. GDH mRNA in the patient's brain was not altered in size or amount.

Aged↗
PubMed1981

The clinical spectrum of hexosaminidase deficiency diseases.

Hexosaminidase deficiency diseases or GM2-gangliosidoses were originally described as infantile encephalopathies. Recently, hexosaminidase deficiencies have been found with different phenotypes, including juvenile and adult encephalopathies, cerebellar ataxias, and motor neuron diseases. Individual cases have resembled Ramsey-Hunt syndrome, olivopontocerebellar ataxia, Friedreich ataxia, amyotrophic lateral sclerosis, Kugelberg-Welander disease, Fazio-Londe disease, and Charcot-Marie-Tooth disease. Tremor, dystonia, spastic paresis, and psychosis have been seen. Since few diagnosable causes for these system atrophies are known, these patients should be tested for hexosaminidase deficiency. These recessive disorders fit a multiple loci/multiple alleles genetic scheme, and a clinical genetic classification is presented.

Adult↗
PubMed1991

The aetiology of dizziness and how to examine a dizzy patient.

Dizziness embraces various sensations of spatial disorientation. A common symptom, it has been experienced by at least one third of the population by the age of 65. Peripheral causes include disorders of the labyrinth and vestibular nerve such as Ménière's disease, benign paroxysmal positional vertigo, acute vestibulopathy (vestibular neuronitis) and acoustic neuroma. The most common lesions of the central nervous system that cause dizziness are infratentorial ischaemia, mechanical trauma, multiple sclerosis and cerebellar atrophy. Other aetiological factors include intoxications, psychogenic causes, cervical problems and cardiovascular diseases. The history and physical examination are the cornerstones of the search for the cause of dizziness. The most valuable otological methods are electronystagmography and audiometry. Of the clinical neurophysiological methods, brainstem auditory evoked potentials are more useful than electroencephalography. Computed tomography and magnetic resonance imaging should be used when a CNS disorder is suspected.

Dizziness↗
PubMed1970

Clinical neurology.

Explore the source record for details and available documents.

Cerebrovascular Disorders↗
PubMed2005

Impaired regulation of gonadotropins leads to the atrophy of the female reproductive system in klotho-deficient mice.

klotho-Deficient mice exhibit a syndrome resembling human premature ageing, with multiple pathological phenotypes in tissues including reproductive organs. It was proposed that Klotho might possess the hormonal effects on many organs. In this study, the female reproductive system of klotho mice was examined to reveal the mechanism that brought the female sterility by histological and molecular approaches. We observed cessation of ovarian follicular maturation at the preantral stage and the presence of numerous atretic ovarian follicles and atrophic uteri. In situ hybridization analysis revealed that LH receptor and aromatase P450 were not expressed in the ovaries. These results suggest the impairment of gonadal development during the antral transition process. We next addressed the responsible organs for the failure of antral transition. Transplantation of klotho ovaries to wild-type mice resulted in the ability to bear offspring. Administration of FSH or GnRH induced advanced maturation of ovaries and uteri in klotho mice. These results indicate that the female reproductive organs in klotho mice are potentially functional and that klotho gene deficiency leads to the atrophy of reproductive organs via impairment of the hypothalamic-pituitary axis. Absence of the estrus cycle and constant low trends of both FSH and LH levels were found in female klotho mice. Immunohistochemical analysis revealed that the production of both FSH and LH were decreased in pituitary gland. Taken together, our findings suggest the involvement of klotho in the regulatory control of pituitary hormones.

Animals↗
PubMed1983

[Striatonigral degeneration associated with olivopontocerebellar atrophy. Anatomo-clinical study of 3 cases. Nosologic discussion].

Results of a clinicopathologic study in 3 cases of nigrostriatal degeneration associated with olivopontocerebellar atrophy are reported. The clinical picture was primarily that of parkinsonism but associated disorders were atypical: sphincter and deglutition disorders, pyramidal signs and action myoclonia; paralysis of vertical gaze (with a deficit of convergence in 1 case) and a cerebellar syndrome in 2 cases; postural hypotension with an invariable pulse, amyotrophy of the hands and a paradoxical response to Dopa: worsening of akineto-hypertonic symptomatology and deglutition disorders in 1 case. Pathologic examination showed similar lesions in the 3 cases: atrophy with pigmentation of the putamen and changes in the substantia nigra, associated with evidence of olivopontocerebellar degeneration. There was marked loss of neurones in the intermediolateral tract in the cord of the patient with the Shy and Drager's syndrome. All cases reported of this abiotrophic association have been of a sporadic nature. The predominance of females, the age of onset and duration of the disease are features that do not differ much from those of pure nigrostriatal degenerative disorders. These cases cannot therefore be considered as a pathological entity but as a particular form of degeneration affecting multiple systems.

Adult↗
PubMed1987

Pathologic features in two siblings with the Pena-Shokeir I syndrome.

Two siblings whose clinical and pathologic features were consistent with the "Syndrome of camptodactyly, multiple ankyloses and pulmonary hypoplasia" originally described by Pena and Shokeir were examined at autopsy. Additional features were intrauterine growth retardation, immaturity of the central nervous system (CNS) and atrophy of skeletal muscles. Our data suggest that CNS damage may cause the complicated phenotypic abnormalities of the syndrome.

Abnormalities, Multiple↗
PubMed2000

Non-carcinogenic effects of TCDD in animals.

Exposure to TCDD and related chemicals leads to a plethora of effects in multiple species, tissues, and stages of development. Responses range from relatively simple biochemical alterations through overtly toxic responses, including lethality. The spectrum of effects shows some species variability, but many effects are seen in multiple wildlife, domestic, and laboratory species, ranging from fish through birds and mammals. The same responses can be generated regardless of the route of exposure, although the administered dose may vary. The body burden appears to be the most appropriate dosimetric. Many of the effects often attributed to TCDD are associated with relatively high doses: lethality, wasting, lymphoid and gonadal atrophy, chloracne, hepatotoxicity, adult neurotoxicity, and cardiotoxicity. Changes in multiple endocrine and growth factor systems have been reported in a manner which is tissue, sex, and age-dependent. The most sensitive adverse effects observed in multiple species appear to be developmental, including effects on the developing immune, nervous, and reproductive systems. Such effects have been observed at maternal body burdens in the range of 30-80 ng/kg in both non-human primates and rodents. Biochemical effects on cytokine expression and metabolizing enzymes occur at body burdens which are within a factor of ten of the clearly adverse developmental responses. Thus, effects on the immune system, learning, and the developing reproductive system of multiple animals occur at body burdens which are close to those present in the background human population.

Acne Vulgaris↗
PubMed1977

Histocompatibility antigens (HLA): associations with immunopathic diseases and with responses to microbial antigens.

Described is the experience from a single histocompatibility typing laboratory sampling, firstly, Australian patients with various immunopathic diseases and, secondly, subjects previously classified as "responders" or "non-responders" to various microbial antigens. The diseases considered included chronic active hepatitis (CAH) and various cirrhoses, "thyrogastric" autoimmune diseases, systemic lupus erythematosus (SLE), rheumatoid arthritis, dermatitis herpetiformis (DH) with intestinal villous atrophy, and multiple sclerosis (MS). The immune responses considered included those to flagellin, candidin, mumps, trichophyton, tuberculin and streptococcal enzymes. The HLA specificities particularly associated with disease included B8 (CAH, thyrotoxicosis SLE, DH, and miscellaneous immunopathic diseases) and B7 (thyrotoxicosis, SLE, DH, and MS). The same specificities were present in excess, although not impressively so, among responders to certain of the microbial antigens, i.e. B7 with high responders to flagellin and B8 (and A1) with responders to trichophyton.

Adult↗
PubMed1987

Nephropathic cystinosis with central nervous system involvement.

Nephropathic cystinosis is associated with end-stage renal failure, retinal damage, and hypothyroidism. Patients may now survive past the first decade of life with the use of dialysis and renal transplantation. Examination of a 24-year-old woman with this disorder revealed ovarian failure, mildly abnormal results on a glucose tolerance test, intermittent confusion, short-term memory loss, and cerebral atrophy on computerized axial tomography. Autopsy examination at age 25 years revealed cystine storage in multiple tissues including the atrophic ovaries, pancreatic islet cells, the aorta, and the brain. Dysfunction of multiple organ systems may develop in patients with cystinosis who survive into adulthood. This emphasizes the need for a systemic therapy for cystinosis.

Adult↗