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Biomedical subjects

C M Poser

Publications and source records attributed to C M Poser.

At least 19 recordsLinked to original sources

Improvement in motor evoked potentials and clinical course post-steroid therapy in multiple sclerosis.

Motor evoked potentials (MEP) were recorded in 23 patients with definite relapsing multiple sclerosis before and after treatment with a short course of high dose of methylprednisolone. MEP were performed together with clinical examination just before treatment, and 6 and 60 days later. The following results were observed: (1) a statistically significant relationship between the corticospinal deficit and the alteration in MEP, (2) a significant improvement in latency of MEP by day 6, (3) a significant correlation between the change in the Kurtzke disability scale rating and the improvement in MEP. The results provide further evidence for the possible effectiveness of short courses of high dose corticosteroids in the treatment of relapses of multiple sclerosis and the usefulness of MEP in its assessment.

Adult

The epidemiology of multiple sclerosis: the Iceland model. Onset-adjusted prevalence rate and other methodological considerations.

The epidemiology of multiple sclerosis (MS) is characterized by the fact that there is an uneven distribution of the disease throughout the world. The two most commonly used indices of its frequency are the incidence and prevalence rates. The incidence rate reflects, to a great extent, the influence of environmental factors in triggering the clinical manifestations of the disease, if it is based upon the actual date of the initiation of symptoms, rather than the date of diagnosis. The prevalence rate is currently based on the date of diagnosis and includes all MS patients who are alive on a particular date, without regard to their ethnic origin, the site and the duration of residence or any other factors that may have influenced the acquisition of the disease. We propose that in order to make the concept of the prevalence rate possibly more meaningful, the term should refer, retrospectively, to all patients whose symptoms eventually led to the diagnosis of MS, even though the diagnosis was not yet established on an earlier prevalence day. In addition, only patients of the same ethnic background who have spent their prepuberal years in the geographical area under study should be included. We are calling this measure the onset-adjusted prevalence rate. Another problem encountered in epidemiological studies of MS relates to the common practice of adjusting prevalence data obtained for age and sex in one area to what is referred to as a "standard" American (or world) population, groups of great ethnic and age diversity. It is also curious that in many studies the bases for comparison of populations are census data obtained many years previously. We suggest that age and sex adjustment should be applied only to similar ethnic groups born and raised under different environmental conditions. We believe that data obtained by calculating an onset-adjusted prevalence rate restricted to a homogeneous group of patients sharing the same environment during the prepuberal years may provide valuable etiological clues.

Age Factors

Multiple sclerosis.

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Adrenal Cortex Hormones

A numerical scoring system for the classification of multiple sclerosis.

A simple, numerical system for the classification of multiple sclerosis into probable and definite categories is proposed. This system is based upon the analysis of the clinical symptomatology of 111 autopsy-proved cases of the disease from England, Norway and the United States. It is not designed to be used as an aid in the diagnosis and should be applied only to those cases where multiple sclerosis is suspected. It permits the incorporation of data obtained by means of evoked responses studies, psychological evaluations and other methods for confirming anamnestic information or delineating some asymptomatic lesions. The numerical system also provides a means for plotting the temporal evolution of the disease strictly in terms of the appearance of new signs and symptoms since recurrences of previously experienced symptoms cannot be scored; thus it has the advantage of not allowing so-called exacerbation due to physiological or psychological factors to falsely suggest disease progression or activity. The major use of this scoring system is to provide a reasonably reliable means of comparing epidemiologic data from different investigators by more clearly defining the probable and definite categories.

Adolescent

Parainfluenza virus type 3: isolation from CSF of a patient with Guillain-Barré syndrome.

We report the isolation of parainfluenza virus type 3 from the CSF of a 19-year-old man with Guillain-Barré syndrome. Although parainfluenza viruses are usually associated with respiratory tract illnesses, some strains show a neurotropism not previously appreciated. Thus, parainfluenza viruses may be responsible for a portion of cases of Guillain-Barré syndrome.

Cerebrospinal Fluid

Recurrent disseminated vasculomyelinopathy.

The monosymptomatic (recurrent infantile hemiplegia) and the polysymptomatic forms of disseminated vasculomyelinopathy that follow various infections and antigenic challenge to the nervous system were seen in two cases. These cases emphasize the importance of vasculopathy as the initial and obligatory component of the postinfectious and postimmunization neurologic syndromes as well as the clinical and pathological variability of the secondary effects on the nervous system. Recurrent infantile hemiplegia occurred in the first patient. In the second patient, after two episodes of postinfectious myelinoclastic encephalopathy, concurrent acute hemorrhagic leukoencephalopathy and an acute Guillain-Barré syndrome following swine flu vaccination developed.

Adrenal Cortex Hormones

Dysmyelination revisited.

Dysmyelination describes an inborn error of metabolism affecting myelinogenesis that causes it to be abnormal, arrested, or delayed. Abiotrophy or myelin as defined by Gowers, due to metabolic failure of the myelin maintenance system, is yet another feature of dysmyelination. In addition to the leukodystrophies, genetically determined conditions such as infantile amaurotic idiocy, hematosidosis, Niemann-Pick's disease and several of the aminoacidopathies are examples of dysmyelinating diseases. In order to reconcile morphological and neurochemical data in these conditions, it is necessary to reexamine a number of pathogenetic hypotheses based on known enzymatic deficiencies, and the interpretation of fragmentary biochemical analyses. The obligatory role of the neuron and axon in myelin formation and maintenance is reviewed. The hypothesis is advanced that gangliosides and their degradative products constitue precursors for the synthesis of the characteristic myelin sphingolipids cerebrosides, sulfatides, and sphingomyelin. Alterations in axoplasmic flow and of ganglioside metabolism must be condidered as important factors in the pathogenesis of dysmyelination.

Axons

Neurological manifestations of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease): report of 2 cases and review of the literature.

Two cases of hereditary hemorrhagic telangiectasia (HHT) with neurological involvement are presented. One patient had multiple vascular malformations including telangiectasias of the brain, medulla, and spinal cord and a berry aneurysm of the internal carotid artery; she also had a large cerebellar abscess, presumably reflecting the presence of a pulmonary arteriovenous fistula. The second patient had an idiopathic subarachnoid hemorrhage. In more than 200 reported patients with HHT involving the nervous system, 61% had lesions seondary to a pulmonary arteriovenous fistula (cerebral hypoxemia, paradoxical and septic emboli, and brain abscess). The findings emphasize the need for early surgical correction of such fistulas. In 36% of the patients with neurological involvement and HHT, vascular malformations of the brain and spinal cord were documented, and in 3%, portal-systemic encephalopathy was noted. Multiple lesions were frequent. HHT should be considered a generalized vascular dysplasia (universal or systemic angiomatosis), and not simply a benign mucocutaneous disease.

Aged

Syphilitic meningomyelitis. A case report.

A 58-year-old man was admitted with a progressive, subacute asymmetrical paraparesis. The patient denied a previous syphilitic infection, but spinal fluid examination disclosed a lymphocytic pleocytosis, hypoglycorrhachia, and a positive serologic test for syphills. Clinical improvement resulted from a 14-day course of penicillin. Syphilitic involvement of the nervous system appears to be increasing in the United States and should be included in the differential diagnosis of progressive paraparesis.

Humans

Dementia.

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Dementia