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Biomedical subjects

D W Mulder

Publications and source records attributed to D W Mulder.

At least 55 records · Page 3Linked to original sources

Familial adult motor neuron disease: amyotrophic lateral sclerosis.

We analyzed the medical records of 103 patients with familial adult motor neuron disease (MND). In the 72 families, 329 members were known to be affected. Observations were compared with the sporadic and Mariana forms of MND. Clinical and laboratory examinations of all three forms were similar in clinical course and findings, but there were minor variations in age at onset, sex ratio, survival, and the frequency with which onset occurred in the lower extremities. Recognition of the familial form still depends on diagnosis of the disease in more than one member of a family.

Adult↗

Guillain-Barré syndrome. Clinicoepidemiologic features and effect of influenza vaccine.

A study of the epidemiologic and clinical features of Guillain-Barré syndrome in the population of Olmsted County, Minnesota, over the 46-year period 1935 through 1980 was conducted through the centralized diagnostic index maintained at Mayo Clinic, Rochester, Minn. A total of 48 cases were identified, giving an age- and sex-adjusted incidence of 1.8 per 100,000 person-years. The rate increased over time from 1.2 in the interval 1935 through 1956 to 2.4 in the interval 1970 through 1980. Males were affected more than females (age-adjusted rates of 2.3 and 1.2, respectively). The rate increased with age from 0.8 in those under 18 years old to 3.2 for those 60 years and older. Antecedent infectious diseases were reported in 65% of the cases. Implications with regard to the incidence of Guillain-Barré syndrome associated with the A/New Jersey/76 (swine flu) vaccine are discussed.

Adolescent↗

Brachial plexus neuropathy in the population of Rochester, Minnesota, 1970-1981.

Brachial plexus neuropathy (BPN) is a clinical entity of unknown cause characterized by the acute or subacute onset of pain and weakness, with occasional atrophy of the arm muscles. Information on the incidence of the disease in a delineated population is lacking, as the data available on BPN have come essentially from case reports or selected series. Using the Mayo Clinic records-linkage system as the source of data, 579 clinical records were reviewed of Rochester, Minnesota, residents in which a diagnosis suggestive of BPN was reported for the period 1970 through 1981. Eleven cases fulfilled all criteria, providing an overall annual incidence rate of 1.64 cases per 100,000 population. An infectious disease and/or tetanus toxoid immunization preceded the onset of BPN in 4 cases. The upper brachial plexus was involved in 6 cases, the lower brachial plexus in 2, and the whole plexus in 3; in 1 case there was bilateral BPN. The neuropathy ran a mild to moderate course in 10 cases, and complete recovery was recorded in 6, with slight residua in the others. The occurrence of antecedent events and the features of the disease are supportive of the concept of an immune-mediated process.

Adult↗

Lack of association of A/NJ/76 (swine flu) vaccine and polymyositis.

There is no indication of an increase in the number of cases of polymyositis/dermatomyositis observed and reported among the 43.3 million civilians who received the A/NJ/76 (swine flu) vaccine from October 1 to December 16, 1976, in the United States. Among nearly 1 million Army and Navy personnel who received the vaccine, there was no increase in the incidence rate of polymyositis/dermatomyositis. Furthermore, there was no indication of an increase in the number of cases of polymyositis/dermatomyositis diagnosed at the Mayo Clinic, the Cleveland Clinic, the Cleveland Metropolitan Hospital or the Massachusetts General Hospital during or following the national immunization program in 1976.

Dermatomyositis↗

Encephalitis and aseptic meningitis, Olmsted County, Minnesota, 1950-1981: I. Epidemiology.

All cases fulfilling stated criteria for encephalitis and aseptic meningitis in Olmsted County, Minnesota, for the period 1950 through 1981 were identified. This is, to our knowledge, the first such incidence and trend study in a delineated population, providing rates per 100,000 person-years of 7.4 for encephalitis (189 cases) and 10.9 for aseptic meningitis (283 cases). These are about twelve and six times higher, respectively, than the rates reported by the Centers for Disease Control. The rates have been stable over successive 5- or 10-year periods except for a recent increase in aseptic meningitis. Both conditions were more common in the summer months, in childhood, and among males. Viral identification using conventional laboratory tests has improved with time; in the period 1970 through 1981, virus type was specified in about one-fourth of the cases. The most common agents identified were California and mumps viruses in encephalitis, and entero and mumps viruses in aseptic meningitis. Antecedent and/or concurrent infections were noted in 42 and 35% of encephalitis and aseptic meningitis cases, respectively. No case due to mumps, measles, or rubella viruses has occurred since 1972, reflecting the impact of immunizations. Recovery was reported at the end of the acute phase in 95% of patients with aseptic meningitis, and there were no deaths. Seventy-eight percent of encephalitis patients recovered completely; the case fatality rate was 3.8%. Of the encephalitis cases, 2% were diagnosed initially postmortem.

Adolescent↗

Rehabilitative operation for motor neuron disease: tendon transfer for segmental muscular atrophy of the upper extremities.

Segmental spinal muscular atrophy of adolescence is a clinical syndrome that can be distinguished from the more common forms of motor neuron disease. A patient with this syndrome who was no longer able to care for herself is described. After selective tendon transfers in her right upper extremity, she has been able to perform many of the activities of daily living. This improved function has been maintained for more than 2 years. We suggest that transfer of tendons and muscles may be indicated in selected patients with slowly progressive muscular atrophy.

Activities of Daily Living↗

Fine structural study of neurofibrillary changes in a family with amyotrophic lateral sclerosis.

Lewy body-like hyaline inclusions in the soma and swollen, cord-like cell processes are characteristic alterations of the anterior horn cells in familial amyotrophic lateral sclerosis (ALS) with posterior column and spinocerebellar tract involvement. A fine structural analysis of these two structures has been performed in two brothers from a family ("C" family) previously described by Kurland and Mulder in 1955. The perikaryal hyaline inclusions consisted of accumulations of randomly oriented neurofilaments interspersed with thick linear densities associated with granular material. Some of the accumulations showed a central condensation. Cord-like, swollen neuronal processes were composed, for the most part, of numerous neurofilaments arranged parallel to the long axes. Dense structures were sometimes observed within the large bundles of filaments. They were composed of ill-defined dense, granular and fibrillar material associated with scattered vesicles and mitochondria. These dense areas were sometimes surrounded by various amounts of fine filaments, approximately 5 nm in diameter.

Amyotrophic Lateral Sclerosis↗

ALS and heavy metals: a pilot case-control study.

We conducted a case-control study of suspected risk factors for ALS among referral patients seen at the Mayo Clinic. Responses to questions about demographic factors, diet, medical history, travel, infectious disease, and other variables were similar in ALS patients and controls. In all but a few items, the responses of the cases and controls did not differ significantly. A significant level of shared exposure to an exogenous agent or environmental insult among cases was noted mainly for heavy metals.

Adult↗

Familial spastic paraplegia with Kallmann's syndrome.

A sibship is reported in which two males have spastic paraparesis and Kallmann's syndrome (hypogonadotrophic hypogonadism and anosmia). One of the brothers also is color blind. The association of familial spastic paraplegia and Kallmann's syndrome has not been described previously.

Adult↗

Motor neuron disease (ALS): evaluation of detection thresholds of cutaneous sensation.

Cutaneous touch-pressure, vibration, and thermal cooling detection thresholds were estimated on the skin of one foot for 80 patients with motor neuron disease (MND) and compared with that of more than 300 site, age, and sex-matched healthy controls. Fourteen of the patients had elevated thresholds (greater than or equal to 95th percentile): 7 showed elevated thresholds of sensitive points, 5 an increased number of insensitive grid points, and 2 a combination of these criteria. Therefore, raised vibration thresholds occur more frequently in MND patients than in controls (p less than 0.001). A detection threshold abnormality was not demonstrated for touch-pressure or for thermal cooling. These results are in keeping with the demonstrated increased cutaneous myelinated fiber degeneration in this condition and with evidence of sensory neural system involvement at postmortem examination. Although motor neurons are selectively vulnerable in MND, afferent neurons may also be affected, albeit less frequently and/or to a lesser degree.

Adult↗

Tongue force in normals and in dysarthric patients with amyotrophic lateral sclerosis.

Tongue force, rate of syllable repetition, and judgments of articulatory defectiveness were measures obtained on 19 dysarthric adults with amyotrophic lateral sclerosis and on 125 normal adults. Anterior and lateral tongue forces were measured by means of a pressure transducer clasped between the teeth; the tongue forces were recorded on a pen-writing ECG apparatus. Audio-recorded syllable repetitions of /p lambda/, /t lambda/, and /k lambda/ also were transcribed on ECG paper and counted. Three listeners rated articulatory precision on a 7-point scale of severity. The normal males had significantly higher tongue forces than normal females; normal subjects had significantly higher tongue forces than dysarthric patients; and anterior tongue forces were significantly greater than lateral in normal and dysarthric patients. There was a high negative correlation between tongue force and severity of articulatory defect. Syllable repetitions were significantly slower in the dysarthric patients than in the normal patients, and a high negative correlation was obtained between syllabic rate and severity of articulatory defect.

Adult↗

ALS in Rochester, Minnesota, 1925-1977.

The incidence, trend, and survivorship of ALS in the population of Rochester, Minnesota, was determined for the years 1925 through 1977. The average annual incidence was 1.76 per 100,000 population. There was a small but nonsignificant increase in the rate during the 53-year period. The male:female ratio was 1.1, and the male:female ratio of incidence rates was 1.6. The median age at onset was 66 years; the incidence rates increased with increasing age. Median survivorship was 22.5 months, and was longer for younger patients than for patients with an advanced age at onset. The demographic characteristics in this total community experience are believed to reflect the pattern of ALS more accurately than previously reported clinical series.

Age Factors↗

Is there a characteristic personality profile in amyotrophic lateral sclerosis? A Minnesota Multiphasic Personality Inventory study.

A comparison of the Minnesota Multiphasic Personality Inventory profiles of 21 men and 17 women who had amyotrophic lateral sclerosis (ALS) with the profiles of 50,500 general medical patients showed no marked deviations. The validating scales of the men and the women were similar to those of the general medical population. There was no evidence of increased defensiveness in the group with ALS, as measured by either the L or the K scale, when compared with the general medical population. Individual patient interviews also showed no characteristic personality profile for ALS patients.

Adult↗

Guillain-Barré syndrome: a 42-year epidemiologic and clinical study.

The central medical-records linkage system at the Mayo Clinic for the population of Olmsted County, Minnesota, provided the necessary data resource for the identification and follow-up of virtually all diagnosed cases of the Guillain--Barré syndrome over a period of 42 years. During this time, a total of 40 Olmsted County residents had clinical signs and symptoms of the disease. The mean annual incidence rate was 1.7 per 100,000 population. There was a significantly higher incidence rate (P less than 0.01) among patients 40 years of age and older. In four patients malignancies developed; however, the occurrence of the Guillain-Barré syndrome had no significant effect on survivorship in these patients. A case-control comparison of suggested etiologic factors supports the view that there is a significant association between antecedent infections and the Guillain-Barré syndrome.

Adolescent↗

Rehabilitation techniques for patients with amyotrophic lateral sclerosis.

Rehabilitation techniques for the management of patients with amyotrophic lateral sclerosis are aimed at maintaining the patients at their optimum functional levels for as long as possible and at preventing complications secondary to disuse of muscles and immobilization. A functional staging of patients is proposed which is helpful in categorizing them in reference to the type of rehabilitative techniques required and the kinds of assistive devices needed.

Amyotrophic Lateral Sclerosis↗

Myasthenia Gravis.

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Antigen-Antibody Complex↗