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Biomedical subjects

J L Teepen

Publications and source records attributed to J L Teepen.

At least 19 recordsLinked to original sources

Critical illness polyneuropathy and myopathy (CIPNM): evidence for local immune activation by cytokine-expression in the muscle tissue.

In a longitudinal prospective study a muscle biopsy was taken from 30/32 (33%) of the 98 patients who developed critical illness polyneuropathy and myopathy (CIPNM). Neuropathic changes were found in 37%, myopathic in 40%, and a combination in 23% of the biopsies. The immunohistopathology showed macrophages and Th-cells in 40% and 60% of the muscle biopsies respectively. Small mainly perivascular infiltrates contained macrophages and Th-cells. ICAM-1, VCAM and MAC were found on the vascular endothelium in 58%, 53% and 79% respectively. In all biopsies there was an upregulation of both HLA-I and HLA-DR. Proinflammatory cytokines and TNFalphaR75 were also produced locally (IL-1beta in 71%, IFN-gamma in 40%, IL-12 in 73%, TNFalphaR75 in 90%). The anti-inflammatory cytokine IL-10 was simultaneously expressed in 96% of the biopsies. HLA-DR, TNFalphaR75 and IL-10 differed significantly when compared with control muscle biopsies. Our data provide evidence that small numbers of activated leukocytes producing both pro- and anti-inflammatory cytokines infiltrate skeletal muscle of CIPNM patients. We propose that the local balance of leukocyte activities is of importance in the pathophysiology of muscle weakness in CIPNM.

Antigens, CD↗

[The indispensable anamnesis: in-vitro fertilization in a woman under treatment for melanoma].

A couple consulted the gynaecologist repeatedly because of a primary fertility disorder. When they had desired children for 8 years, no pregnancy had yet occurred. In-vitro fertilization (IVF) treatment was started. A few days after the beginning of the hormonal treatment that precedes the IVF procedure, the woman noted a swelling in the right inguinal area for which she consulted the surgeon. This swelling was found to be a metastasis of a melanoma that had been removed from her leg 6 months previously. The gynaecologist was not aware of this case history and the surgeon was not informed of the started IVF treatment. As was found later, this was in accordance with the couple's wishes. The first IVF treatment resulted in a pregnancy that ended with the birth of a healthy boy. The placenta showed melanomatous metastases and the mother died 2 months after the birth of her son.

Adult↗

Thrombosis of the deep cerebral veins: CT and MRI findings with pathologic correlation.

Deep cerebral vein thrombosis can present with acute, severe neurological symptoms and may be rapidly fatal as in the 20-year-old woman reported here. Although MRI is superior for establishing the diagnosis, CT is usually the first examination performed in the clinical setting. It is therefore important to recognise certain indicators such as extensive bithalamic low density. These and certain other less specific signs are correlated with the MRI and autopsy findings.

Adult↗

[Acute myopathy during treatment of status asthmaticus].

Two patients with chronic pulmonary disease, a woman of 38 and a man of 54 years old, who had developed a status asthmaticus, had difficulties being weaned from artificial ventilation. They suffered from an acute myopathy caused by a combination of high-dose corticosteroids and muscle relaxants (pancuronium, vecuronium). This acute myopathy is characterised by generalised flaccid quadriplegia with muscle atrophy and areflexia, difficulties being weaned from artificial ventilation, myoglobinuria and high levels of creatine kinase activity in serum. The prognosis is good; almost complete recovery occurs. Muscle biopsy may reveal necrotising myopathy and occasionally, selective loss of thick myofilaments.

Adult↗

[Apolipoprotein E polymorphism and Alzheimer disease].

At present a reliable and specific diagnostic test of Alzheimer's disease is not available. Thus far, diagnosis is based on clinical criteria despite their occasional inadequacy. Post mortem search for neuropathological hallmarks can establish the diagnosis with certainty. In the present case control study we performed an apoE genotyping for 21 patients, divided into an Alzheimer-positive and an Alzheimer-negative group after neuropathological search. As described in the literature, the apoE-epsilon 4 allele was overrepresented in the Alzheimer-positive group, while in the Alzheimer-negative group the apoE-epsilon 3 allele dominated. The epsilon 4 allele of the apoE gene may be considered as a biological risk factor for the development of Alzheimer's disease. Especially in geriatric patients with cognitive impairment, apoE genotyping seems to be a supplementary tool for risk assessment.

Aged↗

Primary leiomyosarcoma of the spine.

We present a 39-year-old man with tumour of the eighth thoracic vertebra, causing compression of the spinal cord. The tumour proved to be a primary leiomyosarcoma (LMS) of bone, an uncommon neoplasm; to our knowledge this is the first report of primary LMS in the spine. The lesion was documented by plain radiography, myelography, CT, MRI and digital subtraction angiography. These investigations did help to focus on the differential diagnosis and demonstrated the extent of the bony lesion, the findings were nonspecific, and the correct diagnosis was established by pathological examination.

Adult↗

The enigmatic origin of olfactory schwannoma.

We present a case of so-called olfactory schwannoma in a 17-year-old male. The diagnostic procedures included computed tomography and digital subtraction angiography. Histological examination with tumor markers, S100, Leu-7 (CD57) and EM showed the tumor tissue to be a schwannoma. Arguments are raised that olfactory schwannomas are comparable to or even occur as intracerebral schwannomas with frontal localisation.

Adolescent↗

A miniaturized cuff electrode for electrical stimulation of peripheral nerves in the freely moving rat.

A bipolar cuff electrode for electrical stimulation of small diameter peripheral nerves is described. The cuff is made of a highly flexible rubber-impression material, and the electrode assembly is suited for chronic implantation. Its manual construction is easy and reliable, utilizing only simple tools. The cuff completely envelopes nerves of varying diameter and requires a minimal amount of manipulations of the nerve, thereby reducing the chance of surgical trauma. The snug envelope prevents the nerve from drying, and minimizes shunting between the two leads by extracellular fluids. Small outer dimensions were achieved: 1.4 x 1.1 x 2.3 mm (width x height x length) when used with nerves of 1 mm diameter, which minimizes pressure and damage to surrounding tissues. Morphometric analysis of nerves enclosed in cuffs for 28-30 h revealed a small decrease in the number of large-diameter fibers. Stimulation thresholds remained, however, constant throughout the experiments.

Animals↗

Familial anaplastic ependymoma: evidence of loss of chromosome 22 in tumour cells.

A family with anaplastic ependymomas, histologically verified in three cases and neuroradiologically suggested in a fourth, is presented. Two healthy brothers both had two affected sons. All four male patients were younger than 5 years at the time of diagnosis. Two boys died before the age of 3 years. Genotype analysis (with polymorphic DNA markers for chromosome 22 and interphase cytogenetic analysis) of one of the tumours showed a subpopulation of tumour cells with monosomy of (part of) chromosome 22. Non-neoplastic cells of this patient showed a normal karyotype. These findings give further evidence for the role of a tumour suppressor gene on chromosome 22 in the pathogenesis of familial ependymal tumours.

Brain Neoplasms↗

Critical illness polyneuromyopathy after artificial respiration.

Up to now, 71 critically ill patients have been reported with neuromuscular complications after artificial respiration. The authors review the literature and present data of a personal series of 22 patients all suffering from severe flaccid tetraparesis and muscle atrophy, which developed after an average of two weeks artificial respiration. The prognosis was relatively good in those surviving the primary disease. The multiconditional causes are discussed with emphasis on the combination of polyneuropathy and myopathy. Tumor necrosis factor (TNF), a key mediator of sepsis, which also has an influence on muscle and nerves, is mentioned as a possible cause of this illness.

Adult↗

Pelizaeus-Merzbacher disease. The Löwenberg-Hill type.

The clinical and neuropathological findings are reported of two sibs with adult type PMD. Clinical features deviating from the usual pattern included: no psychosis, no measurable dementia, no dwarfism, no microcephaly, no (marked) involuntary movements, but conspicuous generalised muscle atrophy and denervation, impairment of vital and gnostic sensation, thoracolumbar vertebral anomalies, and aplasia of os coccygis. Neuropathological findings were as usual, with additional unusual features: pinhead-size areas of acute myelin-abbau products, involvement of grey in addition to white matter, and upon ultrastructure, the new finding of intra-oligodendroglial fingerprint bodies, both in neuronal satellite and in white matter oligoglia, but not in astrocytes, ganglion cells, or pericytes. This excludes the origin of the stored material in the lysosomes as to derive exclusively from demyelination and would possibly imply PMD to be an oligodendroglial lysosomal storage disease.

Adolescent↗