Biomedical subjects

H D Müller

Publications and source records attributed to H D Müller.

12 recordsLinked to original sources

PubMed2003

Inflammatory infiltrates in the spinal cord of patients with Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is defined as an acute inflammatory demyelinating polyradiculoneuropathy (AIDP) of the peripheral nervous system. Reports on central nervous system involvement in patients with GBS are rare and the histopathological analysis was usually restricted to conventional staining techniques. We were able to investigate four cases with GBS at autopsy in respect to the inflammatory infiltrates and histopathological changes in the spinal cord by immunohistochemistry using a panel of antibodies recognizing lymphocytes and different macrophage-activation antigens. There were increased inflammatory cell infiltrates comprising lymphocytes and macrophages in the spinal cord of two cases. In one of these two cases, GBS predominantly affecting the motor system similar to acute motor axonal neuropathy (AMAN) developed following hepatitis B vaccination; in the second one, GBS developed rapidly 4 days after onset of intravenous purified GM1-ganglioside application affecting the motor as well as the sensory system, resembling acute motor sensory axonal neuropathy (AMSAN). Impairment of the spinal anterior horn cells with their axons was suggested to be responsible for prolonged motor symptoms and the predominantly axonal type of neuropathy at least as a late-stage feature in these two cases with fatal outcome. Insignificant cellular infiltrates in the spinal cord were noted in the other two GBS cases. Focal cellular infiltration of spinal nerve roots and meninges was similar in all cases.

Adult↗

PubMed2001

Dominantly inherited myopathy with novel tubular aggregates containing 1-21 tubulofilamentous structures.

Tubular aggregates (TAs) in skeletal muscle fibers have been observed as a nonspecific finding in a number of different conditions such as periodic paralysis, myotonic disorders, hyperaldosteronism, chronic use of drugs, and alcoholism. However, TAs were also found more specifically in well-defined muscle disorders, e.g., exercise-induced cramps, myasthenic syndromes, and even in dominantly or recessively inherited familial myopathies. We report on a presumably dominantly inherited familial myopathy with late onset characterized morphologically by the presence of three types of TAs in type II muscle fibers identified in three affected members of one family (a 86-year-old man and his two sons). The first, novel type was characterized by tubules, 30-200 nm in thickness which included 1-21 tubulofilamentous structures 14-18 nm in diameter. The second type of TAs corresponded to previously well-described tubules and were derived from terminal cisternae, which were rather irregularly arranged or widened, and filled with material of medium electron density. The third type of TAs were occasional, hexagonally arranged TAs of the usual type [type Ib and Ic]. Rare annulate lamellae were also seen. Our findings support the evidence of tubular aggregates as the major finding in certain dominantly inherited myopathies. Tubules of the first type, to the best of our knowledge, have not been recorded in any other myopathy. It is therefore suggested that these tubules characterize a novel type of a benign, slowly progressive myopathy with late onset, muscle pain, cramps, and stiffness.

Adult↗

PubMed2000

Hereditary motor and sensory neuropathy with absence of large myelinated fibers due to absence of large neurons in dorsal root ganglia and anterior horns, clinically associated with deafness, mental retardation, and epilepsy (HMSN-ADM).

Hereditary motor and sensory neuropathy (HMSN) with autosomal recessive inheritance represents a genetically heterogeneous group of disorders with variable clinical, pathologic and electrophysiologic manifestations. A new variant of autosomal recessive HMSN, clinically defined by sensorimotor polyneuropathy associated with deafness and mental retardation, has recently been described. We report on the first autopsy case with this type of HMSN: a girl of non-consanguineous parents with a presumably autosomal recessive type of motor and sensory neuropathy clinically associated with deafness, mental retardation, and epilepsy. The autopsy showed complete absence of large myelinated fibers in peripheral motor and sensory nerves corresponding to a lack of large neurons in dorsal root ganglia and anterior horns of the spinal cord, moderate neurogenic muscle atrophy, and nearly complete absence of neurons in the dentate nucleus of the cerebellum. Molecular genetic analyses in our case revealed neither genetic alterations in the survival motor neuron gene nor in the PMP-22 gene.

Anterior Horn Cells↗

PubMed1985

[The chemical composition of "zymosans" from yeasts].

The chemical composition of "Zymosanes" (complex cell-wall-polysaccharides), prepared by different methods from Baker's yeast, were analyzed by classical and modern techniques, e.g. GLC and TLC. They are very complex natural products consisting of a basic structure of polysaccharides, which proteins and different lipids are bound to. Higher purified zymosanes seem to be free from nucleic acids and do not contain sialic acid.

Amino Acids↗

PubMed1979

[Pilar tumor of the scalp. Case report and study of 59 cases].

The pilar tumor occurs mainly in elderly women. It is a solitary lesion, localized on the scalp in most cases. In spite of the usually long course of the disease, pilar tumors very rarely metastasize. Small tumors look like "sebaceous cysts", large and very large tumors are diagnosed as squamous cell carcinomas. In these cases one could directly come to the right diagnosis if sex, age and localization do correspond. Microscopic examination reveals interlacing lobules of squamous eqithelium that undergo in their center abrupt keratinization of pilar or trichilemmal cysts. The pilar tumor, therefore, keratinizes like the middle portion of the outer root sheath. Our own case shows the typical above-mentioned clinical and histological signs of the pilar tumor.

Carcinoma, Squamous Cell↗

PubMed1978

[Morphea resembling pseudoxanthoma elasticum in progressive scleroderma].

Lesions of circumscribed scleroderma clinically appeared as pseudoxanthoma elasticum. The histological structure, however, revealed circumscribed scleroderma. The sclerodactyly, Raynaud's phenomenon, acrosclerosis and the laboratory results suggested systemic sclerosis.

Adult↗

PubMed1977