Anomalous fusion between the lunate and triquetrum.
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Biomedical subjects
Publications and source records attributed to J Muller.
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A slowly progressive autosomal dominant neuromuscular disease--termed spheroid body myopathy--is described in four successive generations and documented by muscle biopsies in five patients of two generations. With an onset in adolescence, the disease proceeds to some motor incapacitation, but life span is apparently not shortened. The salient morphologic feature is the presence of spheroid bodies, chiefly occurring in type 1 myofibers. Ultrastructurally, these spheroid bodies are composed of tiny filaments but are devoid of organelles; in some cases they resemble cytoplasmic bodies. "Smearing in the 1-band" is a frequent and early finding. At a later age, signs of denervation are also present, both clinically and in muscle biopsies. The clinical and morphologic features justify the designation of this neuromuscular condition as a distinct entity.
Phenylpropanolamine (PPA) is a drug that has been associated with serious side effects including stroke. It is often combined with caffeine in diet preparations and "look-alike" pills. In order to determine if PPA/caffeine can lead to stroke in normotensive and/or hypertensive rats, we administered the combination in six times the allowed human dose calculated on a per weight basis for the rats two times per day for five days. Subarachnoid and cerebral hemorrhage was noted in 18% of the hypertensive rats. A single PPA/caffeine administration (same dose) lead to acute hypertension in both the normotensive and hypertensive animals. These results suggest that PPA/caffeine can lead to cerebral hemorrhage in previously hypertensive animals when administered in greater than the allowed dosage. An acute elevation in blood pressure may be a contributing factor.
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CNS neoplastic angioendothelosis is a treatable primary proliferative disorder of the endothelial cells of blood vessels characterized by a clinical neurological picture of multiple infarct dementia and an inordinate amount of local cerebral edema, so striking that it may simulate primary or metastatic central nervous system tumor. The malignant cells remain within the lumen of the vessels and rarely if ever metastasize or occur in peripheral blood. There is remarkable improvement in symptoms by treating with high dose steroids. Antimetabolites and irradiation are suggested means of additional treatment.
Experimental hydromyelia and syringomyelia, induced by kaolin in dogs, has been investigated by scanning electron microscopy. This study demonstrated that communication between the 4th ventricle and the spinal central canal is normally occluded by a plug of acellular material lying within the central canal. This plug is broken down following the onset of hydrocephalus and communication is rapidly established between the ventricles and the central canal. The canal distends and ruptures dorsally into the spinal gray matter with the development of extensive intramedullary cavities, lined by glial and neuronal elements. The morphological changes demonstrated are consistent with a physical destructive process of the cord resulting from CSF dissection.
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