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Biomedical subjects

B A Kakulas

Publications and source records attributed to B A Kakulas.

At least 91 records · Page 5Linked to original sources

Needle muscle biopsy: will it make open biopsy obsolete?

Seventy-five needle muscle biopsies have been performed in this department over the past two years. Adequate biopsies were obtained in 69 cases. In 40 cases a variety of neuromuscular conditions was seen, broadly categorised as necrotizing myopathy (10), neurogenic atrophy (7), metabolic myopathy (5), vasculitis (2), normal muscle (7), and non-specific changes (9). Twenty-nine cases were for muscular dystrophy carrier detection. There were no complications associated with the procedure. The technique is simple and quick, and can be performed on outpatients or in the ward. Needle biopsy is the method of choice for sampling skeletal muscle in most patients, although open biopsy is still indicated for certain conditions.

Adolescent↗

Multifocal progressive neurological disorder caused by neoplastic angioendotheliosis.

The case of a 46-year-old woman who suffered a multifocal neurological illness, which rapidly progressed to dementia, is reported; the clinical signs were suggestive of multiple sclerosis. At necropsy, polyfocal cerebral white-matter lesions, associated with intravascular plugs of neoplastic cells which appeared to arise from the vascular endothelium, were found. The findings were typical of a rare form of malignant disease of the vascular endothelium known as neoplastic angioendotheliosis, which shows a striking predilection for the central nervous system.

Brain↗

Polymyositis presenting with respiratory failure.

A 36-year-old woman presented with a 3-month history of increasing dyspnoea, culminating in respiratory failure due to paralysis of respiratory muscles. She required assisted ventilation. Subsequently, mild limb and neck weakness was noted. Muscle biopsy of the right vastus lateralis showed polymyositis. The patient died suddenly and the postmortem revealed polymyositis with predominant involvement of the diaphragm and intercostal muscles.

Adult↗

Pathology of spinal injuries.

Information about the neuropathology of spinal cord injury is derived from the personal study of 341 specimens; 225 of these were traumatic, including 123 with massive injuries. Thirty-one were associated with metastases, 38 were degenerative, and 6 were infectious. Included are 41 normal controls. The hyperacute human spinal injury study provides a reference base for animal experiments. A sound knowledge of the pathology of spinal injury is an essential prerequisite for the competent management of patients with these injuries. Because no lesions were found that would be amenable to surgical correction, the study supports the conservative approach, in keeping with the teaching and practice of Sir Ludwig Guttmann and Sir George Bedbrook. In most specimens traumatic necrosis was most severe in the central gray matter and adjoining posterior columns of the cord. Preserved continuity of a proportion of the lateral, anterior, and posterior white matter was usual. Space-taking subdural or extradural hemorrhages and hematomyelia were rare. In patients who survived for more than a few weeks, posttraumatic cysts resulted from removal of necrotic parenchyma by macrophages. Although in very severe injuries complete disruption of both bony and spinal cord tissues was observed, others with equally massive injuries showed some continuity of the spinal cord parenchyma. This somewhat unexpected observation is in accord with physiologic studies in which poly EMG and sensory-evoked potentials demonstrate continuity of long tracts across the lesion in patients who were otherwise clinically complete. Regeneration of nerve roots and to a lesser extent of central axons was evident in patients who survived for more than 5 or 6 months. Complications consisted of ascending or descending necrosis and enlarging cavities. There is clinical and physiologic evidence of remodeling of reflex systems in the spinal patient that manifests as a changing neurologic picture. It is possible that the use of a variety of techniques, such as electrical stimulation, would influence such plastic changes to the benefit of the patient. Little detailed anatomic information is available on this topic as a key area for future investigation.

Axons↗

Response to ionising radiation in Duchenne muscular dystrophy.

Using lymphoblastoid cell cultures the response to gamma (gamma)-radiation, was examined in 6 Duchenne muscular dystrophy (DMD) patients; 2 clinically normal males as negative controls, and 2 patients with ataxia telangiectasia (AT) showing sensitivity to ionising radiation as positive controls. In a series of experiments, cell recovery and growth at day 2 post radiation, was determined after 5 separate gamma-irradiation dose levels: 50, 100, 150, 200 and 300 rads. The DMD cell strains showed a radiation dose response that was significantly greater than in cells from 2 normal males, while both DMD and normal cells were significantly less responsive than were AT-sensitive cell strains.

Adolescent↗

Fifty years survival after cervical fracture and fusion.

A patient who survived for 50 years after two cervical injuries is described. Among other effects he showed remarkable compensatory changes in the vertebrae above and below a cervical fusion operation. He also developed a meningeal pseudocyst following lumbar laminectomy. The history and pathological findings are considered to be instructive.

Aged↗

Vertebral metastases and spinal cord compression.

Clinical interest in spinal compression and resultant paraplegia due to metastases has mounted in recent years. This has stimulated attention to the neuropathology of the condition. Fourteen cases of spinal cord compression due to vertebral metastases are compared with over 100 traumatic cases. In the traumatic lesions there is central haemorrhagic necrosis leading to cavitation and gliosis with nerve root regeneration in the late stages. In the metastatic cases, lesions are often peripheral, pie-shaped and are related to vascular factors. The neuropathology of cord necrosis due to metastatic spinal disease is therefore different to trauma. These observations have clinical importance in planning treatment.

Adult↗

Muscle lipids in Duchenne muscular dystrophy.

The lipids of muscle and adipose tissue from normal males and of muscle from males with Duchenne muscular dystrophy were investigated. Triglyceride, the major neutral lipid, showed similar fatty acid compositions in all tissues examined. When the phospholipids of dystrophic muscle and of normal adipose tissue were compared with those of normal muscle, it was found that there was an increase in the proportion of sphingomyelin in dystrophic muscle, while adipose tissue had higher proportions of sphingomyelin and lysophosphatidylcholine but lower choline phosphoglyceride. In dystrophic muscle only small alterations from normal were observed in the fatty acid compositions of the individual phospholipids, whereas the phospholipids of adipose tissue had quite distinctive fatty acid compositions. An atrophic muscle sample resulting from poliomyelitis consisted almost entirely of connective tissue and fat and had a phospholipid composition similar to that of adipose tissue. From a comparison of the results for all the types of tissue studied, it is evident that the increase in sphingomyelin in dystrophic muscle biopsies and the changes in the fatty acid compositions of individual phospholipids may be accounted for by the increased amounts of fat and connective tissue which are present in dystrophic muscle samples. In a case each of polymyositis, limb girdle muscular dystrophy and an autosomal recessive form of muscular dystrophy, the results obtained for the phospholipid composition of the muscle sample were also normal or consistent with some contamination from fat and connective tissue.

Adipose Tissue↗

Neuropathology of the cortical lesions of the Parkinsonian-dementia (PD) complex of Guam.

The Guam amyotrophic lateral sclerosis and Parkinsonian-dementia (ALS and PD) complex still presents a challenge to research. Previous papers have highlighted the lesions in the hippocampus and basal nuclei to explain the bizarre clinical syndrome. In our study of 20 to 62 cases of PD with or without ALS, significant widespread and often severe lesions were found throughout the entire cerebral cortex. These changes included variable degrees of nerve cell loss, spongy change, gliosis, increased lipofuscin content of nerve cells, the presence of granulovacuolar bodies and neurofibrillary tangles within neurones and, less frequently, Hirano bodies. The lesions show a characteristic topographical distribution. The global involvement of the cerebral cortex correlated well with the loss of higher mental function which was present in the patients.

Adult↗

An experimental study of early ultrastructural changes in the injured spinal cord.

Experimental hemisection injury of rat spinal cord was undertaken in order to examine Wallerian degeneration of ascending and descending tracts, and alterations in neurons and synapses. By light microscopy degeneration of axons was recognized first at 72 hours post-operatively. On the other hand, EM study revealed axonal degeneration 24 hours post-operatively. There were several ultrastructural changes in the early stages after hemisection injury. Increase in number of lysosomes in neurones was believed to be related to "autolysis" of the injured spinal cord. Another noteworthy finding was the "separation" of boutons. The mechanisms considered to account for these findings were deafferentation and oedema.

Afferent Pathways↗

Multiple myeloma with bilateral orbital infiltration and polyneuropathy.

Orbital involvement is a rare complication of myeloma usually presenting as unilateral proptosis without pain. A case is reported of a woman with bilateral orbital infiltration without proptosis, who became blind within three months of the onset diplopia. The blindness was due to visual field obstruction, with eyelids covering both corneas; the eyes themselves remained normal. This is the first case of orbital myeloma reported in Australia and only the second case of bilateral primary orbital infiltration with myelomatosis to be reported in the world literature.

Blindness↗

Skeletal muscle lipids in normal and dystrophic mice.

The lipids of muscle from normal and dystrophic mice of the Bar Harbor strain C57BL/6J were investigated with particular reference to the fatty acid composition of the neutral lipids and phospholipids. Triglyceride, which together with cholesterol comprised most of the neutral lipid fraction, showed no significant changes in fatty acid composition. There was no significant change in the amount of total phospholipid phosphorus in dystrophic muscle, but analyses of the levels of individual phospholipids showed a decrease of 9% in phosphatidylcholine. In both phosphatidylcholine and ethanolamine phosphoglyceride, large decreases were observed in the proportion of the fatty acid 16:0, while 18:0 and 18:1 increased significantly. In phosphatidylcholine 18:2 also increased, and a decrease occurred in 22:6 in ethanolamine phosphoglyceride. Sphingomyelin showed increases in 16:0, 23:0 and 24:1 and decreases in 18:0 and 18:1. The other phospholipid fractions showed less remarkable changes, with a significant increase in 18:0 in lysophosphatidylcholine and a decrease in 16:0 in cardiolipin.

Animals↗

Vertebral metastases and spinal cord compression.

Clinical interest in spinal compression and resultant paraplegia due to metastases has mounted in recent years. This has stimulated attention to the neuropathology of the condition. 14 cases of spinal cord compression due to vertebral metastases are compared with over 100 traumatic cases. In the traumatic lesions there is central haemorrhagic necrosis leading to cavitation and gliosis with nerve root regeneration in the late stages. In the metastatic cases, lesions are often peripheral, pie-shaped and are related to vascular factors. The neuropathology of cord necrosis due to metastatic spinal disease is therefore different from that caused by trauma. These observations have clinical importance in planning treatment.

Adult↗

Experimental kuru in the gibbon and sooty mangabey and Creutzfeldt-Jakob disease in the pigtailed macaque. With a summary of the host range of the subacute spongiform virus encephalopathies.

The experimental host range for the slow virus infections causing subacute spongiform virus encephalopathies is enlarged in primates to include the gibbon for kuru, the pigtailed macaque for Creutzfeldt-Jakob disease, and the sooty mangabey for both diseases. The report is based on neuropathological evidence of the diseases in animals with preclinical lesions. A table lists all the species to which the subacute spongiform virus encephalopathies have been transmitted.

Animals↗

The effect of EDTA on the histochemical myofibrillar ATPase reaction.

The effects of different salts and concentrations of ethylene diamine tetraacetic acid (EDTA) on the histochemically demonstrable myofibrillar ATPase reaction have been investigated. Serial frozen sections from the vastus lateralis muscle of rats, rabbits and guinea pigs were treated with cold (4 degrees) dipotassium EDTA solution and then incubated for myofibrillar ATPase activity. The results showed that all type I muscle fibres exhibited enhanced ATPase activity while type II fibres demonstrated either very low, moderate or intense reaction suggesting the existence of three different sub-populations. None of these effects was observed if the sections were immersed in magnesium chloride solution after preincubation in cold diK+ EDTA. Preincubation in cold non-ionic EDTA, disodium or tetrasodium EDTA did not affect the regular ATPase reaction pattern, while preincubation in warm (19 degrees--21 degrees C) solutions of the same salts resulted in reversal of the regular pattern of ATPase activity. The present histochemical data emphasise the importance of K+, Mg++ and temperature during preincubation of muscle fibres in EDTA before the demonstration of myofibrillar ATPase activity. Moreover, preincubation in a cold solution of diK+ EDTA may be valuable in the histochemical detection of distinct sub-populations of type II muscle fibres and certainly distinguished between type II white and type II red.

Animals↗