Search PubMed⌕ Search

Biomedical subjects

K Skullerud

Publications and source records attributed to K Skullerud.

At least 55 records · Page 3Linked to original sources

Spinal muscular atrophy type I combined with atrial septal defect in three sibs.

All three children of an unrelated Norwegian couple were born with spinal muscular atrophy (SMA I) as confirmed by autopsy in two of them. Two of the children died at birth, whereas one lived for 7 weeks on support systems that included artificial ventilation. All three children had large atrial septal defects (ASD). One had valvular aortic stenosis. Another had arrhinencephaly and transient pleural effusion which was evacuated during pregnancy. The findings may reflect concurrence of unrelated disorders caused by genetic or environmental factors, segregation of linked genes, pleiotropism or the existence of a previously unknown disease.

Abnormalities, Multiple↗

[Pathologic brain damage in male alcoholics dying outside of hospitals].

From 1984 to 1988, 195 male alcoholics aged 30-64 years who died outside hospitals and nursing homes in Oslo were autopsied at the Institute of Forensic Medicine, the National Hospital, Oslo. Of the 127 brains neuropathologically examined, 86 (67.7%) showed abnormalities, and 28 contained lesions of more than one type. One or two lesions associated with alcoholism were found in 61 cases (48%). Thus, 18 (14.2%) showed Wernicke's encephalopathy, 47 (37%) cerebellar atrophy, two central pontine myelinolysis, and one hepatic encephalopathy. Subdural haematoma and/or cortical contusions were found in 30 (23.6%), and cerebrovascular lesions in 19 (15%). Of the 195 cases, 22 had a history of repeated epileptic seizures. Nineteen of them were examined neuropathologically, and 13 had focal damage that might have been responsible for their fits. The results indicate that the frequency of Wernicke's encephalopathy and cerebellar atrophy in male alcoholics who die outside hospital is similar to that previously observed in cases who died in hospital. Although cerebral damage was even more frequent among vagrants and others dependent on social support, half the men living in their own homes were also affected.

Adult↗

Clinicopathological study of germinal matrix hemorrhage, pontosubicular necrosis, and periventricular leukomalacia in stillborn.

The occurrence of germinal matrix hemorrhage (GMH), pontosubicular necrosis (PSN), and periventricular leukomalacia (PVL) was evaluated in 58 stillborn, among whom 23 (40%) showed one or more lesions. A comparative study of neuropathological findings and obstetrical histories in stillborn beyond 26 weeks of gestation showed a greater frequency of damage when the pregnancies had been complicated. The pathogenesis of PSN is disputed. As PSN was found in stillborn (13 cases), hyperoxia is not an essential pathogenetic factor. PSN may represent a form of anoxic encephalopathy. However, PSN was found as an isolated lesion in an infant of a diabetic mother and in most cases of intrauterine growth retardation; hypoglycemia may therefore be involved in the pathogenesis. Ultrasound examinations of living neonates have indicated the prenatal occurrence of GMH in some cases, and our study opens the possibility that there is intrauterine involvement of PVL and PSN as well. Consequently, cerebral lesions diagnosed in newborn may have occurred in utero.

Encephalomalacia↗

Frequency and prognostic significance of germinal matrix hemorrhage, periventricular leukomalacia, and pontosubicular necrosis in preterm neonates.

The occurrence of germinal matrix hemorrhage (GMH), periventricular leukomalacia (PVL), and pontosubicular necrosis (PSN) was evaluated in a material of 96 preterm infants. All cases were born at less than 38 weeks of gestation, and died within 30 days after birth. The frequency of GMH (50%) and PVL (24%) was within the range of previous observations, but the 59% occurrence of PSN argues against the assertion that intraventricular hemorrhage is the most common neuropathological finding in preterm neonates. However, different combinations of these injuries were found in more than half the cases affected. Of the 48 infants with GMH, 36 (75%) showed either PSN (19 cases), PVL (2 cases), or both lesions (15 cases), and the frequency of additional damage was related to the severity of hemorrhage. Thus, neonatal mortality may be more related to additional hypoxic/ischemic lesions than to the severity of hemorrhage per se. Clinical follow-up studies on subpopulations of preterm infants with and without GMH have shown no difference in frequency of mild and moderate psychomotoric deficiencies. The 35% occurrence of PSN as a solitary lesion in the 48 cases without GMH was similar to the frequency of PSN as a single additional lesion in 48 cases with GMH (40%). This finding makes PSN and not GMH the most likely cause of at least less severe handicaps.

Brain↗

Variations in the size of the human brain. Influence of age, sex, body length, body mass index, alcoholism, Alzheimer changes, and cerebral atherosclerosis.

The various factors which determine brain weight and volume of the lateral ventricles were studied in an autopsy material of 467 cases. The material consisted of 64 men and 17 women between 45-54 years and 196 men and 190 women between 70-79 years. The weights of the cerebral hemispheres and of the cerebellum and brainstem were determined separately. The volume of the lateral ventricles was determined by weighing the hemispheres with and without water in the lateral ventricles. The recorded variables were age, sex, body length, body weight, cerebral atherosclerosis, Alzheimer changes and alcoholism. Cerebral atherosclerosis and Alzheimer changes were quantitated by morphometric methods. The results were analysed by conventional and multivariate statistical methods. The following observations were made: In normal brains there was a significant correlation between the weight of the supra- and infratentorial parts. Similarly, there was a significant correlation between the size of the lateral ventricles and the weight of the cerebral hemispheres. Women had smaller brains than men even when the difference in body length was taken into account. The difference was approximately 110-115 g for the whole brain after correction for other variables. Women had also smaller lateral ventricles than men, but this difference was in proportion to the smaller size of their hemispheres. There was a physiologic decline in brain weight and a widening of the lateral ventricles with increasing age. This shrinkage probably started after the age of 55. There was a clear correlation between body length and brain weight. The estimated increase in brain weight was approximately 3 g per cm body length. There was a decreasing brain weight and an increasing ventricular size with a decreasing body mass index. This shows that emaciation leads to a decrease in brain size. Severe Alzheimer changes caused a statistically significant enlargement of the lateral ventricles both in men and women. There was a general trend for brain weight reduction in cases with severe Alzheimer changes but the decrease was statistically significant only in old women, and it could not be entirely excluded that the weight reduction in part was due to a concurrent emaciation rather than to the Alzheimer changes per se. In the majority of the cases, the Alzheimer changes were mild and had probably progressed slowly with age. A few cases had very severe changes.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

Pleomorphic xanthoastrocytoma: report of 5 cases.

Five cases of pleomorphic xanthoastrocytoma are described. The favorable prognosis of these tumors is confirmed. Thus, two patients had postoperative survival of 9 and 12 years, respectively, and three others are still alive and well, one of them 6 years after the operation. All the cases described until now (17 including the ones described here) have involved patients under 30 years of age. Most of the tumors have been located superficially in the brain with extensive adhesions to the meninges. Usually they have been well demarcated from the brain. Histologically, the tumors showed a marked cellular pleomorphism, including bizarre giant cells, but there were few mitoses and only a slight tendency to necrosis. The tumor tissue contained a dense network of reticulin fibers and many cells contained lipid vacuoles. Glial fibrillary acidic protein (GFAP) was demonstrated in the cytoplasm of the tumor cells.

Adolescent↗

Watershed infarcts in the brain caused by microemboli.

Multiple vascular occlusions are frequently found in the leptomeningeal arteries over watershed infarcts in the brain. These occlusions have largely been interpreted as thrombi secondary to slowing of the blood flow. This report suggests that most of the occlusions are microemboli, which may lodge preferentially in these areas, and that they are the cause of the infarcts rather than secondary events. These suggestions are based upon the analysis of three groups of patients. The first group consists of four cases, two of which had atheromatous masses and the other two, tumor emboli in the overlying leptomeningeal arteries. These cases prove beyond doubt that microemboli can lodge preferentially in the watershed areas and cause infarcts in the brain. The second group consists of the cases of watershed infarcts that were precipitated by hypotensive episodes. Only one of these showed occlusion of the overlying arteries, although all of them obviously had slowing of the blood flow during the acute phase. These cases thus discredit the concept that stagnation thrombosis is a frequent event. Finally, three cases with watershed infarcts and vascular occlusions interpreted as platelet microemboli are presented to demonstrate different pathogenetic mechanisms effective in the process of embolization.

Adenocarcinoma↗

The cerebral lesions in a patient with generalized glutathione deficiency and pyroglutamic aciduria (5-oxoprolinuria).

The clinical and pathologic features of a male patient with generalized glutathione deficiency and pyroglutamic aciduria are presented. The patient died at the age of 28 years. He was mentally retarded from infancy and developed progressive tremor, retardation of movement, and ataxia as from the age of 16. Neuropathologic examination of the brain disclosed a selective atrophy of the granule cell layer of the cerebellum and focal lesions in the visual cortex and the thalamus. The type and distribution of the lesions resembled those seen after mercury intoxication. However, in our patient the damage was probably caused by the lack of protection of glutathione against oxidative damage in the brain. Possible treatment of this rare metabolic disorder might include external supply of an antioxidant, e.g., a thiol capable of penetrating the blood brain barrier.

Adult↗

Rheumatoid arthritis of the lumbar spine leading to anterior vertebral subluxation and compression of the cauda equina.

A 71-year-old woman with rheumatoid arthritis suffered a spontaneous anterior subluxation of the fourth lumbar vertebra with compression of the cauda equina. The patient was operated upon with a decompressive lumbar laminectomy and a stabilizing posterior interbody fusion of L4 and L5. Histological examination of the unstable L4-L5 motor segment showed that the apophyseal and discovertebral joints had been destroyed by rheumatoid granulation tissue.

Aged↗

Encephalomyelopathy following intrathecal methotrexate treatment in a child with acute leukemia.

A previously undescribed type of encephalomyelopathy after prophylactic intrathecal methotrexate (MTX) treatment for acute leukemia is reported. The patient was treated systemically with vincristine, MTX, stereoids and mercaptopurine. Radiation therapy was not given. The neurologic symptoms started 24 hours after the completion of the fifth intrathecal MTX instillation and death ensued 18 days later. The lesions consisted of superficial and sharply circumscribed areas of incomplete necrosis with astrocytosis on the base of the brain and along the insula regions, around the foramina of Luschka, and over the superior and inferior colliculi. Similar superficial lesions were found over the surface of the cerebellum and along most of the surface of the spinal cord. Most of the affected areas in the brain were located under the large subarachnoid cisterns. In the cord the lesions were most pronounced along the entrance zones of the roots. There was also a severe loss of anterior horn cells. There were no signs of meningeal leukemia, no pathological changes were caused by the intrathecal MTX treatment.

Acute Disease↗