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Biomedical subjects

M Haltia

Publications and source records attributed to M Haltia.

At least 163 records · Page 9Linked to original sources
PubMed1984

Neurofilament subunit--related proteins in neuronal intranuclear inclusions.

Well-preserved nuclei were isolated from the brain of a patient with neuronal intranuclear inclusion disease ( NIID ). The inclusions in situ stained for proteins and, by immunohistochemical techniques, for the 200K and 68K components of the neurofilament protein triplet but were negative for the 145K neurofilament protein. At the electron microscopic level the inclusions were seen to consist of straight, randomly arranged tubular filaments 8.5 to 9.5 nm in diameter. They were resistant to extraction with hot buffer containing sodium dodecyl sulfate (SDS) and 2-mercaptoethanol, indicating that the proteins of NIID inclusions are probably cross-linked by covalent bonds other than disulfide. This feature is also exhibited by the paired helical filaments occurring in brain in Alzheimer's disease. No major differences were found in the polypeptide composition of nuclei in NIID and control nuclei resolved by SDS-polyacrylamide gel electrophoresis. The inclusions isolated from SDS-extracted nuclei were shown by immunohistochemical techniques to stain for all three neurofilament subunits, indicating that 145K -related antigenic sites were uncovered during the extraction procedure.

Aged↗
PubMed1984

Trilateral retinoblastoma. Pineoblastoma (ectopic intracranial retinoblastoma) associated with bilateral retinoblastoma.

A 5-month-old healthy boy had bilateral retinoblastoma. The left eye was enucleated and the right eye was successfully treated by external radiation. At the time when this treatment was completed, a large midline tumour mass in the sellar and parasellar areas of the brain was discovered by CT scan. This tumour was also treated by external radiation, but a recurrence was observed after three months. The patient died 11 months from the diagnosis of retinoblastoma. At autopsy the orbits showed no extraocular growth and the treated eye revealed only a scarred tumour. There was no evidence of metastatic disease. The brain tumour resembled pineoblastoma or differentiated retinoblastoma. It is concluded that the case shows all clinical features of 'trilateral' retinoblastoma, which could be confirmed by complete autopsy as well as neuropathological and ophthalmopathological studies.

Autopsy↗
PubMed1984

[Not Available].

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Allergy and Immunology↗
PubMed1983

Distribution of endothelial and basement membrane markers in angiogenic tumors of the nervous system.

The distribution of two endothelial cell markers Factor-VIII-related antigen and Ulex europaeus agglutinin was examined by immunoperoxidase and immunofluorescence techniques in paraffin-embedded specimens representing the three main types of angiogenic neoplasms of the nervous system, hemangioblastoma, hemangioendothelioma and hemangiopericytoma. In addition, the distribution of the basement membrane (BM) marker, laminin, was studied in the same tumors. It was found that Ulex europaeus agglutinin was a more sensitive marker of neoplastic endothelial cells than Factor-VIII-related antigen. Both markers only stained endothelial cells, while the tumor cells of hemangiopericytomas and the stromal cells of hemangioblastomas remained unstained. These findings do not support the view that the stromal cells of hemangioblastomas are derived from endothelial cells. With antiserum to laminin a typical staining pattern could be noticed in each tumor, showing the architectural relationships of the cells very clearly. In all three tumor types laminin was only found in the BM of the vessels, not in the interstices of the neoplastic cells outside vessel lumina. Therefore, the reticulin network previously found between the individual cells of hemangiopericytomas does not correspond to BM. It is concluded that both Ulex europaeus agglutinin and laminin antisera could be valuable new aids for the diagnosis of the three tumor types.

Adolescent↗
PubMed1983

A post-mortem comparison of the cortical cholinergic system in Alzheimer's disease and Pick's disease.

Assessment of neurochemical markers in the frontal cortex indicates that choline acetyltransferase is significantly decreased in Alzheimer's and Gerstmann-Straussler dementias but not in Pick's dementia. It therefore appears that the cholinergic innervation of the cortex from the basal forebrain is intact in Pick's disease. Cortical somatostatin was decreased only in Alzheimer's disease (AD), indicating that loss of somatostatin is not a constant feature in different forms of dementia. Muscarinic binding sites were unaltered in Pick's disease and Gerstmann-Straussler syndrome but were decreased in a subpopulation of AD patients. These data suggest that in some cases of AD a significant loss of cholinoceptive neurones in the cortex is evident.

Adult↗
PubMed1983

Lack of cholinergic deficit in the neocortex in Pick's disease.

Choline acetyltransferase activity was decreased in the frontal cortex in Alzheimer's and Gerstmann-Straussler dementias but not in Pick's disease. Cortical somatostatin was only decreased in Alzheimer's dementia. Postsynaptic muscarinic binding sites appeared to be decreased in a subpopulation of Alzheimer's patients. Our data indicate that a loss of cholinergic innervation of the cortex is not common to all dementias.

Choline O-Acetyltransferase↗
PubMed1983

High levels of brain dolichols in neuronal ceroid-lipofuscinosis and senescence.

Dolichols as unesterified alcohols were identified as significant components of lipid extracts from storage cytosomes isolated post-mortem from the brains of patients with the infantile, late infantile, and juvenile types of neuronal ceroid-lipofuscinosis (NCL). Very small amounts of dolichols were present in the corresponding subcellular fractions of non-NCL brains. The nuclear fraction from NCL cerebral cortex contained the highest dolichol content expressed per milligram protein or lipid, whereas the crude mitochondrial fraction was the richest in normal brain. Highly significant elevations of dolichol levels were found in human cerebral cortex of patients with NCL and Alzheimer's disease compared with age-matched controls, but the levels were normal in Pick's disease. In human non-NCL cerebral cortex, dolichols increased from 16 micrograms/g at age 5 to over 200 at age 81. Rat cerebral cortex showed a similar progressive increase in dolichol content with age. The high dolichol values in NCL, Alzheimer's disease, and senescence appears to be related to the increase of lipofuscin in brain. This is the first time a uniform biochemical abnormality has been found in all childhood forms of NCL, but the enzyme defect is still unidentified. It may lie on pathways where dolichols and retinyl compounds are recycled in Golgi membranes and derived organelles during the biosynthesis of glycoproteins.

Adolescent↗
PubMed1983

Accuracy of clinical diagnosis in primary degenerative dementia: correlation with neuropathological findings.

Neuropathological features and causes of death are presented in 27 deceased patients belonging to a prospective series of 71 hospitalised patients with primary degenerative dementia. The clinical criteria for primary degenerative dementia used in the present study were accurate enough to exclude patients with multi-infarct dementia. At necropsy, 82% of the cases had neuropathological changes of Alzheimer's disease. The clinical features of patients with other neuropathological changes are described. The most common immediate cause of death was bronchopneumonia which accounted for 59% of deaths in Alzheimer's disease.

Aged↗
PubMed1983

Dolichols in brain and urinary sediment in neuronal ceroid lipofuscinosis.

Long-chain polyisoprenoid alcohols (dolichols) increase more than tenfold from age 5 to 80 years in human cerebral cortex. The dolichol content of brain from infantile, late infantile, and juvenile forms of neuronal ceroid lipofuscinosis (NCL) was significantly higher than in age-matched patients with other neurologic diseases. Significant increase of dolichols was also found in the urinary sediment in all three types of NCL patients, and this test is useful in making the diagnosis. Dolichol accumulation is the first biochemical marker of NCL and seems to parallel storage of ceroid lipofuscin.

Adolescent↗
PubMed1983

Hyperthyroidism and acromegaly caused by a pituitary TSH- and GH-secreting tumour.

A female patient with acromegaly, TSH-induced hyperthyroidism and a large eosinophilic pituitary adenoma is reported. Granules in the adenoma cells were by immunohistochemical methods shown to contain GH and with monoclonal TSH-antibodies it was shown that 5-10 per cent of the cells secreted TSH. The basal serum TSH was elevated in the hyperthyroid phases and was not suppressible by exogenous T3 but decreased markedly with dexamethasone. There was a small subnormal rise in serum TSH after TRH injection which was totally suppressed by T3 (but not dexamethasone). L-dopa, bromocriptine and somatostatin caused a 20-30 per cent decrease in serum TSH. The alpha-subunit concentration was also elevated and was equally depressed by bromocriptine and somatostatin. The urinary excretion of TRH was within reference limits. This mixed tumour obviously secreted an excess of both GH and TSH causing acromegaly and hyperthyroidism.

Acromegaly↗
PubMed1982

Classic amyotrophic lateral sclerosis with dementia.

Documented cases of classic amyotrophic lateral sclerosis (ALS) combined with severe dementia are few and come from the isolated populations of the Western Pacific. In this report, three women had a clinically and neuropathologically documented combination of ALS and dementia. In all cases the symptoms and signs of ALS were mainly bulbar. In two of them dementia appeared first, followed by ALS. At autopsy, there was a marked discrepancy between the severe degree of dementia of Alzheimer's type and the sight nonspecific neuropathologic findings without Alzheimer's changes. Our cases bear a close resemblance to recently published Japanese cases. It may be concluded that the combination of sporadic ALS and progressive dementia seems to be a clinical entity without consistent neuropathologic changes and that it also occurs in the western countries.

Aged↗
PubMed1982

Trial with human leucocyte interferon and vidarabine in herpes simplex virus encephalitis: diagnostic and therapeutic problems.

A combination therapy of human interferon, vidarabine, and dexamethasone was administered to six patients with proven (Patients I-III) or presumed (Patients IV-VI) herpes simplex virus encephalitis (HSVE). Interferon combined with dexamethasone was given to one patient with presumed HSVE (Patient VII). Leucopenia and elevated serum transaminase levels appeared in all patients and a diffuse bleeding in one of them. Patients II, III and IV died, 26, 43, and 209 days after the onset of encephalitis, respectively. Patients I, V, VI, and VII were left with moderate brain damage although their physical condition was good. HSV encephalitis presents diagnostic difficulties, complementary diagnostic methods are needed, and current therapeutic trials must be considered as preliminary.

Adult↗
PubMed1982

Infantile neuronal ceroid lipofuscinosis: isolation of storage material.

We used ultracentrifugation to isolate autofluorescent storage particles from the brains of patients with infantile type of neuronal ceroid lipofuscinosis (Haltia-Santavuori). The particles differed from those of the late infantile (Jansky-Bielschowsky) and juvenile (Batten-Spielmeyer-Sjögren) types and retained the characteristic appearance--large, partly membrane-bound conglomerates of spherical globules with a uniform finely granular internal structure--despite the vigorous procedure. The sedimentation properties of the particles also differed from the two other forms, but dolichols are found in the particles of all three diseases.

Brain↗
PubMed1982

Polyarteritis confined to lower extremities.

A 32-year-old woman is described with polyarteritic changes concentrated to the calves. The disease has manifested itself in several acute episodes with swelling and pain in the calves, following symptomless phases. The response of to corticosteroid therapy has been good. Biopsy of the gastrocnemius muscle revealed peculiar multiple layers of intense NADH-tetrazolium reductase activity resembling annual rings in muscle fibers, a finding which has not been described earlier.

Adult↗