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Biomedical subjects

G T Bots

Publications and source records attributed to G T Bots.

At least 91 records · Page 5Linked to original sources

Familial Creutzfeldt-Jakob disease.

A Finnish family is described with 9 cases of presenile dementia in 3 generations. The mean age at onset was 52 years (range 46--62 years). Progressive dementia, upper motor neuron signs, muscular rigidity, and twitching, irregular tremors were consistent features in the 6 clinically investigated patients and were associated with spongiform change in the cerebral cortex of one autopsy and two brain biopsy cases. The EEG showed progressive slowing without the occurrence of repetitive high-voltage complexes at any stage of the disease. The average duration of the disease (21 months, range 11--36 months) was longer than in the sporadic form of CJD. The occurrence of CJD within this family follows a pattern consistent with an autosomal dominant mode of inheritance, suggesting the possibility of vertical transmission of the presumptive causative agent for example by genomic integration or transplacental passage. However, the occurrence of the disease only through the paternal line of relationships and the presence of a discordant twin pair argue strongly against transplacental passage or transmission via mother's milk. Simple contact infection also seems unlikely, as conjugal cases were not found among the 7 married patients. The interval between the death of the last affected member in generation IV and the time of onset of the disease in the first affected member of generation V was 10 years. Thus setting a minimum incubation period if case-to-case transmission were occurring. To evaluate the role of a genetically determined susceptibility to infection studies on the HLA antigens and other genetic markers are in progress.

Creutzfeldt-Jakob Syndrome↗

Nervous system complications of herpes zoster: immunofluorescent demonstration of varicella-zoster antigen in CSF cells.

In nine out of 11 patients with herpes zoster and neurological symptoms varicella-zoster antigen was demonstrated in CSF cells by indirect immunofluorescent staining. Cerebrospinal fluid cytology was in most cases highly suggestive of viral infection. This study demonstrates the value of both immunofluorescent staining on CSF cells and CSF cytology, as corroboration of an otherwise exclusively serologically based virological diagnosis.

Adolescent↗

Facial palsy as the initial symptom of a lymphoreticular malignancy. Case report.

In this case report an adult patient is described who initially manifested a peripheral facial palsy mimicking 'Bell's palsy', which later on appeared to be the first manifestation of a lymphoreticular malignancy. Cranial nerve involvement appears to be very rare as a first symptom of malignant systemic disease. The importance of additional investigations including lumbar puncture and CSF cytology in cases of facial nerve palsy is stressed.

Cranial Nerve Neoplasms↗

ECHO 25 focal encephalitis and subacute hemichorea.

ECHO virus 25 focal encephalitis was documented for the first time in a 5-year-old boy with unequivocal hemichorea. Hemichorea caused by enterovirus CNS disease has never been reported. ECHO virus antigen in cerebrospinal fluid (CSF) cells was demonstrated by the indirect immunofluorescent technique and typed by a significant rise in neutralizing antibodies against ECHO virus 25. Sequential computerized tomographic (CT) studies with digital analysis demonstrated the evolution of a focal process in the head of the left caudate nucleus, ultimately leading to replacement of tissue by a CSF-containing cyst. In the unclarified pathogenesis of cerebral disease in enterovirus infection, this case suggests a focal inflammatory process rather than vasculitis, occlusion, and infarction. Finally, the near-complete recovery of our patient demonstrated that the mere loss of neostriatal tissue was not decisive in itself, because the tissue loss was permanent and the symptoms were transient.

Antibodies, Viral↗

Varicella and acute cerebellar ataxia.

In two cases of varicella-associated cerebellar ataxia, varicella-zoster antigens in CSF cells were shown by an indirect immunofluorescent technique. Direct viral invasion in CNS disease complicating varicella plays an important part in pathogenesis and rules out a single immune-mediated mechanism.

Antigens, Viral↗

Viral meningoencephalitis and head injury.

Among 162 children referred with head injury (in a 3-year period) a viral meningoencephalitis was proven to be present in seven cases. Only one patient showed symptoms of infection before the accident. On admission classical signs of viral meningoencephalitis were absent or mixed with the complications of head injury. A diagnosis of viral meningitis/meningoencephalitis was documented by immunofluorescent demonstration of viral antigens in CSF cells and/or pattern of CSF cytology and/or conventional serological methods. Whether the trauma and the infection are coincidental or causally related can not be decided definitively. A hypothesis is presented suggesting diminished alertness in subclinical viral meningoencephalitis, leading to accident proneness.

Antigens, Viral↗

Value of cerebrospinal fluid cytology for the diagnosis of malignancies in the central nervous system.

The authors present a retrospective analysis of the results of the cytological examinations of cerebrospinal fluid (CSF) samples and tumor-cyst aspirates deriving from 262 patients treated for malignant intracranial primary and secondary tumors, and vertebral and peridural metastastic processes. Positive preoperative CSF samples were found in 15.3% of all cases of primary cerebral malignancies (13.9% of all gliomas) and positive postoperative CSF samples were found in 40% (91% of the medulloblastoma cases). In all cases of single or multiple secondary cerebral tumors, positive preoperative CSF samples were found in 20%.

Astrocytoma↗

Acetylcholinesterase-activity in rectal mucosa of children with obstipation.

The results are presented of a clinical-enzyme histochemical evaluation of the activity of acetylcholinesterase in the rectal mucosa of 46 children with obstipation. In four cases abundant and coarse acetylcholinesterase positive nerve fibres were present in the lamina propria of the mucosa. Only in these 4 cases was the diagnosis of Hirschsprung's disease supported by the clinical course. Local accumulations of fine acetylcholinesterase positive nerve fibres or accumulations of acetylcholinesterase positive foamy or structureless material were not correlated with Hirschsprung's disease.

Acetylcholinesterase↗

Characteristics of mononuclear cell populations in chronically inflamed synovial membranes.

Mononuclear cells infiltrating synovial membranes in chronic synovitis were characterised both in situ and in cell suspensions by surface markers and histochemical techniques. T-lymphocytes were the predominant infiltrating cell in rheumatoid arthritis as well as in other forms of chronic arthritis, including ankylosing spondylitis and arthritis associated with Crohn's disease. B-lymphocytes were found exclusively in rheumatoid synovial membranes. These cells were demonstrable both in true germinal centres and, focally and diffusely, in nodular mononuclear infiltrates lacking the histochemical characteristics of germinal centres. The synovial lining cells, unlike mononuclear phagocytes, had no demonstrable receptors for C3 and Fc.

Adult↗

Astrocytoma in three sisters.

Three sisters died of astrocytoma, and the microscopic appearance of the tumor was the same in all three. This finding supports either genetic or environmental influences in the occurrence of brain tumors.

Aged↗

Proliferation of blood vessels and stroma in brain tumours. An enzyme-histochemical study.

Proliferation of the vascular endothelium occurring in brain tumours is accompanied by a proliferation of histiocytes in the peripheral part of the vessel wall. These histiocytes infiltrate the tumour tissue in a very regular pattern. Enzyme-histochemically, there are marked differences between the activities of alkaline phosphatase, 5-nucleotidase, and ATPase in the normal and proliferating blood vessels. The whole process encompasses reactive changes evoked by the destroyed perivascular sheath of astroglial foot processes and the subsequent oedema in the tumour and the surrounding parenchyma. There are often tumour areas where diminished vascular permeability is established by proliferation of perivascular connective tissue. Here the oedema has completely disappeared. A clearcut influx of monocytes from the blood into the vessel wall is seen only in the vicinity of necrotic foci; the number of histiocytes is increased and their turnover is observed in swollen macrophages. In the rest of the tumour influx of monocytes and activity of macrophages are inconspicious.

Adenosine Triphosphatases↗

Use of immunohistochemical and morphologic methods for the identification of human growth hormone-producing pituitary adenomas.

Indirect immunoperoxidase and immunofluorescent techniques were used for the detection of growth hormone in routinely processed human pituitary adenomas. With the immunoperoxidase method, distinct immunoreactive cells could be demonstrated in 15 adenomas; all except for one were associated with acromegaly. Immunofluorescence was less useful because of autofluorescence due to the fixation. In histologic stainings, the immunoreactive cells were acidophyl. With immunohistochemistry and electron microscopy, densely granulated and sparsely granulated adenomas could be distinguished. Sparse granulation was associated with an expanded rough-surfaced endoplasmic reticulum and Golgi complex. Clinically, the sparsely granulated adenomas were characterized both by active hormone secretion and aggressive local growth. It is concluded that the immunoperosidase method is of value for functional classification of pituitary adenomas. For information about secretory activity we suggest that electron micrographs of adenoma cells be examined.

Adenoma↗

[Volitional postural dyskinesia with hypertrophic neuropathy].

Two cases of voluntary postural dyskinesia with hypertrophic neuropathy are reported. Electron-microscope studies revealed in one case a hypertrophic neuropathy with onion bulb formation, in the other axonal degeneration. The findings are compared with those of Pierre Marie-Boveri disease, of Roussy-Lévy disease and with the case of Salisachs and Lapresle (1973).

Aged↗