Biomedical subjects
Publications and source records attributed to G T Bots.
We studied a patient with transcortical motor aphasia resulting from a traumatic interhemispheric left frontal hematoma. The aphasia was caused by compression exerted by the hematoma on the left supplementary motor area, which is known to have a function in speech. This cause of a transcortical motor aphasia has not been described earlier.
Electron-microscopical examination of the nucleus accumbens, caudate nucleus and frontal cortex was performed in 21 patients with Huntington's disease (HD) and 21 age-matched controls. In HD, nuclear membrane indentations (NMI) were found in 25%, 29% and 20%, respectively, of the neurons of these areas. In the control group these figures were 4%, 4% and 38%, respectively. Possible pathogenetic mechanisms of the NMI are discussed.
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The distribution of epidermal Langerhans cells (LC) in erythrokeratodermia variabilis (EKV) was investigated using enzyme-histochemical (ATPase) and immunohistochemical (anti-T6 and anti-HLA-Dr) techniques. Biopsy specimens from lesional skin of five patients were examined before and 8 weeks after treatment with etretinate (RO 10-9359). In addition, electron microscopy studies were carried out in two of these cases. The number of ATPase-positive dendritic cells in lesional epidermis appeared to be remarkably reduced in comparison with normal skin from healthy subjects. After treatment, the number of ATPase-positive dentritic cells had increased but still remained below normal values. Similar but less striking results were found for anti-T6-stained specimens. The HLA-Dr antigen appeared to be unsuitable as a marker for comparative quantitative studies because of the highly variable expression of this antigen in the control specimens. Electron microscopy studies revealed LC in the basal and suprabasal layers of the lesional epidermis. Both before and after treatment, the LC exhibited a normal ultrastructure. In view of the clinical and histologic normalization of the skin lesions during treatment, these findings suggest that the decreased number of epidermal LC may be related to the abnormal keratinization that occurs in EKV.
Eleven patients belonging to two generations of a Dutch family with cerebral and cerebellar haemorrhage, haemorrhagic infarction and infarction are described. Their ages varied from 44 to 58 years. The principal clinical characteristics was recurring cerebral haemorrhages, sometimes preceded by a history of migrainous headaches or mental changes. In 4 of the 6 autopsied cases, old and new multiple cerebral haemorrhagic infarcts and infarcts were found, in one case a single cerebral haemorrhage and in another a cerebellar haemorrhage. In 5 cases this resulted in secondary subarachnoid haemorrhage. In one case the infarcts were only slightly haemorrhagic and did not result in subarachnoid haemorrhage. This patient presented as dementia. Microscopically, in these 6 cases and in one biopsy specimen hyaline thickening of the walls of cortical arterioles was found. The arteries of the arachnoid showed marked tortuosity, concentric proliferation, and focal hyalinization of the walls. Amyloid was found in the hyalinized vessels in 5 cases, but not outside the central nervous system. We believe that we are dealing with an inherited disorder with an autosomal dominated mode of inheritance, in which microangiopathy leads to cerebral haemorrhage and (haemorrhagic) infarction. It seems likely that amyloidosis underlies the angiopathy, and that this family suffers from a condition similar to the one described by Gudmundsson in 1972.
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The case history, skeletal muscle and heart muscle biopsy findings from an affected member of a family suffering from hypokalaemic periodic paralysis associated with permanent muscular weakness are reported. The patient, a female aged 35 years, while treated with 750 mg and later 1000 mg of acetazolamide daily, developed typical exercise angina pectoris. The ECG during exercise showed ST-segment depression. A coronary angiography was performed because coronary artery disease was suspected. To exclude cardiac muscle disease a biopsy of the left ventricular wall was taken. The angiography was normal. Ultrastructural analysis of the biopsy specimen showed an unusual amount of intermyofibrillary glycogen resembling, although far less abundant, the increase of glycogen found in the skeletal muscle biopsy specimen obtained from the same patient. The possible implications of these findings are discussed with reference to the normal echocardiographic findings in the family.
Computed tomography fails to ascertain, or exclude, the presence of intracranial haemorrhage in a considerable number of cases, especially in subarachnoid haemorrhage and haemorrhagic infarcts. A number of other methods, including cerebrospinal fluid spectrophotometry and cytology have, therefore, been tested to define their diagnostic efficacy in 25 cases of confirmed intracranial haemorrhage and in 25 instances of blood-stained cerebrospinal fluid due to traumatic puncture. The combination of spectrophotometry and cytology proved to have a high diagnostic reliability. On the basis of these results a routine scheme of investigation is proposed.
Fibrocartilaginous embolism of the spinal cord has been reported in only 17 cases. We describe three new cases, all seen within a year, of infarction of the spinal cord by fibrocartilaginous emboli in the ramifications of the anterior spinal artery. These emboli probably originate from degenerated intervertebral disks, but full understanding of the pathogenesis is still lacking. The disease has been found more frequently in dogs. Because the pathologist is not likely to attempt to demonstrate the condition unless the clinician has proposed this diagnosis, the true frequency will be known only when the clinical picture is widely recognized.
A case of a malignant neurofibroma of the cauda equina is described. Its location seems to be rare. An extensive surgical resection including the adjacent neural tissue and the enveloping dural sac was carried out, followed by radiotherapy.
We report an instance of progressive multifocal leukoencephalopathy in which cytarabine administration was successful as demonstrated by clinical course and follow-up computerized tomography studies. In this case, simian virus 40 (SV40) antigen was demonstrated in CSF cells by the indirect immunofluorescence technique.
A case of an intrasellar cyst in a 48-year-old woman is reported. The cyst was lined by stratified squamous epithelium, most of which was covered by ciliated columnar epithelium. Most of the stratified squamous epithelium showed necrosis accompanied by signs of an acute inflammatory reaction. After surgical resection aseptic meningitis developed. The lumbar CSF findings were still abnormal four months after the operation.
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Foci of heterotopic nervous tissue, including ganglion-cells, were found in the lungs of an anencephalic foetus. The nine cases in the literature are reviewed. Only in two of these cases was the histological investigation sufficiently detailed to establish the nature of the ectopic tissue. In all but one case the condition was associated with gross malformation of the brain.
The autopsy findings on a 60-year-old man with progressive disturbances of gait, presenile dementia and incontinence, showed Lafora bodies in numerous ganglion cells of the cerebral cortex and in many nuclei of the brain stem. Histochemical analysis of the Lafora bodies revealed the presence of a polysaccharide-protein complex containing phosphate groups. The case closely resembled the one described by Suzuki et al. It is suggested that this type of presenile dementia may be a presenile form of Lafora disease.
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