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Biomedical subjects

L J Endtz

Publications and source records attributed to L J Endtz.

At least 37 records · Page 2Linked to original sources

Persistence of the blink reflex to sudden illumination in a comatose patient. A clinical and pathologic study.

A persistent blink reflex to light (BRL) was observed in a patient who became comatose after a cardiac arrest. Postmortem examination revealed severe hypoxic ganglion cell necrosis of the cerebral and cerebellar cortex, the basal ganglia, the hypothalamus, nuclei in the brain stem, and the superior colliculi. The pretectum was unaffected. These findings suggest that the afferent fibers of the BRL enter the brain stem not in the superior colliculus, but in the pretectum.

Aged↗

Thyroid crisis presenting as coma.

Coma is a rare complication of thyrotoxicosis. A patient with coma due to a thyroid crisis is reported. The EEG showed extremely slow and low voltage activity, which gradually returned to normal. Coma as a presenting symptom of thyroid crisis has not been reported before.

Adult↗

Aphasia with a left frontal interhemispheric hematoma.

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.

Aphasia↗

[Neurology and photographic illustration in the medical book].

The mutual influence between G. B. A. Duchenne (de Boulogne) (1806-1875), who published in 1862 the first known medical books illustrated with photographs, and J.M. Charcot (1825-1893), whose appointment as "Médecin de la Salpêtrière" occurred in the same year, has been of great importance for the history of medical illustration. The development of medical photography at the Salpêtrière has been continuous, absorbing also a stream sprung from another Paris hospital, the Hôpital Saint Louis (A. Hardy and A. de Montmeja, 1868). The first Department of Medical Photography was installed by the "Assistance Publique" at the Salpêtrière (1878). The books and journals were for the greater part published by two publishing houses: J. B. Baillière and Delahaye and his successors. Attention is drawn to the fact that the third edition of Duchenne's major work (1872) is no longer illustrated by photographs but contains engravings from those photographs. Other books and articles are illustrated with photographs but one is increasingly confronted with a great number of drawings of engravings made from photographs. The reasons for this change are discussed. It appears that a photograph may serve as an illustration but that for an educational or a research purpose it has another function: to preserve the image to be studied, the authors preferring to sort out the essential information (or what was thought to be so) in the form of drawings or engravings.

Books, Illustrated↗

Familial cerebral amyloid angiopathy presenting as recurrent cerebral haemorrhage.

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.

Adult↗

Hereditary Pick's disease: second re-examination of the large family and discussion of other hereditary cases, with particular reference to electroencephalography, a computerized tomography.

A large family with Pick's disease was re-examined after twenty years. Five new cases were found, one in the fourth and four in the fifth generation. This family now includes 25 patients with the clinical diagnosis Pick's disease, proven by autopsy in 14, and 7 patients in whom the same diagnosis was considered likely, over six generations. The additional information strongly supports the previously postulated dominant mode of inheritance in this family. In connection with the possible hereditary nature of Pick's disease in general, data on reported families in which the disease occurred in two or more generations and unpublished data on three other families are discussed. Since the diagnosis Pick's disease was certain in affected members of this family, the value of electroencephalography and computerized tomography could be assessed. Persons at risk but without clinical signs of the disease were investigated in the same way, and in 4 cases out of 12, distinct frontal atrophy was found. In one of these cases clinical signs of Picks disease became manifest a year after the investigation.

Brain↗

Chronic myeloid leukemia in myasthenia gravis after long-term treatment with 6-mercaptopurine.

A woman with myasthenia gravis and a thymoma did not respond sufficiently to thymectomy. She was treated with 6-mercaptopurine. Withdrawal of this treatment was several times followed by an aggravation of myasthenic symptoms. After more than 12 1/2 years treatment she developed Ph1-positive chronic myeloid leukemia (CML). No other case of CML following immunosuppressive treatment has been described. Because the therapeutic agent is potentially leukemogenic, the possibility cannot be definitely excluded that the development of CML is not a mere coincidence.

Chromosomes, Human, 21-22 and Y↗

Acute myelopathy caused by fibrocartilaginous emboli.

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.

Acute Disease↗

Studies on asterognosis and amyotrophy of the hand in brainstem syndromes. Relation to the symptomatology of tumours at the spinocranial junction.

Fifteen patients with symptoms of an ischaemic lesion in the vertebrobasilar territory were investigated with respect to the presence of asterognosis and amyotrophy of the hand muscles. Stereognostic disturbances were noted in 4 patients with a medullary lesion and in two patients with brainstem syndromes of less defined level. The findings in these cases suggested a lesion of the medial lemniscus. Amyotrophy of the hand muscles was not seen in these patients. The absence of association of astereognosis and amyotrophy of the hand muscles in syndromes due to lower brainstem lesions supports the view that these symptoms, when occurring conjointly in foramen magnum tumours, originate from two different lesions and are caused by different mechanisms.

Agnosia↗

Peripheral nerve involvement in pernicious anaemia.

Impairment of nervous function and thiamine metabolism were studied in 40 patients suffering from pernicious anaemia, 20 of whom had not been treated and the other 20 were on hydroxocobalamin therapy. Of the untreated patients 13 (65%) showed signs of peripheral nerve dysfunction with reduced conduction velocities, as compared with only 1 (5%) of the treated patients. Reduced blood thiamine levels were found in 16 of the untreated patients (80%), 10 (60%) of whom showed biochemical signs of thiamine deficiency. In the treated group, 7 patients (30%) had reduced blood thiamine levels. Thus, peripheral nerve involvement in pernicious anaemia is much more frequent than would be expected from the literature, and thiamine deficiency was present in the majority of these cases.

Aged↗

Heterotopic nervous tissue in the lungs.

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.

Anencephaly↗