Reye's syndrome and short-chain fatty acids.
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Biomedical subjects
Publications and source records attributed to P Berlit.
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The case of a patient with a radiation lesion of the lumbosacral cord following prophylactic radiation therapy of the paraortic lymph nodes for seminoma is reported. Seven months after radiotherapy he suffered from a pure anterior cell syndrome, followed by a loss of pain and temperature sensation. Ten years later he developed a complete transversal syndrome at the level of L 1. This case indicates the existence of a lumbar radiation myelopathy caused by a lesion at the level of the conus medullaris.
Over a period of 13 years, 353 cases of metastases in the brain, spinal canal or peripheral nerves were treated in 14,350 inpatients. In 79.6% of the cases, the metastases were localized intracranially, in 14.7% spinally, in 2.6% peripherally and in 3.1% in several of these sites. Solitary tumors predominated (65.7%). Of 420 intracranial metastases, 336 were located supratentorially (80%) with a slight preponderance on the left side (54.5%), 15% cerebellar, and 5% in the brainstem. Of the spinal metastases, 80% were located in the thoracic spinal cord. Almost 60% of the cases also displayed metastases outside the nervous system, mainly in the skeletal system and the lungs. The most frequent primary tumor was bronchial carcinoma (26,6%) followed by breast cancer (19.5%) and unknown primary tumor (17.6%), which was also not found on autopsy in 0.8%. Rare primary tumors were parotid and pancreatic carcinomas, testicular and bladder tumors. There are correlations between the primary tumor and the location of the metastases in the nervous system in general and in the brain in particular. The latency between diagnosis of the primary tumor and that of the metastasis was 1-3 years. In one out of three cases, the metastasis in the nervous system was the first sign of the tumor condition. In six cases, the metastasis was removed before the primary tumor and two possible kinds of primary tumors were found in seven cases. Compared to intracranial hypertension focal deficit manifestations including focal convulsions occurred twice as frequently in cerebral metastases. Spinal metastases led to CSF blockade in 20%.(ABSTRACT TRUNCATED AT 250 WORDS)
Immunohistochemical examinations were carried out in a case of thromboangiitis obliterans. A 26-year-old man developed 4 brain infarctions which could be demonstrated by CAT scan. He suffered from a left-sided hemiparesis, major epileptic seizures, and an ischemic optic neuritis. Immunologic studies supported a diagnosis of cerebral thromboangiitis obliterans (CTAO). The serum anti-elastin titer as well as IgE were considerably increased and a biopsy of the temporal artery showed immunohistochemical signs of an acute inflammatory vessel disease. After the diagnosis of CTAO had been made, the patient was treated with azathioprine and dexamethasone. In cases of young stroke patients, a temporal biopsy is important in confirming the diagnosis.
The existence of thromboangiitis obliterans as a clinical entity has been a matter of debate for many years. In contrast to other immunovasculitides there is no organ involvement while peripheral vessels are affected. Heavy smokers under 40 years of age have a high predisposition for the disease. The cerebral form shows relapsing brain infarctions which can be visualized in CCT while panarteriography remains negative. Apart from unspecific inflammatory signs in blood and CSF there are distinctive laboratory findings proving the autoimmunological character of von Winiwarter-Buerger's disease. In the serum anti-elastin antibodies, IgE and anticollagen antibody activity are detectable. In 3 patients the authors detected specific immunohistochemical findings in a biopsy specimen of the temporal artery. In addition to platelet-inhibiting substances corticoids in acute and azathioprine in chronic treatment becomes necessary.
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Urinary organic acids, known to be elevated in children with biotin deficiency, were determined in 7 epileptics under long-term therapy with anticonvulsants and in three controls. Four patients administered phenytoin, primidone, phenobarbital, or carbamazepine, alone or in combination, had reduced plasma biotin levels (less than 250 ng/l) and an elevated excretion of certain organic acids indicating a possibly decreased activity of propionyl CoA carboxylase (3-OH-propionate, methylcitrate) and 3-methylcrotonyl CoA carboxylase (3-methylcrotonate and the glycine conjugate, 3-OH-isovalerate). Two epileptics receiving sodium valproate alone had normal circulating biotin levels and no changes in level of the investigated urinary acids were found. These findings indicate that the reduced biotin levels seen in epileptics receiving other anticonvulsants than sodium valproate lead to an elevated excretion of certain organic acids in urine.
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The case is reported of a patient with incomplete peripheral facial palsy. In addition to hyperacousia and gustation disturbance, he presented with signs of glossitis limited to the region supplied by the chorda tympani. Clinical and laboratory investigations excluded the possibility of a viral infection. Possible connections between this combination of symptoms (apparently "idiopathic" facial palsy and regional glossitis) and the Melkersson-Rosenthal syndrome are discussed.
Plasma arginine vasopressin concentrations were determined by radio-immunoassay in 112 adult epileptics who were taking carbamazepine, phenytoin, primidone, or sodium valproate in long-term monotherapy, and in 19 controls. No significant difference was found between the groups, but some epileptics taking carbamazepine and primidone showed low values. Serum concentrations of carbamazepine did not correlate with the concentrations of plasma arginine vasopressin. In conclusion, there was no evidence of a stimulating effect of chronic carbamazepine medication or a special inhibiting effect of phenytoin on the release of vasopressin arginine from the posterior pituitary.
The case of a patient with occlusion of the external carotid artery is reported; he suffered from vertebrobasilar insufficiency with Wallenberg's syndrome. Arteriography showed stealing of intracranial blood from the vertebral artery via the occipital artery, and thromboendarterectomy was successfully performed. Since no localised processes could be demonstrated could the lateral medullary infarction be due to the haemodynamic changes? In cases of external carotid steal thromboendarterectomy might be the appropriate treatment.
This review summarizes those neurological diseases which are accompanied by a drooping of the upper lid, due to weakness of the m. levator palpebrae or m. tarsalis respectively. After connatal ptosis with or without involvement of other bulbar muscles the different types of muscular dystrophies are mentioned. Myositis, disturbances of potassium regulation and myasthenia gravis are other causes of ptosis. Diseases involving the oculomotor nerve (aneurysm, upper herniation, cavernous sinus thrombosis, orbital cavity processes, superior orbital fissure syndrome) may lead to associated ptosis. Metabolic disturbances, such as diabetes mellitus, Wernicke's encephalopathy and botulism may be accompanied by ptosis. Infectious diseases such as polyneuritis, meningitis or encephalitis can lead to ptosis. Sympatholytic ptosis is due to diseases of the central or peripheral course of the sympathetic nerve from the diencephalon via the cervical medulla, the neck, internal carotid artery to the superior orbital fissure. This type of ptosis is usually accompanied by miosis and often by sweating loss on the same side.
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135 cases of cardiogenic emboli to the brain are reported in view of symptoms, diagnosis, prognosis and therapy. Brain embolism occurred most often in ischemic heart disease with atrial fibrillation, followed by valvular heart disease, myocardial infarction and carditis. Usually there was a sudden onset of neurological symptoms during the morning hours. Initial lapses of consciousness were a bad prognostic sign--they appeared as well as seizures more often in embolic than in thrombotic brain infarctions. The clearly higher incidence of emboli to the left cerebral hemisphere has anatomical reasons. Angiographic findings depend on the time of examination; common are occlusions of the supraclinoid portion of the internal carotid artery, media-occlusions at the trifurcation, media-branch-occlusions and disturbed middle cerebral blood flow. CT and lumbar puncture do not support the diagnosis. Cardiologic examination should include X-ray of the chest, long time electrocardiogram and echocardiography. In view of a mortality of 22 per cent and major neurological residual symptoms in 34 percent the prognosis of brain embolism is serious. Early anticoagulant therapy in the absence of brain hemorrhage in CT seems to improve the prognosis by preventing reembolization.
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Sera of 200 non-transfused healthy male blood donors were tested for antibody reactivity to cell-surface antigens of cultured astrocytoma cells. Positive reactions were observed only rarely by protein-A assay (PA), in about half the cases by immune adherence assay (IA) and in nearly all cases by anti-C3 mixed hemadsorption assay (C3-MHA). In general, titers were low and only seven sera showed reactivity at 1:1,000. Serum 537 showed the strongest reaction. The anti-astrocytoma reactivity in this serum was due to an IgG antibody. Extensive absorption analysis with a panel of cell lines and fresh cells of both benign and malignant origin, as well al fetal cells, revealed that this serum detected an antigen that was present on most neural-crest-derived tumors (astrocytomas, melanomas and neuroblastomas), on very few other malignant tumors and on fetal brain. The antigen detected by serum 537 shows close relationship to the astrocytoma antigen AJ which had been defined by the serum of a patient with astrocytoma. Both antigens appear to be differentiation antigens present predominantly on non-epithelial neoplasms. The antigen detected by serum 537 is heat-stable and pronase-resistant. The sera of two other healthy donors apparently had a similar specificity, whereas the four other high-titered sera and all other sera detected class-III antigens which were non-specific and not tumor-restricted.