Biomedical subjects
Publications and source records attributed to J de Reuck.
Intracranial xanthomata occurring in patients with familial hypercholesterolemia are very rare. We present a young woman with a large intracranial xanthoma. The patient was treated for a familial hyperlipoproteinemia type IIa. MRI and morphological features are shown and discussed on reviewing the literature.
Pancreatic encephalopathy is an uncommon condition found in patients with acute pancreatitis. The present report shows the interest of MRI in the diagnosis of this disorder. Patchy white matter signal abnormalities, resembling plaques seen in multiple sclerosis, may reflect the lesions that are found in the cerebral white matter of post-mortem confirmed cases.
Villaret's syndrome is an extremely uncommon condition in which the four lower cranial nerves and the cervical sympathetic are selectively affected. Most cases described thus far have been attributed to either trauma or space-occupying lesions in the posterior retroparotid space. This report demonstrates a first case of "pure" Villaret's syndrome caused by jugular vein thrombosis secondary to previous mastoiditis.
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The results of a collagen assay performed on cultured skin fibroblasts from a 55-year-old man with multiple cerebral aneurysms are presented. Estimation of the collagen type III production by gel electrophoresis and scanning densitometry shows a reduction of collagen type III levels to 5% of normal. These findings point to the presence of a constitutional connective tissue disorder related to Ehlers-Danlos Syndrome type IV. It is suggested that biochemical collagen studies may be a useful alternative to angiography for detecting relatives at risk in families affected by intracranial aneurysms.
Mitotic figures were observed in 38 cases of a series of 3200 CSF cytograms. Their relative incidence was the highest in cases of tumoural invasion of the CSF, but they also occurred in inflammatory and reactive fluids and did not necessarily indicate tumoural origin. In bacterial infections of the central nervous system they were mainly present during the repair phase of the disease. In haemorrhagic spinal fluid they were absent. These findings suggest that cell division in spinal fluid tends to develop in conditions in which the viability of the cells is not affected by introduction of foreign material such as blood and bacteria.
Parkinsonism is a well known complication of carbon monoxide poisoning. Pharmacological treatment has been regarded as being not successful, as far as levodopa alone is concerned. Three cases are presented with symptomatic parkinsonism after carbon monoxide exposure, who have been treated with bromocriptine (in one case in combination with levodopa). The results are surprisingly good. A possible explanation is discussed.
The clinico-pathological findings in a 38-year-old man with a progressive cauda equina syndrome and with a dorsal dermal fistula in the lower back are presented. The X-rays of the spine revealed fusion of the vertebrae L4-L5 and L5-S1, and the myelogram showed an intradural tumour, extending upto the level of L2. An enterogenous cyst, adherent to the right third lumbar radix was completely removed. The clinical and pathological data are compared to previous published cases and discussed in relation to the origin of the tumour.
Choroid plexus and ependymal cells were only demonstrated in 10 of a series of 2620 CSF cytograms. Choroid plexus cells sometimes stained positive for acid phosphatase while in ependymal cells this enzyme activity was not observed. Choroid plexus and ependymal cells in CSF were found to be associated with various neurological disorders involving mainly the brain stem and spinal cord. This is probably due to the higher chance of sedimentation of viable cells in the lumbar sac in these conditions. Loss of these cells might explain the incidental observation of granular ependymitis in post-mortem brains.
One hundred and twenty samples of haemorrhagic spinal fluid were examined by acid phosphatase staining. This enzyme activity starts to appear in mono-histiocytic cells 2 days after bleeding and increases up to the 5th day. After 1 week the activity decreases rapidly. Similar results are found in mixtures of incubated clear spinal fluid, to which blood is added. Acid phosphatase staining is a useful additional method for determination of the age of a leptomeningeal bleed.
The case of a 57-year-old woman with idiopathic hypoparathyroidism is presented. A CT-scan showed extensive bilateral symmetrical calcification in the region of the basal ganglia, nuclei of the cerebellum and the cerebral and cerebellar white matter. A review of the literature showed that bilateral symmetrical calcification detected by CT is usually small in extent and is most often confined to the globus pallidus. It is most commonly found in patients older than 50 years, who only rarely have symptoms associated with it. The finding is, though, non-specific and may occur in a variety of pathological conditions both with and without an aetiological relationship. Further study of the cerebral parathormone responsive adenylate cyclase enzyme proves hopeful to elucidate the aetiology of idiopathic bilateral symmetrical calcification.
The authors describe three young female patients with generalized livedo racemosa and cerebral infarction. The radiologic and biopsy findings point to a widespread vasculopathy, involving small and mid-sized arteries. The histologic examination of the temporal artery biopsy in two patients gives additional evidence for a chronic endarteritis as possible underlying cause of this disease entity.
Target fibres were demonstrated in the gastrocnemius muscles following section of the Achilles tendon of two rabbits. In five other animals with tenotomized gastrocnemius muscles, a subarachnoid phenol block of the lumbar and sacral spinal cord was performed. Large and small medullary lesions inhibited the occurrence of target fibres in the tenotomized muscles, the smallest one being neurolysis of the dorsal roots. This final experiment suggests that the target phenomenon is the morphological expression of an increased stretch sensitivity of the tenotomized muscle.
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During a period of 10 years, 2782 brains were examined at post-mortem and in 17 cases metastases were attached to the dura mater, 8 presenting as multiple nodules 6 as a single nodule and 3 as diffuse dural thickening. The primary growths were, in equal proportions sarcomas, epitheliomas and lymphoid tumours. In contrast to the frequent location of metastases in the axial skeleton (9 cases), brain involvement was rarely observed (2 cases). The clinical findings were non-specific. The difference in biological behaviour between subdural and intracerebral metastases is stressed.
Out of series of 240 patients, with proved cerebral infarcts at necropsy, 14 with a clinical history of epilepsy were selected. The possible etiology of seizures was analyzed by comparing the clinical and pathological data. It appeared from this series that epileptic spells, preceding for years the occurrence of a stroke, were due to other causes than to cerebrovascular insufficiency. It was also difficult to separate apoplectic seizures from those occurring due to scar formation after a cerebral infarct, as postapoplectic epilepsy was induced either by the occurrence of a new stroke or by hemodynamic disturbances. Severe generalized or local hemodynamic and metabolic disturbances were also noted in cases of convulsions resulting from a cerebral infarct.
From 220 necropsies of patients with cerebral infarcts, 5 were selected, in which the clinical history revealed typical parkinsonian symptoms with a progressive course of the extrapyramidal disorder. No correlation could be found between the location and the size of the infarcts on the one hand and the signs of parkinsonism on the other hand. In the 5 cases presented, the substantia nigra showed patchy areas of neuronal degeneration without any Lewy bodies nor neurofibrillary changes, around a pronounced status cribrosus in areas irrigated by paramedian branches of the mesencephalic arteries. Similar neuropathologic findings were present, but to a minor degree, in 2 of 5 cases of pseudo-bulbar palsy. Some extrapyramidal features were also present. It is proposed that is some rare instances, status cribrosus may be responsible for nigral degeneration and for the occurrence of parkinsonism.
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