[Adoption: ethical bases of an awaited amendment].
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Biomedical subjects
Publications and source records attributed to P Le Coz.
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Spontaneous cervical epidural hematomas are uncommon lesions that usually produce permanent neurological deficit unless there is early surgical decompression. They are now well-recognized by scan X and especially by sagittal MRI of the spine. We describe 2 patients, a 24 year-old man and a 79 year-old woman with no previous history of trauma who were admitted in emergency for a sudden weakness of limbs, respectively a tetraplegia and a right hemiplegia. Both patients complained of inaugural and acute neck pain. Motor deficit completely resolved in few hours and MRI of the spine showed on T1 a signal isointense, extending respectively from C3 to C6 and C5 to C7, consistent with an hematoma. Laboratory data and angiography were normal. Surgery was recused. Neck pain lasted about a week. Follow-up MRI, in one case, was normal two months later. Cervical epidural hematomas revealed by transient neurological findings that completely and permanently resolved are exceptional. They could mimic ischemic myelopathy and should be considered in the differential diagnosis of other painful vascular conditions like symptomatic vertebral dissection to avoid inappropriate anticoagulation. Conservative management in these cases may be proposed if spontaneous neurological resolution is confirmed by MRI.
During Parkinson's disease static contrast sensitivity (C.S.) abnormalities are linked to an impairment of the sensitive visual function. C.S. was tested in twelve parkinsonians and 12 controls without neurological and/or ophthalmological pathology, using a Colored Stripes Electronic Generator (GEPCO). Results for parkinsons showed a general deficiency over the spectrum of spatial frequencies, which was statistically significant and particularly pronounced for intermediate frequencies. This study was repeated for three patients: it showed threshold deterioration for two of them, correlated by evolution in the disease, and an improvement for the third patient after introduction of dopatherapy. C.S. is subjected to dopaminergic control. Among parkinsonians. C.S. deterioration may result in an operating failure of both the visual cortex and retina, and is improved by dopatherapy. The Static Gepco contrast sensitivity test is easy to reproduce and can be used easily to monitor the sensory visual defect in parkinsonian patients under treatment.
The authors report on a Portuguese family with 3 adult brothers affected with GM2-gangliosidosis (B1 variant) in a sibship of 4, and more specifically on one of these brothers with neurological onset at the age of 17. Psychosis, lower motoneuron involvement and dysarthria were predominant in two of the cases; the third had a cerebellar symptomatology. Hexosaminidase A activity, studied in leukocytes, was profoundly deficient when measured using the specific sulfated substrate, but nearly normal using a conventional assay (non-sulfated substrate). These results established the diagnosis of the unusual enzymological form of GM2-gangliosidosis known as the B1 variant, which had so far not been associated with an adult phenotype. Molecular studies are in progress to study genotype/phenotype correlations in this family in comparison with known mutations in the B1 variant and in adult GM2-gangliosidosis. This report also emphasizes that a metabolic etiology, leading to genetic counselling, should be considered in some familial degenerative neurological disorders.
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Twenty-four patients presenting an acute stroke with watershed cerebral infarct on CT scan or MRI were included in this retrospective study. Age was 63 +/- 14 years (mean +/- SD), and sex ratio was 2 men for 1 woman. Main clinical features were: in anterior location, lower limb weakness and frontal syndrome with transcortical motor aphasia in left lesions or spatial dyscalculia in right ones; in posterior location, brachiofacial weakness with constant quadranopsia and hypoesthesia, and Gerstmann syndrome in left lesion. There was no distinctive feature for subcortical and multiple infarcts. In bilateral infarcts, there were one pseudobulbar syndrome, and 2 pseudo brainstem syndromes with neuropsychological signs. Aetiologies were severe carotid artery disease in 14 cases, severe cardiopathy in 6, isolated cerebral angiitis in 1, essential thrombocythemia in 1, protein C deficiency with sickle cell disease in 1, and cholesterol emboli in 1 anatomical case. CBF performed in carotid artery occlusions or tight stenoses showed evidence of haemodynamic changes. Microembolic process can be proposed in the case with cholesterol emboli. Preventive treatment is discussed.
A woman, who presented with clinical and radiological signs of a right temporal mass suggestive of a brain tumour, was found to have granulomatous angiitis associated with cerebral amyloid angiopathy; the diagnosis was confirmed by biopsy. She is still well 13 years after excision of the lesion. The association of granulomatous angiitis and cerebral amyloid angiopathy constitutes a peculiar variety of central nervous system micro-angiopathy. Only a few similar cases have been described.
Three patients from a single family of six siblings had homocystinemia and homocystinuria due to 5,10-methylenetetrahydrofolate reductase deficiency and had severe recurrent strokes in adult life. Two of the patients died 1 year after clinical onset.
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Heroin, cocaine, amphetamines, sympathomimetic drugs can cause cerebral angiopathy. We report 2 patients with cerebrovascular disorders after ingestion of a nasal vasoconstrictor containing phenylpropanolamine (P.P.A.). The first patient had two acute repetitive attacks of severe headache and vomiting, occurring after a daily treatment with 180 mg of P.P.A. during 6 weeks. The second patient had an intracerebral hemorrhage, occurring some hours after taking for the first time 120 mg of P.P.A. In both cases, cerebral angiography, performed in the next week, demonstrated segmental narrowing and dilatations of medium-size intracranial arteries. None of the usual causes of cerebral vasculitis were present. The outcome was favorable and follow-up angiograms showed the disappearance of the beading pattern. P.P.A. is widely used over the counter in diet pills and stimulants. Cerebral vascular complications have been rarely reported, always hemorrhagic and often associated with cerebral vasculitis. They are unrelated to duration or dosage of treatment. The mechanism is unclear but could result from several factors: chronic or paroxystic high blood pressure, immuno-allergic vasculitis, arterial spasm, direct "toxic" effect of the P.P.A. on the arterial wall may be increased by other drugs and caffeine.
A progressive neurological syndrome with cerebellar signs, abnormal proprioception, areflexia and Babinski response was observed in a child with chronic intestinal malabsorption. There was no ophtalmoplegia or retinitis pigmentosa. Electromyography and biopsy showed no axonopathy or myopathy. Two other members of the family were also affected. The serum Vitamin E corrected the serum Vitamin E levels within a few months and led to secondary neurological improvement. The authors underline the importance of searching for Vitamin E deficiency and its cause in patients, especially children, with signs of spino cerebellar degeneration. Substitative therapy may have a favorable influence on the neurological condition even when administered late.
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