Transient achromatopsia in vertebrobasilar insufficiency.
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Biomedical subjects
Publications and source records attributed to J Lapresle.
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The authors report of Argyll-Robertson-like pupils in three patients presenting the neural type of Charcot-Marie-Tooth (CMT) disease with peroneal muscular atrophy. Up to now the light-near dissociation had been reported in the hypertrophic variety of CMT disease. Stress is laid upon the fact that the presence of the light-near dissociation in patients with CMT disease does not help in clinically differentiating the neural from the hypertrophic type of this disease.
The authors begin by enumerating the various syndromes in which painful ophthalmoplegia may be observed (sphenoidal fissure syndrome, Collier's syndrome, syndromes involving the orbital apex, the cavernous sinus and parasellar syndromes; Raeder's syndrome, Gradenigo's syndrome and Fischer-Brugge syndrome). They then discuss the various causes that must be investigated in all cases of painful ophtalmoplegia. They consider in order: -- ophtalmoplegia due to general causes (especially diabetes) and neurological causes (e.g. multiple sclerosis); -- ophtalmoplegia due to common local canses space-occupying processes, vascular malformations, ear, nose and larynx infections); -- painful ophalmoplegia of unknown origin, which includes four entities of very differing importance (Gubler and Charcot's ophthalmoplegic migraine and Tolosa-Hunt syndrome of which the clinical symptoms and course are so different that they can be distinguished as two entities; and, secondarily, inflammatory pseudo-tumours of the orbit and the recurrent multiple cranial nerve palsies that are observed in South-East Asia). (Acta nurol. belg., 1977, 77, 331-350).
The authors report an observation of dominant generalized cortical hyperostosis with multiple and unilateral involvement of a certain number of cranial nerves. In other members of the family, who present with the same bone disorder, involvement of the cranial nerves was limited to the facial nerve, either uni or bilaterally, with a partially or totally regressive evolution. This cranial nerve involvement, which is classic in the recessive form (first described) of generalized cortical hyperostosis, has until now, never been reported in the dominant form of this disorder.
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The authors report a case of myasthenia gravis in which a tumour of the sella turcica, without clinical symptoms, was discovered on X-ray examination of the skull and was removed with success. Histological study showed endocrine and neural type cells with cells of intermediate type. Subsequent thoracic surgery was performed with removal of a thymoma which was not visible on X-ray examination of the chest. The first operation on the sella turcica had no effect on the myasthenia but after the second on the thymoma a definite improvement was noted. The association of myasthenia, thymoma and ganglioneuroma of the sella turcica is unknown. In order to explain this, the authors hypothesize a common origin in the neural crests of both tumours.
The authors present two clinical observations of amyotrophic lateral sclerosis (A.L.S.) in which a loss of voluntary control was associated with retention of automatic and reflex activity of muscles innervated by cranial nerves. In the first case the face and swallowing were affected, in the second the movements of the eyes. These phenomena are exceptional in A.L.S.
A case of Arnold-Chiari malformation plus syringomyelic syndrome with hypertrophy of upper left and lower right limbs is reported. The pathogenesis of this muscular hypertrophy is discussed. The authors retain the idea of an added malformation.
A case of the syndrome of Tolosa-Hunt is presented in which the points of interest were the high number of attacks (four in two years and half) and the varying side of the symptoms with an involvement of the left sixth cranial nerve during the first, third and fourth attacks, and of the right third cranial nerve during the second attack. The trigeminal pain was always on the same side as the ophthalmoplegia. Bilateral carotid and vertebral angiography, orbital phlebography and air encephalography did not reveal anomalies. The sedimentation rate was moderately high during each attack. Corticotherapy begun during the last attack was very effective against the pain, but less so against the paralysis. The authors review the literature concerning this rare syndrome, the cause of which is thought to be an inflammatory process involving the cavernous sinuses.
A man presented at 27 years of age, the first signs of a progressive neurological and mental deterioration which eventually lead to his death at 37, from an intercurrent pulmonary infection. He was a severe alcoholic. The pathological examination of the brain revealed patchy demyelination and deposits of crystalline material, resembling so called calcifications. The biophysical examination showed the presence in the brain and other organs, of aluminum and phosphorus in all deposits, sometimes associated with iron, sulfur and calcium. Nothing in his past history suggests any contact with aluminum. Presumably this is a case of "generalized aluminosis", with the main clinical and pathological features manifesting in the central nervous system.
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