Biomedical subjects
Publications and source records attributed to D Leys.
Three patients developed chorea while receiving oral contraceptives. Two were young patients whose chorea developed long after treatment had been started and disappeared soon after it had been discontinued. The third patient had acute amphetamine-induced chorea after prolonged oral contraception. Prolonged administration of female sex hormones is a possible cause of chorea in women who have not previously had chorea or rheumatic fever.
A previously healthy 35-year-old man suddenly developed vertebrobasilar ischaemia while playing tennis. Cerebral arteriography revealed a dissecting aneurysm of the cervical portion of both vertebral arteries. The neurological deficit and the angiographic features resolved with anticoagulant treatment alone. Only 15 patients with non-traumatic extracranial vertebral artery dissection have been previously reported. It is necessary to recognize the condition very quickly, because the authors consider that anticoagulation is required as an emergency.
The patient reported had had a continuous headache involving the whole skull for 7 years. Many drugs had failed to relieve the pain, but with indomethacin the headache completely disappeared within 3 days. Nine months later the treatment was discontinued without any relapse. This variety of headache, not previously reported, was quite similar to the "hemicrania continua" except for its localization.
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The authors report 6 cases of thoracic disk herniations in patients with Scheuermann's disease. They underline the relationship between the neurological symptomatology and Scheuermann's disease by the herniation and evolution in their treatment by a new surgical approach.
Previously reported cases of peripheral neuropathies occurring during almitrine therapy had only a few weeks follow-up after having stopped the drug. We have studied clinical and electrophysiological data 6 to 12 months after almitrine had been given up in 9 patients from a group of 14 whose epidemiologic, clinical, electrophysiological and pathological data had been registered. In 7 of them, without any chronic respiratory deficiency, almitrine was administered as almitrine bismésilate and raubasine, and in 7 others (6 with chronic respiratory deficiency) as almitrine bismesilate alone. In patients who had another possible cause of neuropathy, clinical disorders appeared after a lesser total quantity of almitrine (p less than 0.05). Clinical data were suggestive of sensory peripheral neuropathies of feet and lower third of legs. Electrophysiological data suggested distal axonopathy in spite of the absence of denervation: amplitudes of sensory potentials were reduced and nerve conduction velocities were moderately decreased. Biopsies revealed mild neurogenic atrophy of muscles and distal axonopathy. Clinical improvement was very slow and 6 to 12 months later, most of the patients still presented decreased vibration sense and ankle reflexes loss, but all of them were still improving. Amplitudes of sensory potentials and sensory nerve conduction velocities were significantly improved (p less than 0.05) but motor nerve conduction velocities were not (p greater than 0.05). Our study shows: 1) clinical, electrophysiological and pathological data similar to those previously reported; 2) subclinical disturbances of motor functions in lower limbs and sensory functions in upper limbs; 3) some patients presented with unusual signs: posture tremor (3 cases), painful legs and moving toes (1 case); 4) peripheral neuropathies may occur during almitrine therapy even in patients without any chronic respiratory insufficiency; 5) peripheral neuropathies occurred with lower doses in patients with other factors predisposing to neuropathies; 6) patients' improvement was very slow; 7) in 9 cases the imputability of these peripheral neuropathies to almitrine is plausible. We suggest not to prescribe almitrine without caution, especially in patients with other factors of neuropathy. Treatment should be regularly interrupted.
We report 4 cases of neuromyelitis optic (Devic's disease), with 3 to 7 year-follow-up for 3 of them. Most of the data agreed with the previously reported cases: subacute onset, association of optic neuritis and myelitis within several weeks or months, absence of relapse, good prognosis for paraplegia but poor prognosis for visual loss, and effectiveness of corticosteroids on spinal cord involvement in 4 cases. Moreover these 4 cases have revealed the following rare features: unilateral visual disturbance (1 case), total recovery of visual signs (1 case) with only a short period of follow-up, onset during childhood (1 case), onset following an acute lymphocytic meningitis (1 case), and dependency from corticosteroid therapy (2 cases). None of the patients had obvious immunological disturbance. N.M.R. imaging revealed in 2 cases, disseminated demyelinated areas which confirmed neuropathological data from the literature, showing that anatomical lesions could be wider than expected on clinical signs. The poor visual prognosis, the serum inflammatory signs, the epidemiologic data, the high cell and protein levels in C.S.F., and the absence of relapse suggested, in our cases and in most of the previously reported ones, that neuromyelitis optic must be differentiated from multiple sclerosis.
Thirty-four patients were operated for dorsal disk pathology by a postero-lateral approach. The first cases have been followed-up for over 4 years and have shown this method to be reliable. When associated with the "harpoon" marker technique, the surgical indications now include not only patients with progressive radiculo-medullary symptoms, but also cases of sudden decompensation and those limited to radicular symptoms.
A 57 year old man presented with a giant, fusiform, partially thrombosed aneurysm, located distally to the right angular gyrus artery, and revealed by a subarachnoïd hemorrhage. There was no history of head trauma, endocarditis or systemic infection, and physical examination was normal. The treatment consisted in simple trapping of the aneurysm, responsible for a transient ischemic parietal syndrome. After a short review of the pathogenesis of peripheral aneurysm of the C.N.S., and previously reported cases of distally located aneurysms of the middle cerebral artery, only one similar case was found concerning a posterior temporal artery aneurysm involving to a giant serpentine aneurysm. The case presented is believed to be a segmentary form of cerebral arterial dolicho ectasia; relations between arterial ectasia or fusiform aneurysms and serpentine aneurysms are discussed.
Diastematomyelia is rarely diagnosed in adulthood, and apparently 8 cases only have been reported. We report here a 59 year-old woman who recently experienced backpain with irradiations to lower limbs, mild distal weakness and sphincter disturbances. Diastematomyelia was suspected because of a history of lumbar "cutaneous malformation" operated when 4 month old, and a hairy patch over the lumbar spine. C.T. scan showed diastematomyelia and revealed a posterior spur of bone protruding into the sagittal plane from the posterior part of L3 and L4 and dividing the posterior part of the low inserted spinal cord. It appears that, when diastematomyelia has been diagnosed in adulthood, the level of the malformation was lower than when diagnosed in childhood.
A 23 year-old man, with a two month-history of back pain, presented with spastic paraplegia and hypesthesia of both lower limbs. A myelo C.T. revealed a spinal epidural dumb bell-shaped mass extending in the intra and extra vertebral spaces through the enlarged intervertebral foramen. Total recovery was observed three months after surgery. Pathological examination of the tumor showed it to be a cavernous angioma. Primary epidural angioma is rare: in 2 reported dumb bell-shaped spinal epidural cavernous angiomas, radiological findings were also suggesting a spinal neurinoma.
A 40 year-old Frenchman had had for 12 years, an amyotrophy of one upper limb. Clinical features were similar to those previously reported in Japan and India i.e. atrophy limited to one hand and forearm, with mild functional discomfort, and slow progression for 2 years after which the disorder did not progress. Electromyography showed disturbances of anterior horn cell type. The cause of this syndrome is unknown, no pathological case has yet been reported. The prognosis appears to be good.
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Neurinomas of the trigeminal nerve are rare. Based on an analysis of the published cases and on six additional cases of our own classification into three types is proposed: Type I: neurinoma of the roots in the posterior fossa, Type II: neurinoma of the Gasserian ganglion, Type III: neurinoma of the trigeminal branches. This classification allows a better adaptation of the surgical procedure to the individual case. The trigeminal nerve originates from a sensory root and a motor root which emerge from the pons and gain the middle cranial fossa floor over the apex of the petrousrigde before forming the Gasserian ganglion. The three branches of the trigeminal nerve originate at the Gasserian ganglion. Neurinomas of this nerve are relatively rare, as there are only 183 published cases. A study of the literature reveals that their incidence among brain tumours is from 0.2 to 0.4% according to the authors. Based on the published material and six personal cases and also on the anatomical, clinical and neuroradiological findings three major types of neurinomas of the trigeminal nerve can be distinguished according to their origin: neurinomas developed in the posterior fossa (type I) on the nerve roots, neurinomas of the Gasserian ganglion (type II) developed in the temporal fossa and neurinomas of the branches of the trigeminal nerve (type III). Transitional forms between the various types are possible and express special conditions of development. Each of these topographical forms has a specific clinical and radiological picture and each requires specific surgical treatment. This will be demonstrated by analysing our own six cases and the cases reported in the literature.
Seven patients with subdural empyema were initially treated by antibiotics without surgery. Six have recovered without sequelae. One required delayed surgery and has recovered with epilepsy. The authors emphasise the use of CT for the diagnosis and follow-up of subdural empyema, the principles and modalities of non-surgical treatment, and the good results, especially for late morbidity.
Nine patients with symptoms and signs of Refsum's disease are reported. In 6 a systemic accumulation of phytanic acid was demonstrated, together with low phytanic acid oxidase activity in skin fibroblasts in 5 of them. In 3, no disorder of phytanic acid metabolism was demonstrated. In 3, the diagnosis was made during the pre-clinical period. The disease seems more frequent in Northern France, which agrees with the hypothesis of a genetic mutation which would have taken place in Scandinavia some centuries ago and was subsequently spread by the Vikings. The effects of a dietary treatment on serum phytanic acid levels and clinical disorders are reported. The general condition of the patients improved remarkably but only partially. The diet is unpalatable and in some patients the level of serum phytanic acid increased, due to the mobilization of body fat. Patients with very high levels of phytanic acid might be initially treated by plasmapheresis. For the same reason, the diet should supply enough calories to keep body weight unchanged, and body weight loss whatever its cause should be avoided.
Three months after having stopped a corticosteroid therapy prescribed for disseminated sarcoidosis, a 23 year-old man presented signs of thoracic spinal cord involvement, with a lymphocytic reaction in the CSF. The hypothesis of a recurrence of sarcoidosis was evoked, and, after a few days course of corticosteroid, the patient recovered; he had no functional sequelae, and did not present any recurrence 2 years after having stopped the treatment. The main characteristics of spinal cord sarcoidosis are summarized: occurrence during a usually previously known sarcoidosis, within weeks or months, located spinal cord involvement, sometimes with a syringomyelic syndrome, and lymphocytic CSF reaction. In this case, the lesion was probably an arachnoidal infiltration without tumorous process of the spinal cord. Corticosteroid must be prescribed at once, and for a long time, because the results of surgery are always poor.