Biomedical subjects
Publications and source records attributed to H Petit.
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.
In earlier open studies, beta-blockers were found to be effective in the treatment of neuroleptic-induced akathisia. In the present study, 16 patients with severe neuroleptic-induced akathisia successively received low doses of three beta-blockers--propranolol, sotalol, and betaxolol. There was rapid and complete improvement in seven of 16 patients (and partial improvement in three patients) treated with betaxolol. The efficacy of propranolol and betaxolol and failure of sotalol in treating neuroleptic-induced akathisia suggest a central mechanism of action.
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.
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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.
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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 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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A case of astereognosis and amyotrophy of the right hand is reported, which was caused by a large extradural neurinoma of the C2 nerve root. The quick and complete recovery which followed surgery suggested a vascular mechanism as the cause of the neurological deficits.
A syringoperitoneal shunt is a one-way drain that empties cerebrospinal fluid from the pathological syringomyelic cavity into the abdomen. This treatment technique is based on the principle that the signs and symptoms of syringomyelia are caused by insidious interference with the condition of nerve signals in the spinal cord tracts due to either tearing or compression of the tracts by the distended cavity. A series of eight patients suffering from this disorder were operated upon using a syringoperitoneal shunt. The initial results seem to indicate that this technique is especially useful when pain, atrophy, or both dominate the clinical picture. Clinical signs, diagnostic studies, and surgical considerations are discussed.
The amnesic syndrome is analysed in 6 infarctions restricted to the thalamic area. Bilateral lesions were linked to more definite deficits; the anterograde forgetting was unequivocal at the initial stage and affected both verbal and visual memory. In unilateral lesions, deficits were far more discrete and there was no evidence enough for assuming a strict hemispheric specialisation, left for verbal memory, and right for visual memory. These cases and others from the literature suggested that amnesia is more important and pure in infarctions of the anterior part of the thalamus.
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.
A case of septo-optic dysplasia, presenting with focal epileptic fits in a 29 year old man, is described. Gas encephalography and CT scan demonstrated the main features of the malformation: a single ventricle overhanging a narrowed third ventricle, abnormal mass in the basal forebrain corresponding to an embryonic cyst, optic atrophy. Angiography revealed deep venous anomalies, and, in particular, absence or hypoplasia of the sinus recti.