Antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors.

BACKGROUND: Antiamphiphysin antibodies react with a 128-kd protein found in synaptic vesicles. They were first described in patients with paraneoplastic stiff-man syndrome and breast cancer, but studies suggest that they can also occur in patients with other tumors and neurological disorders. OBJECTIVE: To determine if antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors. PATIENTS AND METHODS: Of 2800 serum samples tested by routine immunohistochemical procedures on sections of paraformaldehyde-fixed rat brain for the detection of autoantibodies associated with paraneoplastic neurological syndromes, 5 were selected because of labeling suggestive of antiamphiphysin antibodies and subsequently confirmed by the results of Western blot analysis using recombinant amphiphysin protein. Controls consisted of 40 patients with various nonparaneoplastic neurological diseases; 101 patients with cancer but without paraneoplastic neurological syndrome; 9 patients with small cell lung cancer, anti-Hu antibodies, and paraneoplastic neurological syndrome; 3 patients with M2-type antimitochondrial antibodies but no neurological disorder; and 30 normal subjects. RESULTS: Of the 5 patients with antiamphiphysin antibodies, patient 1 had sensory neuronopathy, encephalomyelitis, and breast cancer; patient 2 had limbic encephalitis, and small cell lung cancer was detected in the mediastinum after 24 months of follow-up; patient 3 had encephalomyelitis and ovarian carcinoma; and patients 4 and 5 had Lambert-Eaton myasthenic syndrome and small cell lung cancer (patient 4 subsequently developed cerebellar degeneration). None of the 5 had stiffness. Two patients (Nos. 2 and 4) had antimitochondrial antibodies. The two patients (Nos. 4 and 5) with Lambert-Eaton myasthenic syndrome had antibodies directed against the voltage-gated calcium channel, and patient 2 subsequently developed anti-Hu antibodies. In the controls, antiamphiphysin antibodies were detected by Western blot analysis in 3 of 8 patients with anti-Hu antibodies, but in none of the other groups. CONCLUSIONS: These data indicate that antiamphiphysin antibodies are not specific for one type of tumor or one neurological syndrome and can be associated with other neural and nonneural antibodies. The simultaneous association of several antibodies in some patients suggests multimodal autoantibody production.

[Early diagnosis of Guillain-Barré syndrome by electric stimulations of the nerve roots]. / Diagnostic précoce du syndrome de Guillain-Barré par stimulations électriques radicularies.

Ulnar nerve electrical stimulations from motor roots to wrist were used in the early Guillain-Barré syndrome (GBS), to improve the electrophysiological diagnosis yield. 22 patients with a GBS were investigated with this technique between 3 to 17 days after the onset. Conventional electrophysiological examination was sufficient to diagnose an Acute Inflammatory Demyelinating Polyneuropathy in 12 cases. In 10 other patients, standard examination remained negative or not conclusive, and vertebral electrical stimulations were decisive for the diagnosis, showing conduction bloks. This method could be routinely performed for an early diagnosis, in association with conventional motor conduction measurements. This study confirms the possibility of a pure proximal demyelinating impairment of the peripheral nervous system in the early GBS.

Monitoring of the motor pathway during spinal surgery.

Paraplegia caused by irreversible lesions of the spinal cord is one of the major possible complications after scoliosis surgery. Several monitoring methods have been proposed but none are completely satisfactory. Since 1986 the authors assessed motor pathways during scoliosis surgery, using electrical stimulation of the motor cortex and lower limb muscle recordings (tibialis anterior muscle). Twenty-seven patients were included in this study: 25 with idiopathic scoliosis and 2 with dorsal kyphosis. Recordings in anesthetized patients with hypothermia were performed before and after spinal derotation during the surgical procedure. Magnetic cortical stimulation was carried out in ten awake patients before and after surgery. Reproducible responses were obtained in 22 patients under anesthesia. In eight patients no difference of the latency of the muscle response was detected before and after the correction of the spinal angulation. In 14 patients the increase of latency ranged from 0.4 ms to 5.2 ms. No correlation was found between the slowing of motor conduction and the magnitude of spine correction. No central neurologic complications were seen after surgery. The authors concluded that their study demonstrated that motor pathway assessment in anesthetized patients can be performed at different times during the surgical procedure. This technique should help in the future monitoring spinal function during scoliosis surgery.

Improvement in motor evoked potentials and clinical course post-steroid therapy in multiple sclerosis.

Motor evoked potentials (MEP) were recorded in 23 patients with definite relapsing multiple sclerosis before and after treatment with a short course of high dose of methylprednisolone. MEP were performed together with clinical examination just before treatment, and 6 and 60 days later. The following results were observed: (1) a statistically significant relationship between the corticospinal deficit and the alteration in MEP, (2) a significant improvement in latency of MEP by day 6, (3) a significant correlation between the change in the Kurtzke disability scale rating and the improvement in MEP. The results provide further evidence for the possible effectiveness of short courses of high dose corticosteroids in the treatment of relapses of multiple sclerosis and the usefulness of MEP in its assessment.

Central motor conduction and glutamate deshydrogenase: activity in olivo-ponto-cerebellar atrophy.

Central motor conduction was investigated by way of magneto-electric cortico-spinal stimulation in 6 patients with sporadic olivo-ponto-cerebellar atrophy. Two patients were found to have reduced leucocyte GDH activity. Only the 3 patients with corticospinal deficits displayed increased central conduction rates, which were predominant in the lower limbs. The duration of the disease is statistically longer in patients with corticospinal deficit compared to patients with no corticospinal deficit. In OPCA, evoked motor potentials are useful in assessing the corticospinal deficit which does not appear to be linked to reduced leucocyte GDH activity.

[Epidemiology of amyotrophic lateral sclerosis in the Limousin area]. / Epidémiologie de la sclérose latérale amyotrophique en Limousin.

Several epidemiologic studies of amyotrophic lateral sclerosis have shown a uniform repartition over the world. We report the results of a retrospective study and the preliminary results of a prospective study carried out in the Limousin area. The mean incidence per/year is 0.97/100,000 in the retrospective study and 1.3/100,000 in the prospective study. Such data are similar to those previously reported. The two major findings are the high frequency of bulbar palsy and the high mean age of the patients. This can be associated with the high mean age of the population in the Limousin area as compared to other regions.

[Study of central motor pathways using cortical magnetic stimulation and spinal electrical stimulation: results in 20 normal subjects]. / Etude de la voie motrice centrale par stimulation magnétique corticale et électrique spinale: résultats chez 20 sujets normaux.

Evoked motor potentials can be elicited by magnetic cortical or electric spinal stimulations. The central conduction time (CCT) corresponds to the difference in latencies between the total conduction time (from cortex to muscle) and the peripheral conduction time (from spinal cord to muscle). CCT is the sum of the conduction time in the cortico-spinal fibers, of the spinal synaptic delay, and of the conduction time in the proximal part of the motor roots. CCT values (mean + standard deviation) were determined in 20 healthy subjects ranging from 21 to 56 years of age (mean 31.2). Results of magnetic cortical stimulation were compared to the results of electrical stimulation of the cortex. CCTs after magnetic cortical stimulation were longer than CCTs after electric cortical stimulation. This could be explained by the fact that electrical stimulation elicits a direct response in the cortico-spinal tract whereas magnetic cortical stimulation has indirect effects on the pyramidal cells of the motor cortex through excitatory interneurons. Compared with electrical stimulation, the magnetic stimulation has the great advantage of being painless and allows a safe evaluation of the central motor pathways in man.

Early vesico-ureteral reflux following conus medullaris injury: case report.

The authors describe severe vesico-ureteral reflux and simultaneous renal insufficiency which occurred after a spinal cord injury to the conus medullaris. They point out the misleading character of these injuries when there are only minimal neurological signs in the trunk and limbs. The main clinical consequence may be an isolated neuropathic bladder which, if not detected, delays treatment. Additionally, they underline the role of mixed bladder and sphincter lesions in the development of renal insufficiency; also the role of increased intravesical pressure during filling and emptying with bilateral vesico-ureteral reflux.