Auriculo-ventricular block and distal myopathy with rimmed vacuoles and desmin storage.

A young patient had an auriculo-ventricular block and a distal myopathy with muscle biopsy findings suggestive of inclusion body myositis. What was most unusual was the presence of numerous sarcoplasmic bodies identified as desmin by electron microscopy and immunocytochemistry. The nosological situation of this condition is discussed.     

Altered excitatory and inhibitory amino acid receptor binding in hippocampus of patients with temporal lobe epilepsy.

We examined binding to excitatory amino acid and inhibitory amino acid receptors in frozen hippocampal sections prepared from surgical specimens resected from 8 individuals with medically refractory temporal lobe epilepsy. The excitatory receptors studied included N-methyl-D-aspartate (NMDA), strychnine-insensitive glycine, phencyclidine, and quisqualate. The inhibitory receptors studied were gamma-aminobutyric acid type A (GABAA) and benzodiazepine. Excitatory and inhibitory amino acid receptor binding were differentially altered in the patients with temporal lobe epilepsy in comparison to 8 age-comparable autopsy control subjects, and changes in receptor binding were regionally selective in four areas. Binding to phencyclidine receptors associated with the NMDA channel was reduced by 35 to 70% in all regions in the hippocampi of the patients. In contrast, binding to the NMDA recognition site and its associated glycine modulatory site was elevated by 20 to 110% in the cornu ammonis (CA) 1 area and dentate gyrus of the hippocampus of the patients. Binding to these sites was unaffected in area CA4. Binding to the quisqualate-type excitatory amino acid receptor was unchanged in all regions except the stratum lacunosum moleculare CA1, where it was increased by 63%. GABAA and benzodiazepine receptor binding was reduced by 20 to 60% in CA1 and CA4, but unchanged in dentate gyrus. The data indicate that excitatory and inhibitory amino acid receptors are altered in the hippocampus of patients with temporal lobe epilepsy.     

Increased lymphocyte beta-adrenergic receptor density in progressive multiple sclerosis is specific for the CD8+, CD28- suppressor cell.

Beta-adrenergic receptor density on T cells from healthy humans is greatest on suppressor cells (CD8+, CD28-) and the effect of catecholamines, secreted by the sympathetic nervous system, predominates on this subset. The sympathetic skin response, a measure of sympathetic nervous system function, is absent in most patients with chronic progressive multiple sclerosis (MS). We measured beta-adrenergic receptor density on suppressor cells, cytotoxic cells, and monocytes from patients with chronic progressive MS and healthy control subjects. Control receptor density on suppressor cells was 2.8 +/- 0.3 fmol/10(6) cells versus a density of 5.1 +/- 0.7 fmol/10(6) cells for patients. Cytotoxic cell (CD8+, CD28+) receptor density was 1.4 +/- 0.4 fmol/10(6) cells in control subjects and 0.9 +/- 0.3 fmol/10(6) cells in the patients. Monocytes displayed beta-adrenergic receptor densities of 2.6 +/- 0.4 fmol/10(6) cells in normal individuals and 2.7 +/- 0.4 fmol/10(6) cells in the patient group. CD8 lymphocyte beta-adrenergic receptor densities in patients with relapsing-remitting and those with stable MS were not different from control values, yet were significantly less than the values for patients with chronic progressive MS. We find that mononuclear cells from healthy control subjects and patients with chronic progressive MS proliferate in response to 200 units/ml of recombinant human interleukin-2 (IL-2) similarly. However, IL-2 treatment increased beta-adrenergic receptor density on normal mononuclear cells, but failed to increase it on mononuclear cells from patients with chronic progressive MS.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pleuropulmonary disease associated with dopamine agonist therapy.

Artificial dopamine agonists are widely employed for the treatment of idiopathic parkinsonism. Pleuropulmonary disease has previously been reported to occur with the use of bromocriptine and mesulergine. We report similar adverse effects induced by the newer agonists lisuride and cabergoline. All these agents are tetracyclic ergot derivatives. This suggests a causal link between ergot-derived dopamine agonists and pleuropulmonary disease.     

Postencephalitic acquired Tourette-like syndrome in a child.

We report a 6-year-old girl who developed a Tourette-like syndrome following presumed herpes encephalitis. This case suggests that tics can be acquired in childhood and may be associated with lesions involving basal ganglia or limbic circuitry.     

Dynamics of functional interconnections between cerebral motor cortex neurons of cats under stimulation

In acute experiments on cats the activity of the motor cortex neurons was recorded. The dynamics of interneuronal connections at passive bending and electrostimulation of the limb contralateral to the place of recording was studied by methods of cross-correlation analysis of impulse trains. At passive bending a considerable decrease of the number of common inputs between neighbouring neurons (within 50-100 microns) and an increase in their number between remote (from 100 till 400 microns) cortical neurons. During electro-stimulation a certain increase in the number of direct inhibitory interactions between the neighbouring neurons was observed.     

Use of CT in epilepsy: does it matter how long you wait?

Computer tomography (CT) of the brain is of value for finding potentially correctable lesions in adult patients with new onset seizures. The value of CT is unknown, however, for finding such lesions in adult chronic epileptic patients without prior CT. We compared a group of 177 adult patients who had CT within a year from the onset of seizures to a group of 93 patients who had a history of seizures for more than a year before CT was performed. In the first group, 33 potentially correctable lesions (19%) were found including 17 tumors. The group with chronic epilepsy had 4 (4%) potentially correctable lesions: 3 arteriovenous malformations and 1 meningioma. It seems that CT is of value in discovering potentially removable lesions in chronic epileptic patients, but the likelihood is relatively small. The incidence of stable structural lesions seems to be similar in the two groups.     

Dissection of the extracranial vertebral artery: report of four cases and review of the literature.

Four cases of cervical vertebral artery (VA) dissection are reported. In three patients VA dissection was associated with neck trauma. All patients were young or middle-aged (range 27 to 49 years). In two there was a history of migraine. Pain preceded neurological symptoms from hours to six weeks. Three patients had neurological deficits including elements of the lateral medullary syndrome, and one experienced recurrent transient ischaemic attacks in the vertebrobasilar territory. Angiographic findings included irregular stenosis, occlusion and pseudoaneurysm; in two patients VA abnormalities were bilateral. All patients were treated with anticoagulants and improved. In a review of 28 cases with traumatic dissection and 29 cases with spontaneous dissection of the VA reported in the literature, distinct clinical and angiographic features emerge. Aetiology remains obscure in most cases of spontaneous dissection and management is still controversial.     

Test-retest reliability in EEG frequency analysis.

This study was performed to gain a better understanding of EEG frequency analysis test-retest reliability in normal healthy adults, and to evaluate factors which could influence the measured inter-record differences. Nineteen subjects underwent serial EEG recordings at 5 min and 12-16 week intervals. Records were visually edited using a standardized protocol, and FFT frequency analysis performed on segments of 60, 40, or 20 sec total length. Correlation coefficients for broad band features averaged 0.92 over the 5 min retest interval and 0.84 over the 12-16 week interval. There was essentially no difference between correlation coefficients of absolute and relative power features. Coefficients based on 60 sec records were marginally higher than those of 40 or 20 sec records. On the other hand, test-retest percent differences were typically lower for relative as opposed to absolute power features, and 60 sec records showed consistently lower percent differences than did 40 or particularly 20 sec records. Peak alpha frequency and mean frequency were the most stable EEG features at either interval. Montage had significant effects on test-retest differences at the 12-16 week interval. A significant association between intra-record and inter-record variability could not be demonstrated.