A polygraphic study on resistance to habituation of the somatic, autonomic and EEG components of the orienting reaction elicited by a repetitive auditory stimulus was performed in 39 epileptics with therapy-resistant generalized seizures (TRGS) and in 119 matched subjects in two control groups. The study showed a significantly higher resistance to habituation of the orienting reaction in epileptics with TRGS vs. the normal subjects of control group I. A significantly higher resistance to habituation was also noted in epileptics with therapy-controlled generalized seizures of control group II. The correlational analysis of data showed that the severity of these habituation disturbances in epileptics with TRGS depended on the patients' age, type of electroclinical seizures, pretrial seizure frequency, type of resting EEG, administered treatment (no. of administered antiepileptic drugs/patient), daily dose, as well as on the serum level of these drugs. The multivariate regression analysis showed that the most significant predictive variables for the habituation disturbances were the pretrial seizure frequency, type of electroclinical seizures, daily dose of administered antiepileptic drugs and their serum level. The data also evidenced that the antiepileptic treatment improves the interictal habituation disturbances, the effect being the more marked as the treatment was more sustained. The above-mentioned habituation changes in epileptics with TRGS should be ascribed to some disturbances in nervous excitability.
Tomaculous neuropathy represents the morphological substrate of the recurrent familial neuropathy with liability to pressure palsies. Some ultrastructural changes characterizing the tomaculous neuropathy can occur as incidental aspects in other different neuropathies. Few tomaculous neuropathy cases with clinical aspect of chronic polyneuropathy without paretic episodes have been mentioned in the literature. In the present work, we report four cases who offered the morphological surprise of a true tomaculous neuropathy with 15-37% of the teased fibres bearing tomaculae sized: 55-106 microns/20-23 microns, on the background of a demyelinating neuropathy with 25-56% of the teased fibres showing segmental de- or remyelination. The clinical and electrophysiological diagnoses of these 4 patients were: HSMN type I (2 cases), HSMN type VIII (polyneuropathy associated with a cerebello-extrapyramidal syndrome -1 case), and a neurogenic scapuloperoneal syndrome (1 case). The specificity of the tomaculous neuropathy is discussed.
The influence of acetazolamide in patients with hemorrhagic stroke was assessed in 54 patients in comparison with the influence of other therapies in 68 patients included in a control group. Modified Rankin Scale and mortality rate were evaluated at three different moments: onset, 72 hours and control (3 weeks-one month from the onset). A better outcome was seen when acetazolamide was given. Mortality rate was significantly lower in the group of acetazolamide. This therapy may be safely used in haemorrhagic stroke, especially when hydrocephalus is associated.
The effect of large glucocorticosteroid hormones administered to two groups of adult rabbits with an experimental acute injury of the spinal cord was studied. In the former (10 rabbits), each animal received intravenous 30 mg/kg body weight of prednisolone-21-sodium-hemisuccinate after 30 minutes and further 15 mg/kg b.w. at intervals of 2, 24 and 48 hours since injury. In the latter (40 rabbits), each animal received 15 mg/kg b.w. methyl-prednisolone-21-hydrogen-sodium-hemisuccinate at 30 min, 2, 24 and 48 h postinjury. A group of 20 injured rabbits with the same experimental conditions was used as controls without glucocorticoid treatment. Clinically, both treated groups of rabbits showed a significant improvement of posterior limb movements and in the general state of health compared with the controls. The mean motor score (according to a 5-score scale) indicated a significant difference between the two treated groups (2.3 and 2.6, respectively and 1.7 for the controls). Histologically, the modifications correlated with the clinical posttraumatic symptomatology and severity in all the three groups: histopathological modifications consisted in oedema, ischaemic cells, diffuse microglial hyperplasia, microhaemorrhages, all of them located in the central gray matter. In all cases with a severe clinical evolution (score of 1-3), aggravated histopathological modifications both in the gray and white matter are evidenced: necrosis; vacuolization; cavitation; myelinic and axonal fragmentation, demyelination, mesenchymo-glial scar reaction. In all groups, regenerated nervous fibres were noticed in the dense scar of the injured cord.
The problem of the late EP components generator localization has not yet been solved. This study aims to find out if a focal cortical epilepsy of the frontal (FLE) or of the temporal lobe (TLE) can modify the P300 component of the evoked response (either visual or auditory). Our data rely mainly on the selective differentiation test of an 80 dB click alternatively applied on the right or left ear, the patient having to give a motor response to those stimuli which have been chosen as target stimuli (probability of administration, 1:4). Under these circumstances, in normal subjects, evoked potentials showed deep P300 and an attenuated N2b in case of target stimulation and high, delayed N2b and an attenuated P300 for the nontarget stimulation, FLE did not abolish the late components of the EP; on the contrary, these were significantly augmented by the task. TLE, in exchange, severely attenuated differences between N2b-P300 components recorded from the scalp in case of target and nontarget stimulation. P300 was even higher at rest (possibly an effect of defective habituation). Rare or rapid stimuli application which was separately investigated gave another type of EPs modification compared to that specific for the auditory discrimination task. These facts are corroborated with the complete abolition of late EP components after lateral brain stem lesions. The conclusion is that N2a component has a reticular brain stem generator while N2b and P3 have initial generators in the temporal lobe (hippocampus).
Three cases with Wallenberg's syndrome were reported, in which CT-scan revealed silent old right temporal infarction, right recent cerebellar haematoma and left calcified parietal subdural haematoma evidenced after 10 years since trauma due to a traffic accident. Although Wallenberg's syndrome constitutes a fully clinical diagnosis, CT-scan does not seem to be justified by the management logical deductions of the medical act; however, it is better to perform it in order to identify a possible associated pathology.
Despite many efforts, the etiopathogenesis of ALS remains unknown. During the last decade evidence for an autoimmune involvement in motoneuron degeneration and death has remarkably increased. Multiple reports have documented significant expression of proteins associated with immune function in affected areas of ALS patients. Two animal models of immune-mediated motoneuron destruction have been developed that closely resemble clinical, electrophysiological and morphological features of human ALS. Inflammatory foci within the spinal cord, and IgG at the neuromuscular junction as well as within upper and lower motoneurons found in the animal models support the role of autoimmune mechanisms of motoneuron destruction in this model. IgG from ALS patients and from the animal models can passively transfer physiological changes at the neuromuscular junction in mice. That ALS IgG interact with calcium channels and induce an alteration of their function is now electrophysiologically and biochemically evident. Furthermore, it has been documented that motoneurons may be selectively vulnerable since they have a deficient calcium buffering capacity. Although further research efforts are necessary to elucidate the interaction of the ALS antibodies with the calcium channel function and how defective calcium handling by the motoneurons is important in their degeneration, the current data strongly suggest the involvement of autoimmune mechanisms in ALS etiopathogenesis.
The effect of forearm or calf ischemia on computerized EEG was studied in five patients with peripheral nervous system affections and five patients with cerebral infarction. Spatial analysis of power spectra in various frequency bands was performed in two 8-sec epochs, the former before ischemia and the latter after 5-min from the cessation of circulatory arrest which lasted 15 min. Transient peripheral ischemia resulted in consistent augmentation of alpha activity involving both the anterior and the posterior hemispheric areas, bilaterally. On the other hand, the slower frequency bands (theta, delta) underwent minor, nonsignificant alterations after ischemic stress. The rise of alpha amplitude induced by ischemic stress was ascribed to the activation of nonspecific thalamic system. The activation of this system might also be involved in the favourable effects exerted by ischemic stress on motor recovery of paretic limbs.
The catecholamine (CA) response to upright posture was studied in 30 brainstem infarct patients with orthostatic arterial hypotension; the investigation was made before and after 10 days propranolol therapy (in 15 cases) and before and after 10 days metoclopramide therapy (in other 15 cases). Before treatment almost all patients responded to posture by a rise in adrenaline (A) excretion and by a depression in noradrenaline (NA) excretion. Propranolol therapy prevented the excessive A release produced by standing and normalized their NA response to posture. Metoclopramide administration also prevented the post-orthostatic A discharge but had no significant influence on NA response to posture. Both drugs exerted a favourable influence on postural hypotension of investigated patients. As post-orthostatic A discharge observed in patients with postural hypotension is involved in the pathogeny of this syndrome and both metoclopramide and propranolol are able to correct this disorder one may maintain that the clinical favourable results obtained with these drugs are ascribable at least partly to their blocking effect on A release.
Four patients with severe muscle cramps were reported. In two of them the electrical signs of neuromyotonia were demonstrated. All the patients had undergone gastrectomy and all were immoderate consumers of alcohol. The neuromuscular disorder was reversible in all the patients.
The initial objective of the present study was to investigate the role of excitatory and inhibitory amino acids in generalized as compared to focal epilepsy, both forms being induced by the same convulsant agent, i.e. penicillin. Our attempts to obtain in the rat the generalized epilepsy, constantly induced in cats by systemic administration of penicillin, were unsuccessful. This is probably due to the rudimentary development of the cerebral cortex in rodents as compared to the feline cortex. The tentative conclusion was drawn that the cortex is the brain structure mainly involved in the genesis of petit mal seizures. Penicillin was applied to the cortex of 40 white Wistar rats and the electrical cortical activity was registered. The concentrations of glutamate, aspartate, glycine, GABA and serine were determined in the cerebral cortex, the brain stem and the cerebellum. The same amino acids were determined in the brain of 20 controls. No significant changes in the amino acid contents were obtained in the cerebral cortex. In the brain stem the glutamate level was significantly increased while the glycine content was markedly decreased. These findings are consistent with the involvement of the brain stem structures in seizure activity.
Melodic Intonation Therapy is a well-known method exploring the verbal encoding of aphasics but within this study, it was used to investigate the verbal decoding (the auditory comprehension). Two separate groups, 240 cases each, were investigated, the former with MIT and the latter with other therapy methods. Each group included three subgroups according to the three frequent types of aphasia (Wernicke, Broca and Anomia). The method of semantic fields was associated to treat the second group since it is usually used in the treatment of aphasics with auditory decoding disturbances. All patients were tested twice, before and after therapy.
Sixty-four muscle biopsies obtained from patients with Duchenne muscular dystrophy, limb-girdle dystrophy, congenital muscular dystrophy, or who referred for diagnosis were examined with histochemical methods for dystrophin staining with antidystrophin antibodies. Six atypical cases in whom the dystrophin expression was inconsistent with both clinical and morphopathological pattern were selected: one case of Duchenne muscular dystrophy in whom dystrophin was partially represented, one case referred for investigation in whom dystrophin was absent, despite the lack of clinico-morphological signs supporting the diagnosis of Duchenne disease, two cases of congenital muscular dystrophy in whom dystrophin was absent and one patient with the same diagnosis in whom dystrophin was present, but quantitatively reduced. A patient with limb-girdle muscle dystrophy, in whom immunohistochemical investigation was necessary for the diagnosis is also reported. In conclusion, the immunohistochemical investigation using antidystrophin labelled antibodies of muscle biopsies obtained from patients with various types of muscular dystrophy is mandatory.
Anticardiolipin antibodies (ACL-A) are acquired antiphospholipid antibodies characteristically found in patients with systemic lupus erythematosus or related autoimmune diseases. Several reports have shown that there may be an association between ACL-A and various neurological disorders, in particular cerebral ischemia. Using a micropin enzyme linked immunosorbent assay we measured the levels of ACL-A in the sera of 225 unselected patients with various neurological disorders. The prevalence of ACL-A in the whole group was 4.0% (9/225). However, the prevalence in patients with ischemic cerebrovascular disorders was 9.1% (5/55). With one exception (thrombocytopenia was found more often in ACL-A-positive cases) there was no difference with respect to the prevalence of risk factors for stroke and associated diseases between ACL-A-positive and ACL-A-negative patients with TIA/stroke. High titers of ACL-A were also found in a few patients with epilepsy (n = 2), migraine (n = 1), and intracranial meningioma (n = 1). In patients with ischemic cerebrovascular disorders search for ACL-A may help to identify patients with a possibly higher risk of thrombosis.
Neoplasms are relatively rare in the lumbosacral region. The present study presents two cases with tumors in the lumbosacral region, a chordoma and a tumor with multinucleated giant cells which further showed a presacral development. Some common aspects of the clinical and radiologic symptomatology were evidenced as well as the specific characteristics of each separate tumor in our cases. The diagnosis was possible due to the modern radiologic equipment.
The effect of antihypertensive therapy withdrawal in acute ischaemic stroke patients was assessed in order to establish possible correlations between blood pressure values and the modified Rankin Scale (mRS) outcome. One hundred and twelve consecutive patients with acute ischaemic stroke and hypertension were treated with relatively similar regimen. Seventy-two hours following the onset of stroke, systolic blood pressure decreased to < or = 180 mmHg in 110 patients. Antihypertensive therapy was randomly discontinued in 59 patients. Clinical outcome and mortality were compared in patients with and without antihypertensive therapy withdrawal, no statistically significant differences (p > 0.05) between the two groups being found. Statistically significant positive correlations (p < 0.05) between mRS outcome and the decrease of systolic and diastolic blood pressure values were in patients treated with anticoagulant and antihypertensive therapy. Statistically significant negative correlations (p < 0.05) between mRS outcome and low levels of diastolic blood pressure were noted in patients who discontinued antihypertensive therapy. Our findings emphasize the need to associate antihypertensive and anticoagulant therapy in acute ischaemic stroke patients with hypertension. Spontaneous decrease of diastolic blood pressure values showed poor outcome. The management of hypertension associated with acute ischaemic stroke should be reconsidered.
A polygraphic study of the somatic, autonomic and EEG components of the orienting reaction elicited by an auditory stimulus was performed in 39 epileptics with therapy-resistant generalized seizures (TRGS) and in 119 matched subjects in two control groups. The study showed a significant interictal hyperresponsivity in epileptics with TRGS vs. the normal subjects of control group I, which consisted in a marked increase of the intensity of the orienting reaction components. This hyperresponsivity was also more marked than that noted in epileptics with therapy-controlled generalized seizures of control group II. The correlation analysis of data showed that the severity of these responsiveness disturbances in epileptics with TRGS depended on the patients' age, type of electroclinical seizures, pretrial seizure frequency, type of resting EEG, administered treatment (no. of administered antiepileptic drugs/patient), daily dose, as well as on the serum level of these drugs. The multivariate regression analysis showed that the most significant predictive variables for the responsiveness disturbances were the pretrial seizure frequency, type of electroclinical seizures, daily dose of administered antiepileptic drugs and their serum level. The data also evidenced that the antiepileptic treatment improves the interictal responsiveness disturbances, the effect being the more marked as the treatment was more sustained. The above-mentioned responsiveness changes in epileptics with TRGS should be ascribed to some disturbances in nervous excitability.
