Romanian journal of neurology and psychiatry = Revue roumaine de neurologie et psychiatrie最新文献

Motor conduction velocity (MCV) alone cannot separate all the cases with types I and II of hereditary motor and sensory neuropathy (HMSN). However, sensory conduction velocity (SCV) in sural nerve distinctly separated types I and II of HMSN (8). As in most of our patients with HMSN sural nerve was unexcitable, we introduced SCV estimation in the distal segments of median and ulnar nerves. So, we studied 124 patients from families with typical and uncomplicated cases of Charcot-Marie-Tooth disease (CMTD): 68 patients had the "hypertrophic" form (type I) and 56 cases the "neuronal" form (type II). In this series, 16 patients had median MCV from 35 to 45 m/s, but SCV in the median and ulnar nerves separated 7 cases with type I and 9 patients with type II of HMSN. In conclusion, the type I and II of HMSN were delimited in most of the studied cases by MCV values in the median nerve. Nevertheless, in cases difficult to be classified either into type I or II of HMSN, i.e. patients with MCV from 35 to 45 m/s, only SCV measurements in distal median and ulnar nerves segments can distinctly separate type I (a slowing over 40% from control values), indicating that this type is underlain by a process of segmental demyelination (SD), which also was confirmed by sural nerve biopsy data. In our cases with HMSN type II MCV was either normal or slightly slowed. By contrast SCV was significantly slowed (a slowing of up to 30%). In addition, we have also a few cases of complicated HMSN forms (rare variants), associated with: 1. Isaacs' syndrome; 2. "denervation-reinnervation" muscle hypertrophy; and 3. Marinesco-Sjögren syndrome. 1. In 3 patients, the clinical features of "neuronal" form of CMTD were associated with fasciculation, cramps, impaired muscular relaxation, and percussion myotonia with respective electromyographic (EMG) accompaniments, which were responsive to valproic acid therapy. 2. On the other hand 3 patients developed in addition to the Isaacs' syndrome a significant "denervation-reinnervation" muscle hypertrophy, confirmed by both morphometric data on muscle biopsy and computed tomography. In these patients there was an increased proportion of type I and a decreased one either of type IIB or of type IIA fibres, without myotonic or dystrophic features.(ABSTRACT TRUNCATED AT 400 WORDS)

This paper contains a clinical, classical electroencephalographic and computerized electroencephalographic (by original methods) study of 271 cases with vertebro-basilar strokes, all the cases being verified by transcranial Doppler ultrasound investigations and some of the patients by morphopathological studies. In the first part, the standard EEG modifications in relation with the clinical pictures and with the affected arterial field are presented. In the second part, the aspects of the computerized EEG recordings with the carrying out of the cortical EEG mappings which have brought important contributions in setting down the topography of the infarctions are discussed. The third part presents the results of the polysomnographic recordings of the 70 selected cases with ischaemic vertebro-basilar attacks without disorders of consciousness (32 cases), with attacks accompanied by symptomatic hypersomnias (13 cases), with ischaemic attacks accompanied by insomnia (5 cases) and with ischaemic attacks with disorders of consciousness (20 cases). The opinion of the authors on the possible relations between the disturbances of the vigilance, the perturbations of the polysomnographic organization of sleep-wakefulness cycles (with the realisation of some interesting competitive dysfunctions between the three states of vigilance) and the electrographic anomalies are discussed.

The present paper presents three short clinical observations of patients with cerebral trauma and concussion also showing transient motor deficits of upper limbs but no sensory disorders. The etiopathogenesis of motor brachial deficits is also discussed. The conclusion is that the sudden anterior flexion of the head determines an instantaneous hit of the pyramidal bulbar decussation (the upper part) to the odontoid apophysis or to the anterior margin of the occipital foramen. Recovery depends on the severity of trauma. This clinical entity is very rare. There are other pathologic causes which can trigger a transient brachial diplegia, thus, requiring a differential diagnosis.

Clinical, electrophysiological and morphological (sural nerve and gastrocnemius muscle biopsies) data of a 57-year-old man with a chronic sensorimotor polyneuropathy of Charcot-Marie-Tooth type associated with a progressive cerebello-extrapyramidal syndrome are reported. Patient's family data were negative. Nerve structural and ultrastructural examinations revealed the morphological picture of a tomaculous neuropathy. The association of different clinical syndromes and the specificity of the tomaculous neuropathy are discussed.

The present study is aimed to provide a new possible approach to the treatment of glioblastomas, in an effort to control the extremely severe evolution of these tumors. Thus, from May 28, 1991, we started the administration of IL-2 (produced at the "Victor Babeş" Institute, Bucharest) in 9 glioblastoma patients (operated on at the Neurosurgery department VII of the "G. Marinescu" Hospital, Bucharest) by repeated intraoperative and postoperative local injections. The survival period from the first IL-2 administration was 7 months (1 case), 8 months (2 cases), 11 months (2 cases), 14 months (1 case) and 17 months (1 case). There are only 2 survivors: one 4 months and the other 23 months following the first injection of IL-2. The survivor whose condition 23 months following introduction of IL-2 therapy is very good, demonstrates that IL-2 offers some chance to these hopeless patients.

Transient ischemia of the extremities was applied in compression or traumatic neuropathies affecting radial nerve (17 cases), ulnar nerve (3 cases), upper brachial plexus (4 cases) or peroneal nerve (10 cases). The limb opposite to that displaying paresis was submitted repeatedly to a 15-minute-period of ischemia every other day for two weeks. The procedure induced in most patients (27 out of 34 cases) a motor improvement of variable degree. In some patients (13 cases) the motor recovery occurred two days or more after starting the procedures, while in others (14 cases) during the very day in which the initial session of ischemia was made or even during the first hour of procedure application. The most beneficial effects of peripheral ischemia were noted in compression neuropathy of peroneal nerve palsy, 9 out of 10 patients with such a disorder being improved by the procedure. We suppose that the method of therapy proposed by us restores promptly the motility of patients with compression neuropathy by inducing a long-lasting activation of some central neural mechanisms.

Electrophysiological studies in cats have shown that one of the most common forms of sleep oscillation, spindling, may develop into spike-and-wave discharges through repetitive spike-bursts transmitted along thalamocortical or corticothalamic axons. The lowered frequency (from around 10 Hz to 3 Hz) can be explained by the abnormally increased duration of an IPSP mediated by GABA receptors. The administration of ethosuximide has a twofold effect resulting in a diminished spindling activity of the reticular thalamic nucleus as well as a decrease in the incidence of epileptic spike-and-wave discharges. Self-sustained paroxysmal activity may develop during EEG synchronized states by repetitively setting into action reciprocally connected structures.

A polygraphic study of the somatic, autonomic and EEG components of the orienting reaction elicited by an auditory stimulus was performed in 66 epileptics with therapy-resistant partial seizures (TRPS) and in 135 matched subjects in two control groups. The study showed a significant interictal hyperresponsivity in epileptics with TRPS 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 partial seizures of control group II. The correlational analysis of data showed that the severity of these responsiveness disturbances in epileptics with TRPS 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 TRPS should be ascribed to some disturbances in nervous excitability.

The syndrome of chronic vertebrobasilar insufficiency (VBI) consists in a complex of symptoms, often mild and transient, and lacks a complementary system of objective paraclinical investigations able to certify its existence. The study of somato-sensory, auditory and visual evoked potentials in VBI showed the occurrence of changes regardless of the symptom importance both in disorders due to extrinsic causes: a) compression by a damage of the cervical column (92.9%, 50% and 63.9%, respectively; b) diseases of the cervical spinal cord (92.9% and 46.6%, respectively) and in disorders due to some intrinsic vascular causes such as atherosclerosis (62.5%, 88.8% and 87.5%, respectively). We described: a) local and far field modifications for the somesthetic evoked potentials (SEPs); b) modifications due to brain stem hypoxia (vestibular and auditory centres) or of the internal ear for the early and middle auditory potentials (AEPs); c) modifications due to lower perfusion of the occipital lobes and of the nonspecific centres in the brain stem for the visual evoked potentials (VEPs). The N0-P0-Na component of the middle auditory response was considered to represent a vestibular response and it was significantly affected in cases with VBI and clinical manifestations like: equilibrium disorders, nystagmus and vertigo. Typical images of the sensory evoked responses affected by VBI are reproduced considering their evidence as an elective method in the paraclinical examination of this syndrome.

A computerized EEG topographic analysis (CETA) was carried out in 69 epileptics in order to evaluate the diagnostic value of such an investigation compared to that of the routine interictal EEG. The spectral frequency analysis of the bioelectrical activity in these patients was accomplished both by the fast Fourier transform and by the autoregression method. The obtained data were used for the spatial analysis of the electrical activity which was performed both by the interhemispheric coherence technique and by the color-coded topographic mapping in frequency bands. The data analysis showed that in 58 (84.1%) of the epileptics in whom routine EEG evidenced focal interictal bioelectrical abnormalities CETA also showed such abnormalities. However, CETA revealed focal abnormalities also in 8 (72.7%) out of the 11 epileptics in whom interictal EEG was interpreted as normal. The data analysis also showed that CETA was more efficient than EEG in the detection of focal abnormalities in the epilepsy with slow graphic elements, in the partial epilepsy, in the epilepsy with late onset and in the therapy-resistant epilepsy. The focal abnormalities evidenced by CETA were more expanded than those evidenced by routine EEG and the topographic maps indicated the focal involvement of a significant number of frequency bands. Considering the results, the authors make some appreciation regarding the diagnostic values of CETA in epilepsy.