Neurologija最新文献

Hepatolenticular degeneration (Wilson's disease) is a hereditary disease in which metabolic disorder of copper leads to its accumulation in the liver, brain, cornea and kidneys with consequent pathologic changes in those organs. Hereditary mechanism of the disease is autosomal recessive with prevalence of 30-100 per 1,000,000 inhabitants. Etiology of this disease is not yet explained. There are two hypotheses. The first one is that it is the disorder of ceruloplasmine metabolism caused by insufficient synthesis of normal ceruloplasmine, or synthesis of functionally abnormal ceruloplasmine. The second one is: the block of copper biliar excretion which is the consequence of the liver lysosomes functional defect. Pathogenetic mechanism of disease is firstly long-term accumulation of copper in the liver, and later, when the liver depo is full, its releasing in circulation and accumulation in the brain, cornea, kidneys and bones, which causes adequate pathologic changes. Toxic activity of copper is the consequence of its activity on enzymes, particularly on those with -SH group. There are two basic clinical forms of the disease: liver disease or neurologic disease. Before puberty the liver damage is more frequent, while in adolescents and young adults neurologic form of the disease is usual. The liver disease is nonspecific and characterized by symptoms of cirrhosis and chronic aggressive hepatitis. The only specificity is hemolytic anemia which, in combination with previous symptoms, is important for diagnosis of the disease. Neurologic symptoms are the most frequent consequence of pathologic changes in the basal ganglia. In our patients the most frequent symptoms were tremor (63%); dysarthria, choreoathetosis and rigor (38%); ataxia and mental disorders (31%); dysphagia and dystonia (12%), diplopia, hypersalivation, nystagmus and Babinski's sign (6%). Among pathologic changes in other tissues and organs the most important is the finding of Kayser-Fleischer ring in the cornea as a result of copper accumulation. Its importance for precise diagnosis is great. The diagnosis of the disease is based on anamnesis, clinical examination, specific and nonspecific laboratory tests. The therapy of choice is penicillamine. If we use it early, the result will be good remission in the majority of patients. Late diagnosis or delay in treatment cause death which is the result of bleeding from esophageal varices or basal ganglia disease. Immunologic damages caused by penicillamine demand interruption of therapy and substitution by three-ethyl-tetra-amine (TETA). We also use zinc salts and tetratiomolibdate in therapy of this disease. Pathogenesis, clinical picture and therapy of the disease are based on our own results.

Fifteen patients with spasmodic torticollis were treated with local injections of botulinum--A toxin. All the patients were followed for a period of 4 to 7 months. Thirteen out of 15 patients (86%) improved in the amount of sustained movements of torticollis. In six out of 9 patients presenting with pain complete relief was noted. Beneficial effects of botulinum toxin injections lasted from 2 to 3 months, with reproducible efficacy after repeated injections. The most frequent side effect was dysphagia, presented in 10 patients.

The characteristics of common migraine are not unique, they also appear in other forms of benign headaches. As there are no laboratory tests and biological signs enabling the diagnosis of migraine and its differentiation from the headache due to muscle contractrion, clinical criteria which are simplified and easy to comprehend should be used. We compared the symptoms in 29 patients of both sexes, suffering from common migraine, as well as 29 patients with headache due to muscle contraction (chronic daily headaches). In common migraine the following symptoms appear in greater percentage (statistically significant): nausea, vomiting, unilateral localization of pain, pulsating pain, photophobia and phonophobia. Bad headache is more frequent in patients with common migraine than in those suffering from chronic headache. We should accept Solomon's and Cappa's attitude who suggest at least two of the following five criteria: 1. nausea with or without vomiting, 2. unilaterality, 3. pulsating pain, 4. photophobia or phonophobia, and 5. provocation by menstruation or positive family history. Besides the above mentioned criteria the authors also mention and emphasize the existence of free interval in migraine when the patient has no difficulties and feels well.

Various modalities of six neuropsychological functions (graphia, calculia, finger gnosis, right-left orientation, praxia and constructive praxia) referred to as parietal or nonverbal have been investigated in the light of speech disorders. We examined 20 patients with brain lesion of vascular origin, who met the diagnostic criteria of mild and moderate dysphagia, 13 patients with Wernicke's and 7 with Broca's dysphasia. Verbal and nonverbal functions in patients with ischemic focuses of the speech area of the left hemisphere were investigated the Boston Diagnostic Aphasia Examination (BDAE). The investigation revealed that the presence and the type of mild and moderate dysphasia had a noteworthy role in pathoplasticity of correlated signs, thus implying in clinical practice a parietal lesion. Generally, poorer and at the same time more heterogeneous results were obtained in patients with Wernicke's dysphasia, mostly on calculia and right-left orientation. Finger agnosia was not considered as an authentic parietal sign, while each modality of graphia was impaired to a varying extent in speech disorders caused by presylvian and retrosylvian lesions. The paper also deals with the significance of lobulus parietalis inferior in speech.

Nootropics are psychoactive substances that activate, protect and restore functions of the neuronal cells. Piracetam is the first representative of these compounds, often cited to act as an activator and protector of neuronal cells function, especially in hypoxic conditions. Therefore, we examined the effect of piracetam on the cortical neuronal activity in conditions when both carotid arteries have been ligated. We assumed that oligaemia occurred as the result of arterial obstruction and consequent hypoxic state developed. Evoked somatosensory potentials were used as an objective indicator of neuronal cells functional state. Our results demonstrate that the occlusion of both carotid arteries induces the extension of latency and reduction of the amplitude in the somatosensory evoked potentials of the adult cat. Piracetam (100 mg/kg i.v.) improves the evoked potentials returning both, latencies and amplitude to the control values, i.e. as measured before the ligature of arteries has been settled. Regarding a number of papers claiming that piracetam facilitates cholinergic neurotransmitter system, and that the amplitude of the somatosensory evoked potentials is increasing depending on the amount of the acetylcholine released, we think that the effect of piracetam on the evoked potentials during the occlusion of both carotid arteries, registered in our experiments, is mediated through the action of piracetam on the cholinergic system.

In the paper are presented the results of total enzyme activity investigation: GOT, GPT, LDH and CPK, and of the CPK isoenzymes in the cerebrospinal fluid of 148 examinees and in the serum of 67 examinees with an acute stroke, who were treated at the Intensive Care Unit of the Department of Neurology and Institute of Neuropathology, Clinical Medical center "Rebro". The aim was to determine the reliability of the applied methods in the diagnosis of cerebrovascular diseases, particularly in the differential diagnosis of cerebral hemorrhage, ischemia and subarachnoidal hemorrhage. The highest frequency of pathologic findings of the tested enzymes in the whole group of patients with CVA was obtained in the determination of the CPK total activity assessment, then followed the assessments of LDH activity, isoenzyme CPK profile, GOT and finally GPT activity. A larger number of pathologic findings of all mentioned enzymes and CPK isoenzymes was found in the group of patients with ICH. In the patients with ICH and ISI, who survived stroke a higher incidence of normal findings of the total enzymic activities was found, while in those who died from ICH a higher incidence of pathologic findings could not be established. The correlations between the total CPK activity in the serum and in the cerebrospinal fluid does not exist, as well as the correlation between the CPK isoenzyme profile in the serum and CSF.

By means of the EEG brain mapping method we analyzed electroencephalograms of patients who presented focal abnormalities in standard EEG. A total of 15 patients with various epileptic manifestations was investigated. According to the etiology of the disease, they were divided into 3 groups: microfactor, stationary macrofactor, and non-stationary macrofactor. The brain mapping technique proved to be very useful as imaging method of focal abnormalities, useful for registering asymmetries in reaction to following of intermittent photostimulation. The method is especially suitable for storing findings and chronological comparison of findings during pharmacological therapy. The specificity of the method regarding the etiological factor and type of epileptic manifestation could not be verified in such a small number of patients.

Visual potentials evoked by pattern reversal (PRVEPs) were studied in 64 normal children (age range 7 to 15 years) and in 20 age-matched patients with common and complicated migraine (age range 6 to 16 years). Most of them were studied before and during prophylactic therapy. A Quadristim set (Alvar) was used to present checkerboard patterns on a TV monitor, to amplify the EEG signals and to average and plot the evoked potentials. The potentials were elicited by binocular full-field 2/s checkerboard reversal, recorded from an electrode 4 cm above the inion, and analyzed for latency, amplitude and waveform morphology. The degree of similarity between pairs of PRVEPs was determined by the correlation coefficient. In all our patients no pronounced influence of the disease itself was demonstrated on the PRVEP latencies and amplitudes, but in most of them the correlation coefficients were significantly different from that showing the measure of normal PRVEP variations. Prophylactic medication influenced non-significant increase of the coefficient. This increase seems to be related to recovery from medication in terms of attack frequency. No significant difference in PRVEP results of common and complicated migraine was noticed. The PRVEP distortion can be revealed by the correlation coefficient but not by other PRVEP parameters. Therefore, this parameter may be useful as a sensitive measure of both PRVEP distortion and PRVEP improvement. Our results give further evidence that non-demyelinating disorders, but with ischemic damage and abnormalities in neurotransmitters, can produce measurable changes in PRVEP morphology.