Acta neurologica最新文献

Quantitative assessment of regional cerebral blood flow (rCBF) in the bilateral cerebral and cerebellar cortices was performed in 13 epileptic patients receiving long-term high-dose phenytoin (PHT) therapy, using single photon emission computed tomography (SPECT) with N-isopropyl-(iodine 123) p-iodoamphetamine. In 4 of the 13 patients, both the cerebellar to frontal rCBF ratio and cerebellar to cerebral rCBF ratio showed low values as compared with those in 22 normal subjects matched for sex and age, probably reflecting abnormal relative cerebellar hypoperfusion. None of the 4 patients showed any abnormal findings on X-ray computed tomography or magnetic resonance brain imaging. The patients with a history of acute PHT intoxication tended to show the abnormal relative cerebellar hypoperfusion. These results suggest the utility of SPECT scans for early detection of cerebellar abnormalities known to be often present in epileptic patients, and imply a risk of long-term high-dose PHT therapy.

Quantitative assessment of regional cerebral blood flow (rCBF) in the bilateral cerebral and cerebellar cortices was performed in 22 normal adult subjects consisting of an early twenties group (5 men and 9 women, 20-22 years) and middle age group (3 men and 5 women, 41-52 years), using single photon emission computed tomography (SPECT) with N-isopropyl-(iodine 123) p-iodoamphetamine. Absolute rCBF values in each region in the 22 subjects ranged from 46 to 102 ml/100 g/min (mean 69.1, standard deviation 13.7 ml/100 g/min). The absolute rCBF values were significantly higher in women than in men, and in the early twenties group than in the middle age group. In addition, based on each absolute rCBF value, the interhemispheric asymmetry, anterior to posterior ratio and cerebellar to frontal ratio in rCBF were calculated. These results may be useful as indexes for detecting organic and functional brain abnormalities in various neuropsychiatric diseases.

The effectiveness of Oxiracetam (1600 mg/day) versus placebo was assessed in a group of 96 out-patients suffering from cognitive disorders secondary to primary degenerative dementia. The study lasted twelve months and was performed in two stage, a) double-blind (26 weeks) and b) open study (26 weeks). The assessment of the results obtained at two, six and twelve months was carried out following both the methodology based on neuropsychological tests and scales, and the study of the simple reaction time by any of a computerized portable tachystoscope. The patients treated with Oxiracetam showed a statistically significant improvement of simple reaction time and cognitive function detected by the Attention matrix. In the placebo group after twelve months a significant worsening of cognitive and global function was observed in comparison with baseline scores. The patients themselves appeared in favor of Oxiracetam. The drug tolerability proved to be very good for the whole duration of the treatment. The authors believe that Oxiracetam favorably acts on the symptoms of senile cerebral deterioration and can improve the capability of information processing, as suggested by the better performances obtained at the reaction time test and at the Attentional Matrix test.

Depression is a common psychiatric problem associated with epilepsy. Interictal depressive symptoms are more frequent and severe in epileptic patients than in subjects with comparable chronic neurologic diseases or physical handicaps. Epileptic depression was characterized as major or dysthymic: bipolar depression is rarely described. Several Authors are of opinion that interictal depression is more frequent in epileptics with temporal lobe foci and, in particular, with temporal left hemisphere lesions. The pathogenetic significance of depression in epileptics is unclear. Some suggest the hypothesis that depression represents behavioral effects of neurochemical responses to brain injury for asymmetrical hemispheric distribution of neural substrate for mood. We think that depression in epileptic patients does not represent a psychological reaction to a particular cognitive or physical impairment, but it is in some way related to the type of epilepsy. In addition, some antiepileptic drugs may have psychotropic effects: the most positive findings were associated with carbamazepine.

Sixty Multiple Sclerosis patients hospitalized either in relapse (28) or in chronic progressive (32) phase of the disease were treated with high-dose methylprednisolone infusions (1 g/daily for 6 days). Clinical examinations, scored by Kurtzke's functional systems (FSs) and expanded disability status scale (EDSS), were performed before treatment, immediately after, and thereafter at 1,3,6 and 12 month intervals. In relapsing cases, 22 patients (78.6%) improved and EDSS mean value decreased by 1.39 points after the treatment; 8 patients had a new bout within one year. In chronic progressive cases, 18 patients (56.2%) improved and EDSS mean value decreased by 0.56 points after the treatment; 13 patients showed a new worsening throughout the follow-up period. The treatment proved to be safe and effective both in relapsing and in chronic progressive patients, determining rapid clinical improvement in most of the cases, and a slowing down of progression in some chronic patients.

An 18-year-old female patient with a particular form of intractable motor cortex epilepsy, in which motor partial seizure status occurred only during sleep every night, was reported focusing on the drastic efficacy of sulthiame on the seizure status. Moreover, the present study demonstrated that single photon emission computed tomography (SPECT) and pulse oximetry examinations, both of which were performed in the ictal state, were useful for the regional diagnosis of the epileptic focus and observations of the seizure frequency, respectively. The therapeutic effect, epileptic picture and clinical examinations described here appear to be relatively rare in the literature, and therefore, this case report may be of clinical significance.

A case of Nasu-Hakola disease (membranous lipodystrophy) was reported. The patient is a 33-year-old man who started to have pathological fractures at the age of 24 years. Then, neurological symptoms such as dementia appeared gradually. A biopsy specimen of the bone showed membranocystic changes of the adipose tissue. T2-weighted magnetic resonance images of the head showed low intensity in bilateral putamens, internal capsules, globi pallidi and caudate nuclei. One of his brothers has the same disease. In both patients, fatty acid composition of serum total lipids showed increased nervonic acid and plasma amino acid analysis showed decreased glutamine.

Epileptic seizures and EEG interictal paroxysmal activity (PA) usually occur in an apparently unpredictable fashion, and a small number of patients with truly epileptic seizures may not present any PA on repeated EEGs. With the aim of increasing the possibility of recording interictal or ictal PA, several activation procedures are routinely carried out. In rarer instances, seizures seem to be more or less specifically evoked by unusual triggering procedures, or are chronologically related to biological rhythms. These different activating procedures may have different effectiveness depending on the type of epileptic syndrome. In the present paper the Authors describe the different activating procedures, both routinely employed and unusual ones. Their possible differential use with regard to the different epileptic syndromes and to the specific epileptologic history of the patient are examined. A selective use of these procedures is suggested, as an accurate choice of such techniques may increase the diagnostic usefulness of the EEG, in particular in patients whose basal recording is not contributory.

An electrophysiological study, comprehensive of peripheral sensory and motor conduction velocity (SCV, MCV), motor cortical stimulation (CS), median nerve somatosensory evoked potentials (SSEPs), brainstem evoked potentials (BAEPs) and sural nerve biopsy, was performed on 100 hereditary ataxia patients: 48 with Friedreich's ataxia (FA), 18 with Early Onset Cerebellar Ataxia (EOCA) and 34 with Autosomal Dominant Cerebellar Ataxia (ADCA). An early "peripheral" and "central" sensory impairment was observed in FA probably due to axonal loss and not related to disease severity or duration. On the contrary, BAEP and CS findings suggested a progressive involvement of the auditory and motor pathways. The presence of a non progressive sensory neuropathy allowed a distinction of EOCA patients in two groups: with and without peripheral neuropathy. The clinical and genetic heterogeneity was confirmed by the variability of evoked potential results. The ADCA patients showed the mildest degree of electrophysiologic abnormalities with an involvement of the peripheral pathways, both sensory and motor, more frequent than the central ones.

The authors report preliminary data on cognitive development of 57 children, perspectively followed, who were exposed to antiepileptic drugs in utero for maternal epilepsy. Cognitive impairments are associated with other risk factors in 5 cases, so that a direct AEDs responsibility is not easy to prove.