cover image European Journal of Neurology

European Journal of Neurology

1999 - Volume 6
Issue 6 | November 1999

REVIEW

Abstract

Amyotrophic lateral sclerosis (ALS) is a chronic degenerative disorder of unknown etiology affecting the motor system. Conventional and non‐conventional neuroimaging techniques can provide essential help both to increase the confidence in ALS diagnosis and to assess the disease evolution. Signal abnormalities at the level of the motor crotex and the corticospinal tract on conventional T2‐weighted magnetic resonance (MR> images are a potentially useful marker of ALS pathology. However, the prognostic value of these conventional MR abnormalities is still hampered by their low pathological specificity. Non‐conventional MR techniques with a higher pathological specificity, such as MR spectroscopy, magnetization transfer imaging and diffusion‐weighted imaging, seem to have some potential not only for ALS diagnosis, but also for monitoring disease evolution either naturally or when modified by experimental treatments.

RESEARCH PAPERS

Abstract

An alanin‐9valin (Ala‐Val) polymorphism in the mitochondrial targeting sequence of managanese‐containing superoxide dismutase (Mn‐SOD) has recently been described. We studied this polymorphism in 72 Swedish paients with sporadic motor neuron diseases (MND) and controls unsing an oligonucleotide ligation assay. There were significant differences in genotype between MND patients and controls (= 0.025), and between male and female MND patients (= 0.009). Individuals homozygous for the Ala allele had a higher risk for MND [odds ratio, 2.9; 95% confidence interval (CI), 1.3–6.6], which was increased when including only females in the analysis (odds ratio, 5.0; 95% CI, 1.8–14.0). In classical amyotrophic lateral sclerosis, the odds ratio was 3.8 (95% CI, 1.3–10.0), and 5.5 (95% CI, 1.5–19.9) when including only females. The results suggest that mutations influencing the allocation of Mn‐SOD may be a risk factor in MND, especially in females, and that MND may be a disease of misdistribution of the superoxide dismutase enzymes.

RESEARCH PAPERS

Abstract

Neuropsychological clinical decision‐making is complicated by the fact that variability in test performance increases with advancing age. The research explores the presences of homogeneous subgroups in 120 neurologically healthy individuals, from 55 to 85 years of age. Subjects at risk for dementing diseases were diagnosed as Aging‐Associated Cognitive Decline (AACD) and Mild Cognitive Impairment (MCI). Cluster analysis was applied on 11 neuropsychological variables assessing logical memory immediate recall and retention percentage, visual memory immediate recall and retention, conceptual thinking, naming verbal fluency, constructional functions, motor speed, flexibility and finger tapping. Five clusters were extracted, one representing cognitively successfully aged, and two consisting of individuals with normal or average level performance. One cluster was characterized by older subjects with difficulties in visual memory, visuoconstructional functions, and speed and attention, most of the younger subjects in the same cluster had a diagnosis of AACD or MCI. The fifth cluster represented individuals at risk for dementing diseases; most of them were diagnosed having AACD and more than half had a diagnosis of MCI. Age, activity and intellectual levels, and to a lesser degree education, were significantly related to the cluster solution. The present findings caution against treating samples of elderly individuals as homogeneous.

RESEARCH PAPERS

Abstract

Impairments in executive functions have been related to aging and frontal lobe lesions. Aging also causes slowing of mental processing. We examined whether ischemic stroke in the frontal brain area results in dysexecutive syndrome, or whether the frontal stroke causes increased slowing of mental processing. Neurological, radiological and neuropsychological examinations were carried out 3 months post‐stroke on 250 ischemic stroke patients (55–85 years) and on 39 healthy control subjects. Of the patients, 62 had frontal and 188 had nonfrontal lesions. The neuropsychological examination comprised several cognitive domains, including tests considered to measure executive functions. The frontal group was slower than the nonfrontal group in tasks measuring speed of mental processing which were time‐limited (Trail Making A, Stroop dots and fluency). They were also inferior in the Digit Span backwards task. There were no differences between the groups in other cognitive domains, nor in some tests which are considered to be measures of executive functions (e.g. WCST). Impairments in executive functions were evident in both the frontal and the nonfrontal groups compared with the controls, but no dysexecutive syndrome specifically related to frontal lesions was found. Frontal stroke related mainly to the slowing of mental processing.

RESEARCH PAPERS

Abstract

In this study we compared the performance of 39 multiple sclerosis (MS) patients with 28 age‐, sex‐ and education‐matched controls on both the Mini‐Mental State Examination, a global cognitive assessment tool, and the Sternberg Short‐Term memory scanning task, a standardized test of short‐term memory (STM) processes. While the STM span of our MS patients did not differ from that of our controls, STM scanning time of the MS group was reliably slower than that of the controls and a significat correlation was observed between STM scanning time and duration but not severity of illness. Our results suggest that processing stages other than the manipulation of data within the STM buffer are also affected by MS.

RESEARCH PAPERS

Abstract

Cerebrospinal fluid (CSF) and plasma HIV‐1 RNA levels were prospectively measured by the Roche Amplicor Monitor polymerase chain reaction assay in 30 HIV‐1 infected patients without central nervous system opportunistic infections. All participants completed a global neuropsychological battery consisting of Mattis Dementia Rating Scale. Additional tests were use to better characterize the type of cognitive changes with a specific reference to frontal lobe function. The neuropsychological evaluation confirmed the subcortical pattern of cognitive dysfunction. CSF and plasma HIV‐1 RNA levels were significantly correlated. No correlation was detected with either blood or CSF RNA levels and the global cognitive status, but when stratified in three cognitive subgroups, higher CSF HIV‐1 RNA levels were observed in the more cognitively impaired subjects. Our results provide further evidence that plasma and CSF HIV‐1 RNA level cannot be used as a reliable diagnostic marker for HIV‐1 associated cognitive disorders. Only longitudinal studies will determine whether a high CSF HIV‐1 level could be a risk factor for HIV‐1 dementia.

RESEARCH PAPERS

Abstract

The aim of this study was to determine factors predictive of cerebral infarction subtype from clinical data collected within 48 h of neurologic deficit. All cardioembolic (= 231) and atherothrombotic infarctions (= 369) included in prospective stroke registry of the Sagrat Cor‐Alianza Hospital of Barcelona were analysed. Demographic characteristics, anamnestic findings, cerebrovascular risk factors and clinical data of patients with embolic stroke and patients with thrombotic infarction were compared. Predictors of stroke subtype were assessed by means of a logistic regression model based on 16 clinical variables. After multivariate analysis, atrial dysrhythmia and sudden onset to maximal deficit were significant predictors of embolic stroke, whereas hypertension, chronic obstructive pulmonary disease, diabetes, hypercholesterolemia and/or hypertriglyceridemia and age were independent predictive factors of atherothrombotic stroke. Setting a cut‐off point of 0.50 for predicting mechanism of stroke on admission resulted in a sensitivity of 76% specificity of 87% and total correct classification of 83%. Clinical features alone that are observed at stroke onset can help to distinguish cardioembolic from atherothrombotic infarctions.

RESEARCH PAPERS

Abstract

Objectives: The aim of this study was to investigate the relationship between sympathetic and cardiac parasympathetic function and the side of the lesion in stroke patients. Methods: Thirty‐two patients with stroke and 29 healthy age‐matched control subjects were studied. Sympathetic skin responses (SSR) and RR interval variations (RRIV) during rest and deep breathing were recorded for the assessment of sympathetic and vagal parasympathetic function, respectively. Results: The mean SSR amplitude values in patients compared with controls were 337 ± 244 versus 1897 ± 848 ( < 0.0001) for right hemispheric lesions and 466 ± 398 versus 1873 ± 843 ( < 0.0001) for left hemispheric lesions. The mean SSR latencies in patients compared with controls were 1526 ± 163 versus 1395 ± 109 ( < 0.05) for right hemispheric lesions and 1490 ± 125 versus 1423 ± 112 ( < 0.05) for left hemispheric lesions. RRIV (during deep breathing)/RRIV (at rest) ratios in patients compared with controls were 1.20 ± 0.25 versus 1.84 ± 0.52 ( < 0.0001), and 1.55 ± 0.88 versus 1.84 ± 0.52 ( < 0.05) for right and left hemispheric lesions, respectively. Conclusion: Supression of vagal parasympathetic activity was more apparent in stroke patients with right hemispheric lesions in our series. Therefore, the right hemisphere seems to have a greater effect upon parasympathetic activity.

RESEARCH PAPERS

Abstract

Background: The genetic background for ischemic cerebrovascular disease of the young and the role of lipids and lipoproteins as risk factors are not clear. Methods: We determined five LDL receptor mutations (Trp23Stop, Trp66Gly, Trp556Ser, 313+1G → A, 1846‐1G → A) and three apolipoprotein B mutations (Arg3500Gln, Arg3500Trp, Arg3531Cys), and other risk factors for ischemic cerebrovascular disease in 80 patients (36 women, 44 men) with onset of disease before the age of 50 years compared with 3366 individuals from a general population sample within the same age range. Results: None of the patients were carriers of mutations in the LDL receptor (Trp23Stop, Trp66Gly, Trp556Ser, 313+1G → A, 1846 – 1G → A) or the apolipoprotein B gene (Arg3500Gln, Arg3500Trp, Arg3531Cys) associated with hypercholesterolemia. However, on univariate analysis as well as on logistic regression analysis allowing for age and gender, plasma cholesterol (OR 1.4; < 0.0005), HDL‐cholesterol (OR 0.4; < 0.005), diabetes (OR 5.8; < 0.0001), and hypertension (OR 3.9; < 0.001) were significant predictors of ischemic cerebrovascular disease. Conclusions: The five most common LDL receptor mutations in Danish patients with familial hypercholesterolemia and three mutations in the apolipoprotein B gene did not predispose to ischemic cerebrovascular disease of the young. However, cholesterol and HDL‐cholesterol are important risk factors for ischemic cerebrovascular disease of the young in the present study. The elevation in cholesterol could in some patients be due to rare LDL receptor mutations not tested for, and could in other patients be multifactorial in origin.

RESEARCH PAPERS

Abstract

The aim of the sudy was to investigate the influence of hazardous alcohol drinking on the occurrence of epileptic seizures, the semiology of such seizures, and the extent of the problem. A consecutive sample of 142 acute seizure patients (78 male and 64 female, mean age 46 (16–79) years) was studied. Control groups were 185 consecutive sciatica patients and 254 healthy individuals. Subjects with a hazardous alcohol drinking level were identified by a score>8 in the Alcohol Use Disorders Identification Test (AUDIT). Seizures in AUDIT‐positive individuals occurring withing 72 h of the last drink were considered to be related to alcohol withdrawal. Generalized or partial onset seizures were classified on the basis of history, electroencephalographic (EEG) and neuroradiological findings. Thirty‐five percent of seizure patients were AUDIT‐positive, whereas conversely 27% were abstainers. Two‐thirds of AUDIT‐positive seizure patients met the criteria for withdrawal seizures. Indications of partial onset seizures were found in 25 (51%) of AUDIT‐positive patients, all secondarily generalized seizures. Sixty percent of generalized onset sizure patients were AUDIT‐positive. In conclusion, seizure patients included significantly more AUDIT‐positive subjects, as well as abstainers, than healthy Norwegian controls and consecutive sciatica patients from our hospital. Partial onset seizures are more frequent among hazardous drinkers than hitherto recognized. A generalized onset seizure in adults warrants a high suspicion of alcohol as a provoking factor. Routine screening of acute seizure admission with the Alcohol Use Disorders Identification Test is recommended.

RESEARCH PAPERS

Abstract

Visual system dysfunction has been reported in Parkinson's disease (PD). The objective of the present study was to evaluate a putative association of distorted colour vision and delayed initiation and execution of movement in PD. We performed the Farnsworth‐Munsell 100‐hue test and estimated the total error score in 30 previously untreated parkinsonian patients and 30 age‐ and sex‐matched controls. We then determined slowness in motor readiness and motor programming in the parkinsonian subjects on the same day. Subjects were asked to press a start button and release it after the randomized appearance of a visual stimulus and to move their right index finger to a reaction button as quickly as possible. Reaction time was considered as elapsed time between onset of the stimulus light and release of the start button, movement time was the time period between release of the start button and the pressing of the reaction button. Significant differnces appeared between parkinsonian patients' and controls' reaction times (= 0.007), movement time (= 0.001) and total error score (= 2.23E‐08). A significant relation (Spearman = 0.473, = 0.008) was found between movement time and total error score, but not between reaction time and total error score (Spearman = 0.259, = 0.166). We conclude, that visual dysfunction and execution of movement are more influenced by altered dopaminergic neurotransmission in PD in comparison to the initiation of movement.

CASE REPORTS

Abstract

This study reports a patient with severe, debilitating bilateral thalamic pain caused by bilateral thalamic infarcts. The authors consider it to be a unique case as a further clinically unilateral lesion led to pain relief bilaterally.

CASE REPORTS

Abstract

We report a patient with a progressive brainstem syndrome, who on magnetic resonance imaging had large bilateral, symmetrical, contrast‐enhancing, infratentorial space‐occupying lesons. Biopsy of one of the lesions revealed this unusual appearance to be due to a primary central nervous system (CNS) lymphoma of B‐cell type. Symmetry of lesions may be a clue to the diagnosis, perhaps reflecting the mechanism by which CNS lymphomas spread.

LETTER TO THE EDITOR

Single cerebral Toxoplasma abscess: first manifestation of HIV infection

BOOK REVIEWS

Numb Toes and Aching Soles: Coping with Peripheral Neuropathy

BOOK REVIEWS

The Clinical Neuropsychiatry of Multiple Sclerosis

BOOK REVIEWS

Forensic Neuropsychology: Fundamentals and Practice

RESEARCH PAPERS

Abstract

In this study, hair magnesium (Mg), zinc (Zn), copper (Cu), and manganese (Mn) levels, and serum Zn and Mg levels were measured by atomic absorption spectrophotometer in patients with epilepsy (n = 33) and healthy subjects (n = 21), and results obtained were statistically compared. The mean hair Cu, Mg, and Zn levels of epileptic patients were significantly lower than the levels of control subjects. There was no significant difference between epileptic patients and control subjects in respect to the mean Mn levels. Mean serum Mg levels in epileptic patients showed significant difference, but serum Zn levels were similar among both groups. When the effects of anticonvulsant therapy on Cu, Zn, Mn, and Mg in the hair, and Mg and Zn in the serum were analyzed in epileptics, there was no significant difference between the patients with or without therapy. Likewise, the mean trace element levels in epileptics showed no significant difference according to the type of antielpileptic drug and seizure, and gender. We suggest that the changed element status (Zn, Mg, and Cu) in hair play an indicator role in the diagnosis of epileptic patients.