cover image European Journal of Neurology

European Journal of Neurology

1999 - Volume 6
Issue 4 | July 1999

REVIEW

Abstract

This review discusses the state of neurology and the Internet at the turn of the millennium. First, some basic definitions about the Internet and its component protocols are presented. Next, ways neurologists and patients can use the Internet are enumerated. Internet resources or applications are available or are being created that can aid in the successful fulfillment of a neurologist's core professional activities: clinical care, teaching, research, and practice issues. Currently, the most useful categories of Internet resources for neurologists are electronic communication and access to knowledge bases. They fulfill needs that are not met by traditional, non‐electronic media. There are many other types of Internet applications that supplement traditional medical methodologies. Finally, some problems and prospects concerning medical uses of the Internet are discussed: technological infrastructure including usability, security, meaning, validity/quality, value, outcomes, and responsibility. These issues must be successfully addressed if Internet computing is to become truly useful ‘just in time’ at the point of medical care. Solutions are actively under development today. The prospects are bright for neurology, and medicine in general, on the Internet. The Internet will become an essential medical device in the near future.

RESEARCH PAPERS

Abstract

Controversy exists regarding the apolipoprotein E (ApoE) ɛ4 allele association with vascular dementia (VaD), ranging from increased ɛ4 frequency, similar to that found for Alzheimer's disease (AD), to no association between the ɛ4 allele and VaD. To clarify further the relationship between ApoE alleles polymorphism and cerebrovascular disease (CVD) in demented and cognitively impaired patients, we examined the ApoE phenotypes in a sample of 280 patients: 155 with AD, 21 with VaD, 32 with mixed dementia (MD), 45 with mild cognitive impairment (MCI) but without CVD, and 27 in which vascular disease was the most probable cause of cognitive decline [vascular mild cognitive impairment (VMCI)]. Our results show that the frequency of the ApoE ɛ4 allele in patients over 70 years old with clinically diagnosed VaD and VMCI does not differ significantly from that of controls. In contrast, ApoE ɛ4 allele‐bearing individuals had greater risk of having late‐onset AD (OR=8.8; 95% Cl 3.7–21.0), or non‐vascular cognitive impairment (OR=7.0; 95% Cl 2.5–19.0).

RESEARCH PAPERS

Abstract

Rivastigmine is a carbamate acetylcholinesterase (AChE) inhibitor with central selectivity. Early studies showed that daily doses up to 6 mg/day have some efficacy in patients with dementia of the Alzheimer type (DAT). The present study was designed to assess the safety, tolerability and efficacy of rivastigmine at doses up to 12 mg/day. A total of 114 patients with mild‐moderate DAT were randomly assigned to either rivastigmine (b.i.d. (twice daily) or t.i.d. (three times daily)) or placebo in a double‐blind fashion titrated to their maximum tolerated dose over 10 weeks followed by an eight‐week maintenance phase. The mean maximum tolerated dose was approximately 10 mg/day (b.i.d. or t.i.d.). Gastrointestinal complaints, the majority of which were mild to moderate, were the most frequently reported adverse events. No clinically relevant changes in vital signs, haematology or organ function were detected. Significantly more patients taking rivastigmine b.i.d. were considered improved according to the Clinicians' Interview‐Based Impression of Change‐Plus (CIBIC‐Plus) vs. placebo (57% vs. 16%, respectively; =0.027). The Nurses' Observation Scale for Geriatric Patients (NOSGER) (memory component) and the Alzheimer's Disease Assessment Scale‐cognitive subscale (ADAS‐cog) also improved in the rivastigmine b.i.d. group vs. placebo (mean change from baseline on NOSGER =−0.7 vs. +1.3, respectively; =0.037: mean change from baseline on ADAS‐cog=−2.7 vs. +0.2, respectively; =0.054). Despite the relatively small size and limited duration of the study, the finding that rivastigmine induced changes in the same (positive) direction in all three dimensions measured suggests that rivastigmine at doses of up to 12 mg/day has useful efficacy in patients with mild‐moderate DAT. Reports from larger phase III studies confirm this finding. The results of this study also suggest that b.i.d. is the more efficacious regimen and has comparable tolerability to the t.i.d. regimen.

RESEARCH PAPERS

Abstract

Parkinson's disease (PD) is a common cause of dementia in the elderly. Dementia in Alzheimer's disease (AD) and PD share common biologic and clinical features. The estrogen receptor (ER) gene is one of the susceptibility genes for AD. In order to test the hypothesis that the overlap between AD and PD may have a genetic basis, we determined ER gene polymorphisms in 13 PD patients with dementia (PDD) (age ± SD; 71.9 ± 5.5 years), 71 PD patients without dementia (PDND) (68.4 ± 7.5 years), 86 AD patients (76.8 ± 8.0 years) and 51 control subjects (CTL) (74.9 ± 6.9 years). ER genotypes were classified as a P or p allele on the basis of a Pvu II‐RFLP, and X and x on the basis of a Xba I‐RFLP. The frequency of the P allele in the PDD group as well as the AD group was higher than that in CTL. There was no significant difference in the distribution of the P allele between CTL and PDND. There were no significant differences in the distribution of the X allele among the PDD, PDND and CTL groups, whereas a higher incidence was found in AD. We conclude that the ER gene may be a common susceptibility gene for dementia in PD as well as AD.

RESEARCH PAPERS

Abstract

Ischemic stroke is associated with altered systemic immune responses both early after the onset and in the recovery phase. Interleukin (IL)‐10, a Th2 related cytokine, has multiple effects on different cell types, including T and B lymphocytes, monocytes, neutrophis and mast cells. IL‐4 is another Th2 cytokine that inhibits the synthesis of pro‐inflammatory cytokines by Th1 clones. We used enzyme‐linked immunospot assays to detect and enumerate blood mononuclear cells (MNC) secreting IL‐10 and IL‐4 spontaneously as well as after stimulation with myelin basic protein (MBP), considered to be an autoantigen of possible pathogenic importance in, for example, multiple sclerosis, to evaluate the involvement of anti‐inflammatory cytokines in ischemic stroke. All patients with ischemic stroke and cerebral hemorghage had strongly elevated numbers of IL‐10 secreting blood MNC compared with healthy individuals. Numbers of MBP‐reactive IL‐10 secreting blood MNC were also elevated in a proportion of the patients with stroke and hemorrhage. Levels of IL‐4 secreting blood MNC did not differ in ischemic stroke versus healthy individuals. The anti‐inflammatory IL‐10 could play a pivotal role in ischemic stroke as well as cerebral hemorrhage.

RESEARCH PAPERS

Abstract

: The aim of this study was to assess the effect of Albunex, a vascular contrast agent based on albumin‐coated air microbubbles, on pulsed Doppler and colour‐coded duplex sonography of the cranial vasculature. : Twenty healthy male volunteers received intravenous injections of contrast in single doses ranging from 0.08 to 0.30 ml/kg. Pulsed wave Doppler sonography examination and colour‐coded duplex sonography were carried out in the right internal carotid artery (ICA) and middle cerebral artery (MCA) before and after i.v. contrast. The relative intensity increase of the Doppler signal was measured in decibels. : Transpulmonary passage of contrast occurred in sufficient amounts to enhance the intensity of the Doppler signal significantly, but the duration of this effect was short. Contrast enhancement also improved visualization of both the ICA and MCA in all subjects. For the transcranial examinations, this resulted in visualization of a greater length of the middle cerebral arteries and additional vessels in the Circle of Willis. : These results confirm that contrast enhancement can significantly improve the quality of Doppler examination and colour‐coded duplex sonography of both the intracranial and extracranial vessels. However, the use of Albunex in neurosonology will be of limited value due to its relatively short duration.

RESEARCH PAPERS

Abstract

Knowledge of resource use and associated costs of treatment, care and rehabilitation at hospitals and in the health care and social service sectors is limited. This study presents data on the total resource use during the first year after spontaneous intracerebral hemorrhage. All patients hospitalized because of stroke at a university hospital in Copenhagen, Denmark, during a 1‐year period 1994–1995 were included in a database. The patients were followed until 1 year after the stroke, and data on resource use during and after the hospital stay were collected prospectively. This study investigates a subgroup comprising 90 patients with intracerebral hemorrhage. Resource use is described and costs are calculated. The cost of the hospital stay including acute care and rehabilitation had a mean of 90 200 DKK (US$16 100). The total cost of health care and social services during the first year had a mean of 123 200 DKK (US$22 000). Costs decreased significantly with age, but when differences of 30 days case fatality between age groups were considered, the association between costs and age disappeared. The mean cost of treatment, care and rehabilitation during the first year after intracerebral hemorrhage was 123 200 DKK, of which the primary hospital stay constituted 73%.

RESEARCH PAPERS

Abstract

The aim of the study was to monitor the natural history of new enhancing lesions in multiple sclerosis (MS) by means of serial gadolinium‐enhanced magnetic resonance imaging (MRI). Out of the 63 new enhancing lesions seen on the baseline scan, belonging to 26 relapsing‐remitting MS patients, 26 (40%), nine (14%) and four (6%) lesions showed persisting enhancement at first, second and third follow‐up scan, respectively. At the end of 5 months of follow‐up, 58 (92%) of the new enhancing lesions were detected as T2 hyperintensities, 24 (38%) as T1 hypointensities ('black holes'), and five lesions (8%) disappeared in both T2 and T1 weighted images. Duration of gadolinium enhancement of at least two consecutive scans significantly influenced the development of ‘black holes’. No significant correlation was observed between volume, location, configuration of enhancement at baseline and final outcome of the lesion. In individual cases, different evolution of new enhancing lesions was observed at the same time.

RESEARCH PAPERS

Abstract

Serial single fiber electromyography (SFEMG) examinations of orbicularis oculi muscle in patients with blepharospasm or hemifacial spasm treated with botulinum toxin injections were performed. The aim of the study was to evaluate the impairment of neuromuscular transmission, to follow reinervation after botulinum toxin administration and to find out whether there was a relationship between SFEMG parameters and clinical symptoms. Examinations were performed before injection, during early and late remission of symptoms, and after recurrence of the involuntary movement. Severe impairment of neuromuscular transmission, as revealed by increased jitter and increased presence of abnormal potential pairs and pairs with blocking, was found in early remission, but fiber density remained unchanged when compared with pretreatment values. In late remission, increased fiber density was registered for the first time. The recurrence of involuntary movements was related to the further increase of fiber density and tendency to normalization of jitter parameters. The study therefore suggests that formation of new neuromuscular junctions and their functional maturation is responsible for muscle recovery after botulinum toxin administration.

RESEARCH PAPERS

Abstract

Increases in circulating glucocorticoids promote catabolism, particularly in skeletal muscle. The sensitivity of the muscle to glucocorticoids can be altered by a change in the number of glucocorticoid receptors in the muscle, or by a change in the proportions of activated receptors (between binders IB and II). We have investigated the concentration of glucocorticoid receptors, and the proportions of types IB and II, in healthy and diseased muscle. We found significantly reduced concentrations of glucocorticoid receptors in the group of inflammatory myopathies (51% reduction; <0.05, Wilcoxon signed rank test). No significant changes in the relative proportions of binders IB and II were found in pathological muscle, although the proportion of binder IB tended towards elevated values (especially in the diabetic neuropathies, with a 17% increase). We conclude that the sensitivity of muscle to glucocorticoids can be reduced in neuromuscular diseases, especially in myositis, by a reduction in the number of glucocorticoid receptors in the tissue, but that no relevant shift in the relation between activated receptor types is present. This could be important in relation to the risk of a secondary steroid myopathy and catabolism of skeletal muscle in the treatment of inflammatory myopathies with glucocorticoids.

RESEARCH PAPERS

Abstract

This paper presents a clinical and electrophysiological analysis of type and duration of seizures recorded by means of long‐term video electroencephalogram (EEG) monitoring, a method which enables accurate diagnosis of psychogenic pseudoepileptic seizures occurring with or without epileptic seizures. Analysis is based on 1083 patients, hospitalized at our department between 1990 and 1997, with a preliminary diagnosis of epilepsy. Psychogenic pseudoepileptic seizures were diagnosed in 85 patients (7.8%). In 48 patients, pseudoepileptic seizures alone were diagnosed (group 1). whereas 37 patients had a mixed condition in which pseudoepileptic seizures were accompanied by epileptic seizures (group 2). For comparison of duration of pseudo‐ and epileptic seizures a control group (group 3), consisting of 55 patients randomly selected from the population of patients suffering from epileptic seizures alone, was parceled out. Long‐term video EEG monitoring was performed in 70 patients. In 55 (79%) of these patients 230 seizures (221 pseudoepileptic and nine epileptic) were recorded. In 30 patients (32%), the diagnosis was based on clinical observation of the seizures and on the number of EEG recordings, including activating procedures such as sleep deprivation, photostimulation, hyperventilation and anti‐epileptic drug withdrawal. We found that the duration of epileptic seizures was significantly shorter than the duration of psychogenic pseudoepileptic seizures. Our study has exposed the difficulties involved in the diagnosis of psychogenic pseudoepileptic seizures and the negligible value of neuroimaging techniques and interictal EEG recordings in the differential diagnosis of epileptic versus nonepileptic seizures. In this study, psychogenic seizures were significantly more frequent in women than in men; patient history analysis did not confirm the hypothesis that sexual abuse may cause psychogenic seizures.

RESEARCH PAPERS

Abstract

High blood levels of glucocorticoids are associated with increased mortality, confusion and poor functional outcome in stroke patients. It has been proposed that inhibition of glucocorticoids in acute stroke might be beneficial, but experimental data are conflicting and no long‐term follow‐up study has been reported. We have studied whether pre‐ or postoperative administration of metyrapone, a steroid synthesis inhibitor, can influence long‐term outcome after ligation of the right middle cerebral artery (MCA) distal to the striatal branches in hypertensive rats. Metyrapone (200 mg/kg) was administered either 30 min before or 1, 12 and 24 h after MCA occlusion. Limb placements and ability to traverse a rotating pole were evaluated pre‐ and postoperatively. Infarct size, histology and GFAP immunoreactivity were evaluated on 5 μm coronal sections from brains perfused in situ 4 weeks after the ischemic event. Pretreatment did not influence outcome, whereas postoperative administration of metyrapone significantly increased infarct volume (<0.05). Posttreated rats performed significantly worse than vehicle‐treated rats on the rotating pole 3 weeks after the operation (<0.05). Our results do not support the hypothesis that inhibition of glucocorticoid synthesis improves outcome after cerebral ischemia.

SHORT REPORTS

Quality of life in Alzheimer's disease

SHORT REPORTS

dimorphisms in gammopathy‐associated peripheral neuropathy

SHORT REPORTS

Abstract

To elucidate the possible role of carotenoids and vitamin A as risk factors for Alzheimer's disease (AD), we compared serum levels of β‐carotene and α‐carotene, and vitamin A, measured by isocratic high performance liquid chromatography, of 38 AD patients and 42 controls. The serum levels of α‐carotene did not differ significantly between AD patients and control groups. However, the serum levels of β‐carotene and vitamin A were significantly lower in the AD‐patient group. These values did not correlate to age, age at onset or score on the MiniMental State Examination. Weight and body mass index were significantly lower in AD patients than in controls. These results suggest that low serum β‐carotene concentrations in AD patients could be related to a deficiency in dietary intake of this provitamin, although its possible relationship with risk for AD could not be excluded.

CASE REPORTS

Abstract

There are few reports of Guillain–Barré Syndrome (GBS) occurring in families. We have encountered a mother, who developed acute inflammatory demyelinating polyradiculoneuropathy at age 35 years, whose son developed the bulbar form of GBS 7 years later. Both shared HLA DR2.

CASE REPORTS

Abstract

Down's syndrome (DS) is often associated with autoimmune diseases, although an association with multiple sclerosis (MS) has not been previously reported. A 49‐year‐old male with DS experienced progressively worsening gait and bladder dysfunction. Following Poser criteria, the patient was diagnosed with laboratory‐supported definite MS. Ten days following diagnosis the patient experienced dysestetic paroxysmal pain at the pelvic level (an uncommon complaint in MS) which was initially addressed with carbamazepine, resulting in mid relief and adverse effects consisting of increased motor deficit and decreased daytime alertness. A titration combination of lamotrigine and gabapentin, two relatively new antiepileptic drugs which have been utilized individually for a number of neurological symptoms, resulted in significant reduction in pain frequency and intensity, with no adverse effects. This case study presents details of the first reported association of DS and MS, between which the pathogenetic relationship remains unclear. The presence of a rare symptom complaint in MS, as well as the effective combination of lamotrigine and gabapentin for treating this symptom, without adverse effects is an additional interesting aspect of this case.

CASE REPORTS

Abstract

We report the case of a female patient who was exhibiting slowly progressive, severe, generalized freezing of voluntary movement and disequilibrium. Brain magnetic resonance imaging showed a very low signal intensity in both pallidal nuclei, with a high signal intensity in the central portion, the so called ‘eye‐of‐the‐tiger’ sign. Despite the unusual clinical features, we believe that this is a case of Hallervorden‐Spatz syndrome, although without neuropathological examination we were unable to confirm the diagnosis with certainty. This case further demonstrates the complex role of the pallidum in voluntary movement.

CASE REPORTS

Abstract

We report a 69‐year‐old male patient whose motor aphasia started at the age of 61. The language disability remained isolated and progressed over a period of eight years without any additional cognitive deficits. Computed tomography (CT) and magnetic resonance imaging (MRI) showed moderate cortical atrophy with frontal dominance. Single photon emission tomography (SPECT) showed hypoperfusion in the frontotemporoparietal region, positron emission tomography (PET) demonstrated a global cortical reduction of glucose utilization with a lesser decrement in the occipital lobes. The clinical symptoms and the neuropsychological findings fit the diagnosis of primary progressive aphasia.

CASE REPORTS

Abstract

We report an unusual case of sudden isolated transient amnesia triggered by acute marijuana use. The memory disorder, apart from the long duration, had the characteristics of a transient global amnesia‐like episode. Acute marijuna intoxication can affect memory more globally and severely than previously reported.

LETTERS TO THE EDITOR

Issues relating to the assessment of migraine recurrence following triptan therapy

LETTERS TO THE EDITOR

Answer to Millson and Tepper

LETTERS TO THE EDITOR

Spontaneous erections and libido increase associated with venlafaxine

BOOK REVIEWS

Genetics of Cerebrovascular Disease

BOOK REVIEWS

Fundamentals of Functional Brain Imaging. A guide to the methods and their applications to psychology and behavioral neuroscience