cover image European Journal of Neurology

European Journal of Neurology

1998 - Volume 5
Issue 4 | July 1998
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REPORT

Year of the Brain in European countries. Background and organization

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Authors:
Jes Olesen, Gunilla Öberg and Per Vestergaard
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540325.x

REVIEW

Abstract

The cerebral circulation is innervated by sympathetic, parasympathetic and sensory nerves which store a considerable number of neurotransmitters. The role of these have been evaluated in primary headaches. A clear association between head pain and the release of calcitonin gene‐related peptide (CGRP) was demonstrated. In cluster headache and in a case of chronic paroxysmal headache there was in addition release of vasoactive intestinal peptide (VIP) which was associated with the facial symptoms (nasal congestion, rhinorrhea). In parallel with sumatriptan treatment head pain subsided and neuropeptide release normalised. These data show the involvement of sensory and parasympathetic mechanisms in the pathophysiology of primary headaches.

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Authors:
Lars Edvinsson and Peter J. Goadsby
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540329.x

RESEARCH PAPERS

Abstract

We conducted a pedigree analysis in 222 patients with cluster headaches (CHs) in order to assess a familial predisposition to the disease. Heritability was determined by Falconer's index (from the incidence of CH among first degree relatives of probands compared with a control population), which varies between 0 and 1 (none or full genetic causation). A positive family history (I/II degree relatives) was found in 2.3% of our CH patients. Three generations were affected in one family and two generations in another two families. The calculated Falconer's heritability index was, however, only 0.26 ± 0.25 SD. Our study confirms a higher familial occurrence of CH, some families showing a pattern compatible with autosomal dominant inheritance with reduced penetrance. The low Falconer's index indicates, however, a large non‐genetic causation in most cases of CH.

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Authors:
Pasquale Montagna, Mirella Mochi, Guido Prologo, Simonetta Sangiorgi, Giulia Pierangeli, Sabina Cevoli and Pietro Cortelli
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540343.x

RESEARCH PAPERS

Abstract

The focus of this report is utilization behaviour (UB) conceived as part of the Environmental Dependency Syndrome following frontal damage. An historical sketch is provided on the many conceptualizations of the frontal lobe functions relevant to the understanding of UB. The detailed study of the neuropsychological picture presented by a patient (PG) with massive herpetic damage is then outlined. Emphasis is put on PG's prominent UB as well as on her several dissociations between impaired (as expected) and spared (non expected) cognitive abilities. Such dissociations are discussed with reference to the historical conceptualizations outlined above.

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Authors:
Miriam Brazzelli and Hans Spinnler
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540347.x

RESEARCH PAPERS

Abstract

The prevalence and natural evolution of sleepiness were investigated in a 5‐year follow‐up study in a middle‐aged population in Finland. In the original study a structured sleep questionnaire was completed by 1190 subjects and a 5‐year follow‐up questionnaire was returned by 626.

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Authors:
Kirsti Martikainen, Markku Partinen, Jóel Hasan, Hélka Urponen, IIkka Vuori and Pékka Laippala
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540355.x

RESEARCH PAPERS

Abstract

Myoclonic astatic seizures are well known in children's generalized epilepsy. Some of our patients suffering from Parkinson's disease complained about sudden loss of postural reflexes together with dizziness or short loss of consciousness. When we looked into their electroencephalograms (EEG's), we found polyspikes and polyspike‐wave complexes that were seen especially after provocation by hyperventilation or photic stimulation. An additional therapy with sodium valproate diminished the myoclonic astatic attacks as well as the alterations in the EEG in all of the patients. None of them experienced a deterioration in their Parkinson's disease.

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Authors:
Alexandra E. Henneberg, Irma Schöll and Emanuel Kogan
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540365.x

RESEARCH PAPERS

Abstract

The nervous system may be involved during primary human immunodeficiency virus (HIV) infection. Recognized clinical syndromes include meningitis, meningo‐encephalitis, myelopathy, polyneuropathy and rhabdomyolysis. We report three patients with unusual neurological presentations of primary HIV infection: intracranial hypertension, severe encephalopathy characterized by personality changes and regressive behavior, and iumbosacral radiculoneuropathy. In all, the neurological disorder had a benign course and resolved within a few months. Awareness of the large spectrum of neurological manifestations of primary HIV infection can enable early diagnosis and treatment.

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Authors:
Sharon Hassin‐Baer, Israel Steiner, Anat Achiron, Menachem Sadeh, Ami Vonsover and David Hassin
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540369.x

RESEARCH PAPERS

Abstract

Between 1992 and 1995, the annual incidence of Creutzfeldt–Jakob disease (CJD) in one of the 96 French (adminstrative districts) was found to be about six times higher than the CJD national incidence. Among the 12 definite or probable CJD patients referred during this period within this , nine originated from a small confined area (30 × 30 km) and seven patients carried the E200K mutation in their prion protein gene (). Genealogical data showed that these seven cases, together with three other ones previously referred during the 1970–82 period, probably belonged to different branches of the same family which could be traced to the beginning of the eighteenth century. Interestingly enough, all but two patients presented as sporadic cases before the genealogic and genetic studies. To our knowledge, this study is the first describing in France a focal accumulation of CJD associated with the E200K mutation.

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Authors:
Jacqueline Chatelain, Nicole Delasnerie‐Lauprêtre, Marie‐Hélène Lemaire, Françoise Cathala, Jean‐Marie Launay and Jean‐Louis Laplanche
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540375.x

RESEARCH PAPERS

Abstract

Spinocerebellar ataxia type 6 (SCA6) is an autosomal dominant spinocerebellar degeneration caused by CAG repeat expansions in the human α1A voltage‐dependent calcium channel subunit gene. We analyzed 16 SCA6 patients in 14 unrelated Japanese families, and documented the clinical and molecular properties correlating with the CAG repeat expansion. Three of them were sporadic. The CAG repeat number of the expanded and normal alleles was 22.7 ± 2.0 (mean ± SD, n = 15) and 13.8 ± 2.0 (n = 15), respectively, and the repeat size of the expanded alleles correlated inversely with age at onset. The patients presented here were clinically characterized by a slowly progressive cerebellar ataxia and nystagmus. In leukocytes, the strict pattern of the peak in the expanded allele on polyacrylamide gel electrophoresis did not show the presence of cell mosaicism in SCA6, in contrast to other trinucleotide disorders. Moreover, in each patient, the number of CAG repeats in sperm was the same as in leukocytes, and the expanded alleles in sperm indicated uniform peaks as well. In our geographic area, the frequency of SCA6 was as high as MJD, in contrast to the low frequency of other autosomal dominant cerebellar ataxias. Thus, a geographic difference in the frequency of autosomal dominant spinocerebellar ataxias may be present in Japan.

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Authors:
M. Shizuka, M. Watanabe, Y. Ikeda, K. Mizushima, M. Kanai, T. Tsuda, K. Abe, K. Okamoto and M. Shoji
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540381.x

RESEARCH PAPERS

Abstract

We have identified a novel mutation in exon 4 of the Cu/Zn superoxide dismutase (superoxide dismutase 1: SOD1) gene (GAC to GTC), which resulted in an Asp to Val substitution in a Japanese family with amyotrophic lateral sclerosis (ALS) inherited as an autosomal dominant trait. The patients in this family usually died in 2–3 years without sensory or urinary impairment. The SOD1 activity was lower in the proband as compared to the normal controls. The clinical characteristics of this family resemble those of some patients heterozygous for the Asp90Ala mutation, but both the clinical features and SOD1 activity of this family differ from those of patients homozygous for the ASP90Ala mutation.

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Authors:
Mitsuya Morita, Koji Abe, Mitsu Takahashi, Yoshiaki Onodera, Hitoshi Okumura, Masaaki Niino, Kunio Tashiro, Imaharu Nakano and Yasuto Itoyama
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540389.x

RESEARCH PAPERS

Abstract

Several genetic factors are likely to play a role in the aetiology of multiple sclerosis (MS), although so far only the HLA gene complex has been clearly identified as important. In addition, several studies support the importance of nitric oxide synthase (NOS) as a component in the pathogenesis of MS. We have investigated the role of the neuronal nitric oxide synthase (NOS1) gene in 41 Swedish multiplex MS families by parametric and nonparametric linkage analysis with two polymorphic intragenic markers. Two‐point lod scores were clearly negative, effectively excluding a role of the NOS1 gene in most models tested. Non‐parametric linkage analysis (NPL), affected pedigree member (APM) analysis and extended transmission disequilibrium test (ETDT) also failed to provide evidence for a susceptibility locus in this gene. Thus, a contribution of the NOS1 gene to the genetic background of MS is unlikely in this population.

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Authors:
Chun Xu and Jan Hillert
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540393.x

RESEARCH PAPERS

Abstract

A combination of anti‐epileptic drugs is used for the necessary control of seizures. This results in a modulation of the metabolism in the epileptic patient and a concentration of the drugs and their metabolites in serum and in the brain, the target organ. Carbamazepine‐10,11 epoxide (CBZE) is the main active metabolite of carbamazepine (CBZ). We have studied their interrelationship in concentrations in the plasma of 68 patients receiving CBZ, either as monotherapy or in combination with phenytoin (PHT) and phenobarbital (PB). The rate of CBZ metabolism was modulated in drug co‐administration, which, depending on the grade of the induction of cytochrome p‐450, decreases or increases the concentration of CBZE. A graph plotting the relationship between CBZ and CBZE concentrations in patients stabilized on a regime of CBZ alone is linear. The ratio of concentrations of CBZE/CBZ in serum is 0.12 when CBZ is administered as monotherapy, rising to 0.14 (CBZ + PB), 0.18 (CBZ + PHT) and 0.25 (CBZ + PHT + PB) when administered with the other drugs mentioned. From this it can be hypothesized that the additive of induction activities of PHT and PB operates on the mixed function oxidase system.

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Authors:
D. Divanoglou, A. Orologas, S. Iliadis, V. Nathanael, T.C. Constantinidis and I. Milonas
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540397.x

RESEARCH PAPERS

Abstract

Twenty‐two late‐onset, non‐thymoma myasthenia gravis (MG) patients were selected for study based on the combined presence of MG and involution of the thymus. Thymectomy specimens from all the patients were examined histologically and immunohistologically and compared with an age‐matched control group in which tissue was removed from the thymic gland during operations for various cardiac conditions. We were not able to find any morphological differences between thymi from MG patients and patients in the control group. Germinal centres were present in 7/22 in the patient group and 6/20 in the control group. Seven of our 22 MG patients had auto‐antibodies to titin. Germinal centres in the thymus were found in only one. In contrast germinal centres were present in the thymus of 6/15 MG patients with no detectable antibody response to titin (= 0.01). Our study suggests that late‐onset MG is pathogenetically heterogeneic with different modes of auto‐sensitisation.

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Authors:
Andreas O. Myking, Geir O. Skeie, Jan E. Varhaug, Knut S. Andersen, Nils E. Gilhus and Johan A. Aarli
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540401.x

CASE REPORT

Abstract

A 67‐year‐old woman who developed acute secondary mania immediately after a mixed (perforating and cortical) embolic brain infarction in the territory of the right middle cerebral artery is described. According to neuroradiological findings ischemic lesions involving the basal and medial areas of the temporal lobe and the head of the caudate as well as the putamen were evident. In the pathogenesis of secondary mania due to cerebrovascular disease, the pattern of involved structures is unique and underlines the significance of lesion location after damage of the minor hemisphere.

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Authors:
Christian Börnke, Thomas Postert, Horst Przuntek and Thomas Büttner
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540407.x

CASE REPORT

Abstract

We describe two patients with post‐partum cerebral angiopathy. (Case 1) A 24‐year‐old patient developed vaginal bleeding 1 day post‐partum. She received blood transfusion and ergometrine. Four days later she experienced severe occipital headache, loss of vision and generalized seizures. An MRI showed right occipital lesion. She was treated with phenytoin and prednisone. Her vision returned to normal within 2 days. Cerebral angiography showed multiple segmental narrowing; 6 h later severe occipital headache and cortical blindness recurred. She was treated with nimodipine and prednisone with complete recovery in 10 days. (Case 2) On the 7th post‐partum day a 24‐year‐old patient was given ergometrine. One day later she developed severe headache and hypertension, followed by drowsiness, right hemiplegia and aphasia. A left frontal hematoma was seen on the CT scan. Cerebral angiography showed multiple segemental narrowing. Resolution of this angiography was confirmed on follow‐up angiography. Post‐partum cerebral angiopathy is a rare complication of a normal pregnancy. It may present as an ischemic or hemorrhagic stroke. The transience of this severe complication and its association with the administration of drugs with strong vasoconstrictive properties suggest and etiological relationship. Other factors and the exact mechanism are unclear.

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Authors:
Walid Al‐Sous, Saeed Bohlega, Zuheir Al‐Kawi, Donald McLean and Khalid Shukri
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540411.x

CASE REPORT

Abstract

All cases of selective anomic deficit for proper names described until now are a consequence of focal cerebral lesions. In the present paper, we report the case of a patient, AF, with a deficient access to proper names of persons and normal access to common names probably subsumed by a degenerative process of the left cerebral hemisphere. MRI evidenced an atrophy of the left temporal lobe and SPECT highlighted hypoperfusion of the left hemisphere. Neuropsychological examination documented a deficient production of proper names belonging to famous personalities both on visual presentation and verbal definition. Moreover, on verbal fluency tasks, AF was poor for the categories of famous peoples and some commercial articles. Finally, AF was errorless in a proper name comprehension task.

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Authors:
Lucia Fadda, Patrizia Turriziani, Giovanni A. Carlesimo, Ugo Nocentini and Carlo Caltagirone
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.540417.x

ABSTRACTS

Abstract

The previous decline of the incidence of stroke is now less apparent. Most of this decline was in ischaemic stroke; the changes in haemorrhagic stroke are less certain.

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Authors:
ZAHID I. CHOUDARY, MICHAEL T.Y. CHAN, S. BARSOUM and J.W. NORRIS
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.5404231.x

ABSTRACTS

Abstract

Although the effect of t‐PA treatment in acute cerebral infarction remains controversial, there is evidence based data that it may prove effective if given early and with appropriate criteria.

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Authors:
ZURAB G. NADAREISHVILI, LILIANA T. SMARAWSKA and JOHN W. NORRIS
Article first published online:
10 Feb 2003 | DOI: 10.1046/j.1468-1331.1998.5404232.x