cover image European Journal of Neurology

European Journal of Neurology

1999 - Volume 6
Issue 2 | March 1999
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Editorial

Disability in world leaders

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Authors:
François Boller and Anne‐Marie Petrov
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00001.x

Special Article

Dementia in world leaders and its effects upon international events: the examples of Franklin D. Roosevelt and T. Woodrow Wilson

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Authors:
James F. Toole
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00002.x

Special Article

Abstract

It has been proved that Adolf Hitler suffered from idiopathic Parkinson's disease. No indication for postencephalitic parkinsonism was found in the clinical symptoms or the case history. Professor Max de Crinis established his diagnosis of Parkinson's disease in Hitler early in 1945 and informed the SS leadership, who decided to initiate treatment with a specially prepared ‘antiparkinsonian mixture’ to be administered by a physician. However, Hitler never received the mixture, this implies that the SS intended to remove the severely diseased ‘Leader’.

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Authors:
Franz Gerstenbrand and Elisabeth Karamat
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00003.x

Special Article

Abstract

The sheer scale of Stalin's achievements and institutionalized terror has prompted some authors to label him as a paranoid megalomaniac. Whatever the merits of this diagnosis, his undeniable accomplishments and the rationality of many of his actions cannot be explained by the workings of a disturbed mind. In his last years, however, his lifelong suspiciousness became florid paranoia. He eschewed medical advice, Listening to a veterinarian and treating his hypertension with iodine drops. Stalin feared his own shadow and trusted no‐one, even himself. He increasingly withdrew from official functions and he muttered menacingly to his close associates that it was time for another purge. Stalin suffered at least one stroke prior to his fatal intracerebral haemorrhage in 1953. Given his untreated hypertension and the autopsy report, it is probable that he had a number of lacunar strokes. These tend to predominate in the fronto‐basal areas, and disconnect the circuits that underpin cognition and behaviour. The most plausible explanation of Stalin's late behaviour is the dimming of a superior intellect and the unleashing of a paranoid personality by a multi‐infarct state.

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Authors:
Vladimir Hachinski
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00004.x

Special Article

Neuropsychiatric afflictions of modern French Presidents: Maréchal Henri‐Philippe Pétain and Paul Deschanel

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Authors:
François Boller, Annie Ganansia‐Ganem, Florence Lebert and Florence Pasquier
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00005.x

Special Article

Abstract

Urho Kekkonen, born in 1900, was elected President of Finland in 1956. He stayed in office for 25 years, the longest term for any democratically elected chief of state, until his resignation in 1981. Since he was always a model of good health, news of his cognitive decline while still in office came as a surprise to the whole nation. The impact was aggravated by an attempted cover‐up of his dementia. The attempt failed and the health of presidential candidates became, suddenly, a topic of intense public discussion. No special team of presidential doctors was established but Kekkonen's successor initiated a practice of reporting annually on his health. The ethical dilemma involving mass media was, and still is, its duty to inform people versus its right to self‐censorship, which was practised in Kekkonen's case. The physicians face the question of when, how and whom to inform when they notice that the president or some other powerful leader suffers from cognitive decline or other neuropsychological or mental disability. These are universal problems whose solution depends on the degree of democracy and freedom of expression in each country. Kekkonen died in complete privacy in 1986. Until now, no scientific report of his cognitive decline has been published.

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Authors:
Jorma Palo
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00006.x

Special Article

Abstract

The SELEDO (from plus ) study was carried out as a randomized, prospective, placebo‐controlled, double‐blind, multicenter long‐term, 5‐y‐ear trial to evaluate the possible advantages of combining selegiline and levodopa in the early treatment of Parkinson's disease. One‐hundred‐and‐sixteen patients were randomized either to selegiline or placebo. Before starting the study medication, the levodopa dose was titrated to the individual requirements of each patient. The primary study end point (time when levodopa had to be increased by 50% of the titrated dose) was reached in 23 of 59 patients in the selegiline group and 26 of 48 patients in the placebo group. At the end of the 5 years' treatment period the rates derived from a life‐table analysis were 50.4% in the selegiline group and 74.1% in the placebo group (= 0.027, log‐rank test). The median time to reach the primary end point was 4.9 years in the selegiline group and 2.6 years in the placebo group. In patients treated with selegiline, the mean levodopa dose changed only slightly over the 5 years of treatment compared to the initially titrated dose, but rose markedly in the placebo group, where the dose of levodopa had to be adjusted earlier than in the selegiline group. At the same time, the lower levodopa dosage in the selegiline group was accompanied by at least equal therapeutic efficacy (which is necessary for an unambiguous interpretation). Subgroup analyses showed greater benefit for selegiline treated) patients in the earlier stages. Long‐term side effects appeared later in the selegiline group, although the difference was not significant. The early combination of selegiline and levodopa proved to be clearly superior to levodopa monotherapy.

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Authors:
H. Przuntek, B. Conrad, J. Dichgans, P.H. Kraus, P. Krauseneck, G. Pergande, U. Rinne, K. Schimrigk, J. Schnitker and H.P. Vogel
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00007.x

Special Article

Abstract

In normal subjects, the steady‐state electroretinograrn in response to contrast reversing gratings (PERG), is spatially band‐pass tuned in amplitude, with a maximum at intermediate spatial frequencies and an attenuation at lower and higher ones. The amplitude attenuation at low spatial frequencies is believed to reflect centre‐surround antagonistic interactions in the receptive fields of inner retinal neurons. The aim of this study was to evaluate the PERG spatial tuning in multiple sclerosis (MS) patients without a previous optic neuritis history. Steady‐state PERGs in response to counterphase‐modulated (8 Hz) sinusoidal gratings of variable spatial frequency (0.6, 1.0, 1.4, 2.2 and 4.8 c/deg), were recorded from 18 patients with definite or probable MS and no history of optic neuritis (ON). Nine of them had no signs of subclinical optic nerve demyelination (asymptomatic) in either eye, while nine had symptoms or signs of optic pathways involvement (symptomatic) in one or both eyes. Results were compared with those obtained from 10 MS patients with a previous history of optic neuritis (ON) in one or both eyes, as well as from 21 age‐matched controls. The amplitudes and phases of the responses' 2nd harmonics were measured. Compared with the controls, asymptomatic ON patients showed selective losses in mean PERG amplitudes at medium and high (1.0–4.8 c/deg) spatial frequencies. Symptomatic ONpatients and ON patients had reductions in mean PERG amplitudes, with respect to controls, involving the whole spatial frequency range, but with greater losses at medium‐high (1.0–4.8 c/deg) than at lower spatial frequencies. In all patients' groups, the average PERG spatial tuning function differed significantly from that of the controls, assuming a low‐pass instead of the normal band‐pass shape. The PERG phase was delayed in ON but not in ON patients, as compared to controls. However, the phase delay was independent of spatial frequency. In both ON and ON patients, losses in PERG amplitude and spatial tuning tended to be associated with corresponding abnormalities in perimetric sensitivity, visual acuity, colour vision and transient visual evoked potential (WP) latency. The results indicate that abnormalities of the spatial tuning of steady‐state PERG can be found in MS patients without either optic neuritis or signs of subclinical optic nerve demyelination. These changes may reflect a retinal dysfunction, developing early in the course of MS, due to a loss of specific subpopulations of inner neurons, changes in lateral interactions of their receptive fields, or both.

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Authors:
Benedetto Falsini, Giovanni Porrello, Vittorio Porciatti, Antonello Fadda, Tommaso Salgarello and Marco Piccardi
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00008.x

Special Article

Abstract

Expression of presenilin‐1 (PS‐1) mRNA in an alternative splicing at the 3' end of exon 3 was examined in brain tissue, lymphocytes and cultured skin fibroblasts using the RT‐polymerase chain reaction (RT‐PCR) method. We quantified the relative ratios of the densities of the long form of PS‐1 mRNA, which contains a sequence encoding four amino acids (VRSQ, denoted as VRSQ+) to the short form, which lacks the VRSQ sequence (VRSQ‐). The brain tissue of subjects with sporadic Alzheimer's disease (AD) had reduced levels of the VRSQS form of the PS‐1 mRNA compared to a control group. No significant differences appeared in peripheral tissues, such as lymphocytes or cultured skin fibroblasts in AD with control subjects. Changes in the alternative splicing of exon 3 may be specific to the brain and may play an important role in the pathogenesis of sporadic AD.

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Authors:
Kenji Isoe‐Wada, Katsuya Urakami, Yosuke Wakutani, Yoshidi Adachi, Hiroyuki Arai, Hidetada Sasaki and Kenji Nakashima
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00009.x

Special Article

Abstract

Is Lyme neuroborreliosis, even in its early phase, a parenchymatous disorder in the central nervous system (CNS), and not merely a meningitic process? We quantified cerebrospinal fluid (CSF) levels of four nerve and glial cell marker proteins in Lyme neuroborreliosis patients with pretreatment durations of 7–240 days. AH 23 patients had meningo‐radiculitis, and six had objective signs of encephalopathy. Glial fibrillary acidic protein (GFAp) pretreatment levels in CSF, and the light subunit of neurofilament protein (NFL) levels were related to clinical outcome and declined significantly after treatment ( < 0.001 and < 0.01, respectively). NFL was detectable in 11 out of 22 patients, and pre‐and post‐treatment NFL levels were associated with the duration of neurological symptoms within 100 days prior to treatment. Neuron‐specific enolase (NSE) concentrations also decreased after therapy ( < 0.001), while CSF levels of glial S‐100 protein remained unchanged. The pretreatment duration of disease was related to postinfectious sequelae. GFAp, NSE and NFL levels in CSF are unspecific indicators of astroglial and neuronal involvement in CNS disease. The findings in the present study are in agreement with the hypothesis that early and late stages of Lyme neuroborreliosis damage the CNS parenchyma.

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Authors:
Leif DoteVall, Lars Hagberg, Jan‐Erik Karlsson and Lars E. Rosengren
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00010.x

Special Article

Abstract

We studied specific aspects of speed performance in neuropsychological tests and functional and vocational long‐term outcome among moderate or severe traumatic brain injury (TBI) patients admitted to a rehabilitation programme. A group of 140 patients with mild, moderate or severe TBI was followed up for a minimum of 5 years in a rehabilitation programme. Severity of TBI was estimated using the Glasgow Coma Scale (GCS) scores on emergency hospital admission. The patients, grouped by age at injury into the categories: 7 or younger, 8–16 and 17 years of age or older, were tested five or more years post‐injury with comprehensive neuropsychological tests, including a speed performance test with the Stroop material, the Purdue Pegboard (PB) test and simple visual and additive reaction times. The outcome variables were functional outcome, as measured by the Glasgow Outcome Scale (GOS) score, and capacity for employment at the end of follow‐up, . on average 12 years post‐injury. The patients with mild TBI were excluded from further analysis of outcome. The study was carried out at the Kauniala outpatient neurological clinic. In the Stroop test, patients with a GOS score of 3 and patients who were incapable of employment were slower (= 0.0046 and = 0.0015, respectively) than those with a GOS score of 1 or 2 and those capable of independent or subsidized employment, respectively. The PB test also differentiated significantly between the patients with a GOS score of 3 and those with a GOS score of 1 or 2 at the end of follow‐up (= 0.0413), and predicted incapacity for employment (= 0.032), those with worse outcome being slower. Simple reaction times did not differ significantly between the GOS scores at the end of follow‐up, and neither did they predict capacity or incapacity for employment. Our data suggest that the Stroop and PB tests can help estimate functional outcome, as measured by the GOS, among patients with initially moderate or severe TBI and who were referred to a rehabilitation programme. The same tests could also be useful in predicting long‐term vocational outcome.

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Authors:
I. Asikainen, T. Nybo, K. Müfler, S. Sarna and M. Kaste
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00011.x

Special Article

Abstract

Epidemiological studies were performed in South Estonia to establish the prevalence rate of multiple sclerosis (MS) and motor neurone disease (MND). The case finding method included information from the hospital records of the central hospital in the region‐the University Hospital (for MS from 1942 to 1989), from all neurologists in the region, from the Estonian MS Society and Association of Muscular Disorders, and from nursing homes in the region. The prevalence day was 31 December 1989. MND incidence was established for the period of 1986–1995. The results demonstrated high prevalence rates of MS among native Estonians (55.3 per 100000), somewhat lower prevalence among native‐born representatives of other nationalities (43.6 per 100 000) and the lowest prevalence rate of MS among non‐Estonian immigrants (26.6 per 100000). The differences were not statistically significant. The results for MND demonstrated the opposite pattern. The mean annual incidence rate of MND for 10 years was statistically significantly higher among people of other nationalities (2.5 per 100000) and Russians (2.6 per 100000), and lower in native‐born Estonians (1.1 per 100 000). No differences in health care or clinical picture were established. The reasons for the demonstrated differences in MND incidence remain unclear.

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Authors:
Katrin Gross‐Paju, M. Ööpik, S.‐M. Lüüs, I. Kalbe and Ain‐Elmar Kaasik
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00012.x

Special Article

Abstract

We investigated changes in water diffusion in the cerebral white matter of 14 patients with vascular dementia of the Binswanger type (VDBT) and ten patients with Alzheimer's disease (AD) with periventricular hyperintensity (PVH) lesions using diffusion‐weighted magnetic resonance imaging (MRI) and studied the pathophysiological differences between white matter lesions found in these two conditions. Apparent diffusion coefficients (ADCs) in the anterior and posterior white matter and the genu and splenium of the corpus callosum were significantly higher in both groups of patients than in the 12 age‐matched controls, and ADC values in VDBT and AD groups were almost the same. ADC ratios, defined as diffusion restricted perpendicular to the direction of nerve fibers, were also significantly higher in the patients than in the control subjects. However, there were regional differences in ADC ratios in the two conditions, with ratios in VDBT being higher in the anterior portions of the white matter but ratios in AD were higher in the posterior portions. The diffusion‐weighted MRI technique may be useful in the differential diagnosis of VDBT and AD with white matter lesions.

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Authors:
H. Hanyu, Y. Imon, H. Sakurai, T. Lwamoto, M. Takasaki, H. Shindo, D. Kakizaki and K. Abe
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00013.x

Special Article

Abstract

We examined the neurological differences between human immunodeficiency virus (HIV)‐infected men (= 193) and women (n = 41) receiving primary medical care. There was no difference between men and women in the rate of HIV‐related neurological syndromes (. polyneuropathy, myelopathy, myopathy, HIV‐dementia [HAD]). A logistic regression analysis indicated that low CD4+ cell count predicted all neurological syndromes. In addition, HAD was predicted by intravenous‐drug use and lower education level, while neuropathy was associated with older age and with race. These findings indicate that there are no differences in the rate of neuropsychiatric disorders attributable to gender. The presence of other factors (. drug abuse) colild explain previously reported gender differences in neurological manifestations of HN infection.

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Authors:
Oscar L. Lopez, Jeanne Wess, Jorge Sanchez, Mary Amanda Dew and James T. Becker
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00014.x

Special Article

Abstract

This study investigated the potential interaction between tolcapone, a catechol‐‐methyltransferase (COMT) inhibitor, and the decarboxylase inhibitor, benserazide. In an open‐labelled six‐week study, patients with Parkinson's disease (PD), treated with levodopa/benserazide, were given tolcapone at 200 mg t.i.d. Blood samples for analysis of benserazide, its main active metabolite, trihydroxybenzylhydrazine, levodopa and 3‐‐methyldopa (3‐O‐MD) were collected immediately before and repeatedly after the first drug intake of the day at baseline and after 1–2 and 6 weeks of treatment. Furthermore, animal experiments were performed to determine the levels of benserazide and trihydroxybenzylhydrazine at doses for which safety had previously been established. It was shown that tolcapone can cause an increase in benserazide plasma concentrations and that this effect is dependent on the benserazide dose. When tolcapone was combined with 25 mg benserazide the elevation was small. Although the increase was more pronounced when tolcapone was combined with 50 mg benserazide, the levels were still substantially lower than concentrations causing toxicity in animals. The safety margin derived from this study, together with the absence of any organic toxic effects in clinical trials, show that the observed interaction between tolcapone and benserazide does not represent a safety concern for PD patients treated with this combination.

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Authors:
Karin M. Jorga, Jan P. Larsen, Antonie Beiske, Michael Schleimer, Bärbel Fotteler, Monique Schmitt and Brit Moe
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00015.x

Special Article

Abstract

Prosody or the melody of speech is the process used to alter the meaning (linguistic prosody) or emotional force (affective prosody) of a sentence. The components of prosody are rhythm, pitch, tone and stress and they are articulated by modulation of the acoustic correlates of prosody; frequency, duration and amplitude. Little is known about the development of prosody in normal children other than that it appears to be a precursor to the further acquisition of normal language. In order to examine the development of the perception of prosody in normal children, a group of 40 neurologically normal children aged between 5 and 9 years were subjected to a number of prosodic recognition tasks. The objective was to modify a number of existing tasks and to devise a number of new ones to test both linguistic and affective prosody and the appreciation of affective cues in music. The results indicate a step‐wise improvement in perceptual contours up to 8.5 years old. However the perception of emotion in music appears highly developed early on in development. This study provides normative data and is the first report of a comparison between the development of prosodic and musical appreciation in this age group of normal children.

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Authors:
Colin P. Doherty, M. Fitzsimons, B. Asenbauer and H. Staunton
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00016.x

Case Report

Abstract

Landau‐Kleffner syndrome (LKS) is a rare acquired condition of auditory verbal agnosia and convulsive disorder in children. It has been proposed that there is a functional relationship between electrical disturbance and the speech defect. Prosody or the melody of language, as described by Monrad‐Krohn (1947), is one aspect of non‐verbal communication which is distributed bilaterally in the brain. Prosodic parameters of expression and perception in one 7.5‐y‐ear‐old child were tested to see if they were preserved as a means of communication. The child was observed during video‐electroencephalogram (EEG) monitoring over a 48‐h‐our period. MI utterances were recorded and subject to analysis for the salience and variation of acoustic correlates of prosody. Prosodic comprehension was measured using specific perceptual tasks previously presented to normal children between the ages of 5.5 and 8.5. Despite being unable to meaningfully use or perceive phonemes, the child was able to use variations in fundamental frequency, duration and intensity of utterances, to convey both emotional and propositional intent. Similarly, the child was able to discriminate prosodic contours of a male adult voice to an age equivalent to 5.5 to 6.5 years. This argues in favour of the notion for educating such children not only through the visual channel but also through the auditory channel.

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Authors:
Colin P. Doherty, M. Fitzsimons, B. Asenbauer, D. McMackin, R. Bradley, M. King and H. Staunton
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00017.x

Case Report

Abstract

We describe a patient with an acute illness, with clinical features characteristic of encephalitis lethargica, who responded repeatedly to trials of an apomorphine infusion and subsequently to oral levodopa therapy. This observation implies a direct acute neurotropic effect of the encephalitis on nigral dopaminergic cells. Dopaminergic medication may therefore be helpful in the acute stage of encephalitis lethargica as well as in the delayed post‐encephalitic parkinsonian phase of the syndrome.

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Authors:
John McAuley, Maryam Shahmanesh and Michael Swash
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00018.x

Case Report

Amyotrophic lateral sclerosis syndrome associated with connective tissue disease; improvement after immunosuppressive therapy. A long‐term follow‐up case

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Authors:
Carla Bentes, Mamede de CarValho, Isabel Valente, M.L. Sales Luís and José P. da Silva
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00019.x

Case Report

Abstract

We describe a patient presenting with vasculitic neuropathy associated with cutaneous vasculitis in the absence of other clinical or laboratory evidence of the underlying systemic vasculitis, and showing a favourable prognosis. Although the early proposed criteria for the diagnosis of non‐systemic vasculitic neuropathy (NSVN) exclude the involvement of extraneural tissues, the condition observed in our patient might represent a variant of NSVN, with the vasculitis confined to the small vessels in both the peripheral nerve and the skin.

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Authors:
Jun‐ichi Satoh, Tatsunori Yamamoto and Yasuo Kuroda
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00020.x

Case Report

Abstract

A case is described in which non‐convulsive status epilepticus (NCSE) prompted further investigation leading to the diagnosis of Lafora body disease (LBD). The onset of NCSE was temporally related to the withdrawal of sodium valproate and introduction of carbamazepine, which may have been precipitating factors. NCSE has not previously been reported in LBD. Implications for its drug management are discussed.

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Authors:
Robin G. Corkill and Richard J. Hardie
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00021.x

Case Report

Abstract

We report the case of a woman with axonal sensory and autonomic neuropathy lasting several months who improved in association with steroid administration. During the course of her disease and in the follow‐up, the patient underwent repeated cerebrospinal fluid (CSF) examinations, neurophysiological somatic, autonomic nervous system studies and sural nerve biopsy. Clinical and laboratory assessments demonstrated the occurrence of a monophasic, chronic sensory and autonomic neuropathy. A sural nerve biopsy suggested an axonopathy. After a progressive worsening of symptoms lasting about 6 months, steroid treatment was started and within 6 months a complete recovery, with normalization of the CSF findings, was observed. Although the ‘chronic inflammatory neuropathies’ are still debated entities, the features of this chronic, exclusively sensory and autonomic neuropathy are new, and the occurrence of a high protein level in the CSF, together with the favorable outcome associated with steroid treatment, suggests that our case might be another variant in this debated area.

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Authors:
Guido Cavaletti, Patrizia Santoro, Elio Agostoni, Alessandra Zincone, Chiara Gori, Lodovico Frattola and Giovanni Tredici
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00022.x

Letter to the Editor

Fluctuations of international normalized ratio in patients treated with oral warfarin therapy in Sneddon's syndrome

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Authors:
Manuel Lousa, Jose M. Gobernado and Ana Pardo
Article first published online:
27 Jul 2007 | DOI: 10.1111/j.1468-1331.1999.tb00023.x