bluuebunny/crossref_metadata_2025 huggingface dataset:
Metastatic Angiosarcoma of the Liver Preoperatively Presenting as Giant Hemangioma
Authors: Kirschstein Timo , Aeberli Daniel , Zimmermann Arthur , Uhl Waldemar , Büchler Markus W.
Published: April 2003

Background: Hepatic angiosarcomas are rare tumors most often associated with exposure to vinyl chloride or other carcinogens. Only a few cases have been published without such a history. Case Report: We report the case of a 73-year-old woman who was admitted to our medical department with unclear upper abdominal pain, thrombocytopenia and anemia. Both computed tomography and magnet resonance imaging revealed a giant hemangioma in the right liver with multiple small hemangiomas. To cure the problem of thrombocytopenia due to sequestration of blood cells in the hemangioma, we decided to resect the large tumor. Intraoperatively, however, the diagnosis of angiosarcoma with multiple metastases was made. The patient died 6 weeks after surgery. Conclusion: Problems in diagnosing angiosarcoma include the brief duration of antecedent symptoms, difficulties in radiological diagnosis, and patients without a history of professional exposure to carcinogens.
Hippocampus and Parahippocampal Gyrus Linear Measurements Based on Magnetic Resonance in Alzheimer’s Disease
Authors: Pucci Eugenio , Belardinelli Natascia , Regnicolo Luana , Nolfe Giuseppe , Signorino Mario , Salvolini Ugo , Angeleri Franco
Published: April 2003

MRI-based linear measurements of the hippocampus and parahippocampal gyrus complex (HPC) discriminated 39 subjects with probable Alzheimer’s disease, from 15 patients with other dementias and 33 miscellaneous controls without evidence of dementia. The best discriminating parameter was the left height of the HPC at the level of the mammillary bodies, with a sensitivity of 79.49% and a specificity of 68.75%. The diagnostic value of these results is discussed considering the volumetric data found in the current literature.
Idiopathic Hypertrophic Pachymeningeal Lesions
Authors: Bang Oh Young , Kim Dong Ik , Yoon Sa Rah , Choi Il Saing
Published: April 2003

Nine patients with intracranial pachymeningeal thickening and enhancement on magnetic resonance imaging (MRI) completed a short-term clinical and MRI follow-up study. Based on clinical pictures, 4 of them were found to have spontaneous intracranial hypotension (SIH) and the remaining 5 had idiopathic intracranial pachymeningitis (IIP). Both groups were compared regarding their clinical and MRI characteristics. In 4 patients with SIH, gadolinium-enhanced T1-weighted images (WI) showed a diffuse and even enhancement of the entire intracranial and spinal dura mater. These thickened dura was slightly hyperintense on T2-WI. They had a favorable prognosis. In 2 patients with IIP, MRI demonstrated a relatively focal and even thickening and enhancement of the intracranial dura which was slightly hyperintense with a central hypointense area on T2-WI. These patients showed a very favorable course with or without steroid pulse therapy. In the remaining 3 patients with IIP, MRI depicted a focal, uneven enhancement of the intracranial dura which was relatively hypointense on T2-WI. Two of them with prolonged symptoms had a remitting and relapsing course, and 1 had a favorable outcome. In spite of current limitations in identifying the underlying causes of idiopathic pachymeningeal abnormalities, MRI can characterize the different patterns of pachymeningeal thickening. These findings may also correlate with the clinical picture and may be useful in predicting the response to treatment and prognosis.
Antiganglioside GM1 Antibodies and Their Complement Activating Capacity in Central and Peripheral Nervous System Disorders and in Controls
Authors: Uetz-von Allmen Edith , Sturzenegger Matthias , Rieben Robert , Rihs Frank , Frauenfelder Agnes , Nydegger Urs E.
Published: April 2003

So far, the pathogenic significance and use for diagnosis of antiganglioside GM1 antibodies (anti-GM1) are unclear. We therefore compared serum IgM and IgG antimonosialo ganglioside GM1 levels of 33 patients with presumed immune-mediated neuropathies, 100 patients with various other central or peripheral neurological disorders, and 110 controls by ELISA. We also measured the complement-activating capacity of anti-GM1 by C5b-9-GM1-ELISA to evaluate its value to distinguish between pathogenic and nonpathogenic autoantibodies. Low levels of anti-GM1 were observed in all disease categories and in controls (healthy blood donors). Twenty-four of the controls including the 10 with the highest serum IgM or IgG anti-GM1 were examined for neurological disorders in a double-blind checkup study. In the patients, elevated IgM anti-GM1 levels were predominantly found in those with neuropathies (NP), but barely in patients with central nervous system disease (CNSD). We found elevated IgG anti-GM1 levels predominantly in patients with NP of inflammatory origin (multifocal motor neuropathy, chronic inflammatory demyelinating polyneuropathy or Guillain-Barré syndrome), rarely in patients with NP of noninflammatory origin or CNSD, but not in the control disease group myasthenia gravis (MG). Median levels of IgM-, IgG-, (IgM+IgG)-, and C5b-9-binding anti-GM1 were significantly higher in patients with inflammatory NP as compared to the controls (p < 0.025). In addition, median levels of IgG- and (IgM+IgG)-anti-GM1 were significantly higher in inflammatory NP versus CNSD. Elevated complement-binding activity was associated with low or elevated IgM and/or IgG anti-GM1. Nevertheless, there was a significant correlation between anti-GM1 level (IgM+IgG) and the respective complement-activating capacity (r = 0.758; n = 243). Estimation of anti-GM1 and their respective complement-activating capacity may be helpful in the diagnosis of inflammatory neuropathies. However, neither an elevated anti-GM1 level nor an increased C5b-9 binding seems specific for a given disease category (e.g. peripheral nerve disease) nor a disease process (e.g. demyelination or inflammation).
Neurological and Neuropsychological Bases of Empathy
Authors: Eslinger Paul J.
Published: April 2003

Impairments of social behavior after cerebral damage are often problematic and difficult to assess and manage, with few models addressing evaluation, treatment options and prognosis. Recent studies suggest that a fundamental mechanism of social behavior disturbed by acquired cerebral damage is empathy. Empathy refers to the cognitive and emotional processes that bind people together in various kinds of relationships that permit sharing of experiences as well as understanding of others. Empathic changes are particularly evident after focal prefrontal cortex damage and closed head injury in adults, though early frontal lobe damage is also associated with poor empathic and social development. Although alterations in empathy have been studied in only a handful of neurologic samples thus far, it may be an important outcome variable of brain injury, particularly in patients’ adjustment to family, community and vocational settings. Treatment possibilities are presented, though more comprehensive research is needed.
Pure Sensory Syndromes in Thalamic Stroke
Authors: Paciaroni Maurizio , Bogousslavsky Julien
Published: April 2003

We studied 25 patients with an acute thalamic stroke (infarct or hemorrhage) on CT or MRI scan and sensory dysfunction, among the 3,628 patients with first-time stroke included in the Lausanne Stroke Registry. Twelve patients had a right-sided infarct, 11 a left-sided infarct, and 2 a left-sided thalamic hemorrhage. Sensory symptoms or signs were the only clinical abnormality. The presumed causes of stroke were small artery disease in 21 patients including both cases of hemorrhage, emboligenic heart disease in 2, while the etiology of ischemic stroke was undetermined in 2 patients. Nine patients had a loss of all modalities of sensation with faciobrachiocrural distribution, 5 patients suffered dissociated sensory loss with faciobrachiocrural distribution and 11 patients showed a dissociated involvement of sensation with a partial distribution pattern. The inferolateral region (thalamogeniculate arteries) was involved in all patients. Six patients complained of pain and/or dysesthesias during the stroke; 5 of them had involvement of the nucleus ventrocaudalis (in 3 with damage to the nucleus ventro-oralis intermedius, and in one to the pulvinar) and 1 patient had involvement of the nucleus ventro-oralis intermedius. Eighteen patients complained of paresthesias in the contralateral part of the body; 16 of them had involvement of the nucleus ventrocaudalis (in 4 with damage to the nucleus ventro-oralis intermedius, in 1 with damage to the nucleus ventro-oralis intermedius, and nucleus ventro-oralis externus, and in one with damage to the nucleus parvocellularis and pulvinar). Four patients developed delayed pain and/or dysesthesias; all of them had involvement of the nucleus ventrocaudalis (in 1 with damage to the nucleus parvocellularis and pulvinar). Time lag from stroke onset to developing pain ranged from 2 to 15 days (mean 10.5 days). One patient with dissociated involvement of sensation with a partial distribution pattern had paresthesias and dissociated hemisensory loss involving position sense without pain and temperature sensations. This patient had involvement of the posterolateral part of the nucleus ventrocaudalis. In conclusion, sensory dysfunction and delayed pain are more often found in thalamic lesions that involve the nucleus ventrocaudalis, and nucleus ventro-oralis intermedius. Restricted sensory abnormalities correlate with very small lesions located in critical areas within these nuclei.
Prevalence of Dementia ofAlzheimer Type and Apolipoprotein EPhenotypes in Aged Patients withDown’s Syndrome
Authors: Sekijima Yoshiki , Ikeda Shu-ichi , Tokuda Takahiko , Satoh Shun-ichi , Hidaka Hiroya , Hidaka Eiko , Ishikawa Masayo , Yanagisawa Nobuo
Published: April 2003

We investigated the exact prevalence of dementia of Alzheimer type (DAT) and apolipoprotein E (ApoE) phenotypes in 106 Japanese Down’s syndrome (DS) patients. Among these patients 16 were diagnosed as having DAT. The prevalence of DAT was 0% in the 30- to 39-year-old group, 16% in the 40- to 49-year-old group, and 38% in those over 50 years old. The frequency of the σ4 allele in DS patients with DAT was 18.8%, which was considerably higher than that of nondemented DS patients (4.5%) and Japanese nondemented controls (6.7%). Especially, the frequency of the σ4 allele in DS patients who developed DAT under 50 years was significantly higher (28.6%). DS patients certainly develop DAT at earlier ages but the prevalence of DAT in each group of patients was lower than previously recognized. It is very likely that the ApoE σ4 is a risk factor for DAT even in DS patients with a genetic predisposition to Alzheimer’s disease.
Combination Therapy with Neuroprotectants and Thrombolytics in Acute Ischaemic Stroke
Authors: Steiner Thorsten , Hacke Werner
Published: April 2003

A reduction in neuronal damage following acute ischaemic stroke can be achieved by two major strategies: restoration of cerebral blood flow through the use of thrombolytics, and inhibition of the pathophysiological cascade that occurs as a result of a decreased cerebral blood flow through the use of neuroprotective agents. Therefore, combination therapy with thrombolytic and neuroprotective agents may provide additional benefits to those that can be achieved using the individual agents alone. Indeed, such benefits have already been demonstrated in several animal studies. However, a number of problems in trial design and statistical methodology need to be overcome. Nevertheless, combination therapy offers the potential of reducing the cost of stroke in terms of both health care and social welfare, and as well as limiting disability.
Congenital Muscular Dystrophy with Partial Merosin Deficiency and Late Onset Epilepsy
Authors: Martinello F. , Angelini C. , Trevisan C.P.
Published: April 2003

Merosin, the laminin α2 chain located on the surface of muscle fibers, has recently been shown to be absent in a subset of cases with the classical type of congenital muscular dystrophy (Cl-CMD). By immunocytochemistry and immunoblot analysis, using monoclonal antibodies to both the 80- and the 320-kDa fragments, the same protein was found to be only partially deficient in 3 of our cases. All these 3 patients were able to walk, with evidence of a mild to moderate muscle involvement, as opposed to the merosin-negative cases which are never ambulant because they are affected by severe muscular deficit. All of them also suffered from a late onset form of epilepsy, a clinical expression of brain involvement rarely described in cases with merosin-negative CMD. The 3 patients with CMD and partial merosin deficiency were investigated by brain MRI and pattern reversal visual evoked potentials (VEP) associated with electroretinography (ERG). The results were compared with those obtained by similar studies on 3 of our cases with complete merosin deficiency. In both types of patients, the neuroimaging evaluation showed supratentorial white matter changes, usually of moderate degree, irrespective of the amount of merosin detected in muscle. The VEP were normal in all the 3 cases with partial merosin deficiency, whereas they showed reduced amplitude or prolonged latency in all the 3 cases with the merosin-negative form. ERG was normal in all 6 cases. As a whole, our data indicate that leukoencephalopathy does not seem to distinguish between the two variants of Cl-CMD. On the other hand, a benign muscle involvement and normal VEP findings seem to distinguish CMD patients with partial merosin deficiency from those with complete deficiency of the same protein. Late onset epilepsy, evident in all our 3 cases with partial merosin deficiency, needs to be evaluated in a larger series of patients in order to be considered a characteristic of this variant of CMD.
Patterns of Brain Magnetic Resonance Abnormalities on T2-Weighted Spin Echo Images in Clinical Subgroups of Multiple Sclerosis: A Large Cross-Sectional Study
Authors: van Walderveen Marianne A.A. , Barkhof Frederik , Tas Mechli W. , Polman Chris , Frequin Stephan T.F.M. , Hommes Otto R. , Thompson Alan J. , Valk Jacob
Published: April 2003

To substantiate differences in magnetic resonance (MR) patterns in clinical subgroups of multiple sclerosis (MS), we analyzed T2-weighted MR images of a large regional population of MS patients (n = 188). The patients had already been classified according to recent consensus definitions regarding the clinical course of MS into relapsing-remitting (RR), secondary progressive (SP) or primary progressive (PP). Significant (p < 0.01; Spearman test) differences were present between RR and SP patients regarding total lesion load, size and location of lesions. RR and PP patients showed similar MR patterns. PP and SP patients differed in total lesion load, small and medium-sized lesions. The degree of atrophy was highest for SP patients. The clinical progression rate [Expanded Disability Status Scale (EDSS)/disease duration] was similar for various subgroups; the MR progression rate (total lesion score/disease duration) was significantly larger for SP than for PP patients. The lesions load disability quotient (total lesion load/EDSS) differed between RR and PP patients and also between SP and PP patients. In SP patients, the total lesion load correlated significantly (Spearman rank correlation coefficient of 0.52) with EDSS. We conclude that PP patients differ in MR abnormalities from SP patients, that PP and RR patients have similar MR abnormalities and that RR and SP patients are at a different end of the same spectrum of the disease. As the dynamics and clinical impact of MS lesions are different in the various clinical subgroups, they should be considered separately in clinical trials.
Indicators and Prevalence of Malnutrition in Motor Neurone Disease
Authors: Worwood A.M. , Leigh P.N.
Published: April 2003

We investigated the nutritional status of a cross-sectional sample of 47 patients with motor neurone disease (MND). Anthropometric measurements (mid-arm circumference and triceps skinfold thickness) and dietary analysis were taken by a trained observer. 21% of patients were moderately to severely malnourished. Patients with bulbar onset were no more likely to be malnourished than patients with limb onset. Measurements of mid-upper-arm muscle circumference indicated that men were more malnourished than women (p = 0.002). We conclude that malnutrition is more prevalent than generally appreciated in patients with MND, including those who do not have swallowing difficulties.
Delayed-Onset Ipsilateral Sensory Symptoms in Patients with Central Poststroke Pain
Authors: Kim Jong-Sung
Published: April 2003

Sensory symptoms almost always develop on the side contralateral to the cerebral lesion. Delayed occurrence of ipsilateral sensory symptoms after stroke has not been recognized. I describe 6 patients with unilateral stroke who initially developed painful sensory symptoms on the side contralateral to the lesion. The patients’ central poststroke pain progressively worsened for a certain period of time when sensory symptoms also occurred on the side ipsilateral to the lesion. The delayed-onset ipsilateral sensory symptom was mild, unaccompanied by objective sensory deficits and developed at the body parts mirroring the site of the most severe central poststroke pain. Possible pathogenic mechanisms for this delayed-onset ipsilateral sensory symptom are discussed.
Central Nervous System Involvement in Thrombotic Thrombocytopenic Purpura
Authors: Druschky A. , Erbguth F. , Strauss R. , Helm G. , Heckmann J. , Neundörfer B.
Published: April 2003

Thrombotic thrombocytopenic purpura (TTP) is an uncommon multisystem disease characterized by microangiopathic hemolytic anemia, thrombocytopenic purpura, fluctuating neurologic dysfunctions, renal disease, and fever. Delayed diagnosis and treatment may lead to a high mortality rate that can be as great as 90%. We describe 7 patients in whom CNS involvement was the first clinical manifestation of TTP. Despite early diagnosis and treatment, 3 patients died because of multiple system organ failure. Four patients recovered completely. In patients with focal neurologic disorders in whom infectious CNS diseases were excluded TTP should be considered in the differential diagnosis.
Cerebral Infarction in Young Women: Analysis of 130 Cases
Authors: Barinagarrementeria Fernando , Gonzàlez-Duarte Alejandra , Miranda Luis , Cantú Carlos
Published: April 2003

The aim of this study was to determine the risk factors and mechanism of cerebral infarction in young women. Methods: We evaluated 130 consecutive women younger than 41 years of age with cerebral infarction and compared the risk factors with a control group of 122 healthy, age-matched women. Results: The leading risk factors in patients with cerebral infarction were migraine (15%), tobacco use (15%), and oral contraceptive (OC) use (12%). Cerebral arteriograms were abnormal in 59% of patients (57 of 96). The causes of cerebral infarction were cardiac embolism in 36%, nonatherosclerotic vasculopathy in 25%, hematologic disorders in 8%, and migraine in 8%. The etiology could not be determined in 23% of patients. Conclusion: Migraine and OCs are independent risk factors for cerebral infarction in young women. The leading etiologies were rheumatic valve disease and nonatherosclerotic vasculopathy, hematologic disturbances, and migraine were responsible for a few cases.
Role of Somatosensory Feedback from Tools in Realizing Movements by Patients with Ideomotor Apraxia
Authors: Wada Yuko , Nakagawa Yoshitsugu , Nishikawa Takashi , Aso Noriko , Inokawa Maki , Kashiwagi Asako , Tanabe Hirotaka , Takeda Masatoshi
Published: April 2003

In order to investigate the underlying mechanism of ideomotor apraxia, we studied 9 patients who could not mime using tools despite the ability to manipulate actual tools normally. In all the mime tasks, visually presented tools or model gestures by examiners were fundamentally ineffectual in improving the patients’ performances. Even the remarkable improvement demonstrated when using actual tools disappeared immediately after the subjects took their hands off them. In a further experiment, 4 of the 9 patients were required to pretend to use tools while holding a stick, resulting in significant improvements or normal miming. These findings suggest that the somatosensory feedback continuously supplied from a handheld tool is a crucial component in enabling patients with ideomotor apraxia to actually use tools.
Analysis of CAG Repeat Expansion in Huntington’s Disease Gene (IT 15) in a Hungarian Population
Authors: Jakab Katalin , Gárdián Gabriella , Endreffy Emôke , Kalmár Tibor , Bachrati Csanád , Vécsei L. , Raskó I.
Published: April 2003

Huntington’s disease (HD) is a neurodegenerative disorder with autosomal dominant inheritance. The genetic defect is a CAG trinucleotide repeat expansion at the 5′ end of the IT 15 gene on chromosome 4. This gene has not been analyzed in the Hungarian population yet. To obtain data DNA from 26 HD patients, 18 members of their families and 70 normal controls was amplified in the involved region by polymerase chain reaction. The CAG repeat numbers varied from 37 to 70 (median: 43) in HD patients and asymptomatic carriers, while individuals of the normal control group had 10–36 CAG repeat numbers (median: 18). The length of CAG repeat expansion in Hungarian HD patients was similar to that reported from other countries. The group of normal controls had the same CAG repeat expansion as populations reported from Western European countries. It is a useful piece of data for population genetics to prove that the population of Hungary is a mélange of different nations that influenced the history of the country in the last 11 centuries. As opposed to this, the only closely related nation, the Finnish, was genetically more isolated during this time, so the frequency of HD (and also the number of CAG repeats in normal individuals) proved to be exceptionally low.
An Audit of the Perceived Efficacy and Tolerability of Gabapentin Therapy in an Out-Patient Cohort with Chronic Epilepsy
Authors: Langan Y. , Duncan J.S. , Sander J.W.A.S.
Published: April 2003

A retrospective audit of the perceived efficacy and tolerability of adjunctive gabapentin (GBT) therapy in patients with intractable epilepsy was carried out. The case-notes of 263 consecutive patients attending a tertiary referral centre in whom GBT had been added to concomitant anti-epileptic drugs were reviewed. There were 119 males (45%); the median age was 35 years (range 15–81 years) and the median duration of epilepsy was 20 years. The median dose of GBT administered was 1,600 mg (range 300–4,800 mg). Twenty-nine patients (11%) had a reduction in seizure frequency of greater than 50% and 7 (3%) achieved seizure freedom of 6 months’ duration at least while taking GBT. Side-effects were reported by 169 patients (61%) and in 40 (15%) an increase in seizure frequency of 50% occurred, this being the commonest reason for GBT withdrawal. The median duration of treatment was 4 months (range 0.03–47 months). Ten patients, in whom GBT therapy had proven ineffective in doses of 900–1,800 mg, were rechallenged with doses of 300–4,800 mg. One individual experienced a 50% reduction in seizure frequency and 1 an increase in seizures of at least 50%. GBT was generally well tolerated at high doses and rechallenge of a small number with GBT in doses up to 4,800 mg did not result in further improvement in seizure frequency in the majority.
Posturographic Findings in Patients with Idiopathic Cervical Dystonia before and after Local Injections with Botulinum Toxin
Authors: Wöber Christian , Schnider Peter , Steinhoff Nikolaus , Trattnig Siegfried , Zebenholzer Karin , Auff Eduard
Published: April 2003

In this posturographic study, whole-body postural control was impaired in more than 75% of patients with idiopathic cervical dystonia (ICD) before local injections with botulinum toxin type A (BTX-A) and the impairment was independent of the direction of the torticollis. Six weeks after therapy with BTX-A, the number of pathological posturographic parameters had decreased by almost 30%, and the improvement reached statistical significance for sway path, sway area and anteroposterior sway during stance on foam with eyes closed. From a pathophysiological point of view, this improvement may be explained – at least in part – by a reduction of abnormal proprioceptive input from the neck. Accordingly, it seems possible that neck proprioceptive input plays a role in whole-body postural control in ICD patients, even though previous studies suggested that the neck input is relatively ignored in these patients.
The 14-3-3 Protein Detectable in the Cerebrospinal Fluid of Patients with Prion-Unrelated Neurological Diseases Is Expressed Constitutively in Neurons and Glial Cells in Culture
Authors: Satoh Jun-ichi , Kurohara Kazuhiro , Yukitake Motohiro , Kuroda Yasuo
Published: April 2003

The 14-3-3 protein belongs to a family of 30-kD proteins originally identified by two-dimensional analysis of brain protein extracts. Recently, the detection of the 14-3-3 protein in the cerebrospinal fluid (CSF) is utilized as a highly reliable test for the premortem diagnosis of prion diseases such as Creutzfeldt-Jakob disease. For the initial step, to clarify the biological implication of the CSF 14-3-3 protein in these diseases, its expression was investigated in neural tissues and cultures and CSF samples from patients with a variety of neurological diseases by Western blot analysis and immunocytochemistry. The constitutive expression of the 14-3-3 protein was identified in all neural and nonneural tissues examined. It was expressed in all neurons, astrocytes, oligodendrocytes, and microglia in culture with its location in both cytoplasmic and nuclear regions. The 14-3-3 protein was detected in the CSF of 8 out of 71 patients, including 1 Gerstmann-Sträussler-Scheinker disease patient and 7 patients with prion-unrelated neurological diseases, such as meningoencephalitis of viral, bacterial, or tuberculous origin, multiple sclerosis, and mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes. These results suggest that the 14-3-3 protein expressed constitutively at substantial levels in both neurons and glial cells might be released into the CSF as a disease-nonspecific consequence of the extensive brain damage and indicate that the analysis of the 14-3-3 protein in the CSF is not useful as a screening test for prion diseases.
Delayed Movement Disorders after Carbon Monoxide Poisoning
Authors: Choi Il Saing , Cheon Hwa Young
Published: April 2003

Of 242 patients with carbon monoxide (CO) poisoning examined between 1986 and 1996, delayed movement disorders were diagnosed in 32 (13.2%). There were 15 men and 17 women. Ages at insult ranged from 9 to 69 years (mean 45.3 years). Of the 32 patients with delayed movement disorders, 23 (71.9%) had parkinsonism, 5 dystonia, 3 chorea and 1 myoclonus. All were associated with delayed CO encephalopathy. The median latency between CO poisoning and the onset of movement disorders was 4 weeks for parkinsonism, 51 weeks for dystonia, 4 weeks for chorea and 8 weeks for myoclonus. The latency of dystonia onset after CO poisoning was longer than that of other types of movement disorders. The CT findings in delayed movement disorders after CO poisoning were variable, and there was no correlation between the sites of imaging and the development of movement disorders. Abnormal dyskinesias disappeared within 8 weeks, and patients recovered from parkinsonism within 6 months. In conclusion, delayed movement disorders after CO poisoning are not rare, and usually appear as a part of delayed CO encephalopathy. The prognosis is good.
Risk Factors for Stroke in Different Levels of Cerebral Arterial Disease
Authors: Kim Jong Sung , Choi-Kwon Smi
Published: April 2003

Background and Purpose: Despite recent studies on risk factors of stroke, it is still unclear whether certain risk factors differently affect different levels of cerebral arteries in stroke patients. We attempted to study risk factors and lifestyle factors in patients with cerebrovascular diseases affecting different levels of the cerebral arteries. Methods: We studied 343 Korean patients (222 men and 121 women) with acute stroke. Strokes were subdivided into large vessel infarction (LVI, n = 156), small vessel infarction (SVI, n = 105), intracerebral hemorrhage (ICH, n = 82) according to modified TOAST criteria. Patients with cardiogenic embolic infarction, undetermined causes, and miscellaneous causes were excluded. Among the patients with LVI and SVI, 104 and 49 underwent angiographic studies, respectively, who were designated as having definite LVI and definite SVI. Definite LVI was further subdivided into proximal LVI when the internal carotid artery (n = 25), vertebral artery (n = 13) or basilar artery (n = 14) were primarily involved, and distal LVI when the middle cerebral artery (n = 35), anterior cerebral artery (n = 7) or posterior cerebral artery (n = 10) were involved. Using a structured interview, we assessed the risk factors and lifestyles: hypertension, diabetes mellitus (DM), cigarette smoking (CS), alcohol consumption, physical activity and exercise, and the amount of consumption of salt, vegetables, fat, fish and fruits. Serum cholesterol, body mass index and total body fat were also measured. Results: Multivariate analysis showed that in men, DM was a discriminant factor favoring brain infarction (BI) over ICH, definite LVI or ICH and proximal LVI over ICH. Decreased fruit intake differentiated distal LVI from definite SVI, and distal LVI from ICH. In women, DM and abdominal obesity differentiated BI from ICH, while DM favored definite LVI over ICH, proximal LVI over ICH and definite SVI over ICH. Multivariate analysis including sex as a separate variate showed that DM was a factor favoring BI over ICH, definite LVI over ICH, proximal LVI over ICH, distal LVI over ICH and definite SVI over ICH. CS was a factor favoring BI over ICH, while heavy CS was a factor discriminating distal LVI from definite SVI. Male sex was a factor favoring definite LVI over ICH and proximal LVI over ICH. A lesser amount of fruit consumption favored definite SVI over distal LVI. Conclusion: Our results demonstrate that DM in all sexes and central obesity in women are factors discriminating BI from ICH. However, these factors as well as others were not found to discriminate the sizes of the vessels involved in the patients with ischemic stroke, except that being male may be a factor favoring proximal large vessel disease over ICH. Genetic factors or other not yet identified risk factors may be responsible for the differences in the subtypes of ischemic stroke among different ethnic populations.
Ictal Vomiting in Partial Seizures of Temporal Lobe Origin
Authors: Chen Chien , Yen Der-Jen , Yiu Chun-Hing , Shih Yang-Hsin , Yu Hsiang-Yu , Su Ming-Shung
Published: April 2003

We report 3 cases presenting ictal vomiting during partial seizures of temporal lobe origin. Two patients had complex partial seizures accompanying vomiting characteristics. Ictal vomiting occurred early in the course of the seizure when rhythmic discharges involved predominantly the left hemispere, the language dominance hemispere. The other patient had ictal vomiting in simple partial seizures which originated from the right temporal lobe or the language nondominant side. All 3 patients underwent anterior temporal lobectomy with promising outcomes. Pathologic diagnosis included hippocampal sclerosis in 2 patients and astrocytoma in 1 patient. In our patients, ictal vomiting does not lateralize temporal lobe epilepsy and is not specific to pathology.
Pontine Atrophy in Spinocerebellar Ataxia Type 6
Authors: Sugawara Masashiro , Toyoshima Itaru , Wada Chizu , Kato Kazumaro , Ishikawa Kazuo , Hirota Koichi , Ishiguro Hideaki , Kagaya Hajime , Hirata Yutaka , Imota Tsuyoshi , Ogasawara Masumi , Masamune Osamu
Published: April 2003

To investigate the clinical range of spinocerebellar ataxia type 6 (SCA6), we screened CAG repeat expansion in the voltage-dependent alpha 1A calcium channel gene (CACNL1A4) in 71 ataxic patients in 60 families; 54 patients in 43 families with hereditary ataxia and 17 sporadic patients. Thirteen patients with SCA6 were detected to have elongated CAG in CACNL1A4. Of these, 7 patients had been diagnosed as having hereditary cerebellar cortical atrophy, and 6 patients had been found to have sporadic occurrence. One patient showed distinct pontine atrophy with prominent horizontal or oblique gaze nystagmus which is an unusual feature in sporadic olivopontocerebellar atrophy. For the efficient screening of SCA6, we would propose testing CAG repeat expansion in CACNL1A4, in patients with one of two markers: (1) horizontal or oblique gaze nystagmus without other eye movement disorders, (2) pure cerebellar atrophy, even if occurrence is sporadic. We should note that the pontine atrophy could also be caused by CAG repeat expansion in CACNL1A4.
Hereditary Cerebellar Ataxia with Peripheral Neuropathy and Mental Retardation
Authors: Tachi Nobutada , Kozuka Naoki , Ohya Kazuhiro , Chiba Shunzo , Sasaki Kimio
Published: April 2003

We present here 5 patients with hereditary cerebellar ataxia with peripheral neuropathy and mental retardation as determined by clinical, pathological, and molecular studies. The most characteristic features of this disorder, in contrast to Friedreich’s ataxia, were early onset of ataxic gait, mental retardation, and a marked atrophy of the cerebellum. Sural nerve biopsy showed a reduction of myelinated fibers. The expansion of a GAA triplet repeat within the first intron of the frataxin gene, which causes Friedreich’s ataxia, was not identified in any of the patients. Hereditary cerebellar ataxia with peripheral neuropathy and mental retardation represents a specific clinical entity that so far has only been described in Japan.
Chronic Fatigue Syndrome in Patients with Lyme Borreliosis
Authors: Treib Johannes , Grauer Markus T. , Haass Anton , Langenbach Jürgen , Holzer Gerhard , Woessner Ralph
Published: April 2003

Several authors have reported a chronic fatigue-like syndrome in patients that have suffered from Lyme borreliosis in the past. To further investigate this suspicion of an association without sample bias, we carried out a prospective, double-blind study and tested 1,156 healthy young males for Borrelia antibodies. Seropositive subjects who had never suffered from clinically manifest Lyme borreliosis or neuroborreliosis showed significantly more often chronic fatigue (p = 0.02) and malaise (p = 0.01) than seronegative recruits. Therefore we believe it is worth examining whether an antibiotic therapy should be considered in patients with chronic fatigue syndrome and positive Borrelia serology.
Characteristic Expression of Thrombomodulin in the Muscle Sarcoplasm in Patients with the Acute Phase of Rhabdomyolysis
Authors: Hu Jing , Higuchi Itsuro , Inose Masaru , Niiyama Takahito , Uchida Yuichi , Osame Mitsuhiro
Published: April 2003

The expression of thrombomodulin and neural cell adhesion molecule (NCAM) was studied immunocytochemically in biopsied muscle specimens from 10 patients with rhabdomyolysis with different etiologic factors, including 5 with malignant hyperthermia. We have already reported that thrombomodulin was expressed on regenerating muscle cell membranes as well as on vessel walls in patients with various neuromuscular diseases, including Duchenne muscular dystrophy, Becker muscular dystrophy and inflammatory myopathy. We found increased expression of thrombomodulin not only on the sarcolemma, but also in the sarcoplasm of a fair number of muscle fibers in the acute phase of rhabdomyolysis. The granular pattern of thrombomodulin expression in the sarcoplasm seems to be a characteristic finding in the acute phase of rhabdomyolysis. Most muscle fibers which expressed NCAM on the sarcolemma also expressed thrombomodulin. However, the muscle fibers which expressed thrombomodulin in the sarcoplasm did not express NCAM, and showed a degenerative appearance on electron microscopic examination. These results suggest that thrombomodulin is expressed in the sarcoplasm during the acute degeneration phase of rhabdomyolysis in addition to the expression on the sarcolemma during the muscle fiber regeneration as shown in our previous study, and the former process, which is characterized by the granular expression of thrombomodulin in the sarcoplasm, may be a characteristic finding in rhabdomyolysis.
Contribution of PET Studies in Diagnosis of Corticobasal Degeneration
Authors: Lutte Isabelle , Laterre Christian , Bodart Jean-Michel , De Volder Anne
Published: April 2003

Five patients with clinical features of corticobasal degeneration (CBD) were studied with PET imaging. The main clinical findings included a unilateral extrapyramidal motor disorder, without significant response to levodopa, as well as clumsiness, dysarthria, apraxia and a clear asymmetry of neurological signs. PET studies with 18F-labeled 2-deoxy-2-fluoro-D-glucose disclosed mainly a significant hypometabolism in the thalamus and motor cortex controlateral to the more affected limbs. Additional relationships between individual clinical signs and PET data were also found. We concluded that PET findings supported the clinical diagnosis of CBD, although the specific pattern related to this condition needs to be more precisely defined. Further studies are especially needed to correlate clinical data and PET results with pathological examination.
Serum Markers of Type I Collagen Synthesis and Degradation in Amyotrophic Lateral Sclerosis
Authors: Ono Seiitsu , Imai Takashi , Shimizu Natsue , Nakayama Megumi , Yamano Takahiko , Tsumura Mayumi
Published: April 2003

Collagen abnormalities in the skin and spinal cord have been reported in amyotrophic lateral sclerosis (ALS) patients. Serum carboxyterminal propeptide of type I procollagen (PICP) and the carboxyterminal cross-linked telopeptide of type I collagen (ICTP) reflect type I collagen synthesis and degradation, respectively. However, there has been no study concerning PICP or ICTP in ALS. We studied collagen contents of the skin and measured serum levels of PICP and ICTP in patients with ALS and control subjects. Serum PICP levels were significantly lower in ALS patients than in controls. Serum ICTP levels were significantly higher in ALS patients than in controls, and there was an appreciable positive correlation between serum ICTP levels and the duration of illness in ALS patients. In ALS patients, the collagen content of the skin was significantly smaller than in controls and indicated a progressive decrease in relation to illness. In addition, there was a significant negative correlation between serum ICTP concentrations and the collagen content of the skin in ALS patients. These data suggest that increased ICTP levels and decreased serum PICP levels may reflect unique changes in the skin, with a predominance of degradation compared to the synthesis of type I collagen in ALS.
Cardiac Autonomic Function in Patients Suffering from Primary Focal Hyperhidrosis
Authors: Birner Peter , Heinzl Harald , Schindl Monika , Pumprla Jiri , Schnider Peter
Published: April 2003

Cardiac autonomic function in patients (n = 63) with primary focal hyperhidrosis and healthy controls (n = 28) was investigated by short-term frequency domain power spectral analysis of heart rate variability. The power of the very-low-frequency band (0.01–0.05 Hz) was significantly lower in patients with axillary hyperhidrosis than in controls. No differences between groups could be observed at investigation of the low-frequency band (0.05–0.15 Hz), which was a surprising finding because this band represents also sympathetic cardiac innervation. At the high-frequency band (0.15–0.5 Hz), which represents parasympathetic cardiac innervation, an interaction of type and position influencing spectral power was detected. Our highly interesting findings indicate that primary focal hyperhidrosis is based on a much more complex autonomic dysfunction than generalised sympathetic overactivity and seems to involve the parasympathetic nervous system as well.
Long-Term Remission of Idiopathic Cervical Dystonia after Treatment with Botulinum Toxin
Authors: Giladi N. , Meer J. , Kidan H. , Honigman S.
Published: April 2003

Botulinum toxin type A (BTX-A) treatment for cervical dystonia is traditionally considered a purely symptomatic treatment. BTX-A blocks acetyl choline exocytosis for 3–6 months and most patients require reinjection after this period. We report on 6 patients (mean age 41.6 years, range 18–69) with idiopathic cervical dystonia who were treated with BTX-A injections and became asymptomatic for 2–4 years. Four patients showed remission after the first BTX-A treatment, 1 patient after the second set of injections and 1 after the third session. Amelioration of neck dystonia was observed within 1–4 weeks after the last BTX-A treatment and all 6 patients are symptom-free, off antidystonic medications for over 2 years. The possibility that BTX-A treatment may increase the chances of development of clinical remission in patients with idiopathic cervical dystonia is discussed.
Relationship of p53 and Vascular Endothelial Growth Factor Expression to Clinicopathological Factors in Human Scirrhous Gastric Cancer
Authors: Baba M. , Konno H. , Maruo Y. , Tanaka T. , Kanai T. , Matsumoto K. , Matsuura M. , Nishino N. , Maruyama K. , Nakamura S. , Baba S.
Published: April 2003

Although scirrhous cancer has the highest malignant potential among various types of gastric cancer, its pathogenesis is still unclear. The relationship between expression of p53 or vascular endothelial growth factor (VEGF) and clinicopathological variables was investigated by immunohistochemical analysis of archival specimens from 40 patients with scirrhous gastric cancer. Staining for p53 and VEGF was observed in the nuclei and cytoplasm of the tumor cells, respectively. There was no significant association between expression of p53 or VEGF and sex, age, depth of invasion, lymph node metastasis or histological stage. Peritoneal dissemination was the most frequent mode of recurrence, and the depth of tumor invasion was a crucial factor. The recurrence rate was 83.9% (26/32) in patients with serosal invasion, whereas it was 22.2% (2/9) in patients without serosal invasion. Only 7 out of 40 patients (17.5%) survived without recurrence. Among them, the VEGF-positive rate was 14.3% (1/7), whereas it was 52.6% (10/19) in the patients with recurrence. There was no correlation between p53 and VEGF staining. These findings suggest that the progression of scirrhous gastric cancer may be promoted by VEGF overexpression, which is not upregulated by p53 mutation.
Protection of Xeno-Hepatocytes from Complement-Mediated Cytolysis by Transduction with Homologous Restriction Factor 20 Gene Using Retroviral Vector
Authors: Hayashi S. , Isobe K. , Emi N. , Okada H. , Yokoyama I. , Takagi H.
Published: April 2003

Xeno-hepatocyte transplantation has the possibility to substitute for clinical liver transplantation in certain restricted hepatic diseases such as inherited metabolic disorders. To overcome human complement-dependent cytotoxicity on xeno-hepatocytes, the effectiveness of ex vivo transfer with the homologous restriction factor 20 (HRF20, CD59) gene was examined on primary-cultured xeno-hepatocytes using a retroviral vector. Primary-cultured rat hepatocytes transduced with HRF20 cDNA expressed HRF20 antigen by flowcytometric analysis and showed the integration of HRF20 cDNA to the genomic DNA by the polymerase chain reaction. The viability of rat hepatocytes incubated with 50% human serum was decreased due to complement-dependent cytotoxicity, whereas that of the transfectant was significantly protected (77.2 ± 9.4 vs. 97.8 ± 5.2%, p < 0.01). It was concluded that primary-cultured xeno-hepatocytes transduced with HRF20 cDNA using a retroviral vector could escape complement-dependent cytolysis by human serum.
Hyperactive Cytokine Response after Partial Hepatectomy in Patients with Biliary Obstruction
Authors: Kimura F. , Miyazaki M. , Suwa T. , Itoh H. , Ambiru S. , Shimizu H. , Nakagawa K.
Published: April 2003

The relation of interleukin-6 (IL-6) levels in serum to the postoperative complications was assessed in 50 patients following an extensive hepatectomy. In addition, the postoperative levels of IL-6, tumor necrosis factor-α, and interleukin-1β in peritoneal exudative fluid were determined in 22 patients. The patients with preoperative biliary obstruction showed a higher incidence of postoperative infection compared to those without (44 vs. 12%, p = 0.043). The postoperative serum IL-6 levels on day 1 were significantly higher in patients with postoperative infection than in those without (p < 0.01). Biliary obstruction and intraoperative blood loss contributed to the increase in the serum IL-6 levels in a multiple regression analysis (p = 0.01 and p = 0.044, respectively). Further, the fluid levels of inflammatory cytokines were significantly higher in patients with biliary obstruction than in those without (p < 0.05). These results suggest that in patients with biliary obstructions a hyperactive cytokine response occurs after an extensive hepatectomy and results in a high incidence of postoperative infection.
Measurement of (1→3)-β-D-Glucan in an Experimental Model of Systemic Candidiasis
Authors: Kawagoe T. , Nakao A. , Kanbe T. , Tamura H. , Tanaka S. , Takagi H.
Published: April 2003

To investigate the utility of measuring blood concentrations of (1→3)-β-D-glucan, a component of the fungal cell wall, as an auxiliary diagnostic method for systemic candidiasis, rats were inoculated with Candida albicans and the number of C. albicans in the viscera and glucan in the blood were quantitated. The concentration of blood glucan and the number of C. albicans in the viscera were also measured both under leukopenia and with deteriorated reticuloendothelial system cell function, and when the liver and spleen had been excised. As a result, systemic candidiasis appeared in the group with leukopenia, and the number of living C. albicans increased in the kidney and liver. Together with this increase in the number of C. albicans, there was an increase in blood (1→3)-β-D-glucan. Measurements of blood (1→3)-β-D-glucan well reflect a proliferation of C. albicans in vivo, which would make this a useful auxiliary for the clinical diagnosis of systemic mycosis.
Metabolism of Superoxide Dismutase during the Adynamic Ileus of Endotoxemia
Authors: Cullen J.J. , Ephgrave Kimberly S. , Hemann Lynda L. , Hinkhouse Marilyn , Conklin J.L.
Published: April 2003

Endotoxemia is accompanied by gastrointestinal motor disturbances while administration of oxygen free radical scavengers following endotoxemia ameliorates these motor disturbances. We hypothesized that the motor disturbance produced by endotoxemia may be associated with a change in the activity or production of superoxide dismutase (SOD). Strain gauge transducers were placed on the stomach and small bowel of dogs to determine fasting gastrointestinal motility. Manganese and copper/zinc SOD enzyme activities in gut smooth muscle were measured and protein expression was analyzed by Western blotting. Following completion of the baseline studies, dogs were given a single dose of Escherichia coli lipopolysaccharide, 200 µg/kg i.v., and the studies repeated for the next 3 days. Following endotoxin bolus, the migrating motor complex (MMC) was delayed for 2 days while gastric and jejunal smooth muscle levels of Cu/Zn SOD activity were decreased for 1 day in the stomach and for 2 days in the jejunum. Western blotting indicated that endotoxin did not change the amount of SOD enzyme produced. We conclude that endotoxemia results in a temporary prolongation of the MMC and a decrease in Cu/Zn SOD activity. Expression of Cu/Zn SOD and Mn SOD does not seem suppressed by endotoxin as measured by Western blotting. However, the impairment of Cu/Zn SOD activity may contribute to ileus associated with sepsis by altering oxygen-free metabolism in both the stomach and jejunum.