bluuebunny/crossref_metadata_2025 huggingface dataset:
Outcome of intracerebral hemorrhage: clinical and CT findings in 326 patients
Authors: Berlit P. , Tornow K.
Published: January 2011

Clinical symptoms and findings in cranial computed tomography (CT) were evaluated in 326 patients with intracerebral hemorrhage (ICH). Localizations of ICH were the lobes (n = 254), the basal ganglia (n = 46), the pons and brain stem (n = 13) and the cerebellum (n = 8). Multiple hematomas were present in nine patients. An initial coma (n = 225) was most frequent in ICH of the pons (n = 7), cerebellum (n = 6), and the frontal (n = 71) and temporal (n = 66) lobes. Epileptic seizures (n = 70) were most common in hematomas of the frontal (n = 24), temporal (n = 19) and parietal (n = 12) lobes and the basal ganglia (n = 6). A history of hypertension was given in 140 patients; 119 of these had an ICH with a size of ≥3 cm. Mortality (n = 162) was high with ICH in the pons and brain stem (10 out of 13), in the frontal (54 out of 98) and parietal (32 out of 58) lobes and the basal ganglia (n = 23). A size of the ICH of 3 cm or more in cranial CT and an associated ventricular hemorrhage were associated with a bad outcome. An initial disturbance of consciousness was the only reliable clinical predictor of outcome (chi‐square, p < 0.001). Katamnestic evaluation of 66 of the 164 survivors after 5.2 years revealed seizures in 20 patients and mild neurological deficits in 41. Another 14 patients were partially, and nine totally dependent Nineteen patients had died in between; there was only one death attributable to another ICH.
Interictal “patchy” regional cerebral blood flow patterns in migraine patients. A single photon emission computerized tomographic study
Authors: Friberg L. , Olesen J. , Iversen H. , Nicolic I. , Sperling B. , Lassen N.A. , Olsen T. Skyhøj , Tfelt‐Hansen P.
Published: January 2011

In 92 migraine patients and 44 healthy control subjects we recorded regional cerebral blood flow (rCBF) with single photon emission computerized tomography and 133Xe inhalation or with i.v. 99mTc‐HMPAO. Migraine patients were studied interictally. A quantitated analysis of right‐left asymmetry indices in a fixed set of regions of interest was compared with the normal asymmetry indices in the healthy controls. An asymmetry index deviating more than ± 2.5 S.D.s in normals was defined as pathological asymmetry. By quantitated analysis 47% of images from patients with aura attacks and 48% of images from patients without aura attacks were established to contain higher rate of asymmetries, the difference being statistically significant (p < 0.05, Wilcoxon). A blinded visual analysis and scoring by a four level scale were done by four experienced observers. rCBF images from 18% of patients having attacks with aura and from 19% of patients without aura attacks was scored as containing abnormal right‐left asymmetries by the visual analysis. Images from healthy controls were all scored to be normal. In 37% of the images (all from patients) there was lack of consensus among observers (κ = 0.28). There was no correlation between visual or quantitated abnormalities and age, duration of migraine, frequency of attacks or prophylactic medication. No correlation could be established between asymmetries and the usual side of headache or aura symptoms. Two conclusions emerged: (1) visual evaluation of interictal migraine rCBF images is insufficient to pick up abnormalities; (2) almost 50% of the migraine sufferers had abnormal rCBF/asymmetries. However, these are discrete compared with those typically seen during the aura phase of a migraine attack. One explanation to the patchy rCBF patterns might be that they reflect interictal cerebrovascular dysregulation which might to be a common feature in both types of migraine.
Evaluation of gelatinases and IL‐6 in the cerebrospinal fluid of patients with optic neuritis, multiple sclerosis and other inflammatory neurological diseases
Authors: Paemen L , Olsson T. , Söderström M. , van Damme J. , Opdenakker G.
Published: January 2011

The activities of the metalloproteinase gelatinase B, and the presence of IL‐6, an inducer of metalloproteinase inhibitors, were investigated in CSF samples of 190 patients with multiple sclerosis (MS; n = 55), optic neuritis (ON; n = 46), other inflammatory neurological diseases (OIND; n = 27) or control patients (CON) with non‐inflammatory neurological diseases (n = 62). IL‐6, measurable as hybridoma growth factor activity (detection limit 3 pg/ml), was found in only four of these 190 CSF samples (three OIND, one CON). Elevated CSF gelatinase B levels were detected in 40%, 35% and 54% of the patients with MS, ON and OIND, respectively, while all control CSFs were devoid of gelatinase B activity. Clinical and laboratory data were compared with gelatinase B levels. No correlation was found between the CSF cytoses and gelatinase B levels, suggesting that this enzyme in the CSF originates from CNS lesions rather than from CSF cells. However, the occurrence of the gelatinase B significantly correlated with the IgG index in the MS patient group. This study stimulates further investigation into the possible usage of protease inhibition in demyelinating diseases.
A nitric oxide donor (nitroglycerin) triggers genuine migraine attacks
Authors: Thomsen L.L. , Kruuse C. , Iversen H.K. , Olesen J.
Published: January 2011

Supersensitivity to induction of headache and arterial dilatation by a donor of nitric oxide (nitroglycerin) has recently been demonstrated in migraine sufferers. The aims of the present study were to examine whether the nitric oxide donor nitroglycerin may induce a typical migraine attack, to exclude placebo‐related effects and to describe the relation between middle cerebral artery dilatation and provoked migraine. Nitroglycerin (0.5 μg/kg/min for 20 min) or placebo was infused into 12 migraine patients in a double‐blind cross‐over trial. Blood velocity in the middle cerebral artery was measured with transcranial Doppler and characteristics of headache and accompanying symptoms were recorded frequently. Headache occurred during the nitroglycerin infusion as previously described but peak headache intensity did first occur 5.5 h after infusion. At this time the induced headaches in 8 of 10 completing patients fulfilled the diagnostic criteria for migraine without aura of the International Headache Society. Furthermore, all patients who normally had unilateral spontaneous migraine attacks also had unilateral headaches after nitroglycerin. Only one subject developed migraine after placebo (p< 0.03). The time pattern of headache and estimated middle cerebral artery dilatation corresponded well. The study therefore demonstrates that activation of the nitric oxide cGMP pathway may cause typical migraine attacks.
Cognitive profiles and regional cerebral blood flow patterns in dementia of the Alzheimer type
Authors: Waldemar G. , Bruhn P. , Schmidt E. , Kristensen M. , Lassen N.A. , Paulson O.B.
Published: January 2011

Individual cognitive profiles and correlations between cognitive functions and regional cerebral blood flow (rCBF) were analyzed in 20 consecutive patients with a clinical diagnosis of probable Alzheimer's disease (AD). CBF was measured with high resolution single photon emission computed tomography (SPECT) and [99mTc]d,l‐HMPAO. The analysis of cognitive profiles was based on the composite scores for six cognitive domains, derived from a detailed neuropsychological test battery, as compared with corresponding test data obtained in a control group of 28 age‐matched healthy volunteers. The cognitive profiles displayed a marked heterogeneity as concerned the general level of cognitive impairment, the number of significantly affected cognitive domains, the spectrum of affected and non‐affected cognitive domains, and the severity of each cognitive dysfunction. Statistically significant correlations with rCBF were found for memory scores (right frontal and temporal cortex), abstraction scores (frontal/parietal ratio of rCBF), language scores (left frontal and temporal cortex), visual perception scores (rCBF throughout the right hemisphere), and for visuo‐construction scores (side‐to‐side asymmetry of parietal rCBF). We conclude that the previously observed topographical heterogeneity of rCBF distribution patterns in probable AD was reflected by differences in cognitive profiles. The observed heterogeneities stress the relevance of analyzing individual cognitive and rCBF data, as a supplement to group comparisons of data, in the investigation of diseases with potential heterogeneous affections of the brain.
Quantified electroencephalographic changes in Parkinson's disease with and without dementia
Authors: Pozzi D. , Petracchi M. , Sabe L. , Dancygier G. , García H. , Starkstein S.
Published: January 2011

We examined the presence of quantified electroencephalographic (qEEG) differences between Parkinson's disease (PD) patients with and without dementia, and a group of age‐comparable normal controls. While there were no significant differences in relative power in any of the qEEG bands between PD patients without dementia and normal controls, PD patients showed a significantly greater reactivity in the alpha band. On the other hand, PD patients with dementia showed significantly less alpha and more theta relative power than both the normal control and the PD without dementia groups. When PD patients with dementia were compared with a group of patients with Alzheimer's disease (AD) and a similar severity of dementia, no significant between‐group differences were found in any of the qEEG bands. In conclusion, while our study demonstrated no significant qEEG differences between non‐demented PD patients and normal controls, PD patients with dementia showed qEEG changes similar to those observed in patients with AD.
Loss of taste and carbamazepine
Authors: Iñiguez C. , Perez‐Trullen J.M. , Jimenez‐Escrig A.
Published: January 2011

Carbamazepine is the drug of choice for many partial and generalized seizures. Neurotoxic manifestations are the most common side effect. We present an unrecognized neurological complication associated with carbamazepine occurring probably as an idiosyncratic event.
Cerebral amyloid angiopathy, leukoencephalopathy and dementia of acute onset. Clinicopathological study of a new case
Authors: Muñoz J.E. , Saiz A. , Ribalta T. , Graus F. , Tolosa E.
Published: January 2011

Cerebral amyloid angiopathy (CAA) is a well‐established cause of brain hemorrhage in the elderly. The association of CAA with other structural brain lesions is less well known. We report a 69 year old diabetic woman with dementia of acute onset Cranial CT and MRI showed diffuse leukoencephalopathy with predominant right parieto‐occipital distribution. Pathological study demonstrated severe CAA. We review the association of CAA with dementia and leukoencephalopathy.
The premorbid personality of patients with Parkinson's disease: evidence with the Tridimensional Personality Questionnaire
Authors: Kostić V.S. , Lečić D. , Kovačević Z. , Filipović S. , Šternic N.
Published: January 2011

The premorbid personality traits of 102 patients with Parkinson's disease (PD) and 57 age‐matched healthy controls were studied by the recently developed Tridimensional Personality Questionnaire. We found significantly fewer (p < 0.05) of a group of traits called “novelty seeking” (NS), but no changes in “harm avoidance” (HA) and “reward dependence” (RD), in PD patients, for the period approximately 5–10 years before the onset of the disease, compared to controls. Individuals who are lower than average in NS and average in HA and RD are described as reflective, rigid, loyal, stoic, slow‐tempered, frugal, orderly, and persistent Since NS is thought to be directly related to central dopaminergic reactivity, the premorbid expression of these behaviors may be the reflection of neurochemical deficits (hypodopaminergic tone) accompanying the presymptomatic phase of PD.
Stroke epidemiology in Europe
Authors: Inzitari D. , Lamassa M. , Amaducci L.
Published: January 2011

The information on epidemiology of stroke in Europe comes from mortality statistics and incidence studies. The purpose of this paper was to review the data on mortality, incidence, case fatality and stroke care in Europe, and to discuss geographical and temporal trends, taking into account methodological problems. The available updated studies on mortality and incidence were reviewed. Considering methodological characteristics, incidence studies were evaluated separately for “ideal” and “nonideal” studies. The preliminary data of the ongoing EC Stroke Project were also examined. Marked differences are observed in mortality rates across European countries. Rates are higher in Eastern compared to Western countries. Temporal trends are decreasing in Western and increasing in Eastern countries. Incidence data are scanty and probably not fully representative. Preliminary observations show that marked differences exist in stroke care across different countries. Geographical and temporal trends in mortality and morbidity may reflect both methodological inhomogeneity and differences in prevalence of risk factors, incidence and survival. Differences in socio‐economical level may have an impact on stroke care, and may indirectly influence stroke frequency measures. A collaborative effort is needed for standardizing the methodologies, checking the reliability of data, and examining more extensively trends in risk factors and stroke care. This may make it possible to optimize the cost effectiveness of management of stroke patients in Europe.
Carbamazepine and phenytoin: combination versus single drug therapy
Authors: Lorenzo N.Y. , Bromfield E.B. , Theodore W.H.
Published: January 2011

The records of 18 patients with intractable partial seizures who were observed on an in‐patient epilepsy unit during single drug treatment with carbamezepine (CBZ) or phenytoin (PHT) and during combination therapy with both drugs were evaluated retrospectively. Seizure frequency was significantly lower during combination therapy (p < 0.01) and toxicity, as measured by an eight point objective scale, did not increase significantly (p > 0.10). In addition subjective signs of clinical toiicity (e.g. nausea, ataxia, etc.) increased only slightly during combination versus monotherapy. These findings were consistently seen whether the data were evaluated in total (i.e. treatment periods at least 7 weeks) or evaluated by using standardized 35 day treatment periods. In the absence of a blinded clinical trial evaluating PHT/CBZ combination, these findings support consideration of this combination in Intractable patients who have failed rigorously administered mono‐therapy trials, recognizing however that only a small percentage of patients will improve on combination therapy.
Low dose bromocriptine‐induced pleural effusion and pleuropulmonary fibrosis
Authors: Brunt E.R.P. , Boeree M. J.
Published: January 2011

We describe a parkinsonian patient who developed a slight asymptomatic pleural effusion during prolonged therapy with low dose bromocriptine (BCR) in addition to levodopa, following prior treatment with CQA 206–291. A moderate increase of BCR dosage prompted a severe pleuropulmonary inflammatory reaction with polyclonal activation and elevated serum liver enzymes, which normalized following withdrawal of the drug. The clinical syndrome and close relation to BCR treatment are in keeping with a diagnosis of BCR‐related pleuropulmonary fibrosis (PPF). Features of this case are compared with previous reports on dopamine (DA) agonist‐related PPF. This case supports earlier suggestions of polyclonal activation in DA agonist‐related PPF and suggests hepatic involvement and dose dependency.
The neurology of autism: many unanswered questions
Authors: Rapin I. , Dunn M.
Published: January 2011

Autism is a behaviorally defined developmental disorder of the brain almost always presenting in infancy or the preschool years. Its symptoms persist life‐long, although partial compensation is possible through targeted special education that addresses children's deficits in sociability, verbal and non‐verbal communication, and atypical range of interests, activities, and cognitive skills. Although a majority of autistic individuals are mentally deficient, IQ is not a defining feature and verbal autistic persons of normal intelligence are increasingly being identified, referred to as Asperger syndrome. Meager neuropathologic data have disclosed subtle prenatal cellular limbic and cerebellar abnormalities. Autism is associated with a variety of defined genetic and acquired conditions, with multifactorial genetic traits, alone or interacting with environmental events, presumably responsible for most unexplained cases. Autistic regression is frequent and poorly understood and may be associated with clinical or subclinical epilepsy. Unravelling the neurobiologic basis of a disorder that may affect 1–2 in 1000 children will require a concerted multidisciplinary attack.
Impaired water metabolism and cerebral oedema following experimental subarachnoid haemorrhage in rats1
Authors: László A. , Varga C. , Dóczi T.
Published: January 2011

The acute phase of subarachnoid haemorrhage (SAH) is frequently associated with an impaired water metabolism and a disposition to cerebral oedema. For study of the pathogenesis of brain oedema following SAH, an experimental model of SAH was created in the rat The diuretic curves after water loading, the brain water and electrolyte contents and the plasma and cerebrospinal fluid arginine vasopressin (AVP) levels were examined in rats with SAH. Following a water load, a significant water retention and increases in brain water and sodium contents were observed after subarachnoid bleeding. Plasma and cerebrospinal fluid AVP levels were also elevated following SAH. It can be concluded that AVP plays important roles in the development of antidiuresis after water loading and in the disturbance of the brain water and electrolyte balance following experimental SAH.
Neurological involvement in antiphospholipid syndrome: clinical and instrumental evaluation in 21 consecutive cases
Authors: Anzola G.P. , Tincani A. , Magoni M. , Spatola L. , Bonetti A.
Published: January 2011

Twenty‐one consecutive women with the antiphospholipid syndrome were investigated with a full cardiological and neurological examination including a semi‐structured interview. They were also systematically studied with magnetic resonance imaging (MRI), transcranial Doppler (TCD), duplex sonography of neck vessels and transesophágeal echocardiography (TEE). Nearly 67% of patients had signs or symptoms suggestive of CNS involvement Migraine‐like headache, stroke, ocular disorders and epilepsy were the most frequent disturbances. MRI was abnormal in 65% of patients who underwent the examination and showed two distinct patterns of abnormality: either territorial infarctions or multiple subcortical spotty lesions. TCD showed in one case a stenosis affecting the main trunk of the middle cerebral artery. TEE demonstrated potentially emboligenic mitral vegetations in 72% of patients. These findings suggest that antiphospholipid syndrome is burdened with a high rate of complications affecting the CNS, which are likely to be mostly thromboembolic. It is possible that both cardiogenic embolism and in situ thrombosis of cerebral vessels occur. Given the relative absence of conventional vascular risk factors, antiphospholipid antibodies are likely to represent a true risk factor for cerebrovascular disease through the mechanism of an immunologically mediated hypercoagulable state.
A prospective study of “undiagnosed” isolated myelopathy: II. Value of magnetic resonance imaging, evoked potentials and CSF analysis
Authors: Blumhardt L.D. , Miller D.H. , Sandercock P.A.G. , Moore A.P. , Brock L. , Nixon T. , Kendall B. , Moseley I. , McDonald W.I.
Published: January 2011

We aimed to determine the sensitivity of available “diagnostic” tests in detecting subclinical abnormalities characteristic of multiple sclerosis (MS) in patients with unexplained isolated myelopathy, and any relationship between test results and level of disability. The trial investigations were carried out in 69 prospectively selected patients with acute or chronic noncompressive myelopathies. Magnetic resonance scans were the most sensitive individual tests, revealing asymptomatic brain lesions in 51 patients (74%, C.I. 64–84). An abnormal IgG/albumin ratio (IgG/A) was present in 29 (42%, C.I. 30–54), oligoclonal bands (OCB) in 27 (39%, C.I. 17–39) and abnormal evoked potentials (EP) (visual and/or auditory brain stem) in 19 cases (28%, C.I. 17–39). OCB and abnormal EP were found significantly less often than in control patients with clinically definite MS (CDMS) and significantly more often than in patients with myelopathy due to other conditions. The number of anatomical brain areas with lesions on magnetic resonance imaging (MRI) was significantly associated with CSF abnormalities; abnormal EP were correlated with abnormal MRI and elevated CSF immunoglobulins. Clinical classifications, age, symptom duration, disability levels and genetic factors did not appear to influence the prevalence of abnormal MRI or CSF. For the exclusion of compressive and structural diseases of the spinal cord, myelography has been superceded by cervical and thoracic MRI. In addition, MRI of the brain is the investigation of choice in patients with myelopathies that remain unexplained after spinal MRI. However, whether used alone or in combination with other tests, the specificity and predictive value of brain MRI abnormalities for the risk of developing MS, as well as the associated “false positive” rates, remain to be defined by long‐term follow‐up of prospectively ascertained and representative cases.
Subcortical aphasia: a specific clinical picture?
Authors: Demeurisse G. , de Beuckelaer R. , Derouck M. , Capon A. P.
Published: January 2011

100 aphasic patients were examined with a scorable aphasia battery looking at the frequency of various aphasia types and the possible specificity of clinical pictures in deep‐seated lesions. One month after onset, “atypical” aphasiological syndromes proved to be rare and to have the same frequency in patients with cortico‐subcortical or capsulostriatal deep‐seated lesions. In the latter condition, no specific clinical syndrome was brought out, although verbal comprehension disorders were usually less severe than in cortico‐subcortical lesions.
Methylmalonic acid in serum from patients with neurological symptoms consistent with cobalamin deficiency
Authors: Johannsen P. , Østergaard K. , Christensen J. Erik Just , Worm M. , Rasmussen K.
Published: January 2011

Patients may have neurological symptoms due to cobalamin deficiency in spite of a normal plasma cobalamin level. The aim of the present study was to test if serum‐methylmalonic acid (S‐MMA) can identify these cobalamin deficient patients. Patients with neurological symptoms consistent with cobalamin deficiency and normal plasma cobalamin had an estimation of S‐MMA. If S‐MMA was elevated, treatment with by droxycobalamin was offered. The patients were followed for one year in a prospective study with blood tests, clinical assessment and neurophysiological tests. Serum‐MMA was above 0.37 μmol/l (+2 SD of normal mean) in 15 out of 134 patients (11.2%) tested. Twelve of these patients were treated with hydroxycobalamin injections. A significant decrease in S‐MMA below 0.37 μmol/l was found in 11 patients treated, indicating a functional cobalamin deficiency. Five patients were followed for one year. Documented improvement of neurological symptoms and nerve conduction velocity was shown in three of these patients. Of the remaining two patients treated one did not change and one deteriorated. S‐MMA can identify patients with neurological symptoms due to cobalamin deficiency in spite of a normal plasma cobalamin level.
Weber's syndrome due to a possible solitary brainstem toxoplasmosis as a presenting sign of AIDS
Authors: Orefice G. , Volpe G. , Liuzzi G. , D'Abbraccio M. , Coppola M. , Cataldo P. Tullio
Published: January 2011

We report the case of a young man, with a previous history of parenteral drug abuse, who developed a Weber's syndrome. Brain computed tomographic scan and nuclear magnetic resonance imaging showed a single ring enhancing lesion in the right mesencephalic site. After the demonstration of seropositivity for human immunodeficiency vims, a presumptive diagnosis of cerebral toxoplasmosis in an AIDS patient was made and a specific treatment was started. A partial neuroradiological and clinical improvement were obtained. Opportunistic cerebral lesions, as first manifestation of AIDS, should be always considered in subjects at risk for AIDS who present a brainstem syndrome.
Abnormalities of carnitine metabolism in chronic fatigue syndrome
Authors: Majeed T. , de Simone C. , Famularo G. , Marcellini S. , Behan P.O.
Published: January 2011

Carnitine may be involved in the pathogenesis of the chronic fatigue syndrome (CFS). However, no information about the cellular metabolism of carnitine in CFS patients is currently available. Therefore, we aimed to measure the levels of carnitine (total, free and short‐chain) in both peripheral blood lymphocytes (PBLs) and sera from patients with CFS. The serum levels of total, free and short‐chain were comparable in CFS patients, considered as the whole group, to those in healthy control subjects, even though a trend indicating slightly reduced serum concentrations of free carnitine was observed in male patients with CFS. In contrast, the concentrations of total, free and short‐chain carnitine in PBLs from patients with CFS were significantly lower than in cells from healthy controls. Our study indicates that patients with CFS require exogenous carnitine supplementation. The low carnitine concentrations in PBLs from patients with CFS probably reflect the carnitine deficiency occurring in other tissues, including the skeletal muscles. The low cellular concentrations of carnitines may help to explain both the immunological abnormalities and the impaired energy metabolism in skeletal muscles.
The pathophysiology of parkinsonism in multiple system atrophy
Authors: Tison F. , Wenning G.K. , Daniel S.E. , Quinn N.P.
Published: January 2011

Parkinsonism is the principal motor disorder of multiple system atrophy (MSA). In order to explore the pathophysiology of parkinsonism in MSA, we analysed 182 pathological reports from the literature and 35 from the United Kingdom Parkinson's Disease Society Brain Bank, and have also examined published post‐mortem and in vivo neurochemical data. This review indicates that the brains of MSA patients who had parkinsonism in life show degeneration principally in anatomically related regions of the nigrostriatal dopaminergic pathway and of the striatum as well as in the globus pallidus. This degeneration affects both “direct” and “indirect” striatal outflow pathways. The degree of dopaminergic response in any one subject at any one point in the evolution of the disease probably relates both to the severity and extent of striatal pathology and also to the balance between degeneration in “indirect” and “direct” striatal outflow pathways and in the pallidum, especially its external part Abnormal oligodendroglial cytoplasmic inclusions are also found in large numbers in most of the basal ganglia structures that show cell loss and gliosis. The precise consequences of their additional presence in motor cortex remain unknown, but disturbance of function in supplementary motor area could also contribute to the extrapyramidal syndrome of MSA.
Epidermal growth factor receptor and p53 protein expression in human glioblastomas
Authors: Kordek R. , Biernat W. , Alwasiak J. , Yanagihara R. , Liberski P.P.
Published: January 2011

p53 mutations and amplification of epidermal growth factor receptor (EGFR) gene are the most frequently detected genetic alterations in glioblastomas; thus, these changes seem to delineate two subgroups of glioblastomas: those originated de novo and those originated from preexistent low grade astrocytomas. Paraffin‐embedded surgical specimens of 30 human glioblastomas were analyzed immunohistochemically for the presence of p53 protein and EGFR. Approximately half of the cases were p53 protein‐positive while one‐third were EGFR positive. Only three cases were positive for both p53 protein and EGFR. There was no difference between the average ages of patients with only‐p53‐positive, and double‐negative tumors, while three glioblastomas with both p53 protein and EGFR immunopositivity occured in older patients (mean age 67.0 years, p < 0.02). Patients with only‐EGFR‐positive tumors were younger, but not significantly (44.3 years, p < 0.1). This study supports the notion that there are two main subpopulations of glioblastoma—with EGFR and with p53 protein overexpression.
Intrafamilial heterogeneous clinical presentation of the mitochondrial 3243 MELAS mutation; molecular investigations among four generations
Authors: Degoul F. , Diry M. , Viader F. , Boitier E. , Marsac C. , Eymard B. , Romero N. , Delisle M. B. , Lechevalier B. , Chapon F.
Published: January 2011

We have identified the A3243G heteroplasmic point mutation in mitochondrial DNA from a female patient with headache as the main clinical feature. The mitochondrial origin of her disease was only suspected because of her brother with MELAS syndrome. Morphological and biochemical studies failed to reveal mitochondrial respiratory chain dysfunction in her muscle which contained 65% of mutated mitochondrial DNA molecules. Molecular studies performed among four generations (in the blood of seven subjects) showed the variable transmission of mutated molecules and pointed out the difficulty in giving genetic counsel.
Mixed germ cell tumour of the central nervous system with multiple metastases—a 5‐year history. A case report
Authors: Áfra J. , Jelencsik I. , Gyergyai F. , Slowik F. , Kenéz J.
Published: January 2011

After a 4‐month history of intermittent headache an intrasellar benign teratoma was found and totally removed in a 19‐year‐old male patient who became symptom‐free. Three years later a third ventricle germinoma developed which was also totally removed, followed by local radiotherapy. Eight months later a spinal tumour evolved, chemotherapy and radiotherapy resulted in full recovery. Eighteen months later a frontal meningeal tumour appeared and was treated by chemotherapy. Three months later—with chemotherapy—tumorous meningitis developed and the patient died 5 years after the first operation. The results of tumour‐markers monitoring, histology, electronmicroscopy and immunohistochemistry referred to a germinoma with multiple metastases and meningeal seeding after total removal of an intrasellar mature teratoma.
Fluency deficits in patients with Alzheimer's disease and frontal lobe lesions
Authors: Phillips L.H. , Sala S. Delia , Trivelli C.
Published: January 2011

Fluency tests are widely used in clinical settings to assess cognitive function. Fluency deficits In patients with Alzheimer's disease (AD) are generally attributed to deteriorated language storage. In contrast, patients with lesions to the frontal lobes (FL) of the brain are thought to have poor fluency due to executive deficits of retrieval. This study examined the relationships between fluency performance and cognitive measures of language and executive function in both AD and FL patients. In both groups, fluency performance related to measures of language comprehension and executive control of attention. However, in AD patients, fluency deficits were most closely associated with language and verbal memory deterioration, while in FL patients fluency deficits were more strongly associated with executive measures of strategic planning and attention. Qualitatively different patterns of functional deficits may influence fluency performance in different neuropsychological groups. Caution is therefore urged in the interpretation of poor fluency scores as indicative of either language or executive dysfunction, without additional information about the reasons for poor performance.
Lyme neuroborreliosis: cerebrospinal fluid contains myelin protein‐reactive cells secreting interferon‐γ
Authors: Wang W.‐Z. , Fredrikson S. , Xiao B.‐G. , Sun J.‐B. , Kostulas V. , Link H.
Published: January 2011

The mechanisms causing neurological manifestations and influencing the outcome in patients infected with the spirochete Borrelia burgdorferi are unknown. To study the involvement of autoimmune mechanisms in patients with Lyme neuroborreliosis (LN), ELIspot assays were used to determine the numbers of T lymphocytes which, upon stimulation with the myelin components myelin basic protein (MBP) and proteolipid protein (PLP) and two MBP peptides, responded by the secretion of interferon‐γ (IFN‐γ). LN patients had compared to patients with other neurological diseases and tension headache in CSF elevated numbers of MBP‐ and PLP‐reactive T helper type 1 (Th1) like IFN‐γ secreting cells at mean frequencies of about 1 per 1300 and 1 per 1500 CSF cells, respectively. Numbers were elevated up to 5 months after successful treatment of LN, and they did not correlate with clinical findings. Autoreactive T cells were not elevated in the LN patients' blood. These findings match the previously reported elevation of anti‐MBP IgG antibody secreting cells exclusively in CSF in LN. The IFN‐γ release by autoreactive cells and the secretion of autoantibodies in the CSF may have relevance for development and outcome of LN.
Neurosarcoidosis, stroke and antiphospholipid antibodies: a case report
Authors: Zadra M. , Brambilla A. , Erli L.C. , Grandi R. , Finazzi G.
Published: January 2011

Stroke is uncommon in neurosarcoidosis, despite histological evidence of vessel involvement by granulomatous process. We describe recurrent cerebral ischemias in a young man affected by sarcoidosis. Haematological investigations revealed the presence of anticardiolipin antibodies and lupus anticoagulant. Stroke in neurosarcoidosis is probably due to several different mechanisms, one of which could be the presence of a hypercoagulability state.
Ceftriaxone in amyotrophic lateral sclerosis
Authors:   The Italian ALS Study Group
Published: January 2011

One hundred and eight patients with amyotrophic lateral sclerosis (ALS) received ceftriaxone 2 g daily i.v. (62) or i.m. (34) or by both routes (12), for 21‐day cycles on an open basis. Baseline MRC and Norris scores were similar to those at the end of the first 21‐day cycle of therapy. Seven patients showed remarkable clinical improvement, mostly segmental, which started during the first week of treatment and lasted up to 2 months after its completion. Improvement was also noted in seven out of 21 cases given a subsequent cycle of treatment. Based on these findings, the drug is supposed to act by altering the neurochemical transmission at the neuromuscular junction and/or by facilitating the presynaptic uptake of glutamate at the synaptic junction. This hypothesis positively correlates with the results of in vitro experiments showing that ceftriaxone increases 3H‐glutamate uptake in rat spinal cord synaptosomes.
Vascular dementia: persisting controversies and questions
Authors: Moncayo J. , Bogousslavsky J.
Published: January 2011

The concept of vascular dementia remains controversial. Indeed, in many instances what is called vascular dementia may neither be vascular nor dementia. However, while overdiagnosis may be frequently made, underrecognition of vascular factors in dementia also seems common. Recent works show that focal/multifocal cognitive and executive dysfunction which may follow sequential or more rarely single‐strategic stroke should be differentiated from more diffuse progressive intellectual decline mimicking a degenerative disorder (such as Alzheimer's disease) which may smoothly develop in over one fourth of stroke patients.
A diagnostic approach to ischemic stroke in young and middle‐aged adults
Authors: Fieschi C. , Rasura M. , Anzini A. , De Castro S. , Di Gianfilippo G. , Valesini G. , Violi F. , Zanette E.M.
Published: January 2011

We prospectively studied 160 patients (18–47 years of age) with TIA (18) or ischemic stroke (142). Eighty‐five subjects were under the age of 40. All patients underwent noninvasive ultrasound studies (transcranial doppler and echocardiography), plus a battery of laboratory studies including coagulation and antibodies tests and blood lactate‐pyruvate. Angiographic studies were performed in 42% of patients (33% with DSA and 9% with MRA). The most common etiologies were found to be cardioembolic (more common in the 18–39 age group) and atherothromboticic (more common in the 40–47 age group). Autoimmune conditions affected 12.5% of patients, while arterial dissections affected 11%. In 10% of patients the etiology of the cerebral ischemic event could not be determined, in spite of an extensive and expensive workout Hence, a set of guidelines aimed at optimizing, in terms of cost‐benefit, a protocol of investigations in young adults with ischemic stroke is tentatively proposed.
Myasthenia gravis and chronic inflammatory demyelinating polyneuropathy
Authors: Sempere A.P. , Claveria L.E. , Cruz‐Martinez A. , Duarte J.
Published: January 2011

We describe a patient with the previously unreported association of chronic inflammatory demyelinating polyneuropathy (CIDP) and myasthenia gravis (MG). Immunosuppressive treatment with azathioprine and prednisone achieved clinical and electrophysiological remission of MG and improvement of CIDP. As ophthalmoplegia occurs infrequently in CIDP, the possibility of MG should be considered when this sign is present in a patient with CIDP. Since current therapy with corticosteroids, plasma exchange and other immunomodulating agents is effective against both diseases, their association may be undereported.
Plasmapheresis in Guillain‐Barré syndrome
Authors: Korczyn A.D. , Nisipeanu P.
Published: January 2011

The use of plasmapheresis in Guillain‐Barré syndrome is reviewed. The clinical data, based on open‐label studies, suggest that PP – if instituted early – prevents further deterioration and accelerates the recovery, e.g. the time until the patient becomes ambulatory. The data do not suggest any effect of PP to patients with axonal damage to those with preceding gastroenteritis or Campylobacter jejuni infections has not been tested. The use of PP should be weighed against the potential complications, especially in units inexperienced with this procedure.
Comparison of cerebral atrophy evaluated by MRI in Parkinson's disease and multiple system atrophy
Authors: Durif F. , Albuisson E. , Debilly B. , Le Bas J.F. , Pollak P.
Published: January 2011

Cerebral atrophy was evaluated in 31 patients with Parkinson's disease (PD) and 24 patients with clinically probable multiple system atrophy (MSA). Measurements of ventricular and brainstem areas, obtained from axial and sagittal MRI sections, were performed using a computer digitalizing system. Cortical and cerebellar atrophies were subjectively assessed. All measures were scored by one observer who was blind to the diagnosis (BD). Age, sex, levodopa dosage, baseline motor Parkinsonian disability (i.e. without levodopa treatment) and neuropsychological impairment were not significantly different between the two groups of patients. The total brainstem area (p < 0.001), the mesencephalon (p < 0.05) and the pons (p = 0.05) areas were significantly smaller in MSA as were the lobar (p < 0.05) and the vermian (p < 0.01) parts of the cerebellum. In the MSA group, significant correlations were observed between the brainstem area and items from the motor part of the Unified Parkinson's Disease Rating Scale (dysarthria, posture, arising from a chair), and between the cerebellar atrophy and the gait disturbance. A progressive discriminant analysis using radiologic variables correctly classified 79% of patients in their own diagnosis group with a significant difference (p = 0.01). From these results, it appears that MRI could help to differentiate MSA from PD, especially when MRI detects a severe infratentorial atrophy suggestive of MSA. However, brainstem and cerebellum atrophies are not sufficient criteria to differentiate MSA from PD because they lack both specificity and sensitivity.
Peduncular hallucinosis: three cases with thalamic involvement
Authors: Yalçin A. Destina , Yapici Z. , Forta H.
Published: January 2011

Three cases with peduncular hallucinosis are described. The occurrence of complex visual hallucinations in each case were abrupt at the onset of the stroke. Two patients were free of hallucinations after a few days, while in the other they persisted. Magnetic resonance imaging (MRI) or computerized tomographic (CT) brain scan findings revealed thalamic involvement in these three patients.
Chronic inflammatory demyelinating polyneuropathy (CIDP) with ophthalmoplegia: a case with asymmetric limb weakness and high titers of anti‐GM1 antibody
Authors: Ozaki I. , Baba M. , Kurihara A. , Saitoh T.
Published: January 2011

We describe a patient with high titers of anti‐GM1 antibody but not anti‐GQ1b antibody who had recurrent asymmetric limb weakness with little sensory deficit and developed left abducens palsy. Nerve conduction study showed multifocal conduction block or dispersion of motor and sensory axons in the same mixed nerve. Pulse steroid followed by oral predonisolone treatment rapidly resolved his symptoms.
Sexual dysfunction in male multiple sclerosis patients in relation to clinical findings
Authors: Ghezzi A. , Zaffaroni M. , Baldini S. , Zibetti A.
Published: January 2011

Seventy‐seven consecutive male patients with clinically definite or laboratory supported definite multiple sclerosis were submitted to a questionnaire and a clinical interview in order to assess the frequency, the nature of sexual dysfunction and its relationship with clinical findings. Twenty‐six patients (35%) complained of erectile dysfunction: the defect was partial and inconstant in 11 patients (14%), complete and fixed in 15 (21%). Six patients complained of premature ejaculation, in three of whom the symptom was already present before the onset of the disease. Taking the series as a whole, sexual life was poor or not satisfying at all in 34 patients. Erectile dysfunction was associated with the presence and severity of bladder dysfunction and with disability. The relationship with sensory impairment of legs was slight, no relationship was found with motor involvement of legs, type of course, age and disease duration.
APOE‐ε4 in age‐related memory complaints and Alzheimer's disease
Authors: Treves T. A. , Chapman J. , Bornstein N. M. , Verchovsky R. , Asherov A. , Veshchev I.O. , Klimovitzki S. , Korczyn A.D.
Published: January 2011

APOE‐ε4 has been consistently found to be frequent in patients with senile dementia of the Alzheimer type (SDAT). Since forgetfulness may represent an early stage of dementia, APOE‐ε4 frequency can be expected to be high in such subjects, particularly in those who later develop dementia. We examined here the proportion of ε4 alleles in patients with SDAT (n = 179), controls (n = 154) and subjects with age‐related memory complaints (ARMC, n = 167); 16 of them developed dementia of Alzheimer type and three of them dementia of vascular type. We also evaluated the relative risk of dementia (of Alzheimer type) in ARMC subjects who are ε4‐carriers, using a Cox proportional hazards model. The APOE‐ε4 allele frequency was 27% in SDAT, 25% in ARMC patients who became demented, 15% in ARMC subjects who remained such after at least 1 year of follow‐up, and 10% in controls. APOE‐ε4 allele was significantly more frequent in SDAT than in controls or than in stable ARMC (OR = 3.7 [2–6.3] and OR = 2.5 (1.5–4], respectively, p > 0.01). The risk of dementia in ARMC subjects carrying an ε4 allele was three‐fold that of those without (expβ = 3.1 [0.98–10], p = 0.05). Older age at onset of memory decline and lower minimental scores at initial visit were also associated with development of dementia in ARMC subjects (exp β = 1.1 [1.0–1.2], p = 0.05 and exp = 0.76 [0.6–0.9]), p = 0.008). In conclusion, the APOE‐ε4 allele was found to occur frequently in ARMC who subsequently develop dementia, therefore it can indicate a predisposition for dementia in such patients.
White matter high‐signal areas on MRI associated with chronic hypoxia
Authors: Castillo J. , Rodríguez J. R. , Corredera E. , Alvarex J. M. , Purmar J. M. , Noya M.
Published: January 2011

Neuroradiological alterations in patients with chronic hypoxia is an area yet to be explored. The purpose of our study was to evaluate the possibility of changes in cerebral magnetic resonance imaging (MRI) secondary to chronic hypoxia. Using healthy persons as control subjects, we studied the MRI findings associated with chronic pulmonary disease. The presence of high‐signal areas in cerebral white matter was investigated in patients with hypoxia due to chronic stable pulmonary disease (41 obstructive, three restrictive and six with a mixed pattern) and in 50 control subjects. We recorded the gasometric (Pao2 Paco2; pH and CO3H) and spirometric parameters (forced vital capacity [FVC], forced expiratory volume in 1/s [FEV1], and analytical data for erythrocytes, platelets, hemoglobin and hematocrit in the patients and the vascular risk factors in patients and controls. The findings of the MRI were classified into five groups according to the number and extension of the lesions (0, no lesions; I, isolated spotted lesions; II, more than 10 spotted lesions; III, partially confluent lesions; and IV, bilateral confluent lesions). Vascular risk factors other than respiratory disease were detected in 42% of the patients and 48% of the controls (p > 0.05). Patients with chronic hypoxia showed a higher number and extension of high‐signal areas in cerebral white matter (94% in patients as compared to 38% in the control group, p > 0.001). The presence of this lesions on MRI was related only to age (OR 1.2; 95% confidence interval, 1.17–1.41; p = 0.008) and intensity of hypoxia (OR‐0.08; 95 % confidence interval, 0.026 0.086; p = 0.031), but was independent of the duration of illness, hypercapnia and hematocrit. In conclusion, the association between chronic pulmonary disease and the occurrence of high‐signal areas on the MRI in white matter was demonstrated in our patients.
The cerebrospinal fluid in multiple sclerosis. Quantitative assessment of intrathecal immunoglobulin synthesis by empirical formulae
Authors: Sellebjerg F. , Christiansen M. , Rasmussen L. S. , Jaliachvili I. , Nielsen P. M. , Frederiksen J. L.
Published: January 2011

Intrathecal synthesis of IgG occurs in more than 90% of patients with clinically definite multiple sclerosis. The prevalence and significance of intrathecal synthesis of IgA and IgM are, however, less thoroughly characterized. We estimated intrathecal synthesis of IgG, IgA and IgM with various empirical formulae. The concentrations of albumin, IgG, IgA and IgM and the presence of IgG oligoclonal bands were determined in CSF and serum from 350 patients, including 97 with clinically definite multiple sclerosis. Intrathecal synthesis of IgG oligoclonal bands was detected in 95% of patients with multiple sclerosis (95% confidence interval 88–98%). The IgG‐index, an extended IgG‐index, and a hyperbolic IgG formula performed approximately equally in identifying patients with MS, but they were all inferior to the detection of IgG oligoclonal bands. In quantitative measurements, the extended immunoglobulin indices appeared to perform well; studies comparing the extended IgA‐ and IgM‐indices to qualitative analyses (electrophoresis or isoelectric focusing) are, however, needed to confirm this. Detection of intrathecal synthesis of IgA of IgM was of little value in the diagnosis of multiple sclerosis.