Glucose metabolism in the brain in LMNB1-related autosomal dominant leukodystrophy.

Abstract:

OBJECTIVE:LMNB1-related autosomal dominant leukodystrophy is caused by an overexpression of the protein lamin B1, usually due to a duplication of the LMNB1 gene. Symptoms start in 5th to 6th decade. This slowly progressive disease terminates with death. We studied brain glucose metabolism in this disease using 18 F-fluorodeoxyglucose positron emission tomography (PET). METHODS:We examined 8 patients, aged 48-64 years, in varying stages of clinical symptomatology. Two patients were investigated with quantitative PET on clinical indications after which six more patients were recruited. Absolute glucose metabolism was analyzed with the PVElab software in 6 patients and 18 healthy controls. A semiquantitative analysis using the CortexID software was performed in seven investigations, relating local metabolism levels to global glucose metabolism. RESULTS:The clinical quantitative PET revealed low global glucose metabolism, with the most marked reduction in the cerebellum. In the PVElab analysis, patients presented low mean glucose metabolism in the cerebellum, brainstem and global grey matter. In the semiquantitative analysis, 2 patients showed a decreased metabolism in the cerebellum and 4 patients a relatively higher metabolism in parts of the temporal lobes. Since none of the patients showed an increased metabolism in the quantitative analysis, we interpret these increases as "pseudo-increases" related to a globally reduced metabolism. CONCLUSIONS:Global reduction of grey matter glucose metabolism in this white matter disease most likely depends on a combination of cortical afferent dysfunction and, in later stages, neuronal loss. The lowest metabolism in the cerebellum is consistent with histopathological findings and prominent cerebellar symptoms.

journal_name

Acta Neurol Scand

authors

Finnsson J,Lubberink M,Savitcheva I,Fällmar D,Melberg A,Kumlien E,Raininko R

doi

10.1111/ane.13024

subject

Has Abstract

pub_date

2019-02-01 00:00:00

pages

135-142

issue

2

eissn

0001-6314

issn

1600-0404

journal_volume

139

pub_type

杂志文章
  • Cerebrospinal fluid cytotoxicity does not affect survival in amyotrophic lateral sclerosis.

    abstract:OBJECTIVES:Cerebrospinal fluid (CSF) from some patients with amyotrophic lateral sclerosis (ALS) has been demonstrated to significantly reduce the neuronal viability of primary cell cultures of motor neurons. We aimed to study the potential clinical consequences associated with the cytotoxicity of CSF in a cohort of pa...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/ane.12717

    authors: Galán L,Matías-Guiu J,Matias-Guiu JA,Yáñez M,Pytel V,Guerrero-Sola A,Vela-Souto A,Arranz-Tagarro JA,Gómez-Pinedo U,García AG

    更新日期:2017-09-01 00:00:00

  • Delusions in Alzheimer's disease and multi-infarct dementia.

    abstract::Neuropsychiatric symptoms such as delusions and misidentifications have been reported in dementia ranging from 10% to 73% in Alzheimer's disease (AD) patients and up to 40% in multi-infarct dementia (MID) patients. The aim of this study was to investigate in 61 AD and 31 MID patients both the frequency and the content...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1993.tb04177.x

    authors: Binetti G,Bianchetti A,Padovani A,Lenzi G,De Leo D,Trabucchi M

    更新日期:1993-07-01 00:00:00

  • Diseases associated with multiple sclerosis and epilepsy. A population cohort study of 159,200 middle-aged, urban, native Swedes observed over 10 years (1970-79).

    abstract::A defined general population of 159,200 male and female native Swedes born in the period 1911-1940 from an urban catchment area of the then only general hospital, was followed over a decade (1970-79) with regard to in-patient hospitalization for all kinds of diagnoses. As a part of this population cohort study, multip...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:

    authors: Lindegård B

    更新日期:1985-04-01 00:00:00

  • Mode of action of dantrolene sodium in spasticity.

    abstract::The mode of action of dantrolene sodium was studied in 11 multiple sclerosis patients with spastic paresis of the legs by measurements of changes in electromyographic and mechanomyographic proprioceptive reflex responses and in voluntary power. Dantrolene sodium 0.5 mg per kg body weight given intravenously clearly re...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1979.tb02941.x

    authors: Mai J,Pedersen E

    更新日期:1979-06-01 00:00:00

  • Astheno-emotional disorder after aneurysmal SAH: reliability, symptomatology and relation to outcome.

    abstract:OBJECTIVES:Neuropsychiatric symptoms commonly found after aneurysmal SAH are covered in the astheno-emotional disorder (AED) of Lindqvist & Malmgrens diagnostic system for organic psychiatry. This study aims to describe the reliability and symptomatology of AED and its relationship with social outcome. MATERIAL AND ME...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1034/j.1600-0404.2001.103006379.x

    authors: Rödholm M,Starmark JE,Svensson E,Von Essen C

    更新日期:2001-06-01 00:00:00

  • Concentrations of sulfur-containing free amino acids in lumbar cerebrospinal fluid from patients with consciousness disturbances.

    abstract::The concentrations of sulfur-containing amino acids (taurine, methionine, cystine, cystathionine, homocystine) in lumbar CSF from patients with consciousness disturbances and controls were measured, and it was investigated whether there was any correlation between the sequential changes of these amino acid concentrati...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1983.tb04848.x

    authors: Yanai Y,Shibasaki T,Kohno N,Mitsui T,Nakajima H

    更新日期:1983-12-01 00:00:00

  • Buccally absorbed vs intravenous prochlorperazine for treatment of migraines headaches.

    abstract:OBJECTIVE:To compare the efficacy of buccally absorbed prochlorperazine (BAP) to intravenous prochlorperazine (IVP) for the abortive treatment of migraine headaches. METHODS:Randomized double-blind trial. Eighty subjects aged 18-65 presenting with migraines to the ED of a safety-net, urban hospital. Subjects were rand...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章,随机对照试验

    doi:10.1111/ane.13104

    authors: Fernando T,Lumanauw DD,Youn S,Shimada M,Yadav K,Chappell B,Horeczko T,Tanen DA

    更新日期:2019-07-01 00:00:00

  • Epilepsy in an Italian community as assessed by a survey for prescriptions of antiepileptic drugs: epidemiology and patterns of care.

    abstract::The prescriptions of antiepileptic drugs in the years 1985 and 1986 were used to identify all possible cases of epilepsy in a population of about 50,000 people. The general practitioners of the study area filled out a questionnaire for each user of antiepileptic drugs registered as a patient of theirs. This informatio...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1992.tb03991.x

    authors: Giuliani G,Terziani S,Senigaglia AR,Luccioni G,Foschi N,Maffei C

    更新日期:1992-01-01 00:00:00

  • Superior cerebellar artery territory stroke.

    abstract:OBJECTIVES:The purpose of this study was to clarify etiologic factors, prior symptoms and clinical features of isole superior cerebellar artery (SCA) territory infarcts. METHODS:All data were collected from consecutive 21 patients with isole SCA infarcts involved on computerized tomography. RESULTS:The risk factors i...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1998.tb07310.x

    authors: Erdemoglu AK,Duman T

    更新日期:1998-10-01 00:00:00

  • Large striatocapsular infarcts: clinical presentation and pathogenesis in comparison with lacunar and cortical infarcts.

    abstract::In a well-defined prospective series of 247 patients with a first-ever supratentorial brain infarct, 15 patients (6%; 95% confidence interval 3-9%) had a large striatocapsular infarct. Twelve (80%) had signs of cortical dysfunction, whereas risk factor profile, frequency of significant carotid stenosis, and frequency ...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1992.tb05089.x

    authors: Boiten J,Lodder J

    更新日期:1992-09-01 00:00:00

  • Pre-traumatic conditions can influence cortisol levels before and after a brain injury.

    abstract:OBJECTIVE:Satisfactory anabolic reactions, including the activation of the hypothalamic-pituitary-adrenal (HPA) axis, are essential following severe traumatic brain injury (TBI) and aneurysmal subarachnoid hemorrhage (SAH). Many factors may influence this activation. This study aimed to investigate whether individuals ...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/ane.13212

    authors: Sörbo A,Eiving I,Theodorsson E,Rydenhag B,Jonsdottir IH

    更新日期:2020-04-01 00:00:00

  • The neurotoxicity of ethanol.

    abstract::Alterations in nervous system functioning following acute and chronic ethanol exposure have been studied in a great number of experimental investigations. Results from many of these investigations can be difficult to interpret, particularly since a variety of techniques and exposure models are employed. This review em...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章,评审

    doi:10.1111/j.1600-0404.1983.tb04556.x

    authors: Melgaard B

    更新日期:1983-03-01 00:00:00

  • CSF lymphocyte subsets in aseptic meningitis: dual-labelling analysis with flow cytometry.

    abstract::In order to characterize the CSF (cerebrospinal fluid) lymphocytes in CNS (central nervous system) inflammation, we examined paired samples of CSF and PB (peripheral blood) of 19 patients with acute aseptic meningitis, performing the dual labelling method on flow cytometry. Significantly higher percentages of CD3+ (T ...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1990.tb00997.x

    authors: Uchihara T,Ikeda M,Takahashi H,Yoshida S,Tsukagoshi H

    更新日期:1990-05-01 00:00:00

  • Estimate of parkinsonism prevalence through drug prescription histories in the Province of Rome, Italy.

    abstract:INTRODUCTION:The objective of the study was to estimate the prevalence of parkinsonism in the Province of Rome using antiparkinsonian prescription histories from 1986 to 1991. METHODS:A subject was defined as a case of parkinsonism if he/she had received "specific" and "consistent" antiparkinsonian therapy in the stud...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1995.tb00465.x

    authors: Menniti-Ippolito F,Spila-Alegiani S,Vanacore N,Bonifati V,Diana G,Meco G,Raschetti R

    更新日期:1995-07-01 00:00:00

  • Treatment of steroid-unresponsive optic neuritis with plasma exchange.

    abstract:OBJECTIVES:Until now, the significance of plasma exchange (PE) as a treatment for steroid-unresponsive optic neuritis (ON) is still unclear because placebo-controlled and larger studies are missing. We report our experience with 23 patients treated by PE due to steroid-unresponsive ON. MATERIALS AND METHODS:Patients w...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.2011.01612.x

    authors: Roesner S,Appel R,Gbadamosi J,Martin R,Heesen C

    更新日期:2012-08-01 00:00:00

  • N20 and P40 somatosensory evoked potentials: thalamic lesions and subcortical origin.

    abstract::Three patients with well-defined clinical and radiographic lesions have been studied with somatosensory evoked potentials (SSEPs). The data indicate that the primary scalp potentials from stimulation of both the arm and leg (N20 and P40) can be lost with thalamic lesions and would be consistent with these potentials b...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1985.tb03162.x

    authors: Fisher MA,Perlik SJ

    更新日期:1985-01-01 00:00:00

  • Effects of prolonged supratentorial mass expansion on regional blood flow and cardiovascular parameters during the Cushing response.

    abstract::To clarify the central nervous and the cardiovascular mechanisms mediating the Cushing response (CR) during intracranial hypertension, the effects of continuous and prolonged expansion of an epidural supratentorial balloon was studied in six dogs. Regional cerebral blood flow, cardiac output, extracerebral tissue bloo...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1985.tb00872.x

    authors: Schrader H,Hall C,Zwetnow NN

    更新日期:1985-09-01 00:00:00

  • European neuroborreliosis: quality of life 30 months after treatment.

    abstract:OBJECTIVES:The prognosis after Lyme neuroborreliosis (LNB) is debated. The aim of this study was to assess health-related Quality of Life (QoL) and neurological symptoms 30 months after treatment in European patients with LNB. MATERIALS AND METHODS:In a prospective case-control designed study, we investigated 50 well-...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.2010.01482.x

    authors: Eikeland R,Mygland A,Herlofson K,Ljøstad U

    更新日期:2011-11-01 00:00:00

  • The Gulf War era multiple sclerosis cohort: 3. Early clinical features.

    abstract:OBJECTIVES:To present clinical features at diagnosis for a large nationwide incident cohort of multiple sclerosis (MS) among those serving in the US military during the Gulf War era (GWE). MATERIALS & METHODS:Medical records and databases from the Department of Veterans Affairs (VA) for cases of MS with onset in or af...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/ane.12810

    authors: Wallin MT,Culpepper WJ,Maloni H,Kurtzke JF

    更新日期:2018-01-01 00:00:00

  • Cerebral hemodynamics in orthostatic intolerance with normal head-up tilt test.

    abstract:OBJECTIVES:Orthostatic hypotension (OH) and postural orthostatic tachycardia syndrome (POTS) are well-known causes of orthostatic intolerance (OI). In addition, there are OI patients who are characterized by the symptoms of OI and lack of abnormal findings in head-up tilt (HUT) test. The aim of this study was to determ...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/ane.12516

    authors: Shin KJ,Kim SE,Park KM,Park J,Ha SY,Kim SE,Kwon OY

    更新日期:2016-08-01 00:00:00

  • Screening for thrombophilic risk factors among 25 German patients with cerebral venous thrombosis.

    abstract:OBJECTIVES:In this study the frequency of inherited thrombophilic risk factors in a population of German CVT patients and their influence on clinical outcome were evaluated. MATERIAL AND METHODS:Twenty-five patients (age 37.1 +/- 16.3 years) with CVT were screened for inherited coagulation disorders. All participants ...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1034/j.1600-0404.2000.102001031.x

    authors: Stolz E,Kemkes-Matthes B,Pötzsch B,Hahn M,Kraus J,Wirbartz A,Kaps M

    更新日期:2000-07-01 00:00:00

  • Sensitivity of transcranial Doppler for confirming brain death: a prospective study of 270 cases.

    abstract:OBJECTIVE:The reported sensitivity of transcranial Doppler ultrasonography (TCD) for confirming brain death (BD) ranges from 91% to 100%. We assessed the frequency and causes of false-negative results in TCD examination in a series of patients with BD and in the literature. METHODS:We carried out a prospective TCD exa...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.2006.00645.x

    authors: de Freitas GR,André C

    更新日期:2006-06-01 00:00:00

  • Assessing quality and normalization of microarrays: case studies using neurological genomic data.

    abstract:BACKGROUND:Genomic analysis using microarray tools has the potential benefit of enhancing our understanding of neurological diseases. The analysis of these data is complex due to the large amount of data generated. Many tools have been developed to assist with this, but standard methods of analysis of these tools have ...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.2007.00979.x

    authors: Hershey AD,Burdine D,Liu C,Nick TG,Gilbert DL,Glauser TA

    更新日期:2008-07-01 00:00:00

  • Quality of life in newly-debuted epilepsy. An empirical study.

    abstract:OBJECTIVES:This study aimed to illuminate adult's experienced quality of life in newly-debuted epilepsy and to test the American instrument Quality of Life Index (QLI) for the first time on an epilepsy population. A second aim was to find appropriate questions to measure patient perceptions in epilepsy. MATERIAL AND M...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1999.tb00384.x

    authors: Räty L,Hamrin E,Söderfeldt B

    更新日期:1999-10-01 00:00:00

  • Studies on the clustering of multiple sclerosis in Finland I: Comparison between the domiciles and places of birth in selected subpopulations.

    abstract::Further studies pm yjr rofr,op;phu pg ,i;yo;r dv;rtpdod (MS) IN Finland were carried out in three different types of geographical unit: in counties, combined clerical districts, and single clerical district. For longitudinal studies, the prevalence of MS by present domicile was compared to that by place of birth, and ...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1975.tb01362.x

    authors: Wikström J,Palo J

    更新日期:1975-02-01 00:00:00

  • Nasal spray desmopressin treatment of bladder dysfunction in patients with multiple sclerosis.

    abstract::Bladder dysfunction with increased voiding frequency and incontinence is a common problem in patients with multiple sclerosis (MS). In the present study, the effect of the synthetic vasopressin analogue, desmopressin, was evaluated on the voiding frequency in 26 patients with MS suffering from socially handicapping vo...

    journal_title:Acta neurologica Scandinavica

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1111/j.1600-0404.1996.tb00035.x

    authors: Fredrikson S

    更新日期:1996-07-01 00:00:00

  • Recurrent herpes zoster myelitis treated with human interferon alpha: a case report.

    abstract::Recurrent herpes zoster myelitis is very rare. However, a case was recently observed in our hospital. A 43-year-old woman developed myelitis 2 weeks after development of shingles. Her condition was improved by methylprednisolone. Seven months later, she developed myelitis after development of shingles again. Antibody ...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1992.tb04064.x

    authors: Nakano T,Awaki E,Araga S,Takai H,Inoue K,Takahashi K

    更新日期:1992-05-01 00:00:00

  • The increasing prevalence of Parkinson's disease in Estonia.

    abstract:OBJECTIVES:A previous epidemiological study of Parkinson's disease (PD) in the county of Tartu, Estonia, found an adjusted prevalence rate of 152/100 000 persons. We aimed to determine PD prevalence almost 20 years later, as well as evaluate any dynamic changes in disease frequency compared to the first study. METHODS...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/ane.12948

    authors: Kadastik-Eerme L,Taba N,Asser T,Taba P

    更新日期:2018-09-01 00:00:00

  • Entrapment neuropathy in patients with spastic cerebral palsy.

    abstract:OBJECTIVES:We performed nerve conduction and needle electromyographic tests in 29 patients with spastic cerebral palsy (SCP) and severe limb deformities. Nerve conduction abnormalities were detected in 32 of 400 sensory or motor nerves, while 11 of 29 patients (37.9%) showed abnormal nerve conduction, indicating one or...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.2005.00468.x

    authors: Frascarelli M,Frascarelli F,Gentile MG,Serrao M,De Santis F,Pierelli F,Santilli V

    更新日期:2005-09-01 00:00:00

  • Clinical features of multiple sclerosis in north-east Scotland.

    abstract::The clinical features of multiple sclerosis (MS) in north-east Scotland have been examined along with an epidemiological study to determine the prevalence of the disease on 1st December, 1970. Patient information was collected from all available diagnostic indices, both retrospectively and prospectively from 1965. Eve...

    journal_title:Acta neurologica Scandinavica

    pub_type: 杂志文章

    doi:10.1111/j.1600-0404.1979.tb02973.x

    authors: Shepherd DI

    更新日期:1979-10-01 00:00:00