当前位置： SCI文献检索 > JOURNAL OF THE NEUROLOGICAL SCIENCES期刊下所有文献 > Dominant psychiatric manifestations in Wilson's disease: a diagnostic and therapeutic challenge!

Dominant psychiatric manifestations in Wilson's disease: a diagnostic and therapeutic challenge!

Abstract：

INTRODUCTION:Recognition of psychiatric manifestations of Wilson's disease (WD) has diagnostic and therapeutic implications. OBJECTIVE:To describe the clinical features and psychopathology of patients with WD who had initial or predominant psychiatric manifestations. PATIENT AND METHODS:Records of 15 patients with WD (M:F: 11:4), from a large cohort of 350 patients, with predominant psychiatric manifestations at onset were reviewed. Their initial diagnosis, demographic profile, family history, pre-morbid personality, clinical manifestations, treatment and outcome were recorded. RESULTS:Their mean age at diagnosis was 19.8+/-5.8 years. Six patients were born to consanguineous parentage and two patients each had family history of WD and past history of psychiatric illness. Diagnosis of WD was suspected by detection of KF rings (all), observing sensitivity to neuroleptics (n=2), history of jaundice (n=2) and family history suggestive of WD (n=9). Psychiatric manifestations could be classified as affective disorder spectrum (n=11) and schizophreniform-illness (n=3). While the psychiatric symptoms improved in five patients with de-coppering therapy, seven patients needed symptomatic treatment as well. Three of the four patients who responded to de-coppering therapy were sensitive to neuroleptics. Long-term follow up of 10 patients revealed variable recovery. CONCLUSIONS:Young patient with psychiatric manifestations with clues like history of jaundice, family history of neuropsychiatric manifestations and sensitivity to neuroleptics should be evaluated for WD to avoid delay in diagnosis and associated morbidity. SIGNIFICANT OUTCOMES:The study reemphasizes the importance of behavioral manifestations in Wilson disease in terms of diagnosis and management difficulties. LIMITATIONS:Retrospective nature of the study.

journal_name

J Neurol Sci

journal_title

Journal of the neurological sciences

authors

Srinivas K,Sinha S,Taly AB,Prashanth LK,Arunodaya GR,Janardhana Reddy YC,Khanna S

doi

10.1016/j.jns.2007.09.009

subject

Has Abstract

pub_date

2008-03-15 00:00:00

pages

104-8

issue

1-2

eissn

0022-510X

issn

1878-5883

pii

S0022-510X(07)00636-3

journal_volume

266

pub_type

杂志文章

相关文献

JOURNAL OF THE NEUROLOGICAL SCIENCES文献大全
  • Symptomatic female carriers of Duchenne muscular dystrophy (DMD): genetic and clinical characterization.

    abstract：:Duchenne muscular dystrophy (DMD) is an X-linked recessive disease caused by mutations in the dystrophin gene and is characterized by muscle degeneration and death. DMD affects males; females being asymptomatic carriers of mutations. However, some of them manifest symptoms due to a translocation between X chromosome a...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2013.09.036

    authors： Giliberto F,Radic CP,Luce L,Ferreiro V,de Brasi C,Szijan I

    更新日期：2014-01-15 00:00:00

  • Zika virus infection and risk of Guillain-Barré syndrome: A meta-analysis.

    abstract：OBJECTIVE:Findings from studies of the association between Zika virus (ZIKV) infection and Guillain-Barré syndrome (GBS) are inconsistent. I conducted a systematic review and meta-analysis to clarify the nature of this association. METHODS:I searched PubMed, Scopus, Cochrane, CINAHL, Web of Science, Scielo, and DOAJ f...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章,meta分析

    doi：10.1016/j.jns.2019.06.019

    authors： Bautista LE

    更新日期：2019-08-15 00:00:00

  • Risk factors for mental disorders in patients with hypertensive intracerebral hemorrhage following neurosurgical treatment.

    abstract：BACKGROUND:Mental disorders are commonly observed among surgically treated patients with hypertensive intracerebral hemorrhage (HICH), leading to serious negative impacts on the patient's treatment, rehabilitation, and prognosis. The study aimed to establish the prevalence rates and risk factors for mental disorders fo...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2014.04.021

    authors： Li F,Chen QX

    更新日期：2014-06-15 00:00:00

  • Unsafe rear-end collision avoidance in Alzheimer's disease.

    abstract：:Drivers with cognitive impairment are at increased odds for vehicular crashes. Rear-end collisions (REC) are among the most common crash types. We tested REC avoidance in 61 drivers with mild Alzheimer's disease (AD) and 115 elderly controls using a high-fidelity interactive driving simulator. After a segment of uneve...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2006.08.011

    authors： Uc EY,Rizzo M,Anderson SW,Shi Q,Dawson JD

    更新日期：2006-12-21 00:00:00

  • Nurse led telephone assessment of expanded disability status scale assessment in MS patients at high levels of disability.

    abstract：BACKGROUND:In clinical trials drop out bias reduces the validity of results. This is a particular problem in long-term multiple sclerosis (MS) studies, particularly when patients become progressively disabled and have increasing difficulty attending assessment clinics. OBJECTIVE:To assess the validity of nurse led tel...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章,多中心研究

    doi：10.1016/j.jns.2016.01.011

    authors： Huda S,Cavey A,Izat A,Mattison P,Boggild M,Palace J

    更新日期：2016-03-15 00:00:00

  • Central and peripheral SEP defects in neurologically symptomatic and asymptomatic subjects with low vitamin B12 levels.

    abstract：:Somatosensory evoked potentials (SEPs) following median nerve stimulation were abnormal in 7 patients with sensory impairment due to vitamin B12 deficiency. Extensor plantar reflexes indicated a central sensory pathway lesion in 4 cases and absent tendon jerks suggested peripheral neuropathy in 4, but median nerve SEP...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(87)90006-2

    authors： Jones SJ,Yu YL,Rudge P,Kriss A,Gilois C,Hirani N,Nijhawan R,Norman P,Will R

    更新日期：1987-12-01 00:00:00

  • SNCA variants and alpha-synuclein level in CD45+ blood cells in Parkinson's disease.

    abstract：:Parkinson's disease (PD) is the second most frequent neurodegenerative disorder. Impaired metabolism of alpha-synuclein (SNCA) and its aggregation are implicated in PD pathogenesis. SNCA has been identified as a highly significant genetic risk loci associated with the sporadic form of PD in across populations in GWAS ...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2018.10.002

    authors： Emelyanov A,Kulabukhova D,Garaeva L,Senkevich K,Verbitskaya E,Nikolaev M,Andoskin P,Kopytova A,Milyukhina I,Yakimovskii A,Timofeeva A,Prakhova L,Ilves A,Vlasova I,Pchelina S

    更新日期：2018-12-15 00:00:00

  • Effect of mild cognitive impairment on balance.

    abstract：OBJECTIVE:To investigate the effect of mild cognitive impairment (MCI) on balance. METHODS:87 subjects with subjective memory impairment were enrolled, and subdivided into two groups, MCI and non-MCI, according to diagnostic criteria of amnestic subtype of MCI according to the 1999 MCI international panel (Current Con...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2011.02.031

    authors： Shin BM,Han SJ,Jung JH,Kim JE,Fregni F

    更新日期：2011-06-15 00:00:00

  • Muscular dystrophy in the mdx mouse: histopathology of the soleus and extensor digitorum longus muscles.

    abstract：:We have used light microscopic histomorphometry to quantify the developmental histopathological changes induced by muscular dystrophy in the soleus and extensor digitorum longus (EDL) muscles of the mdx mouse. We find that this X-linked disease exhibits early fibre necrosis with foci of invasive cells, clustering of a...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(87)90219-x

    authors： Carnwath JW,Shotton DM

    更新日期：1987-08-01 00:00:00

  • Characterization of membrane markers of isolated oligodendrocytes and clonal lines of the nervous system.

    abstract：:We show here that the major glycolipid in myelin, galactocerebroside, is not only a useful marker for isolated bovine oligodendrocytes but that its quantitation can serve as a probe for cell differentiation. We have produced in the rabbit antisera to bovine oligodendroglia, bovine myelin and galactocerebroside. The bi...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(80)90032-5

    authors： Steck AJ,Perruisseau G

    更新日期：1980-07-01 00:00:00

  • Onset, natural history and outcome in idiopathic adult motor neuron disease.

    abstract：:Cases of adult-onset idiopathic motor neuron disease (MND) identified from January 1970 through December 1986 were studied in a defined area of California. The patients were followed prospectively throughout the illness in 99% of cases. Among 708 cases aged 25-74 years at onset, the most common type (86%) was typical,...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(93)90245-t

    authors： Norris F,Shepherd R,Denys E,U K,Mukai E,Elias L,Holden D,Norris H

    更新日期：1993-08-01 00:00:00

  • Pathological findings in a patient with amyotrophic lateral sclerosis and multifocal motor neuropathy with conduction block.

    abstract：:We studied a 53-year-old woman with progressive weakness of the left arm, gradually spreading to the other limbs. Neurological examination revealed a motor neuron syndrome with paresis, fasciculations and atrophy. Electrophysiological studies showed multiple motor conduction blocks. The anti-GM1 IgM titer was elevated...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(95)00295-d

    authors： Veugelers B,Theys P,Lammens M,Van Hees J,Robberecht W

    更新日期：1996-03-01 00:00:00

  • Kynurenic acid and its analogue can alter the opioid receptor G-protein signaling after acute treatment via NMDA receptor in rat cortex and striatum.

    abstract：:Previously, we have shown that the N-methyl d-aspartate (NMDA)-receptor antagonist kynurenic acid (KYNA) and its analogue KYNA1 do not bind directly to mu, kappa and delta opioid receptors in vitro. On the other hand, chronic administration of KYNA and KYNA1 resulted in region (cortex vs striatum) and opioid receptor-...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2017.02.053

    authors： Samavati R,Zádor F,Szűcs E,Tuka B,Martos D,Veres G,Gáspár R,Mándity IM,Fülöp F,Vécsei L,Benyhe S,Borsodi A

    更新日期：2017-05-15 00:00:00

  • Differential recording of upper and lower cervical N13 responses and their contribution to scalp recorded responses in median nerve somatosensory evoked potentials.

    abstract：:To distinguish the different origins of cervical N13 potentials in median nerve somatosensory evoked potentials (SSEPs), cervical N13 potentials were recorded by two different montages. The abnormal patterns of the SSEPs were compared to the abnormal evoked spinal cord responses (ESCPs) recorded from posterior epidura...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/s0022-510x(01)00509-3

    authors： Fujimoto H,Kaneko K,Taguchi T,Ofuji A,Yonemura H,Kawai S

    更新日期：2001-06-15 00:00:00

  • Magnetic resonance imaging based morphologic evaluation of the pineal gland for suspected pineoblastoma in retinoblastoma patients and age-matched controls.

    abstract：PURPOSE:The purpose of this study was to evaluate the morphologic magnetic resonance imaging (MRI) characteristics of the pineal gland in retinoblastoma (Rb) patients without and with pineoblastoma in comparison to age-matched controls to improve early identification of pineoblastomas (trilateral retinoblastoma, TRb). ...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2015.10.046

    authors： Pham TT,Siebert E,Asbach P,Willerding G,Erb-Eigner K

    更新日期：2015-12-15 00:00:00

  • Motor neuron disease presenting with respiratory failure.

    abstract：:Respiratory failure accounts for the majority of deaths in amyotrophic lateral sclerosis (ALS) but only rarely is ALS diagnosed on the basis of respiratory insufficiency. We report four ALS patients presenting with acute respiratory failure. In three patients we have performed EMG needle examination of both hemidiaphr...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(96)00089-5

    authors： de Carvalho M,Matias T,Coelho F,Evangelista T,Pinto A,Luís ML

    更新日期：1996-08-01 00:00:00

  • Platelet amyloid precursor protein processing: a bio-marker for Alzheimer's disease.

    abstract：:The amyloid precursor protein (APP) in brain is processed either by an amyloidogenic pathway by beta-secretase and gamma-secretase to yield Abeta (beta-amyloid 4 kDa) peptide or by alpha-secretase within the beta-amyloid domain to yield non-amyloidogenic products. We have studied blood platelet levels of a 22-kDa frag...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2005.09.002

    authors： Tang K,Hynan LS,Baskin F,Rosenberg RN

    更新日期：2006-01-15 00:00:00

  • N-methyl-D-aspartate receptor subunit NR2A and NR2B messenger RNA levels are altered in the hippocampus and entorhinal cortex in Alzheimer's disease.

    abstract：:The N-methyl-D-aspartate (NMDA) receptor is a subtype of ionotropic glutamate receptor that is involved in synaptic mechanisms of learning and memory, and mediates excitotoxic neuronal injury. In this study, we tested the hypothesis that NMDA receptor subunit gene expression is altered in Alzheimer's disease (AD), esp...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/s0022-510x(02)00087-4

    authors： Bi H,Sze CI

    更新日期：2002-08-15 00:00:00

  • Dipyridamole and headache--a pilot study of initial dose titration.

    abstract：:Headache is reported by about one third of patients using dipyridamole (DP) after stroke or TIA. No study has systematically examined if initial dipyridamole titration may affect this headache. We therefore randomised patients to (1) standard aspirin and dipyridamole treatment b.i.d. for 2 weeks or (2) titration with ...

    journal_title：Journal of the neurological sciences

    pub_type： 临床试验,杂志文章,随机对照试验

    doi：10.1016/j.jns.2004.05.011

    authors： Lindgren A,Husted S,Staaf G,Ziegler B

    更新日期：2004-08-30 00:00:00

  • The effects of hydrocephalus upon the developing brain. Histological and quantitative studies of the ependyma and subependyma in hydrocephalic rats.

    abstract：:A suspension of kaolin was injected into the cisterna magna of 44 rats at 2 weeks of age. Animals killed at intervals from 5--19 weeks of age showed varying degrees of hydrocephalus. Light microscopy, scanning and transmission electron microscopy revealed stretching and flattening of the ependymal cells but no signifi...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(78)90046-1

    authors： Weller RO,Mitchell J,Griffin RL,Gardner MJ

    更新日期：1978-05-01 00:00:00

  • Sensitivity and specificity of cerebrospinal fluid CXCL13 for diagnosing Lyme neuroborreliosis - a study on 1410 patients and review of the literature.

    abstract：INTRODUCTION:The B-cell chemoattractant CXCL13 has been suggested as a cerebrospinal fluid (CSF) biomarker for Lyme neuroborreliosis (LNB). Our aim was to substantiate the value of CXCL13 in a large unselected cohort and determine a practical cut-off value to diagnose LNB. METHODS:We retrospectively studied clinical a...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2020.116843

    authors： Lintner H,Hochgatterer-Rechberger P,Pischinger B,Seier J,Vollmann P,Haushofer A,Rittner H,Sommer C,Topakian R

    更新日期：2020-07-15 00:00:00

  • Role of Rho-mediated ROCK-Semaphorin3A signaling pathway in the pathogenesis of Parkinson's disease in a mouse model.

    abstract：OBJECTIVE:The present study aims to elucidate the role of Rho-mediated ROCK-Semaphorin3A signaling pathway in the pathogenesis of Parkinson's disease (PD) in a mouse model. METHODS:One-hundred twelve eight-week male C57BL/6 mice were selected. The mouse model of PD was constructed by intraperitoneal injection of MPTP....

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2016.08.061

    authors： Qi L,Tang YG,Wang L,He W,Pan HH,Nie RR,Can Y

    更新日期：2016-11-15 00:00:00

  • CaMKII antisense oligodeoxynucleotides protect against ischemia-induced neuronal death in the rat hippocampus.

    abstract：:The present study was performed to investigate the effects of Ca(2+)/calmodulin-dependent protein kinase II (CaMKII) antisense oligodeoxynucleotides (ODNs) on the assembly of the CaMKII·GluR6·PSD-95 signaling module, GluR6 serine phosphorylation and c-Jun N-terminal kinase 3 (JNK3) activation. A further aim was to det...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2011.10.012

    authors： Liu Z,Xu J,Shen X,Lv C,Xu T,Pei D

    更新日期：2012-03-15 00:00:00

  • Correlation between regional brain volume and olfactory function in very mild amnestic patients.

    abstract：BACKGROUND & AIMS:We aimed to determine neural correlates of olfactory detection and identification and analyze associations between cognitive function and olfactory identification or detection in very mild amnestic patients. METHODS:We recruited 70 patients with chief complaints of memory impairment diagnosed as amne...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2020.116686

    authors： Kashibayashi T,Takahashi R,Fujita J,Kamimura N,Okutani F,Kazui H

    更新日期：2020-04-15 00:00:00

  • Immunological relationship between a demyelinating RNA enveloped budding virus (Semliki Forest) and brain glycolipids.

    abstract：:A monoclonal antibody (MAb) raised against central nervous system (CNS) myelin (212) and a MAb (308) raised against brain with Semliki Forest virus (SFV) were both found to react against the same CNS glycolipids. Both these MAbs were also found to react strongly with SFV and against certain brain glycolipid fractions ...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(86)90144-9

    authors： Khalili-Shirazi A,Gregson N,Webb HE

    更新日期：1986-11-01 00:00:00

  • Risk factors for stroke among patients with hypertension: a case-control study.

    abstract：BACKGROUND:In some African countries, more than half of the adult population are estimated to be hypertensive leading to an escalated burden of stroke in the continent. We conducted the first study to unravel the major risk factors for stroke among hypertensive patients (Nigerian-Africans) using a case-control design w...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2012.11.016

    authors： Owolabi MO,Agunloye AM

    更新日期：2013-02-15 00:00:00

  • Evaluation of MS related central fatigue using MR neuroimaging methods: Scoping review.

    abstract：BACKGROUND:Fatigue is a common and debilitating symptom in multiple sclerosis (MS). Over the past decade, a growing body of research has focussed on the pathophysiological mechanisms underlying central (cognitive and physical) fatigue in MS. The precise mechanisms causing fatigue in MS patients are complex and poorly u...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章,评审

    doi：10.1016/j.jns.2019.03.007

    authors： Arm J,Ribbons K,Lechner-Scott J,Ramadan S

    更新日期：2019-05-15 00:00:00

  • Relationship between poor decision-making process and fatigue perception in Parkinson's disease patients.

    abstract：BACKGROUND:Fatigue is a common non-motor symptom in Parkinson's disease patients. The reasons for its perception are not completely understood. One suggested possibility might be that perceived fatigue is related with abnormal interpretation of somatic symptoms. It has been described that somatic markers misinterpretat...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2013.12.003

    authors： Sáez-Francàs N,Hernández-Vara J,Corominas-Roso M,Alegre J,Jacas C,Casas M

    更新日期：2014-02-15 00:00:00

  • "Cap disease"--a failure in the correct muscle fibre formation.

    abstract：:Four unrelated patients are reported with muscle hypotonia, weakness, skeletal dysmorphism and respiratory insufficiency since childhood. Muscle tissues were found to contain a number of muscle fibres with abnormal structure. Peripherally located structures such as a cap lacking in ATP-ase and fast myosin activity, ri...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/s0022-510x(02)00156-9

    authors： Fidziańska A

    更新日期：2002-09-15 00:00:00

  • Neurological involvement and characterization in acquired hemophagocytic lymphohistiocytosis in adulthood.

    abstract：OBJECTIVE:To describe the neurological and neuroradiological features of acquired hemophagocytic lymphohistiocytosis (HLH) in adulthood by reporting a series of cases. METHODS:Ten consecutive patients who were diagnosed with HLH at Medstar Georgetown University Hospital and Walter Reed National Military Medical Center...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2015.07.017

    authors： Gratton SM,Powell TR,Theeler BJ,Hawley JS,Amjad FS,Tornatore C

    更新日期：2015-10-15 00:00:00