Limbic encephalitis associated with anti-voltage-gated potassium channel complex antibodies mimicking Creutzfeldt-Jakob disease.

Abstract:

IMPORTANCE:Limbic encephalitis that is associated with anti-voltage-gated potassium channel complex (VGKCC) antibodies (VGKCC syndrome) is an autoimmune, usually nonparaneoplastic form of encephalitis that is responsive to immunotherapy. Differentiating this treatable disease from others that have a similar presentation is thus important. OBSERVATIONS:We present the case of a 58-year-old man who had a rapid onset of progressive confusion, twitching of the face and hand, and abnormal basal ganglia detected by magnetic resonance imaging. His conditions were initially diagnosed as Creutzfeldt-Jakob disease (CJD). Faciobrachial dystonic seizures, possibly pathognomonic for the VGKCC syndrome, had been misdiagnosed as myoclonus. Treatment led to a complete resolution of his symptoms. CONCLUSIONS AND RELEVANCE:Given the similarities of the clinical features and, at times, the neuroimaging findings of VGKCC syndrome to CJD, recognizing VGKCC syndrome and the highly associated and distinctive faciobrachial dystonic seizures is very important. Because this syndrome is the most common treatable condition that mimics CJD, we believe that it is crucial to screen all patients with presumed CJD for this reversible condition.

journal_name

JAMA Neurol

journal_title

JAMA neurology

authors

Yoo JY,Hirsch LJ

doi

10.1001/jamaneurol.2013.5179

subject

Has Abstract

pub_date

2014-01-01 00:00:00

pages

79-82

issue

1

eissn

2168-6149

issn

2168-6157

pii

1769294

journal_volume

71

pub_type

杂志文章
  • Association Between Prehospital Tranexamic Acid Administration and Outcomes of Severe Traumatic Brain Injury.

    abstract:Importance:The development and expansion of intracranial hematoma are associated with adverse outcomes. Use of tranexamic acid might limit intracranial hematoma formation, but its association with outcomes of severe traumatic brain injury (TBI) is unclear. Objective:To assess whether prehospital administration of tran...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2020.4596

    authors: Bossers SM,Loer SA,Bloemers FW,Den Hartog D,Van Lieshout EMM,Hoogerwerf N,van der Naalt J,Absalom AR,Peerdeman SM,Schwarte LA,Boer C,Schober P,BRAIN-PROTECT collaborators.

    更新日期:2020-12-07 00:00:00

  • Blood pressure and progression of brain atrophy: the SMART-MR Study.

    abstract:IMPORTANCE:Studies have shown that both high and low blood pressure (BP) may play a role in the etiology of brain atrophy. High BP in midlife has been associated with more brain atrophy later in life, whereas studies in older populations have shown a relation between low BP and more brain atrophy. Yet, prospective evid...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2013.217

    authors: Jochemsen HM,Muller M,Visseren FL,Scheltens P,Vincken KL,Mali WP,van der Graaf Y,Geerlings MI,SMART Study Group.

    更新日期:2013-08-01 00:00:00

  • The aging brain and cognition: contribution of vascular injury and aβ to mild cognitive dysfunction.

    abstract:IMPORTANCE:β-Amyloid (Aβ) deposition and vascular brain injury (VBI) frequently co-occur and are both associated with cognitive decline in aging. Determining whether a direct relationship exists between them has been challenging. We sought to understand VBI's influence on cognition and clinical impairment, separate fro...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/2013.jamaneurol.405

    authors: Marchant NL,Reed BR,Sanossian N,Madison CM,Kriger S,Dhada R,Mack WJ,DeCarli C,Weiner MW,Mungas DM,Chui HC,Jagust WJ

    更新日期:2013-04-01 00:00:00

  • Prognostic Value of Spreading Depolarizations in Patients With Severe Traumatic Brain Injury.

    abstract:Importance:Advances in treatment of traumatic brain injury are hindered by the inability to monitor pathological mechanisms in individual patients for targeted neuroprotective treatment. Spreading depolarizations, a mechanism of lesion development in animal models, are a novel candidate for clinical monitoring in patie...

    journal_title:JAMA neurology

    pub_type: 杂志文章,多中心研究

    doi:10.1001/jamaneurol.2019.4476

    authors: Hartings JA,Andaluz N,Bullock MR,Hinzman JM,Mathern B,Pahl C,Puccio A,Shutter LA,Strong AJ,Vagal A,Wilson JA,Dreier JP,Ngwenya LB,Foreman B,Pahren L,Lingsma H,Okonkwo DO

    更新日期:2020-04-01 00:00:00

  • Assessment of Incidence and Risk Factors of Intracerebral Hemorrhage Among Participants in the Framingham Heart Study Between 1948 and 2016.

    abstract:Importance:Intracerebral hemorrhage (ICH) has the highest mortality of all stroke types and is the most serious complication of anticoagulation. Data regarding trends in ICH incidence and location-specific risk factors on the population level are conflicting. Objective:To assess long-term population-based trends in th...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2020.1512

    authors: Lioutas VA,Beiser AS,Aparicio HJ,Himali JJ,Selim MH,Romero JR,Seshadri S

    更新日期:2020-06-08 00:00:00

  • Preventing Cognitive Decline in Black Individuals With Mild Cognitive Impairment: A Randomized Clinical Trial.

    abstract:Importance:Mild cognitive impairment (MCI) is a transition state between normal cognitive aging and dementia that increases the risk for progressive cognitive decline. Preventing cognitive decline is a public health priority. Objective:To determine whether behavioral activation prevents cognitive and functional declin...

    journal_title:JAMA neurology

    pub_type: 杂志文章,随机对照试验

    doi:10.1001/jamaneurol.2018.2513

    authors: Rovner BW,Casten RJ,Hegel MT,Leiby B

    更新日期:2018-12-01 00:00:00

  • Episodic bradycardia as neurocardiac prodrome to voltage-gated potassium channel complex/leucine-rich, glioma inactivated 1 antibody encephalitis.

    abstract:IMPORTANCE:Voltage-gated potassium channel complex antibody (VGKCc-Ab) encephalitis is an immunotherapy-responsive syndrome usually associated with causative antibodies that target the leucine-rich, glioma inactivated 1 (LGI1) protein. Although it is expressed throughout the brain, LGI1 is not known to be expressed in ...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2014.1234

    authors: Naasan G,Irani SR,Bettcher BM,Geschwind MD,Gelfand JM

    更新日期:2014-10-01 00:00:00

  • Natalizumab therapy for highly active pediatric multiple sclerosis.

    abstract:IMPORTANCE:Given the high frequency of failure of first-line therapies, there is an urgent need for second-line treatment strategies for pediatric patients with multiple sclerosis (MS). OBJECTIVE:To report the use of natalizumab in pediatric MS. Natalizumab, a humanized monoclonal antibody targeting α4 integrin, is ef...

    journal_title:JAMA neurology

    pub_type: 杂志文章,多中心研究

    doi:10.1001/jamaneurol.2013.923

    authors: Kornek B,Aboul-Enein F,Rostasy K,Milos RI,Steiner I,Penzien J,Hellwig K,Pitarokoili K,Storm van's Gravesande K,Karenfort M,Blaschek A,Meyer A,Seidl R,Debelic D,Vass K,Prayer D,Kristoferitsch W,Bayas A

    更新日期:2013-04-01 00:00:00

  • Autoimmune aquaporin-4 myopathy in neuromyelitis optica spectrum.

    abstract:IMPORTANCE:Documentation of muscle pathology compatible with targeting of sarcolemmal aquaporin-4 (AQP4) by complement-activating IgG implies involvement of organs beyond the central nervous system in neuromyelitis optica spectrum disorders. OBSERVATIONS:We report on a 51-year-old woman who had relapsing optic neuriti...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2014.775

    authors: Guo Y,Lennon VA,Popescu BF,Grouse CK,Topel J,Milone M,Lassmann H,Parisi JE,Pittock SJ,Stefoski D,Balabanov R,Lucchinetti CF

    更新日期:2014-08-01 00:00:00

  • Skin α-Synuclein Aggregation Seeding Activity as a Novel Biomarker for Parkinson Disease.

    abstract:Importance:Deposition of the pathological α-synuclein (αSynP) in the brain is the hallmark of synucleinopathies, including Parkinson disease (PD), Lewy body dementia (LBD), and multiple system atrophy (MSA). Whether real-time quaking-induced conversion (RT-QuIC) and protein misfolding cyclic amplification (PMCA) assays...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2020.3311

    authors: Wang Z,Becker K,Donadio V,Siedlak S,Yuan J,Rezaee M,Incensi A,Kuzkina A,Orrú CD,Tatsuoka C,Liguori R,Gunzler SA,Caughey B,Jimenez-Capdeville ME,Zhu X,Doppler K,Cui L,Chen SG,Ma J,Zou WQ

    更新日期:2020-09-28 00:00:00

  • Clinical Characteristics of Patients With Double-Seronegative Myasthenia Gravis and Antibodies to Cortactin.

    abstract:IMPORTANCE:Double-seronegative myasthenia gravis (dSNMG) includes patients with myasthenia gravis (MG) without detectable antibodies to the nicotinic acetylcholine receptor (AChR) or to muscle-specific tyrosine kinase (MuSK). The lack of a biomarker hinders the diagnosis and clinical management in these patients. Corta...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2016.2032

    authors: Cortés-Vicente E,Gallardo E,Martínez MÁ,Díaz-Manera J,Querol L,Rojas-García R,Illa I

    更新日期:2016-09-01 00:00:00

  • Association of Apolipoprotein E ε4 With Medial Temporal Tau Independent of Amyloid-β.

    abstract:Importance:Apolipoprotein E ε4 (APOEε4) is the single most important genetic risk factor for Alzheimer disease. While APOEε4 is associated with increased amyloid-β burden, its association with cerebral tau pathology has been controversial. Objective:To determine whether APOEε4 is associated with medial temporal tau pa...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2019.4421

    authors: Therriault J,Benedet AL,Pascoal TA,Mathotaarachchi S,Chamoun M,Savard M,Thomas E,Kang MS,Lussier F,Tissot C,Parsons M,Qureshi MNI,Vitali P,Massarweh G,Soucy JP,Rej S,Saha-Chaudhuri P,Gauthier S,Rosa-Neto P

    更新日期:2020-04-01 00:00:00

  • Comprehension of an Elevated Amyloid Positron Emission Tomography Biomarker Result by Cognitively Normal Older Adults.

    abstract:Importance:The goal of Alzheimer disease (AD) prevention together with advances in understanding the pathophysiology of AD have led to clinical trials testing drugs in cognitively unimpaired persons who show evidence of AD biomarkers. Data are needed to inform the processes of describing AD biomarkers to cognitively no...

    journal_title:JAMA neurology

    pub_type: 杂志文章,多中心研究

    doi:10.1001/jamaneurol.2017.2954

    authors: Mozersky J,Sankar P,Harkins K,Hachey S,Karlawish J

    更新日期:2018-01-01 00:00:00

  • Posterior cortical atrophy as an extreme phenotype of GRN mutations.

    abstract:IMPORTANCE:Posterior cortical atrophy (PCA) is characterized by progressive visuoperceptual and visuospatial deficits and commonly considered to be an atypical variant of Alzheimer disease. Mutations of the GRN gene are responsible for a large phenotypic spectrum, but, to our knowledge, the association of PCA with GRN ...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2014.3308

    authors: Caroppo P,Belin C,Grabli D,Maillet D,De Septenville A,Migliaccio R,Clot F,Lamari F,Camuzat A,Brice A,Dubois B,Le Ber I

    更新日期:2015-02-01 00:00:00

  • Retinal Architecture and Melanopsin-Mediated Pupillary Response Characteristics: A Putative Pathophysiologic Signature for the Retino-Hypothalamic Tract in Multiple Sclerosis.

    abstract:Importance:A neurophysiologic signature of the melanopsin-mediated persistent constriction phase of the pupillary light reflex may represent a surrogate biomarker for the integrity of the retinohypothalamic tract, with potential utility for investigating alterations in homeostatic mechanisms associated with brain disor...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2016.5131

    authors: Meltzer E,Sguigna PV,Subei A,Beh S,Kildebeck E,Conger D,Conger A,Lucero M,Frohman BS,Frohman AN,Saidha S,Galetta S,Calabresi PA,Rennaker R,Frohman TC,Kardon RH,Balcer LJ,Frohman EM

    更新日期:2017-05-01 00:00:00

  • Ischemic stroke and transient ischemic attack in young adults: risk factors, diagnostic yield, neuroimaging, and thrombolysis.

    abstract:BACKGROUND:Approximately 10% to 14% of ischemic strokes occur in young adults. OBJECTIVE:To investigate the yield of diagnostic tests, neuroimaging findings, and treatment of ischemic strokes in young adults. DESIGN:We retrospectively reviewed data from our Get with the Guidelines-Stroke database from 2005 through 20...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2013.575

    authors: Ji R,Schwamm LH,Pervez MA,Singhal AB

    更新日期:2013-01-01 00:00:00

  • A Histologic Study of the Circadian System in Parkinson Disease, Multiple System Atrophy, and Progressive Supranuclear Palsy.

    abstract:Importance:Circadian dysfunction may be associated with the symptoms and neurodegeneration in Parkinson disease (PD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP), although the underlying neuroanatomical site of disruption and pathophysiological mechanisms are not fully understood. Objective...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2018.0640

    authors: De Pablo-Fernández E,Courtney R,Warner TT,Holton JL

    更新日期:2018-08-01 00:00:00

  • Outcomes of Intensive Systolic Blood Pressure Reduction in Patients With Intracerebral Hemorrhage and Excessively High Initial Systolic Blood Pressure: Post Hoc Analysis of a Randomized Clinical Trial.

    abstract:Importance:The safety and efficacy of intensive systolic blood pressure reduction in patients with intracerebral hemorrhage who present with systolic blood pressure greater than 220 mm Hg appears to be unknown. Objective:To evaluate the differential outcomes of intensive (goal, 110-139 mm Hg) vs standard (goal, 140-17...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2020.3075

    authors: Qureshi AI,Huang W,Lobanova I,Barsan WG,Hanley DF,Hsu CY,Lin CL,Silbergleit R,Steiner T,Suarez JI,Toyoda K,Yamamoto H,for ATACH-II trial investigators.

    更新日期:2020-09-08 00:00:00

  • The growth and impact of Alzheimer disease centers as measured by social network analysis.

    abstract:IMPORTANCE:Alzheimer disease (AD) is a neurodegenerative disorder with no effective therapies. In 1984, the National Institute on Aging created the first 5 AD centers (ADCs) in an effort to coordinate research efforts into the pathology and treatment of the disease. Since that time, the ADC program has expanded to incl...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2013.6225

    authors: Hughes ME,Peeler J,Hogenesch JB,Trojanowski JQ

    更新日期:2014-04-01 00:00:00

  • An inflection point in gene discovery efforts for neurodegenerative diseases: from syndromic diagnoses toward endophenotypes and the epigenome.

    abstract::We are at an inflection point in our study of the human genome as it relates to neurodegenerative disease. The sequencing of the human genome, and its associated cataloging of human genetic variation and technological as well as methodological development, introduced a period of rapid gene discovery over the past deca...

    journal_title:JAMA neurology

    pub_type: 杂志文章,评审

    doi:10.1001/jamaneurol.2013.275

    authors: De Jager PL,Bennett DA

    更新日期:2013-06-01 00:00:00

  • Intractable Epilepsy and Progressive Cognitive Decline in a Young Man.

    abstract::A young man with normal neurodevelopment presented with 3 years of medically refractory, progressive epilepsy and myoclonus. Initial examination included neuroimaging, electroencephalography, and biochemical analyses, all of which were unremarkable except for mildly enlarged ventricles. Over the following year, the pa...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2016.3195

    authors: Cohen AL,Jones LK,Parisi JE,Klaas JP

    更新日期:2017-06-01 00:00:00

  • Efficacy and Safety of Deep Brain Stimulation in Tourette Syndrome: The International Tourette Syndrome Deep Brain Stimulation Public Database and Registry.

    abstract:Importance:Collective evidence has strongly suggested that deep brain stimulation (DBS) is a promising therapy for Tourette syndrome. Objective:To assess the efficacy and safety of DBS in a multinational cohort of patients with Tourette syndrome. Design, Setting, and Participants:The prospective International Deep Br...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2017.4317

    authors: Martinez-Ramirez D,Jimenez-Shahed J,Leckman JF,Porta M,Servello D,Meng FG,Kuhn J,Huys D,Baldermann JC,Foltynie T,Hariz MI,Joyce EM,Zrinzo L,Kefalopoulou Z,Silburn P,Coyne T,Mogilner AY,Pourfar MH,Khandhar SM,Auyeung

    更新日期:2018-03-01 00:00:00

  • Identification of Risk Loci for Parkinson Disease in Asians and Comparison of Risk Between Asians and Europeans: A Genome-Wide Association Study.

    abstract:Importance:Large-scale genome-wide association studies in the European population have identified 90 risk variants associated with Parkinson disease (PD); however, there are limited studies in the largest population worldwide (ie, Asian). Objectives:To identify novel genome-wide significant loci for PD in Asian indivi...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2020.0428

    authors: Foo JN,Chew EGY,Chung SJ,Peng R,Blauwendraat C,Nalls MA,Mok KY,Satake W,Toda T,Chao Y,Tan LCS,Tandiono M,Lian MM,Ng EY,Prakash KM,Au WL,Meah WY,Mok SQ,Annuar AA,Chan AYY,Chen L,Chen Y,Jeon BS,Jiang L,Lim J

    更新日期:2020-06-01 00:00:00

  • Outcomes in mild acute ischemic stroke treated with intravenous thrombolysis: a retrospective analysis of the Get With the Guidelines-Stroke registry.

    abstract:IMPORTANCE:Mild strokes have been poorly represented in thrombolytic trials and only a few series have reported outcomes after treatment with intravenous (IV) recombinant tissue plasminogen activator (rtPA) after mild stroke. OBJECTIVE:To report treatment complications and short-term outcomes in patients with mild str...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2014.4354

    authors: Romano JG,Smith EE,Liang L,Gardener H,Camp S,Shuey L,Cook A,Campo-Bustillo I,Khatri P,Bhatt DL,Fonarow GC,Sacco RL,Schwamm LH

    更新日期:2015-04-01 00:00:00

  • Incidence and pathology of synucleinopathies and tauopathies related to parkinsonism.

    abstract:IMPORTANCE:The frequency and distribution of synucleinopathies and tauopathies manifesting with parkinsonism in the general population are poorly understood, thus affecting health care planning and research. OBJECTIVE:To investigate the incidence and distribution of specific types of parkinsonism and related proteinop...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2013.114

    authors: Savica R,Grossardt BR,Bower JH,Ahlskog JE,Rocca WA

    更新日期:2013-07-01 00:00:00

  • Vascular complications of fungal meningitis attributed to injections of contaminated methylprednisolone acetate.

    abstract:IMPORTANCE:Fungal meningitis due to injections of contaminated methylprednisolone acetate can present with vascular sequelae in immunocompetent individuals. This is particularly germane to neurologists because better recognition of the clinical characteristics of patients with fungal meningitis and ischemic stroke will...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2013.3586

    authors: Kleinfeld K,Jones P,Riebau D,Beck A,Paueksakon P,Abel T,Claassen DO

    更新日期:2013-09-01 00:00:00

  • Swiss cheese striatum: clinical implications.

    abstract:IMPORTANCE:Markedly enlarged Virchow-Robin spaces throughout the striatum appear occasionally on magnetic resonance imaging (MRI) scans of the elderly, and this type of striatum is known as the Swiss cheese striatum (SCS); however, its clinical impact is unknown. OBJECTIVE:To determine the clinical features associated...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2014.286

    authors: Burnett MS,Witte RJ,Ahlskog JE

    更新日期:2014-06-01 00:00:00

  • Characterization of Gene Expression Phenotype in Amyotrophic Lateral Sclerosis Monocytes.

    abstract:Importance:Amyotrophic lateral sclerosis (ALS) is a common adult-onset neurodegenerative disease characterized by selective loss of upper and lower motor neurons. Patients with ALS have persistent peripheral and central inflammatory responses including abnormally functioning T cells and activated microglia. However, mu...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2017.0357

    authors: Zhao W,Beers DR,Hooten KG,Sieglaff DH,Zhang A,Kalyana-Sundaram S,Traini CM,Halsey WS,Hughes AM,Sathe GM,Livi GP,Fan GH,Appel SH

    更新日期:2017-06-01 00:00:00

  • Association of Pharmacological Interventions With Symptom Burden Reduction in Patients With Mild Traumatic Brain Injury: A Systematic Review.

    abstract:Importance:Mild traumatic brain injury (TBI) is experienced by 55.9 million people globally each year. The symptoms of mild TBI are diverse and sometimes long-lasting, requiring frequent use of pharmacological interventions to mitigate them. A thorough understanding of the data supporting pharmacological interventions ...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2020.5079

    authors: Feinberg C,Carr C,Zemek R,Yeates KO,Master C,Schneider K,Bell MJ,Wisniewski S,Mannix R

    更新日期:2021-01-19 00:00:00

  • Sex and Race Differences in the Association of Incident Ischemic Stroke With Risk Factors.

    abstract:Importance:Race-specific and sex-specific stroke risk varies across the lifespan, yet few reports describe sex differences in stroke risk separately in black individuals and white individuals. Objective:To examine incidence and risk factors for ischemic stroke by sex for black and white individuals. Design, Setting, ...

    journal_title:JAMA neurology

    pub_type: 杂志文章

    doi:10.1001/jamaneurol.2018.3862

    authors: Howard VJ,Madsen TE,Kleindorfer DO,Judd SE,Rhodes JD,Soliman EZ,Kissela BM,Safford MM,Moy CS,McClure LA,Howard G,Cushman M

    更新日期:2019-02-01 00:00:00