Infantile spasms: III. Prognostic implications of bitemporal hypometabolism on positron emission tomography.


:Positron emission tomography (PET) of brain glucose utilization is highly sensitive in detecting focal cortical abnormalities in patients with infantile spasms even when the computed tomographic (CT) and magnetic resonance imaging (MRI) scans are normal. Of 110 infants with spasms evaluated for potential surgical intervention during an 8-year period, we encountered 18 infants (7 males, 11 females; age range, 10 mo to 5 yr) with a common metabolic pattern on positron emission tomography (PET) consisting of bilateral hypometabolism in the temporal lobes. CT and MRI scans did not reveal any focal abnormalities in the 18 infants. Video-electroencephalographic monitoring indicated either bilateral or multifocal epileptogenicity, or failed to show any epileptic focus, so that none of the 18 infants were considered candidates for resective surgery. These patients were then enrolled in a prospective study aimed at determining long-term outcome in the presence of bilateral temporal PET hypometabolism. Analysis of outcome in 14 of the 18 subjects (follow-up period, 10 mo to 10 yr 5 mo; mean, 3 yr 11 mo +/- 2 yr 4 mo [SD]) revealed the following: (1) all had severe developmental delay and had failed to gain significant milestones; (2) language development had been minimal or absent; (3) 10 of the 14 met the DSM-IV criteria for autistic disorder. Our findings indicate that patients with infantile spasms and bitemporal glucose hypometabolism on PET comprise a relatively homogeneous group and are typically not candidates for cortical resection. The long-term outcome of these infants is particularly poor and the majority are autistic.


Ann Neurol


Annals of neurology


Chugani HT,Da Silva E,Chugani DC




Has Abstract


1996-05-01 00:00:00












  • Glatiramer acetate in primary progressive multiple sclerosis: results of a multinational, multicenter, double-blind, placebo-controlled trial.

    abstract:OBJECTIVE:To determine whether glatiramer acetate (GA) slows accumulation of disability in primary progressive multiple sclerosis. METHODS:A total of 943 patients with primary progressive multiple sclerosis were randomized to GA or placebo (PBO) in this 3-year, double-blind trial. The primary end point was an intentio...

    journal_title:Annals of neurology

    pub_type: 临床试验,杂志文章,多中心研究,随机对照试验


    authors: Wolinsky JS,Narayana PA,O'Connor P,Coyle PK,Ford C,Johnson K,Miller A,Pardo L,Kadosh S,Ladkani D,PROMiSe Trial Study Group.

    更新日期:2007-01-01 00:00:00

  • The neuropathology of probable Alzheimer disease and mild cognitive impairment.

    abstract:OBJECTIVE:Mixed pathologies are common in older persons with dementia. Little is known about mixed pathologies in probable Alzheimer disease (AD) and about the spectrum of neuropathology in mild cognitive impairment (MCI). The objective of this study was to investigate single and mixed common age-related neuropathologi...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Schneider JA,Arvanitakis Z,Leurgans SE,Bennett DA

    更新日期:2009-08-01 00:00:00

  • Autoimmune response of patients with paraneoplastic cerebellar degeneration to a Purkinje cell cytoplasmic protein antigen.

    abstract::Sera from 6 of 12 patients with paraneoplastic cerebellar degeneration (PCD) contained anti-Purkinje cell antibodies, as determined by indirect immunofluorescence on frozen sections of normal human cerebellum. Samples of cerebrospinal fluid from 2 of the patients with serum antibodies were tested, and both specimens c...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Jaeckle KA,Graus F,Houghton A,Cardon-Cardo C,Nielsen SL,Posner JB

    更新日期:1985-11-01 00:00:00

  • The time course and magnitude of spontaneous recovery of parkinsonism produced by intracarotid administration of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine to monkeys.

    abstract::We studied rhesus monkeys with hemiparkinsonism or bilateral parkinsonism produced by unilateral or bilateral intracarotid administration of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine. Using a standardized clinical rating scale, 4 hemiparkinsonian monkeys showed a 13 to 56% (mean, 36%) spontaneous improvement during...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Kurlan R,Kim MH,Gash DM

    更新日期:1991-06-01 00:00:00

  • Metabolic causes of myoglobinuria.

    abstract::To evaluate the proportion of cases of myoglobinuria that can be ascribed to specific metabolic defects, we have studied eight enzymes--phosphorylase, phosphorylase kinase, phosphofructokinase (PFK), phosphoglycerate kinase (PGK), phosphoglycerate mutase (PGAM), lactate dehydrogenase (LDH), carnitine palmitoyltransfer...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Tonin P,Lewis P,Servidei S,DiMauro S

    更新日期:1990-02-01 00:00:00

  • HTLV-I-associated leukemia: a model for chronic retroviral diseases.

    abstract::Human T-lymphotropic virus type I (HTLV-I) has been associated with adult T-cell leukemia/lymphoma (ATL), a malignancy of mature CD4-positive lymphocytes, and with tropical spastic paraparesis (TSP), a demyelinating neurological syndrome. This article describes the clinical and pathological features of ATL and reviews...

    journal_title:Annals of neurology

    pub_type: 杂志文章,评审


    authors: Murphy EL,Blattner WA

    更新日期:1988-01-01 00:00:00

  • Development and Validation of Forecasting Next Reported Seizure Using e-Diaries.

    abstract:OBJECTIVE:There are no validated methods for predicting the timing of seizures. Using machine learning, we sought to forecast 24-hour risk of self-reported seizure from e-diaries. METHODS:Data from 5,419 patients on (including seizure count, type, and duration) were split into training (3,806 patien...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Goldenholz DM,Goldenholz SR,Romero J,Moss R,Sun H,Westover B

    更新日期:2020-09-01 00:00:00

  • A white matter disorder in dementia of the Alzheimer type: a pathoanatomical study.

    abstract::In cases of Alzheimer's presenile and senile dementia, Alzheimer's disease (AD) and senile dementia of the Alzheimer type (SDAT), respectively, we have observed, in addition to the gray matter degeneration, a lesion that has the character of an incomplete infarction confined to the white matter. It is encountered in 6...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Brun A,Englund E

    更新日期:1986-03-01 00:00:00

  • Dopamine agonists and neuroprotection in Parkinson's disease.

    abstract::There is increasing interest in the potential of dopamine agonists to provide a neuroprotective effect and to alter the natural course of levodopa-treated Parkinson's disease (PD). Theoretically, such a protective effect might derive from (a) a levodopa sparing effect, (b) stimulation dopamine autoreceptors resulting ...

    journal_title:Annals of neurology

    pub_type: 杂志文章,评审


    authors: Olanow CW,Jenner P,Brooks D

    更新日期:1998-09-01 00:00:00

  • A mutant PSEN1 causes dementia with Lewy bodies and variant Alzheimer's disease.

    abstract::We report early-onset parkinsonism and dementia of 18 years' duration in a 52-year-old man whose grandfather and father had suffered from a similar neurological disease. In this patient, we found neuronal loss in various brain regions including the substantia nigra and cerebral cortex, Lewy bodies, cotton wool plaques...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Ishikawa A,Piao YS,Miyashita A,Kuwano R,Onodera O,Ohtake H,Suzuki M,Nishizawa M,Takahashi H

    更新日期:2005-03-01 00:00:00

  • Cerebral blood flow following normovolemic hemodilution in patients with high hematocrit.

    abstract::The effects on cerebral hemodynamics of venisection and a 4% albumin-saline infusion were studied in six patients with high hematocrit (mean, 51.5%). Cerebral blood flow (CBF) was measured using the xenon 133 intracarotid injection method. Blood gases were measured in arterial and jugular venous blood. Rapid two-stage...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Henriksen L,Paulson OB,Smith RJ

    更新日期:1981-05-01 00:00:00

  • Tau reduction prevents disease in a mouse model of Dravet syndrome.

    abstract:OBJECTIVE:Reducing levels of the microtubule-associated protein tau has shown promise as a potential treatment strategy for diseases with secondary epileptic features such as Alzheimer disease. We wanted to determine whether tau reduction may also be of benefit in intractable genetic epilepsies. METHODS:We studied a m...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Gheyara AL,Ponnusamy R,Djukic B,Craft RJ,Ho K,Guo W,Finucane MM,Sanchez PE,Mucke L

    更新日期:2014-09-01 00:00:00

  • Heat opens axon initial segment sodium channels: a febrile seizure mechanism?

    abstract:OBJECTIVE:A number of hypotheses have been put forward as to why humans respond to fever by seizing. The current leading hypotheses are that respiratory alkalosis produces an as yet unidentified change in neural excitability or that inflammatory mediators potentiate excitatory synaptic transmission. However, it is well...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Thomas EA,Hawkins RJ,Richards KL,Xu R,Gazina EV,Petrou S

    更新日期:2009-08-01 00:00:00

  • Strategic Considerations for Applying the Flipped Classroom to Neurology Education.

    abstract::Nowadays, the "flipped classroom" approach is taking the center stage within medical education. However, very few reports on the implementation of the flipped classroom in neurology have been published to date, and this educational model still represents a challenge for students and educators alike. In this article, n...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Sandrone S,Berthaud JV,Carlson C,Cios J,Dixit N,Farheen A,Kraker J,Owens JWM,Patino G,Sarva H,Weber D,Schneider LD

    更新日期:2020-01-01 00:00:00

  • Environmental enrichment slows disease progression in R6/2 Huntington's disease mice.

    abstract::Huntington's disease is a genetic disorder that causes motor dysfunction, personality changes, dementia, and premature death. There is currently no effective therapy. Several transgenic models of Huntington's disease are available, the most widely used of which is the R6/2 mouse, because of its rapid disease progressi...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Hockly E,Cordery PM,Woodman B,Mahal A,van Dellen A,Blakemore C,Lewis CM,Hannan AJ,Bates GP

    更新日期:2002-02-01 00:00:00

  • Surgically correctable lesions solely detected by CT scan in adult-onset chronic epilepsy.

    abstract::A prospective study of 162 patients with adult-onset chronic epilepsy revealed 4 in whom computed tomographic scanning was the only diagnostic test that detected the presence of a surgically correctable cerebral lesion. These patients had suffered from seizures for 1 to 14 years and at the time of operation demonstrat...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Jabbari B,Huott AD,Di Chiro G,Martins AN,Youngblood LA,Harper MG

    更新日期:1980-04-01 00:00:00

  • Mitochondrial dysfunction in Parkinson's disease.

    abstract::This review discusses the etiology and pathogenesis of Parkinson's disease (PD). Mitochondrial respiratory failure and oxidative stress appear to be two major contributors to nigral neuronal death in PD. Complex I deficiency has been reported by several groups and appears to be one of the basic abnormalities responsib...

    journal_title:Annals of neurology

    pub_type: 杂志文章,评审


    authors: Mizuno Y,Yoshino H,Ikebe S,Hattori N,Kobayashi T,Shimoda-Matsubayashi S,Matsumine H,Kondo T

    更新日期:1998-09-01 00:00:00

  • Synergistic immunomodulatory effects of interferon-beta1b and the phosphodiesterase inhibitor pentoxifylline in patients with relapsing-remitting multiple sclerosis.

    abstract::Subcutaneous application of interferon-beta1b (IFN-beta1b) is an established therapy for patients with relapsing-remitting multiple sclerosis (RRMS), but early side effects are still a major concern. In vitro studies with myelin basic protein (MBP)-specific T-cell lines revealed a synergistic suppressive effect of IFN...

    journal_title:Annals of neurology

    pub_type: 临床试验,杂志文章


    authors: Weber F,Polak T,Günther A,Kubuschok B,Janovskaja J,Bitsch A,Poser S,Rieckmann P

    更新日期:1998-07-01 00:00:00

  • Demyelination affects temporal aspects of perception: an optic neuritis study.

    abstract:OBJECTIVE:Visual Evoked Potentials (VEPs) following optic neuritis (ON) remain chronically prolonged, although standard visual tests indicate full recovery. We hypothesized that dynamic visual processes, such as motion perception, may be more vulnerable to slowed conduction in the optic nerve, and consequently be bette...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Raz N,Dotan S,Chokron S,Ben-Hur T,Levin N

    更新日期:2012-04-01 00:00:00

  • Polygenic hazard scores in preclinical Alzheimer disease.

    abstract::Identifying asymptomatic older individuals at elevated risk for developing Alzheimer disease (AD) is of clinical importance. Among 1,081 asymptomatic older adults, a recently validated polygenic hazard score (PHS) significantly predicted time to AD dementia and steeper longitudinal cognitive decline, even after contro...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Tan CH,Hyman BT,Tan JJX,Hess CP,Dillon WP,Schellenberg GD,Besser LM,Kukull WA,Kauppi K,McEvoy LK,Andreassen OA,Dale AM,Fan CC,Desikan RS

    更新日期:2017-09-01 00:00:00

  • Alexander's law: its behavior and origin in the human vestibulo-ocular reflex.

    abstract::Alexander's law refers to the phenomenon in which the spontaneous nystagmus of a patient with a vestibular lesion is more intense when the patient looks in the quick-phase than in the slow-phase direction. Alexander's law was investigated in normal subjects as well as patients with vestibular lesions. During sinusoida...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Robinson DA,Zee DS,Hain TC,Holmes A,Rosenberg LF

    更新日期:1984-12-01 00:00:00

  • Post-Traumatic Stress Disorder is Associated with further Increased Parkinson's Disease Risk in Veterans with Traumatic Brain Injury.

    abstract:OBJECTIVE:Determining if traumatic brain injury (TBI) and post-traumatic stress disorder (PTSD) are risk factors for Parkinson's disease (PD). This constitutes a research priority for the Veterans Administration (VA) with implications for screening policy and prevention. METHODS:Population-based, matched case-control ...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: White DL,Kunik ME,Yu H,Lin HL,Richardson PA,Moore S,Sarwar AI,Marsh L,Jorge RE

    更新日期:2020-07-01 00:00:00

  • Cutaneous allodynia in the migraine population.

    abstract:OBJECTIVE:To develop and validate a questionnaire for assessing cutaneous allodynia (CA), and to estimate the prevalence and severity of CA in the migraine population. METHODS:Migraineurs (n = 11,388) completed the Allodynia Symptom Checklist, assessing the frequency of allodynia symptoms during headache. Response opt...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Lipton RB,Bigal ME,Ashina S,Burstein R,Silberstein S,Reed ML,Serrano D,Stewart WF,American Migraine Prevalence Prevention Advisory Group.

    更新日期:2008-02-01 00:00:00

  • Identification of neuronal outgrowth cells from peripheral blood of stroke patients.

    abstract:OBJECTIVE:Recent studies have identified a subset of outgrowth cell population with endothelial phenotype in long-term cultures of peripheral blood mononuclear cells. The concept that peripheral blood-derived cells participate in neuronal regeneration remains highly controversial, and no specific cell type has been ide...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Jung KH,Chu K,Lee ST,Song EC,Sinn DI,Kim JM,Kim SJ,Kim JH,Kang KM,Park HK,Lee SH,Kim M,Lee SK,Roh JK

    更新日期:2008-03-01 00:00:00

  • Site of autonomic deficit in harlequin syndrome: local autonomic failure affecting the arm and the face.

    abstract::The site of autonomic deficit was investigated in 4 patients with loss of thermoregulatory flushing and sweating on one side of the face but no associated miosis (harlequin syndrome). In 2 patients the sudomotor deficit included the hand. Signs of postganglionic sympathetic deficit included pathological lacrimal sweat...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Drummond PD,Lance JW

    更新日期:1993-12-01 00:00:00

  • Penumbral tissue alkalosis in focal cerebral ischemia: relationship to energy metabolism, blood flow, and steady potential.

    abstract::The effect of focal ischemia on tissue pH was studied at various times up to 6 hours after permanent middle cerebral artery occlusion in rats. Tissue pH was imaged by using umbelliferone fluorescence and correlated with cerebral blood flow, ATP content, and recordings of the steady potential. Circumscribed foci of all...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Back T,Hoehn M,Mies G,Busch E,Schmitz B,Kohno K,Hossmann KA

    更新日期:2000-04-01 00:00:00

  • The localizing value of depth electroencephalography in 32 patients with refractory epilepsy.

    abstract::Clinical seizure manifestations, physical examination, radiological studies, neuropsychological tests, and scalp and depth electroencephalographic (EEG) studies were done to localize seizure foci in 32 patients, 23 of whom have undergone surgery with more than one year of follow-up. Of 16 patients with unlocalized sca...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Spencer SS,Spencer DD,Williamson PD,Mattson RH

    更新日期:1982-09-01 00:00:00

  • Pure hemidystonia with basal ganglion abnormalities on positron emission tomography.

    abstract::We present a patient with hemidystonia and an abnormality of the contralateral basal ganglion seen only with positron emission tomography. A 50-year-old sinistral man suffered minor trauma to the right side of his head and neck. Within 20 minutes he developed paroxysmal intermittent dystonic posturing of his right fac...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Perlmutter JS,Raichle ME

    更新日期:1984-03-01 00:00:00

  • Subcomplexes of human ATP synthase mark mitochondrial biosynthesis disorders.

    abstract:OBJECTIVE: METHODS:We describe biochemically and clinically relevant aspects of mitochondrial ATP synthase, the enzyme that supplies most ATP for the cells energy demand. RESULTS:Analyzing human Rho zero cells we could identify three subcomplexes of ATP synthase: F1 catalytic domain, F1 domain with bound natural IF1 ...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Carrozzo R,Wittig I,Santorelli FM,Bertini E,Hofmann S,Brandt U,Schägger H

    更新日期:2006-02-01 00:00:00

  • Molecular genetic studies of muscle lactate dehydrogenase deficiency in white patients.

    abstract::We identified two new mutations in 2 white patients with muscle lactate dehydrogenase deficiency. Both patients had exercise intolerance, cramps, and recurrent myoglobinuria. One patient was homozygous for a 2-bp deletion in exon 5, resulting in a frameshift with premature termination of translation. The second patien...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Tsujino S,Shanske S,Brownell AK,Haller RG,DiMauro S

    更新日期:1994-10-01 00:00:00