Proton magnetic resonance spectroscopic imaging in patients with cerebellar degeneration.


:Using proton magnetic resonance spectroscopic imaging, we studied the cerebellum of 9 patients with cerebellar degeneration and of 9 age-matched normal control subjects. This technique permits the simultaneous measurement of N-acetylaspartate, choline-containing compounds, creatine/phosphocreatine, and lactate signal intensities from four 15-mm slices divided into 0.84-ml single-volume elements. Because patients with cerebellar degeneration often show substantial atrophy on magnetic resonance imaging (MRI), we specifically chose to analyze the spectroscopic signals only from tissue that did not have an atrophic appearance on the MRI. The spectroscopic findings showed a significant reduction of N-acetylaspartate in all parts of the cerebellum, a significant correlation with MRI scores of cerebellar atrophy, and a significant correlation with clinical rating scores of cerebellar disturbance. Our method of analysis suggests the presence of a neurodegenerative process in cerebellar areas that do not appear to be atrophic on the MRI. Some limitations of proton magnetic resonance spectroscopic imaging in the present study were related to the partial field inhomogeneity characteristics of the posterior fossa, the anatomical location of the cerebellum, and the particularly severe cerebellar atrophy in some of the patients.


Ann Neurol


Annals of neurology


Tedeschi G,Bertolino A,Massaquoi SG,Campbell G,Patronas NJ,Bonavita S,Barnett AS,Alger JR,Hallett M




Has Abstract


1996-01-01 00:00:00












  • Multiple mutations in the GALC gene in a patient with adult-onset Krabbe disease.

    abstract::A 53-year-old man was diagnosed 8 years earlier with globoid cell leukodystrophy (GLD, Krabbe disease) by his severe deficiency of galactocerebrosidase (GALC) activity. He was found to have eight nucleotide changes on the two copies of his GALC gene, including two in the leader sequence, four considered polymorphisms,...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Luzi P,Rafi MA,Wenger DA

    更新日期:1996-07-01 00:00:00

  • Brachial plexus involvement in familial pressure-sensitive neuropathy: electrophysiological and morphological findings.

    abstract::Two family members with hereditary pressure-sensitive neuropathy are reported. One patient presented atypically with acute brachial plexus neuropathy following transaxillary removal of the first rib. Electrophysiological studies showed slowing of motor nerve conduction in clinically affected and unaffected nerves. In ...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Bosch EP,Chui HC,Martin MA,Cancilla PA

    更新日期:1980-12-01 00:00:00

  • Mitochondrial abnormalities in Alzheimer brain: mechanistic implications.

    abstract::Reductions in cerebral metabolism sufficient to impair cognition in normal individuals also occur in Alzheimer's disease (AD). The degree of clinical disability in AD correlates closely to the magnitude of the reduction in brain metabolism. Therefore, we tested whether impairments in tricarboxylic acid (TCA) cycle enz...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Bubber P,Haroutunian V,Fisch G,Blass JP,Gibson GE

    更新日期:2005-05-01 00:00:00

  • Developmental dyslexia in women: neuropathological findings in three patients.

    abstract::Brains from male cases with dyslexia show symmetry of the planum temporale and predominantly left-sided cerebrocortical microdysgenesis. We now report on three women with dyslexia. In all brains, the planum temporale was again symmetrical. Also, in two of the brains, multiple foci of cerebrocortical glial scarring wer...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Humphreys P,Kaufmann WE,Galaburda AM

    更新日期:1990-12-01 00:00:00

  • Organ-specific autoantigens induce transforming growth factor-beta mRNA expression in mononuclear cells in multiple sclerosis and myasthenia gravis.

    abstract::Multiple sclerosis (MS) is characterized by patchy accumulations of inflammatory cells combined with demyelination. There are mononuclear cells in blood and cerebrospinal fluid of patients with MS that produce interferon-gamma and interleukin-4 in response to myelin basic protein (MBP) and proteolipid protein (PLP). H...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Link J,Fredrikson S,Söderström M,Olsson T,Höjeberg B,Ljungdahl A,Link H

    更新日期:1994-02-01 00:00:00

  • Selective neuronal nitric oxide synthase inhibitors and the prevention of cerebral palsy.

    abstract:OBJECTIVE:To design a new class of selective neuronal nitric oxide synthase (NOS) inhibitors, and demonstrate that administration in a rabbit model for cerebral palsy (CP) prevents hypoxia-ischemia-induced deaths and reduces the number of newborn kits exhibiting signs of CP. METHODS:We used a novel computer-based drug...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Ji H,Tan S,Igarashi J,Li H,Derrick M,Martásek P,Roman LJ,Vásquez-Vivar J,Poulos TL,Silverman RB

    更新日期:2009-02-01 00:00:00

  • Outcome of neonatal intraventricular hemorrhage with periventricular echodense lesions.

    abstract::The incidence of periventricular-intraventricular hemorrhage (PV-IVH) in a group of 460 preterm infants with birth weight less than 2,250 gm, studied by cranial ultrasonography, was 39%. Sixty-four (36%) of the infants with periventricular-intraventricular hemorrhage had, in addition, periventricular intraparenchymal ...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: McMenamin JB,Shackelford GD,Volpe JJ

    更新日期:1984-03-01 00:00:00

  • Hereditary ceruloplasmin deficiency with hemosiderosis: a clinicopathological study of a Japanese family.

    abstract::A hereditary ceruloplasmin deficiency associated with severe iron deposition in visceral organ and brain tissues found on histopathological examination at autopsy is discussed. Three siblings of consanguineous Japanese parents were studied. Their clinical symptoms were progressive dementia, extrapyramidal disorders, c...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Morita H,Ikeda S,Yamamoto K,Morita S,Yoshida K,Nomoto S,Kato M,Yanagisawa N

    更新日期:1995-05-01 00:00:00

  • No evidence for genetic association or linkage of the cathepsin D (CTSD) exon 2 polymorphism and Alzheimer disease.

    abstract::Two recent case-control studies have suggested a strong association of a missense polymorphism in exon 2 of the cathepsin D gene (CTSD) and Alzheimer disease (AD). However, these findings were not confirmed in another independent study. We analyzed this polymorphism in two large and independent AD study populations an...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Bertram L,Guénette S,Jones J,Keeney D,Mullin K,Crystal A,Basu S,Yhu S,Deng A,Rebeck GW,Hyman BT,Go R,McInnis M,Blacker D,Tanzi R

    更新日期:2001-01-01 00:00:00

  • TigarB causes mitochondrial dysfunction and neuronal loss in PINK1 deficiency.

    abstract:OBJECTIVE:Loss of function mutations in PINK1 typically lead to early onset Parkinson disease (PD). Zebrafish (Danio rerio) are emerging as a powerful new vertebrate model to study neurodegenerative diseases. We used a pink1 mutant (pink(-/-) ) zebrafish line with a premature stop mutation (Y431*) in the PINK1 kinase d...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Flinn LJ,Keatinge M,Bretaud S,Mortiboys H,Matsui H,De Felice E,Woodroof HI,Brown L,McTighe A,Soellner R,Allen CE,Heath PR,Milo M,Muqit MM,Reichert AS,Köster RW,Ingham PW,Bandmann O

    更新日期:2013-12-01 00:00:00

  • Narcolepsy onset is seasonal and increased following the 2009 H1N1 pandemic in China.

    abstract:OBJECTIVE:Narcolepsy is caused by the loss of hypocretin/orexin neurons in the hypothalamus, which is likely the result of an autoimmune process. Recently, concern has been raised over reports of narcolepsy in northern Europe following H1N1 vaccination. METHODS:The study is a retrospective analysis of narcolepsy onset...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Han F,Lin L,Warby SC,Faraco J,Li J,Dong SX,An P,Zhao L,Wang LH,Li QY,Yan H,Gao ZC,Yuan Y,Strohl KP,Mignot E

    更新日期:2011-09-01 00:00:00

  • Passive joint position sense after total hip replacement surgery.

    abstract::Passive joint position sense was tested in 10 subjects after unilateral total hip replacement surgery (which included capsulectomy). Varied initial limb position, amplitude, and velocity of movement were used. The unoperated side was used as a control. Statistically barely significant errors (p less than 0.025) on the...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Karanjia PN,Ferguson JH

    更新日期:1983-06-01 00:00:00

  • Comparative cranial CT enhancement in a primate model of cerebral infarction.

    abstract::The value of various enhancement techniques as opposed to nonenhanced CT scanning was compared in 15 baboons with cerebral infarction secondary to embolization of the left middle cerebral artery. The most prominent CT findings in infarction included an area of low absorption in the opercula--basal ganglia--centrum sem...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Drayer BP,Dujovny M,Wolfson SK Jr,Boehnke M,Cook EE,Rosenbaum AE

    更新日期:1979-01-01 00:00:00

  • Gut Microbiome Signatures of Risk and Prodromal Markers of Parkinson Disease.

    abstract:OBJECTIVE:Alterations of the gut microbiome in Parkinson disease (PD) have been repeatedly demonstrated. However, little is known about whether such alterations precede disease onset and how they relate to risk and prodromal markers of PD. We investigated associations of these features with gut microbiome composition. ...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Heinzel S,Aho VTE,Suenkel U,von Thaler AK,Schulte C,Deuschle C,Paulin L,Hantunen S,Brockmann K,Eschweiler GW,Maetzler W,Berg D,Auvinen P,Scheperjans F

    更新日期:2020-08-01 00:00:00

  • Neuronal migration abnormality in peroxisomal bifunctional enzyme defect.

    abstract::Patterns of brain dysgenesis that resemble those in the Zellweger syndrome were demonstrated in a boy with an isolated defect of the peroxisomal bifunctional enzyme. There was bilateral centrosylvian pachygyria and polymicrogyria, diffuse hemispheric hypomyelination with heterotopic neurons, Purkinje cell heterotopias...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Kaufmann WE,Theda C,Naidu S,Watkins PA,Moser AB,Moser HW

    更新日期:1996-02-01 00:00:00

  • Clinical and genetic spectrum of pyruvate dehydrogenase deficiency: dihydrolipoamide acetyltransferase (E2) deficiency.

    abstract::Pyruvate dehydrogenase deficiency is a major cause of primary lactic acidosis and neurological dysfunction in infancy and early childhood. Most cases are caused by mutations in the X-linked gene for the E1alpha subunit of the complex. Mutations in DLAT, the gene encoding dihydrolipoamide acetyltransferase, the E2 core...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Head RA,Brown RM,Zolkipli Z,Shahdadpuri R,King MD,Clayton PT,Brown GK

    更新日期:2005-08-01 00:00:00

  • No evidence for a role of rare CYP27B1 functional variations in multiple sclerosis.

    abstract::Association studies have implicated common variants in the 12q14.1 region containing CYP27B1 in multiple sclerosis (MS). Rare CYP27B1 mutations cause autosomal recessive vitamin D-dependent rickets type 1, and it has recently been reported that heterozygous CYP27B1 mutations are associated with increased MS susceptibi...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Barizzone N,Pauwels I,Luciano B,Franckaert D,Guerini FR,Cosemans L,Hilven K,Salviati A,Dooley J,Danso-Abeam D,di Sapio A,Cavalla P,Decallonne B,Mathieu C,Liston A,Leone M,Dubois B,D'Alfonso S,Goris A

    更新日期:2013-03-01 00:00:00

  • Short- and long-term survival and function of unilateral intrastriatal dopaminergic grafts in Parkinson's disease.

    abstract::Six patients with Parkinson's disease were followed for 10 to 72 months after human embryonic mesencephalic tissue from four to seven donors was grafted unilaterally into the putamen (4 patients) or putamen plus caudate (2 patients). After 8 to 12 months, positron emission tomography showed a 68% increase of 6-L-[18F]...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Wenning GK,Odin P,Morrish P,Rehncrona S,Widner H,Brundin P,Rothwell JC,Brown R,Gustavii B,Hagell P,Jahanshahi M,Sawle G,Björklund A,Brooks DJ,Marsden CD,Quinn NP,Lindvall O

    更新日期:1997-07-01 00:00:00

  • Somatotopical organization of striatal activation during finger and toe movement: a 3-T functional magnetic resonance imaging study.

    abstract::The present study aimed at determining the distribution and somatotopical organization of striatal activation during performance of simple motor tasks. Ten right-handed healthy volunteers were studied by using a 3-T whole-body magnetic resonance unit and echo planar imaging. The tasks consisted of self-paced flexion/e...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Lehéricy S,van de Moortele PF,Lobel E,Paradis AL,Vidailhet M,Frouin V,Neveu P,Agid Y,Marsault C,Le Bihan D

    更新日期:1998-09-01 00:00:00

  • Anticerebellar antibodies in serum and cerebrospinal fluid of a patient with oat cell carcinoma of the lung and paraneoplastic cerebellar degeneration.

    abstract::A 56-year-old man was seen with subacute cerebellar degeneration and was found to have oat cell carcinoma of the lung. Antibodies to cerebellar Purkinje cells and granule cells were detected in both serum and cerebrospinal fluid (CSF), and intrathecal antibody synthesis was suggested by serum CSF antibody ratios, CSF ...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Greenlee JE,Lipton HL

    更新日期:1986-01-01 00:00:00

  • Evidence-based path to newborn screening for Duchenne muscular dystrophy.

    abstract:OBJECTIVE:Creatine kinase (CK) levels are increased on dried blood spots in newborns related to the birthing process. As a marker for newborn screening, CK in Duchenne muscular dystrophy (DMD) results in false-positive testing. In this report, we introduce a 2-tier system using the dried blood spot to first assess CK w...

    journal_title:Annals of neurology

    pub_type: 杂志文章,多中心研究


    authors: Mendell JR,Shilling C,Leslie ND,Flanigan KM,al-Dahhak R,Gastier-Foster J,Kneile K,Dunn DM,Duval B,Aoyagi A,Hamil C,Mahmoud M,Roush K,Bird L,Rankin C,Lilly H,Street N,Chandrasekar R,Weiss RB

    更新日期:2012-03-01 00:00:00

  • Dopa-responsive parkinsonism phenotype of Machado-Joseph disease: confirmation of 14q CAG expansion.

    abstract::The subtype IV of Machado-Joseph disease (MJD), characterized by parkinsonism variably combined with ataxia, distal atrophy, and sensory loss, has been all but ignored in recent reports of MJD, including those describing the molecular biologic substrate of the disease. We have demonstrated expansion of the CAG trinucl...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Tuite PJ,Rogaeva EA,St George-Hyslop PH,Lang AE

    更新日期:1995-10-01 00:00:00

  • Coenzyme Q10 levels correlate with the activities of complexes I and II/III in mitochondria from parkinsonian and nonparkinsonian subjects.

    abstract::The activities of complex I and complex II/III in platelet mitochondria are reduced in patients with early, untreated Parkinson's disease. Coenzyme Q10 is the electron acceptor for complex I and complex II. We found that the level of coenzyme Q10 was significantly lower in mitochondria from parkinsonian patients than ...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Shults CW,Haas RH,Passov D,Beal MF

    更新日期:1997-08-01 00:00:00

  • Descending paralysis resulting from occult wound botulism.

    abstract::A 30-year-old male drug abuser developed ophthalmoplegia, bulbar paralysis, and limb weakness responsive to edrophonium. However, potentiation of a low-amplitude evoked muscle action potential was produced with repetitive nerve stimulation at 10 Hz, and the clinical and electrophysiological data suggested the diagnosi...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Rapoport S,Watkins PB

    更新日期:1984-09-01 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

  • Retrograde reactions of Clarke's nucleus neurons after human spinal cord injury.

    abstract::Successful axon regeneration depends on the expression of regeneration-associated genes by axotomized neurons. Here, we demonstrate, for the first time to our knowledge, the expression of regeneration-associated genes by axotomized human CNS neurons. In situ hybridization and immunohistochemistry showed a transient in...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Schmitt AB,Breuer S,Polat L,Pech K,Kakulas B,Love S,Martin D,Schoenen J,Noth J,Brook GA

    更新日期:2003-10-01 00:00:00

  • Association of IgM type anti-GM1 antibodies and muscle strength in chronic acquired demyelinating polyneuropathy.

    abstract::The pathogenetic role of anti-GM1 in chronic acquired demyelinating polyneuropathy (CADP) is uncertain. An association between antibodies and disease activity has not yet been established. In 8 patients with CADP followed longitudinally, anti-GM1 antibodies were monitored with a standardized enzyme-linked immunosorben...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Bech E,Andersen H,Orntoft TF,Jakobsen J

    更新日期:1998-01-01 00:00:00

  • Human immunodeficiency virus (HIV)-associated myopathy: immunocytochemical identification of an HIV antigen (gp 41) in muscle macrophages.

    abstract::In a patient with acquired immunodeficiency disease syndrome (AIDS) and muscle weakness, a muscle biopsy specimen disclosed degeneration of muscle fibers, regeneration, and focal endomysial mononuclear inflammation. A conspicuous feature was the presence of perivascular macrophages within the endomysium that showed po...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Chad DA,Smith TW,Blumenfeld A,Fairchild PG,DeGirolami U

    更新日期:1990-10-01 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

  • Postmortem observations on beta-glucuronidase deficiency presenting as hydrops fetalis.

    abstract::This study reports a case of type VII mucopolysaccharidosis (beta-glucuronidase deficiency) presenting as lethal hydrops fetalis. Skin fibroblast cultures established postmortem revealed deficient beta-glucuronidase activity. Mucopolysaccharides were stored in various cells of the brain, heart, kidney, liver, and sple...

    journal_title:Annals of neurology

    pub_type: 杂志文章


    authors: Irani D,Kim HS,El-Hibri H,Dutton RV,Beaudet A,Armstrong D

    更新日期:1983-10-01 00:00:00