Meningioma presenting with Cushing's syndrome: an unusual clinical presentation.

abstract：OBJECTIVE:The polyglutamine diseases, including Huntington's disease (HD) and multiple spinocerebellar ataxias (SCAs), are among the commonest hereditary neurodegenerative diseases. They are caused by expanded CAG tracts, encoding glutamine, in different genes. Longer CAG repeat tracts are associated with earlier ages ...

journal_title：Annals of neurology

authors： Bettencourt C,Hensman-Moss D,Flower M,Wiethoff S,Brice A,Goizet C,Stevanin G,Koutsis G,Karadima G,Panas M,Yescas-Gómez P,García-Velázquez LE,Alonso-Vilatela ME,Lima M,Raposo M,Traynor B,Sweeney M,Wood N,Giunti P,SPA

更新日期：2016-06-01 00:00:00

abstract：:A 51-year-old man with non-HLA-DR2 histocompatibility developed classic signs and symptoms of the narcoleptic tetrad soon after recovering from an episode of cardiopulmonary insufficiency, which occurred during induction of surgical anesthesia. Symptoms included excessive daytime sleepiness, hypnagogic hallucinations,...

journal_title：Annals of neurology

authors： Rivera VM,Meyer JS,Hata T,Ishikawa Y,Imai A

更新日期：1986-05-01 00:00:00

abstract：:The term Charcot-Wilbrand syndrome (CWS) denotes dream loss following focal brain damage. We report the first case of CWS, in whom neuropsychological functions, extension of the underlying lesion, and sleep architecture changes were assessed. A 73-year-old woman reported a total dream loss after acute, bilateral occip...

journal_title：Annals of neurology

authors： Bischof M,Bassetti CL

更新日期：2004-10-01 00:00:00

abstract：:A unique phenotype of Waardenburg-Hirschsprung disease (WS4) accompanied by peripheral neuropathy and central dysmyelination has been recognized recently in association with SOX10 mutations. We report an infant boy with lethal congenital hypomyelinating neuropathy and WS4 who had a heterozygous SOX10 mutation (Q250X)....

journal_title：Annals of neurology

authors： Inoue K,Shilo K,Boerkoel CF,Crowe C,Sawady J,Lupski JR,Agamanolis DP

更新日期：2002-12-01 00:00:00

abstract：:We studied local cerebral blood flow, as measured by autoradiography with digital image processing and by tissue morphology, in six rats 4 hours after occlusion of the proximal middle cerebral artery. A consistent, three-dimensional pattern of graded reductions in local cerebral blood flow involved the affected hemisp...

journal_title：Annals of neurology

authors： Tyson GW,Teasdale GM,Graham DI,McCulloch J

更新日期：1984-06-01 00:00:00

abstract：:Dopa-responsive dystonia (DRD) is one form of childhood-onset idiopathic torsion dystonia. Adult-onset parkinsonism has appeared in several previously unaffected members in families with DRD suggesting that this may be an additional phenotypical expression of the disease. We report a family with DRD in which 2 women a...

journal_title：Annals of neurology

authors： Nygaard TG,Takahashi H,Heiman GA,Snow BJ,Fahn S,Calne DB

更新日期：1992-11-01 00:00:00

abstract：OBJECTIVE:Seizure at onset (SaO) has been considered a relative contraindication for intravenous thrombolysis (IVT) in patients with acute ischemic stroke, although this appraisal is not evidence based. Here, we investigated the prognostic significance of SaO in patients treated with IVT for suspected ischemic stroke. ...

journal_title：Annals of neurology

authors： Polymeris AA,Curtze S,Erdur H,Hametner C,Heldner MR,Groot AE,Zini A,Béjot Y,Dietrich A,Martinez-Majander N,von Rennenberg R,Gumbinger C,Schaedelin S,De Marchis GM,Thilemann S,Traenka C,Lyrer PA,Bonati LH,Wegener S,R

更新日期：2019-11-01 00:00:00

abstract：OBJECTIVE:To determine the clinical consequences of the PMP22 point mutation, T118M, which has been previously considered to either cause an autosomal recessive form of Charcot-Marie-Tooth (CMT) disease or be a benign polymorphism. METHODS:We analyzed patients from five separate kindreds and characterized their periph...

journal_title：Annals of neurology

authors： Shy ME,Scavina MT,Clark A,Krajewski KM,Li J,Kamholz J,Kolodny E,Szigeti K,Fischer RA,Saifi GM,Scherer SS,Lupski JR

更新日期：2006-02-01 00:00:00

abstract：:B cells have long played an enigmatic role in the scenario of multiple sclerosis pathogenesis. This review summarizes recent progress in our understanding of B-cell trafficking, survival, and differentiation in the central nervous system (CNS). We propose four possible routes of intrathecal immunoglobulin-producing ce...

journal_title：Annals of neurology

authors： Meinl E,Krumbholz M,Hohlfeld R

更新日期：2006-06-01 00:00:00

abstract：:To evaluate reports of abnormal levels of free amino acids (AA) in patients with amyotrophic lateral sclerosis (ALS), we studied serum, cerebrospinal fluid, and urine AA in 12 patients with ALS and 12 controls matched for age, sex, and severity of disability. ALS patients had statistically significant elevations in se...

journal_title：Annals of neurology

authors： Patten BM,Harati Y,Acosta L,Jung SS,Felmus MT

更新日期：1978-04-01 00:00:00

abstract：:Guillain-Barré syndrome (GBS) is an immune-mediated neuropathy, in which leukocytes and humoral components of the immune system proposedly initiate localized inflammation. An important pathogenic role for anti-GM1 ganglioside antibodies has been suggested. Therefore, we evaluated anti-GM1 IgG antibody-induced leukocyt...

journal_title：Annals of neurology

authors： van Sorge NM,van den Berg LH,Geleijns K,van Strijp JA,Jacobs BC,van Doorn PA,Wokke JH,van de Winkel JG,Leusen JH,van der Pol WL

更新日期：2003-05-01 00:00:00

abstract：:Mutations in AbetaPP cause deposition of Abeta amyloid fibrils in brain parenchyma and cerebral vessels, resulting in Alzheimer's disease (AD) and/or cerebral amyloid angiopathy (CAA). We report a novel mutation (L705V) within the Abeta sequence of AbetaPP in a family with autosomal dominant, recurrent intracerebral h...

journal_title：Annals of neurology

authors： Obici L,Demarchi A,de Rosa G,Bellotti V,Marciano S,Donadei S,Arbustini E,Palladini G,Diegoli M,Genovese E,Ferrari G,Coverlizza S,Merlini G

更新日期：2005-10-01 00:00:00

abstract：:Alterations of nodal and paranodal axolemma of the rat sciatic nerve were investigated in antigalactocerebroside serum-induced demyelination. A ferric ion-ferrocyanide (FeFCN) stain that appears to stain the regions with a high sodium channel density in nerve fibers was applied. When acute conduction block was initiat...

journal_title：Annals of neurology

authors： Saida K,Saida T,Kayama H,Nishitani H

更新日期：1984-06-01 00:00:00

abstract：OBJECTIVE:Cognitive impairment is common in epilepsy, particularly in memory function. Interictal spikes (IISs) are thought to disrupt cognition, but it is difficult to delineate their contribution from general impairments in memory produced by etiology and seizures. We investigated the transient impact of focal IISs o...

journal_title：Annals of neurology

authors： Kleen JK,Scott RC,Holmes GL,Lenck-Santini PP

更新日期：2010-02-01 00:00:00

abstract：:Four infants had bilateral hippocampal sclerosis by magnetic resonance scans, including oblique coronal fast spin echo images of the temporal lobes; [18F]fluorodeoxyglucose-positron emission tomographic scans, done in 2 infants, showed isolated bilateral anterior temporal lobe hypometabolism. All had epilepsy with epi...

journal_title：Annals of neurology

authors： DeLong GR,Heinz ER

更新日期：1997-07-01 00:00:00

abstract：:Splice-site and missense mutations have been identified in tau associated with frontotemporal dementia with parkinsonism linked to chromosome 17. In this study we assessed the genetic contribution of tau mutations to three patient series with non-Alzheimer's (non-AD) degenerative dementia. The groups included (1) a co...

journal_title：Annals of neurology

authors： Houlden H,Baker M,Adamson J,Grover A,Waring S,Dickson D,Lynch T,Boeve B,Petersen RC,Pickering-Brown S,Owen F,Neary D,Craufurd D,Snowden J,Mann D,Hutton M

更新日期：1999-08-01 00:00:00

abstract：:The objective of this study was to assess which features of temporal lobe proton magnetic resonance spectroscopic imaging (1H-MRSI) are associated with satisfactory surgical outcome in patients with intractable temporal lobe epilepsy and bilateral hippocampal atrophy. We studied 21 patients with intractable temporal l...

journal_title：Annals of neurology

authors： Li LM,Cendes F,Antel SB,Andermann F,Serles W,Dubeau F,Olivier A,Arnold DL

更新日期：2000-02-01 00:00:00

abstract：:Malignant tumors are known to have a "remote" or nonmetastatic effect on the central and peripheral nervous systems. Eight patients were seen with proximal muscle weakness in association with bronchogenic carcinoma (5), carcinoma of breast (2), and leukemia (1). Electromyography demonstrated small polyphasic motor uni...

journal_title：Annals of neurology

authors： Barron SA,Heffner RR Jr

更新日期：1978-09-01 00:00:00

abstract：:The integrity of the hypothalamic neuroendocrine dopaminergic neurons in Parkinson's disease was assessed by comparing the numbers and distribution of melanin-pigmented neurons in the arcuate and periventricular nuclei in 7 parkinsonian and 5 normal brains. No significant differences were observed. In contrast to theo...

journal_title：Annals of neurology

authors： Matzuk MM,Saper CB

更新日期：1985-11-01 00:00:00

abstract：OBJECTIVE:Migraine with aura is a severe debilitating neurological disorder with few relatively specific therapeutic options. METHODS:We used amiloride, a blocker of epithelial sodium channels, to evaluate its pharmacological potential and explored the biology of a potential mechanism of action in well-established exp...

journal_title：Annals of neurology

authors： Holland PR,Akerman S,Andreou AP,Karsan N,Wemmie JA,Goadsby PJ

更新日期：2012-10-01 00:00:00

abstract：:Regional patterns of cerebral glucose utilization (rCMRglc) and blood flow (rCBF) were examined in the early recovery period following transient forebrain ischemia in order to correlate early postischemic physiological events with regionally selective patterns of ischemic neuropathology. Wistar rats were subjected to ...

journal_title：Annals of neurology

authors： Ginsberg MD,Graham DI,Busto R

更新日期：1985-10-01 00:00:00

abstract：:A prospective study was made of changes in the electroencephalogram after the use of metrizamide for myelography and posterior fossa cisternography in 61 patients (62 studies). The EEG changed in 21 patients (34%); 3 of these had had previously abnormal records, and the abnormalities were accentuated on the second rec...

journal_title：Annals of neurology

authors： Ropper AH,Chiappa KH,Young RR

更新日期：1979-09-01 00:00:00

abstract：:The role of mitochondrial DNA (mtDNA) mutations in the pathogenesis of Leber's hereditary optic neuropathy (LHON) has yet to be characterized. Several clinical features of the disease imply that nuclear genes might also be involved in its expression. We have confirmed the presence of a severe NADH:coenzyme Q1 reductas...

journal_title：Annals of neurology

authors： Cock HR,Tabrizi SJ,Cooper JM,Schapira AH

更新日期：1998-08-01 00:00:00

abstract：:The regional metabolic rate for glucose (rMRglu) was studied in macaque monkeys after spinal cord transection at T10, both during spinal shock and after reflexes had returned. The rMRglu was measured in all Rexed layers in cord segments both caudal and rostral to the level of the transection utilizing the quantitative...

journal_title：Annals of neurology

authors： Schwartzman RJ,Eidelberg E,Alexander GM,Yu J

更新日期：1983-07-01 00:00:00

abstract：:Hereditary canine spinal muscular atrophy (HCSMA) features rapidly progressive muscle weakness that affects muscles in an apparent proximal-to-distal gradient. In the medial gastrocnemius (MG) muscle of homozygous HCSMA animals, motor unit tetanic failure is apparent before the appearance of muscle weakness and appear...

journal_title：Annals of neurology

authors： Balice-Gordon RJ,Smith DB,Goldman J,Cork LC,Shirley A,Cope TC,Pinter MJ

更新日期：2000-05-01 00:00:00

abstract：OBJECTIVE:Lymphocytic choriomeningitis virus (LCMV) is a human pathogen and an emerging neuroteratogen. When the infection occurs during pregnancy, the virus can target and damage the fetal brain and retina. We examined the spectrum of clinical presentations, neuroimaging findings, and clinical outcomes of children wit...

journal_title：Annals of neurology

authors： Bonthius DJ,Wright R,Tseng B,Barton L,Marco E,Karacay B,Larsen PD

更新日期：2007-10-01 00:00:00

abstract：:A child with neurofibromatosis exhibited gigantism and acromegaly in association with a hypothalamic mass lesion. Bromocriptine, 5 mg daily, reduced somatic growth rate and restored biochemical homeostasis but had no effect on tumor growth. Radiation therapy arrested tumor enlargement and stabilized deteriorating visu...

journal_title：Annals of neurology

authors： Duchowny MS,Katz R,Bejar RL

更新日期：1984-03-01 00:00:00

abstract：:Layer II of the entorhinal cortex contains the cells of origin for the perforant path, plays a critical role in memory processing, and consistently degenerates in end-stage Alzheimer's disease. The extent to which neuron loss in layer II of entorhinal cortex is related to mild cognitive impairment without dementia has...

journal_title：Annals of neurology

authors： Kordower JH,Chu Y,Stebbins GT,DeKosky ST,Cochran EJ,Bennett D,Mufson EJ

更新日期：2001-02-01 00:00:00

abstract：:Aggregated tau proteins constitute the basic matrix of neuronal inclusions specific to numerous neurodegenerative disorders. Monodimensional and two-dimensional Western blot analyses performed on cortical brain homogenates allowed discrimination between disease-specific tau protein profiles. These observations raised ...

journal_title：Annals of neurology

authors： Delacourte A,Sergeant N,Wattez A,Gauvreau D,Robitaille Y

更新日期：1998-02-01 00:00:00

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

authors： Humphreys P,Kaufmann WE,Galaburda AM

更新日期：1990-12-01 00:00:00