Acne fulminans with inflammatory myopathy.

abstract：:Genomic triplication of the alpha-synuclein gene recently has been associated with familial Parkinson's disease in the Spellman-Muenter kindred. Here, we present an independent family, of Swedish-American descent, with hereditary early-onset parkinsonism with dementia due to alpha-synuclein triplication. Brain tissue ...

journal_title：Annals of neurology

authors： Farrer M,Kachergus J,Forno L,Lincoln S,Wang DS,Hulihan M,Maraganore D,Gwinn-Hardy K,Wszolek Z,Dickson D,Langston JW

更新日期：2004-02-01 00:00:00

abstract：:We describe a young child with tuberous sclerosis and cardiac rhabdomyoma who developed reversible heart block after being placed on carbamazepine treatment for seizures. Patients of any age with known or suspected intrinsic cardiac disease should be monitored for conduction disturbance if they are treated with carbam...

journal_title：Annals of neurology

authors： Weig SG,Pollack P

更新日期：1993-10-01 00:00:00

abstract：:The worldwide epidemiology of Creutzfeldt-Jakob disease (CJD) is presented from an analysis of 1,435 patients. In the United States, the average annual mortality rate is at least 0.26 deaths per million. Temporal-spatial clustering of cases was not found in the United States, but reports from other countries indicate ...

journal_title：Annals of neurology

authors： Masters CL,Harris JO,Gajdusek DC,Gibbs CJ Jr,Bernoulli C,Asher DM

更新日期：1979-02-01 00:00:00

abstract：:A patient with generalized convulsions noted that seizures were reliably precipitated by mental arithmetic. The interictal electroencephalogram revealed only a mild, diffuse, nonspecific disturbance, but bursts of generalized epileptiform activity with no obvious clinical expression accompanied efforts at mental arith...

journal_title：Annals of neurology

authors： Wilkins AJ,Zifkin B,Andermann F,McGovern E

更新日期：1982-06-01 00:00:00

abstract：:We report the clinical, imaging, and laboratory features of 8 patients with Devic's neuromyelitis optica. All patients had severe myelopathy and optic neuritis. In no patient was the brain, the brainstem, or the cerebellum affected, even after several years of disease. Various immunosuppressive treatments failed to be...

journal_title：Annals of neurology

authors： Mandler RN,Davis LE,Jeffery DR,Kornfeld M

更新日期：1993-08-01 00:00:00

abstract：:Recently, the excitatory amino acid neurotransmitter glutamate was implicated in the pathogenesis of a variety of chronic degenerative neurological diseases in humans and animals. This report describes abnormalities in excitatory amino acids in the central nervous system of 18 patients with amyotrophic lateral scleros...

journal_title：Annals of neurology

authors： Rothstein JD,Tsai G,Kuncl RW,Clawson L,Cornblath DR,Drachman DB,Pestronk A,Stauch BL,Coyle JT

更新日期：1990-07-01 00:00:00

abstract：:Regional cerebral blood flow (rCBF) was measured in human subjects during saccadic eye movements by a 254-channel dynamic gamma camera. Focal rCBF increases were repeatedly observed in an area within the middle precentral and premotor regions which corresponds to the frontal eye field in humans. Our findings suggest t...

journal_title：Annals of neurology

authors： Melamed E,Larsen B

更新日期：1979-01-01 00:00:00

abstract：:D2 dopamine receptor densities were measured in postmortem samples of the caudate nucleus and putamen from 36 parkinsonian patients. The relationship between the age of the patient, duration of the disease, and duration of L-dopa therapy versus density of brain D2 dopamine receptors was examined using [3H]spiperone. R...

journal_title：Annals of neurology

authors： Guttman M,Seeman P,Reynolds GP,Riederer P,Jellinger K,Tourtellotte WW

更新日期：1986-05-01 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:Friedreich ataxia (FRDA), an autosomal recessive neurodegenerative disease caused by mutations in the gene encoding for the mitochondrial protein frataxin, is characterized by ataxia and gait instability, immobility, and eventual death. We evaluated corneal confocal microscopy (CCM) quantification of corneal ...

journal_title：Annals of neurology

authors： Pagovich OE,Vo ML,Zhao ZZ,Petropoulos IN,Yuan M,Lertsuwanroj B,Ciralsky J,Lai E,Kiss S,D'Amico DJ,Mezey JG,Malik RA,Crystal RG

更新日期：2018-12-01 00:00:00

abstract：:To study distribution and patterns of nerve hypertrophy in chronic inflammatory demyelinating polyneuropathy (CIDP), magnetic resonance neurography with 3-dimensional reconstruction of short tau inversion recovery images was performed in 33 patients. This technique clearly showed longitudinal morphological changes fro...

journal_title：Annals of neurology

authors： Shibuya K,Sugiyama A,Ito S,Misawa S,Sekiguchi Y,Mitsuma S,Iwai Y,Watanabe K,Shimada H,Kawaguchi H,Suhara T,Yokota H,Matsumoto H,Kuwabara S

更新日期：2015-02-01 00:00:00

abstract：OBJECTIVE:Migraine aura is sparsely studied due to the highly challenging task of capturing patients during aura. Cortical spreading depression (CSD) is likely the underlying phenomenon of aura. The possible correlation between the multifaceted phenomenology of aura symptoms and the effects of CSD on the brain has not ...

journal_title：Annals of neurology

authors： Arngrim N,Hougaard A,Ahmadi K,Vestergaard MB,Schytz HW,Amin FM,Larsson HBW,Olesen J,Hoffmann MB,Ashina M

更新日期：2017-12-01 00:00:00

abstract：OBJECTIVE:The objective of this study was to evaluate patients with ganglionic acetylcholine receptor antibody (gAChR-Ab) positive autoimmune autonomic ganglionopathy using a multimodal testing protocol to characterize their full clinical phenotype and explore biomarkers to quantify immunotherapy response. METHODS:We ...

journal_title：Annals of neurology

authors： Koay S,Vichayanrat E,Bremner F,Panicker JN,Lang B,Lunn MP,Watson L,Ingle GT,Hagen EM,McNamara P,Jacobson L,Provitera V,Nolano M,Vincent A,Mathias CJ,Iodice V

更新日期：2021-01-13 00:00:00

abstract：:The North American study of plasmapheresis in Guillain-Barré syndrome (GBS) included early, standardized electrodiagnostic testing in 210 of the 245 patients. To determine the types of abnormalities and the relation to outcome, we analyzed the prospectively collected motor conduction data obtained during the first 30 ...

journal_title：Annals of neurology

authors： Cornblath DR,Mellits ED,Griffin JW,McKhann GM,Albers JW,Miller RG,Feasby TE,Quaskey SA

更新日期：1988-04-01 00:00:00

abstract：:We carried out a comprehensive assessment of eye movements in 2 subjects with impaired facial learning and recognition to determine if the defect might be associated with abnormal scanning of faces. Standard electroculogram showed that fixation, pursuit, saccades, and scanning of salient features of scenes and faces w...

journal_title：Annals of neurology

authors： Rizzo M,Hurtig R,Damasio AR

更新日期：1987-07-01 00:00:00

abstract：:The phenotype of severe congenital hydrocephalus secondary to neural cell adhesion molecule L1 (L1CAM) gene mutations includes the distinct finding of brainstem corticospinal tract hypoplasia. Using diffusion-weighted imaging (DWI), we failed to demonstrate anisotropy in the corticospinal tracts of the basis pontis in...

journal_title：Annals of neurology

authors： Graf WD,Born DE,Shaw DW,Thomas JR,Holloway LW,Michaelis RC

更新日期：2000-01-01 00:00:00

abstract：:A 65-year-old woman with subacute global deterioration of neurological function had bilateral occlusion of the internal carotid arteries demonstrated by angiography. Postmortem examination showed isolated giant cell arteritis involving the carotid siphon bilaterally. ...

journal_title：Annals of neurology

authors： Howard GF 3rd,Ho SU,Kim KS,Wallach J

更新日期：1984-02-01 00:00:00

abstract：:A patient is described who suffered a bilateral hemorrhagic infarction in the parasagittal cerebrum following neonatal asphyxia. This case confirms experimental results that show the parasagittal cerebrum is especially vulnerable to ischemic damage in newborns. ...

journal_title：Annals of neurology

authors： Pasternak JF

更新日期：1987-02-01 00:00:00

abstract：:The cause and pathophysiology of dystonia remain unknown. The recent identification of mitochondrial complex I deficiency in platelets from patients with sporadic focal dystonia suggests that a defect of energy metabolism may be relevant in a proportion of patients. We have addressed the possible contribution of mitoc...

journal_title：Annals of neurology

authors： Tabrizi SJ,Cooper JM,Schapira AH

更新日期：1998-08-01 00:00:00

abstract：:We reviewed the records of 160 consecutive patients with glioblastoma and anaplastic astrocytoma to evaluate the long-term consequences of radiation therapy and chemotherapy. We defined long-term survivors as those patients with glioblastoma or anaplastic astrocytoma who lived at least 100% longer than median survival...

journal_title：Annals of neurology

authors： Imperato JP,Paleologos NA,Vick NA

更新日期：1990-12-01 00:00:00

abstract：OBJECTIVE:Axonal degeneration is a main contributor to disability in progressive neurodegenerative diseases in which oxidative stress is often identified as a pathogenic factor. We aim to demonstrate that antioxidants are able to improve axonal degeneration and locomotor deficits in a mouse model of X-adrenoleukodystro...

journal_title：Annals of neurology

authors： López-Erauskin J,Fourcade S,Galino J,Ruiz M,Schlüter A,Naudi A,Jove M,Portero-Otin M,Pamplona R,Ferrer I,Pujol A

更新日期：2011-07-01 00:00:00

abstract：:Basis pontis lacunes cause contralateral but rarely ipsilateral ataxia. We explored this phenomenon with isotope tract tracing in the rhesus monkey. Labeled pontocerebellar fibers cross midline and disperse widely in the opposite hemipons before coalescing in the brachium pontis. This anatomical arrangement suggests t...

journal_title：Annals of neurology

authors： Schmahmann JD,Rosene DL,Pandya DN

更新日期：2004-04-01 00:00:00

abstract：:To determine the influence of chronic ethanol intake on the central nervous system, we studied 40 asymptomatic, well-nourished, chronic alcoholics (mean age, 42.6 +/- 9.1 years) and 20 age-, sex-, and education-matched control subjects. Studies included neuropsychological testing, visual and short-latency auditory evo...

journal_title：Annals of neurology

authors： Nicolás JM,Estruch R,Salamero M,Orteu N,Fernandez-Solà J,Sacanella E,Urbano-Márquez A

更新日期：1997-05-01 00:00:00

abstract：:To investigate the potential role of drug therapy in preventing or exacerbating seizure-related brain injury in the prepubescent brain, we administered kainic acid to rats at postnatal day 35. Therapy with daily phenobarbital was started directly before or 1 day after kainic acid was administered, and was continued th...

journal_title：Annals of neurology

authors： Mikati MA,Holmes GL,Chronopoulos A,Hyde P,Thurber S,Gatt A,Liu Z,Werner S,Stafstrom CE

更新日期：1994-09-01 00:00:00

abstract：OBJECTIVE:Determine the probability of attaining complete remission in children with nonsyndromic epilepsy (NSE) over the course of ≥10 years from initial diagnosis; identify early predictors of complete remission; and assess the risk of relapse after achieving complete remission. METHODS:In a prospective community-ba...

journal_title：Annals of neurology

authors： Berg AT,Testa FM,Levy SR

更新日期：2011-10-01 00:00:00

abstract：:Auditory evoked magnetic fields to noise/square-wave sequences, presented once every 2.2 seconds, were recorded in 8 patients who had ischemic lesions in the auditory cortex or in its vicinity. In 2 patients with large temporoparietal lesions, the magnetic 100-msec deflection (N100m) was absent over the damaged side. ...

journal_title：Annals of neurology

authors： Mäkelä JP,Hari R,Valanne L,Ahonen A

更新日期：1991-07-01 00:00:00

abstract：:Intraneural injection of antisera from rabbits with high antigalactocerebroside antibody levels into rat sciatic nerve produced acute nerve conduction block. This was first apparent in some motor axons between 30 and 60 minutes after injection and progressed to completion within 2 to 4 hours. Concurrent morphological ...

journal_title：Annals of neurology

authors： Sumner AJ,Saida K,Saida T,Silberberg DH,Asbury AK

更新日期：1982-05-01 00:00:00

abstract：:Eight patients with recent cerebral hemispheric infarction were studied with positron emission tomography and the oxygen-15 steady-state inhalation and [18F]deoxyglucose techniques to obtain values of regional cerebral blood flow, oxygen consumption, and glucose metabolism. The Sokoloff equation, used to calculate glu...

journal_title：Annals of neurology

authors： Wise RJ,Rhodes CG,Gibbs JM,Hatazawa J,Palmer T,Frackowiak RS,Jones T

更新日期：1983-12-01 00:00:00

abstract：:A 45-year-old woman was diagnosed as having the unclassified form of botulism. Her intestines may have been predisposed to colonization with Clostridium botulinum because of a jejunoileal bypass procedure that had been done several years earlier. One other similar case has been reported. ...

journal_title：Annals of neurology

authors： Freedman M,Armstrong RM,Killian JM,Boland D

更新日期：1986-11-01 00:00:00

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

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

更新日期：1990-10-01 00:00:00