MELAS: Clinical and pathologic correlations with MRI, xenon/CT, and MR spectroscopy.


:We describe the clinical, imaging, and pathologic findings in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). The patient experienced her first stroke-like episode at age forty-four. Brain MRI, obtained at symptom onset, at 3 weeks, and at 1 year, revealed migrating T2-weighted hyperintensities in the temporal/parietal and occipital cortices and later revealed atrophy. Abnormal cerebrovascular reserve was evident on xenon/CT four days after the first MRI. MR spectroscopy at 1 year revealed increased lactate in both the occipital and temporal lobes. Histologic sections demonstrated spongy degeneration of the cortex that was most prominent at the crests of the gyri. Electron microscopy of the blood vessels showed increased numbers of abnormal mitochondria within the vascular smooth muscle and in endothelial cells. We hypothesize that the stroke-like episodes in MELAS may be due to impaired autoregulation secondary to the impaired metabolic activity of mitochondria in the endothelial and smooth muscle cells of blood vessels.






Clark JM,Marks MP,Adalsteinsson E,Spielman DM,Shuster D,Horoupian D,Albers GW




Has Abstract


1996-01-01 00:00:00












  • Deep brain stimulation of the VIM thalamic nucleus modifies several features of essential tremor.

    abstract:BACKGROUND:Pharmacologic interventions (e.g., beta blockers) and thalamic lesions have failed to alter the pathophysiology of essential tremor (ET) beyond a reduction in tremor amplitude. Deep brain stimulation (DBS) of the ventral intermediate (VIM) nucleus of the thalamus successfully reduces tremor rating scores. It...


    pub_type: 临床试验,杂志文章


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    更新日期:2003-10-14 00:00:00

  • Depiction of the neuroscientific principles of human motion 2 millennia ago by Lucretius.

    abstract::Titus Lucretius Carus was an ancient Roman philosopher of the Epicurean school whose epic poem On the Nature of Things described numerous aspects of the natural world. In fact, much contemporary scientific understanding is consistent with or inspired by his work. Among Lucretius's contributions to neurology were his d...


    pub_type: 传,历史文章,杂志文章


    authors: Hyam JA,Paterson DJ,Aziz TZ,Green AL

    更新日期:2011-09-06 00:00:00

  • Symptomatic spinal epidural lipomatosis in a patient with Cushing's disease.

    abstract::Spinal epidural lipomatosis (SEL) is a rare complication of iatrogenic Cushing's syndrome (CS). There is only one case reported of symptomatic SEL in association with endogenous CS. We present a patient with compressive myeloradiculopathy due to SEL and Cushing's disease and suggest that in SEL of hypercortisolism, th...


    pub_type: 杂志文章


    authors: Sivakumar K,Sheinart K,Lidov M,Cohen B

    更新日期:1995-12-01 00:00:00

  • Treatable neurotransmitter deficiency in mild phenylketonuria.

    abstract::The authors describe a case of neurologic involvement in mild hyperphenylalaninemia (HPA), not due to tetrahydrobiopterin (BH(4)) deficiency, with low levels of monoamine neurotransmitter metabolites in CSF. The combined BH(4)-Phe loading test suggested a BH(4) response, confirmed by clinical improvement after BH(4) t...


    pub_type: 杂志文章


    authors: Bonafé L,Blau N,Burlina AP,Romstad A,Güttler F,Burlina AB

    更新日期:2001-09-11 00:00:00

  • Laryngeal botulinum toxin injections for disabling stuttering in adults.

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    pub_type: 临床试验,杂志文章


    authors: Brin MF,Stewart C,Blitzer A,Diamond B

    更新日期:1994-12-01 00:00:00

  • Neurofilament light and tau as blood biomarkers for sports-related concussion.

    abstract:OBJECTIVE:To compare neurofilament light (NfL) and tau as blood-based biomarkers for acute sports-related concussion (SRC) and determine whether their concentrations at different time points after the injury are associated with prolonged time to return to play (RTP). METHODS:A total of 288 professional hockey players ...


    pub_type: 杂志文章


    authors: Shahim P,Tegner Y,Marklund N,Blennow K,Zetterberg H

    更新日期:2018-05-15 00:00:00

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    pub_type: 杂志文章


    authors: Cranford RE,Leppik IE,Patrick B,Anderson CB,Kostick B

    更新日期:1979-11-01 00:00:00

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    pub_type: 杂志文章


    authors: Orkaby AR,Cho K,Cormack J,Gagnon DR,Driver JA

    更新日期:2017-10-31 00:00:00

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    pub_type: 杂志文章


    authors: Diamond SG,Markham CH,Hoehn MM,McDowell FH,Muenter MD

    更新日期:1989-09-01 00:00:00

  • Comprehensive analysis of the predictors of neurobehavioral symptom reporting in veterans.

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    pub_type: 杂志文章


    authors: Andrews RJ,Fonda JR,Levin LK,McGlinchey RE,Milberg WP

    更新日期:2018-08-21 00:00:00

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    pub_type: 杂志文章


    authors: Merlin LR,Horak HA,Milligan TA,Kraakevik JA,Ali II

    更新日期:2014-07-29 00:00:00

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    pub_type: 临床试验,杂志文章


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    更新日期:1998-07-01 00:00:00

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    pub_type: 杂志文章,多中心研究,随机对照试验


    authors: French JA,Krauss GL,Biton V,Squillacote D,Yang H,Laurenza A,Kumar D,Rogawski MA

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    pub_type: 杂志文章


    authors: Kischka U,Mandir AS,Ghika J,Growdon JH

    更新日期:1993-03-01 00:00:00

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    更新日期:2010-03-30 00:00:00

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    更新日期:2004-06-08 00:00:00

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    pub_type: 杂志文章,评审


    authors: Devinsky O,Spruill T,Thurman D,Friedman D

    更新日期:2016-02-23 00:00:00

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    authors: Fromm GH,Glass JD,Chattha AS,Martinez AJ,Silverman M

    更新日期:1980-02-01 00:00:00

  • Genesis of Alzheimer's disease.

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    更新日期:1997-05-01 00:00:00

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    pub_type: 杂志文章


    authors: Keane JR

    更新日期:1986-01-01 00:00:00

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    pub_type: 杂志文章


    authors: Mellies U,Ragette R,Schwake C,Baethmann M,Voit T,Teschler H

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    更新日期:2017-09-05 00:00:00

  • Acute late-onset encephalopathy after radiotherapy: an unusual life-threatening complication.

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  • Regulatory and reimbursement issues in treating patients with immune-mediated neuropathies.

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    pub_type: 杂志文章,评审


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    pub_type: 杂志文章


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    pub_type: 杂志文章


    authors: Charletta D,Gorelick PB,Dollear TJ,Freels S,Harris Y

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    pub_type: 杂志文章


    authors: Carey LM,Abbott DF,Puce A,Jackson GD,Syngeniotis A,Donnan GA

    更新日期:2002-09-10 00:00:00

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    pub_type: 杂志文章


    authors: Dobkin BH,Verity MA

    更新日期:1976-08-01 00:00:00