Evidence map›Paper›PMID 33402525›Full record

ArticleNeurology(R) neuroimmunology & neuroinflammation2021

Unilateral Relapsing Primary Angiitis of the CNS: An Entity Suggesting Differences in the Immune Response Between the Cerebral Hemispheres.

Mahmoud A AbdelRazek, James M Hillis, Yanjun Guo, Maria Martinez-Lage, Taha Gholipour, Jacob Sloane, Tracey Cho, Marcelo Matiello

Open access · goldAbstract readCase Reports
PubMed Full text at PMC Publisher
In one paragraph

Article in Neurology(R) neuroimmunology & neuroinflammation, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.

0numbers the graph read from it
0cells of the map it votes in
11citing papers in PubMed
1.5field-weighted citation impact, top 18% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

11 citing papers in PubMed, 14 citations in OpenAlex.

  1. Article
  2. Tumor-mimicking primary CNS vasculitis: illustrative case.Journal of neurosurgery. Case lessons · 2026
    Article
  3. Unilateral Primary Angiitis of the Central Nervous System (PACNS): a case report of unusual radiological presentation.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2026
    Article
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors at 1 institution in 2 countries.

Mahmoud A AbdelRazekFrom the Neurology Department (M.A.A.), Mount Auburn Hospital, Harvard Medical School, Cambridge, MA; Neurology Department (J.M.H., M.M.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (Y.G.), Beijing Tongren Hospital, Capital Medical University, China; Department of Pathology (M.M.-L.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (T.G.), The George Washington University, DC; Neurology Department (J.S.), Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA; and Neurology Department (T.C.), University of Iowa. mabdelrazek@mah.harvard.edu.ORCID 0000-0002-2391-3502
James M HillisFrom the Neurology Department (M.A.A.), Mount Auburn Hospital, Harvard Medical School, Cambridge, MA; Neurology Department (J.M.H., M.M.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (Y.G.), Beijing Tongren Hospital, Capital Medical University, China; Department of Pathology (M.M.-L.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (T.G.), The George Washington University, DC; Neurology Department (J.S.), Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA; and Neurology Department (T.C.), University of Iowa.
Yanjun GuoFrom the Neurology Department (M.A.A.), Mount Auburn Hospital, Harvard Medical School, Cambridge, MA; Neurology Department (J.M.H., M.M.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (Y.G.), Beijing Tongren Hospital, Capital Medical University, China; Department of Pathology (M.M.-L.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (T.G.), The George Washington University, DC; Neurology Department (J.S.), Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA; and Neurology Department (T.C.), University of Iowa.
Maria Martinez-LageFrom the Neurology Department (M.A.A.), Mount Auburn Hospital, Harvard Medical School, Cambridge, MA; Neurology Department (J.M.H., M.M.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (Y.G.), Beijing Tongren Hospital, Capital Medical University, China; Department of Pathology (M.M.-L.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (T.G.), The George Washington University, DC; Neurology Department (J.S.), Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA; and Neurology Department (T.C.), University of Iowa.ORCID 0000-0002-5859-7562
Taha GholipourFrom the Neurology Department (M.A.A.), Mount Auburn Hospital, Harvard Medical School, Cambridge, MA; Neurology Department (J.M.H., M.M.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (Y.G.), Beijing Tongren Hospital, Capital Medical University, China; Department of Pathology (M.M.-L.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (T.G.), The George Washington University, DC; Neurology Department (J.S.), Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA; and Neurology Department (T.C.), University of Iowa.
Jacob SloaneFrom the Neurology Department (M.A.A.), Mount Auburn Hospital, Harvard Medical School, Cambridge, MA; Neurology Department (J.M.H., M.M.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (Y.G.), Beijing Tongren Hospital, Capital Medical University, China; Department of Pathology (M.M.-L.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (T.G.), The George Washington University, DC; Neurology Department (J.S.), Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA; and Neurology Department (T.C.), University of Iowa.ORCID 0000-0003-0028-3031
Tracey ChoFrom the Neurology Department (M.A.A.), Mount Auburn Hospital, Harvard Medical School, Cambridge, MA; Neurology Department (J.M.H., M.M.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (Y.G.), Beijing Tongren Hospital, Capital Medical University, China; Department of Pathology (M.M.-L.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (T.G.), The George Washington University, DC; Neurology Department (J.S.), Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA; and Neurology Department (T.C.), University of Iowa.
Marcelo MatielloFrom the Neurology Department (M.A.A.), Mount Auburn Hospital, Harvard Medical School, Cambridge, MA; Neurology Department (J.M.H., M.M.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (Y.G.), Beijing Tongren Hospital, Capital Medical University, China; Department of Pathology (M.M.-L.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (T.G.), The George Washington University, DC; Neurology Department (J.S.), Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA; and Neurology Department (T.C.), University of Iowa.
University of Iowa · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectiveTo determine whether studying patients with strictly unilateral relapsing primary angiitis of the CNS (UR-PACNS) can support hemispheric differences in immune response mechanisms, we reviewed characteristics of a group of such patients.

methodsWe surveiled our institution for patients with UR-PACNS, after characterizing one such case. We defined UR-PACNS as PACNS with clinical and radiographic relapses strictly recurring in 1 brain hemisphere, with or without hemiatrophy. PACNS must have been biopsy proven. Three total cases were identified at our institution. A literature search for similar reports yielded 4 additional cases. The combined 7 cases were reviewed for demographic, clinical, imaging, and pathologic trends.

resultsThe median age at time of clinical onset among the 7 cases was 26 years (range 10-49 years); 5 were male (71%). All 7 patients presented with seizures. The mean follow-up duration was 7.5 years (4-14.1 years). The annualized relapse rate ranged between 0.2 and 1. UR-PACNS involved the left cerebral hemisphere in 5 of the 7 patients. There was no consistent relationship between the patient's dominant hand and the diseased side. When performed (5 cases), conventional angiogram was nondiagnostic. CSF examination showed nucleated cells and protein levels in normal range in 3 cases and ranged from 6 to 11 cells/μL and 49 to 110 mg/dL in 4 cases, respectively. All cases were diagnosed with lesional biopsy, showing lymphocytic type of vasculitis of the small- and medium-sized vessels. Patients treated with steroids alone showed progression. Induction therapy with cyclophosphamide or rituximab followed by a steroid sparing agent resulted in the most consistent disease remission.

conclusionsCombining our 3 cases with others reported in the literature allows better clinical understanding about this rare and extremely puzzling disease entity. We hypothesize that a functional difference in immune responses, caused by such discrepancies as basal levels of cytokines, asymmetric distribution of microglia, and differences in modulation of the systemic immune functions, rather than a structural antigenic difference, between the right and left brain may explain this phenomenon, but this is speculative.

Indexed as

AdultCerebrumChildFemaleFollow-Up StudiesHumansImmunityMaleMiddle AgedRecurrenceVasculitis, Central Nervous SystemYoung Adult

Identifiers

PMID33402525
PMCPMC7862090
OpenAlexW3120241875

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.