Evidence map›Paper›PMID 38772930›Full record

ArticleJournal of neurology2024

Clinical and serological insights into paraneoplastic brachial amyotrophic diplegia.

Haidara Kherbek, Christopher Y Itoh, Catherine Daley, Scott D Eggers, Shannon Hinson, Pallab Sarker, Nathan P Staff, Sean J Pittock, Divyanshu Dubey

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Article in Journal of neurology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.

0numbers the graph read from it
0cells of the map it votes in
6citing papers in PubMed
–field-weighted citation impact
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

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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

6 citing papers in PubMed.

  1. Article
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4 · The record

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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

9 authors.

Haidara Kherbek *Department of Neurology, Mayo Clinic, 200 1St Street, SW, Rochester, MN, 55906, USA.
Christopher Y Itoh *Department of Neurology, Mayo Clinic, 200 1St Street, SW, Rochester, MN, 55906, USA.
Catherine DaleyDepartment of Neurology, Mayo Clinic, 200 1St Street, SW, Rochester, MN, 55906, USA.
Scott D EggersDepartment of Neurology, Mayo Clinic, 200 1St Street, SW, Rochester, MN, 55906, USA.
Shannon HinsonDepartment of Laboratory Medicine and Pathology, Mayo Clinic, 200 1St Street, SW, Rochester, MN, 55906, USA.
Pallab SarkerDepartment of Neurology, Mayo Clinic, 200 1St Street, SW, Rochester, MN, 55906, USA.
Nathan P StaffDepartment of Neurology, Mayo Clinic, 200 1St Street, SW, Rochester, MN, 55906, USA.
Sean J PittockDepartment of Neurology, Mayo Clinic, 200 1St Street, SW, Rochester, MN, 55906, USA.
Divyanshu DubeyDepartment of Neurology, Mayo Clinic, 200 1St Street, SW, Rochester, MN, 55906, USA. dubey.divyanshu@mayo.edu.ORCID http://orcid.org/0000-0001-6865-9045

Funding

State of Minnesota's David J. Tomassoni ALS Research Grant Program 238183
6 · The paper itself

Abstract

backgroundBrachial amyotrophic diplegia (BAD) is typically linked to a neurodegenerative etiology such as amyotrophic lateral sclerosis (ALS). Clinical and serological characterizations of paraneoplastic neurologic syndromes resembling BAD are limited.

methodsA retrospective chart review of patients with BAD-like presentations was conducted. Clinical/paraclinical features of paraneoplastic BAD and neurodegenerative BAD cases were compared.

resultsBetween 2017 and 2023, 13 cases of BAD were identified, of these 10 were neurodegenerative BAD (ALS variant), and 3 cases associated with paraneoplastic autoimmunity. An additional paraneoplastic BAD case diagnosed in 2005 was included. LUZP4-IgG was detected in all four paraneoplastic cases, with coexisting KLHL11-IgG in three cases and ANNA1 (anti-Hu)-IgG in one case. Out of the four paraneoplastic cases, two patients had seminoma, while the remaining two had limited cancer investigation. Three patients exhibited bi-brachial weakness as the initial symptom before the onset of brainstem symptoms or seizures. Compared to BAD patients with a neurodegenerative etiology, a higher proportion of paraneoplastic cases had ataxia (75% vs 0%, p = 0.011). Other clinical features only detected in the paraneoplastic BAD group were vertigo (n = 2), hearing loss (n = 2) and ophthalmoplegia (n = 2). Electrodiagnostic studies in these patients revealed cervical myotome involvement, supportive of motor neuronopathy. All paraneoplastic cases but none of the neurodegenerative BAD cases exhibited inflammatory cerebrospinal fluid (CSF) findings (lymphocytic pleocytosis and/or supernumerary oligoclonal bands; p = 0.067). Despite the administration of immunotherapy and/or cancer treatment, none of the paraneoplastic patients reported clinical improvement. DISCUSSION: BAD or bi-brachial neurogenic weakness is a rare phenotypic presentation associated with paraneoplastic autoimmunity. Co-existing features of brainstem dysfunction or cerebellar ataxia should prompt further paraneoplastic evaluation. Common serological and cancer associations among these cases include LUZP4-IgG and KLHL11-IgG, along with testicular germ cell tumors, respectively.

Indexed as

Paraneoplastic Syndromes, Nervous SystemAdultAgedAutoantibodiesBrachial Plexus NeuropathiesCarrier ProteinsFemaleHumansMaleMiddle AgedRetrospective StudiesAutoantibodiesCarrier ProteinsKLHL11 protein, humanAmyotrophic lateral sclerosisBi-brachial amyotrophic diplegiaFlail arm syndromeKelch-Like Protein 11Leucine Zipper Protein 4Paraneoplastic neurologic syndrome

Identifiers

PMID38772930

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