Progressive Muscular Atrophy

Liewluck, Teerin; Saperstein, David S.

doi:10.1016/j.ncl.2015.07.005

Cited by 65Amyotrophic Lateral Sclerosis ResearchNeurogenetic and Muscular Disorders ResearchPeripheral Neuropathies and Disorders

Progressive Muscular Atrophy

Liewluck, Teerin, Saperstein, David S.·2015·Neurologic Clinics

DOI 10.1016/j.ncl.2015.07.005

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Abstract

Progressive muscular atrophy (PMA) is a rare, sporadic, adult-onset motor neuron disease, clinically characterized by isolated lower motor neuron features; however, clinically evident upper motor neuron signs may emerge in some patients. Subclinical upper motor neuron involvement is identified pathologically, radiologically, and neurophysiologically in a substantial number of patients with PMA. Patients with subclinical upper motor neuron involvement do not fulfill the revised El Escorial criteria to participate in amyotrophic lateral sclerosis clinical trials. Intravenous immunoglobulin therapy is only marginally beneficial in a small subgroup of patients with lower motor neuron syndrome without conduction block.

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Informal caregivers of patients with ALS or PMA face stressful demands due to the prospect of an early death and severe impairments of the patient.
“Informal caregivers of patients with ALS or PMA face stressful demands due to the prospect of an early death and severe impairments of the patient.”
From A blended psychosocial support program for partners of patients with amyotrophic lateral sclerosis and progressive muscular atrophy: Protocol of a randomized controlled trial (2018) · cites this paper as [2]

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Authors: Liewluck, T., & Saperstein, D. S.
Affiliations: Mayo Clinic (US)
University of Arizona (US)
Corresponding author: Liewluck, Teerin
ORCID: Liewluck, Teerin
Saperstein, David S.
Venue: Neurologic Clinics
Publisher: Elsevier BV
Published: 2015-11
Volume: 33
Issue: 4
Pages: 761-773
ISSN: 0733-8619 (ISSN-L)
DOI: 10.1016/j.ncl.2015.07.005
OpenAlex: W1826779307
Cited by: 65
References: 81
Citations by year: 2026: 3 · 2025: 7 · 2024: 7 · 2023: 9 · 2022: 3 · 2021: 9 · 2020: 8 · 2019: 5 · 2018: 4 · 2017: 5 · 2016: 2 · 2015: 3
FWCI: 0.61
Type: journal article
Language: en
Open access: closed
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations: Neurologic Clinics (publishedVersion)
PubMed (publishedVersion)
Topics: Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
Health Sciences › Medicine › Genetics › Neurogenetic and Muscular Disorders Research
Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
Keywords: progressive muscular atrophy, motor neuron disease, lower motor neuron disease, upper motor neuron involvement, El Escorial criteria, intravenous immunoglobulin therapy, conduction block, adult-onset disease
MeSH terms: Diagnosis, Differential, Humans, Muscular Atrophy, Spinal, Severity of Illness Index, Motor Neuron Disease, Immunoglobulins, Intravenous, Disease Management, Neuroimaging

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Progressive Muscular Atrophy

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