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Article

Motor neuron involvement in multisystem proteinopathy: implications for ALS.

Neurology - 14 May 2013

Benatar Michael, Wuu Joanne, Fernandez Catalina, Weihl Conrad C, Katzen Heather, Steele Julie, Oskarsson Bjorn, Taylor J Paul

Abstract excerpt

OBJECTIVE: To explore the putative connection between inclusion body myopathy, Paget disease, frontotemporal dementia (IBMPFD) and motor neuron disease (MND). METHODS: Clinical, genetic, and EMG characterization of 17 patients from 8 IBMPFD families. RESULTS: Limb weakness was the most common clinical manifestation (present in 15 patients, median onset age 38 years, range 25-52), with unequivocal evidence of...

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Share to Amyotrophic Lateral Sclerosis
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Heterogeneous-Nuclear Ribonucleoprotein Group A-B

Share to Motor Neurons

Share to Muscular Dystrophies, Limb-Girdle

Share to Myositis, Inclusion Body

Share to Osteitis Deformans

Share to Valosin Containing Protein

Source

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Identifiers

PMID: 23635965
DOI: 10.1212/WNL.0b013e3182929fc3
PMCID: PMC3908355

Genes

VCP
HNRNPA1

Variants

Provenance

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