What does this research mean for the field?

A reticular pattern of fat infiltration is exclusive to distal hereditary motor neuropathies, while a moth-eaten pattern predominates in distal myopathies. Novelty: ClaimNovelty.NOVEL_FINDING. Consensus alignment: ConsensusAlignment.NEUTRAL.

What question did this study set out to answer?

The aim is to identify characteristic muscle MRI features that can aid in distinguishing distal myopathies from distal hereditary motor neuropathies.

January 10, 2026European Journal of NeurologyOpen Access

Muscle MRI Contributes to the Differential Diagnosis Between Distal Myopathies and Distal Hereditary Motor Neuropathies

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Key result

Muscle MRI identifies distal hereditary motor neuropathies via reticular fat and distal myopathies via moth-eaten patterns.

Why the study?

Distinguishing between distal myopathies and distal hereditary motor neuropathies can be challenging due to overlapping clinical, EMG, and biopsy findings.

Does muscle MRI help distinguish between distal myopathies and distal hereditary motor neuropathies?

Comparison

Distal myopathies vs distal hereditary motor neuropathies

Authors

MPMaría PayáHospital Universitari i Politècnic La Fe SPSofía PortelaHospital Universitari i Politècnic La Fe RSRafael Sivera

Discussion

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Implication

Muscle MRI fat patterns may aid differentiation of dHMN from DM; leaves open prospective validation before clinical adoption.

Key Points

The aim is to identify characteristic muscle MRI features that can aid in distinguishing distal myopathies from distal hereditary motor neuropathies.
Collected clinical, genetic, and muscle MRI data from patients with confirmed diagnoses of DM and dHMN.
Analyzed MRI characteristics to differentiate between the two conditions and guide molecular diagnosis.
Assessed texture and patterns of fat infiltration in muscle imaging.
Seventy-eight patients diagnosed with DMs and thirty-one with dHMNs were included.
A length-dependent pattern of muscle involvement was more common in dHMNs, while muscle hypertrophy was observed in DMs.
A reticular pattern of fat infiltration was exclusive to dHMNs, while a moth-eaten pattern was more prevalent in DMs.
Muscle islands were identified in 54.8% of dHMNs and in 32% of DMs.

Structured PICO

Does muscle MRI help distinguish between distal myopathies and distal hereditary motor neuropathies?

Population

109 patients with a confirmed molecular diagnosis of either distal myopathy (DM) (n=78, 71.5%) or distal hereditary motor neuropathy (dHMN) (n=31, 28.4%) evaluated at a neuromuscular reference center.

Intervention

Muscle magnetic resonance imaging (MRI) including T1-weighted and STIR-T2w sequences of the pelvis, thighs, lower legs, and foot muscles.

Outcome

Distinctive muscle MRI features (texture, pattern of fat infiltration, muscle islands, and foot muscle involvement) to distinguish between DMs and dHMNs.surrogate

Muscle MRI, particularly the assessment of fat infiltration patterns (reticular vs. moth-eaten) and foot muscle involvement, provides distinctive features that can help differentiate between distal myopathies and distal hereditary motor neuropathies.

Limitations

Small number of patients with specific genetic variants (e.g., DNAJB2 and SOD1)

Cite This Study

Payá et al. (2026) studied this question. A reticular pattern of fat infiltration was exclusive to distal hereditary motor neuropathies, while a moth-eaten pattern was predominant in distal myopathies.

synapsesocial.com/papers/696321fc91e05aa366cb854f https://doi.org/10.1111/ene.70456

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Muscle MRI Contributes to the Differential Diagnosis Between Distal Myopathies and Distal Hereditary Motor Neuropathies

Discussion

Member takes

Implication

Key Points

Structured PICO

Limitations

Cite This Study

Muscle MRI Contributes to the Differential Diagnosis Between Distal Myopathies and Distal Hereditary Motor Neuropathies

Discussion

Member takes

Implication

Key Points

Structured PICO

Limitations

Cite This Study

Also Consider

Also Consider