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Updated: Jul 14, 2025

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells

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[Monomelic amyotrophy].

A A Shulman¹, B R Samigullin^1,2, M E Baltin¹

¹Kazan (Volga Region) Federal University, Kazan, Russia.

Zhurnal Nevrologii I Psikhiatrii Imeni S.S. Korsakova

|October 5, 2023

Summary

This summary is machine-generated.

Hirayama disease, a rare neurological disorder, typically affects the upper limbs. This case highlights a patient experiencing weakness in both upper and lower extremities, expanding the known symptom profile.

Keywords:

Hirayama disease electromyography manual muscle testing monomelic amyotrophy motor neuron disease

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Area of Science:

Neurology
Neuroscience
Clinical Neurology

Background:

Monomelic amyotrophy, or Hirayama disease, is a rare, self-limiting motor neuron disease.
It typically presents in young individuals with progressive, focal weakness and atrophy, primarily in the distal upper limbs.
The condition is characterized by the absence of sensory, bulbar, or pyramidal signs.

Observation:

This report details a unique case of a patient presenting with symptoms beyond the typical upper limb involvement.
The patient exhibited weakness not only in the upper extremities but also in the lower extremities.
This presentation deviates from the classic description of Hirayama disease.

Findings:

The case expands the clinical spectrum of Hirayama disease.
It suggests that lower extremity weakness may occur in some patients with this condition.
This challenges the previously understood localized nature of the disease.

Implications:

This case broadens the diagnostic considerations for Hirayama disease.
It may prompt further research into the underlying pathophysiology and potential variations in disease expression.
Understanding these variations is crucial for accurate diagnosis and patient management.