Electrophysiological differences between Hirayama disease, amyotrophic lateral sclerosis and cervical spondylotic amyotrophy.

Xiang Jin,Jian-Yuan Jiang,Xin-Lei Xia,Fei-Zhou Lu,Li-Xun Wang,Chao-Jun Zheng

doi:10.1186/1471-2474-15-349

Xiang Jin, Jian-Yuan Jiang + Show 4 more

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https://doi.org/10.1186/1471-2474-15-349

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Abstract

BackgroundHirayama disease (HD), amyotrophic lateral sclerosis (ALS) or cervical spondylotic amyotrophy (CSA) may result in atrophy of intrinsic hand and forearm muscles. The incidence of HD is low, and it is rarely encountered in the clinical setting. Consequently, HD is often misdiagnosed as ALS or CSA. It is important to differentiate these diseases because HD is caused by a benign focal lesion that is limited to the upper limbs.MethodsThe thenar and hypothenar compound muscle action potential (CMAP) amplitude of the upper limbs of 100 HD, 97 ALS and 32 CSA cases were reviewed; 35 healthy individuals were included as controls. Seventy-eight percent, 38% and 69% of patients with HD, ALS or CSA had unilateral involvement; the remaining patients were affected bilaterally. Thenar and hypothenar CMAP amplitude evoked by ulnar stimulation was compared with CMAP evoked by median stimulation.ResultsThe ulnar/median CMAP ratio was found to be lower in HD (0.55 ± 0.41, P < 0.0001), higher in ALS (2.28 ± 1.15, P < 0.0001) and no different in CSA (1.21 ± 0.53, P > 0.05) compared with the normal range from previous studies (0.89-1.60) and with the healthy controls (1.15 ± 0.23). Conduction velocities of the sensory and motor nerves, the amplitude of the sensory nerve action potential, and the CMAP amplitude of the unaffected limb were all normal.ConclusionsThe hand muscles were differentially affected between patients with HD, ALS and CSA. The ulnar/median CMAP ratio could be used to distinguish these three diseases.

Highlights

Hirayama disease (HD), amyotrophic lateral sclerosis (ALS) or cervical spondylotic amyotrophy (CSA) may result in atrophy of intrinsic hand and forearm muscles
Hirayama disease (HD), known as juvenile muscular atrophy of the distal upper extremity, was first reported by Hirayama et al in 1959 as a benign focal motor neuron disease characterized by unilateral atrophy of the forearm ulnar muscle [1]
It is important to differentiate these diseases because HD is caused by a benign focal lesion that is limited to the upper limbs

Summary

Introduction

Hirayama disease (HD), amyotrophic lateral sclerosis (ALS) or cervical spondylotic amyotrophy (CSA) may result in atrophy of intrinsic hand and forearm muscles. Hirayama disease (HD), known as juvenile muscular atrophy of the distal upper extremity, was first reported by Hirayama et al in 1959 as a benign focal motor neuron disease characterized by unilateral atrophy of the forearm ulnar muscle [1]. In rare cases, further amyotrophy progression has been reported. The clinical features of HD are similar to those of amyotrophic lateral sclerosis (ALS) and cervical spondylotic amyotrophy (CSA). The clinical features are limb spasticity, focal or multifocal limb weakness, wasting, dysarthria, dysphagia and dysmasesis [2]. In about 40% of patients, ALS first affects the upper limbs, and limb weakness is usually asymmetric in the early stages of the disease

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