Chaojun Zheng

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

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.

A study of dynamic F-waves in juvenile spinal muscular atrophy of the distal upper extremity (Hirayama disease)

OBJECTIVE The study aimed to analyse changes in the upper limb F-waves during neck flexion in patients with Hirayama disease (HD). MATERIAL AND METHODS This study included 41 healthy subjects, 38 HD patients and 24 patients with amyotrophic lateral sclerosis (ALS). Bilateral F-waves were consecutively recorded 20 times with the neck both in the standard position and after persistent neck flexion for 30min. The persistence, minimal latencies, chronodispersion, F/M ratios and amplitudes of the F-waves and repeater F-waves were compared between the standard neck and neck flexion positions. RESULTS During neck flexion, repeater F-waves were found in more HD patients, the percentage of both the ulnar and median repeater F-waves increased significantly, and higher F/M ratios were observed on the symptomatic side (P<0.05). No differences in the F-waves were observed in the healthy subjects or the ALS patients during neck flexion (P>0.05). DISCUSSION HD might be more likely to present as a position-related dysfunction rather than a spinal cord-intrinsic disease. Thus, HD patients could be counselled to avoid neck flexion for long periods of time to prevent further damage, especially in the progressive stage of the disease.

Compound Muscle Action Potential Decrement to Repetitive Nerve Stimulation Between Hirayama Disease and Amyotrophic Lateral Sclerosis.

Purpose: To compare repetitive nerve stimulation (RNS) between Hirayama disease (HD) and amyotrophic lateral sclerosis (ALS). Methods: The 3-Hz RNS test was performed on bilateral abductor pollicis brevis, abductor digiti minimi, middle deltoid, and upper trapezius muscles in 33 HD patients and 37 ALS patients. Results: In HD patients, none of tested muscles showed any abnormal decrements (≥10%). Significant decrements were observed in 73% of the ALS patients, and decrements were more frequently observed in proximal muscles (deltoid: 70.3%; trapezius: 48.6%). Illness duration did not correlate with decrement percentage in either patient group, and there was no relationship between decrement incidence and ALS diagnostic category (P > 0.05). Conclusions: The significantly different RNS results between ALS and HD patients support the application of RNS, especially performing RNS in proximal muscles, as a supplementary test in distinguishing these two diseases, even in the early stages. These results may also imply a difference in underlying pathophysiology between ALS and HD.

Diagnostic advantage of S1 foramen-evoked H-reflex for S1 radiculopathy in patients with diabetes mellitus

Hoffmann reflex to tibial nerve stimulation at the popliteal fossa (tibial H-reflex) is routinely used to evaluate S1 radiculopathy. However, it lacks sensitivity because other lesions along this reflex circuit affect the H-reflex bilaterally. This study was undertaken to determine whether the H-reflex evoked by stimulating proximally at the S1 foramen (S1 foramen H-reflex) could improve S1 root lesion evaluation sensitivity in patients with diabetes mellitus. A randomized paired study was designed to evaluate tibial and S1 foramen H-reflexes; bilateral H-M interval (HMI) and H-reflex latency were compared in 22 diabetic patients with unilateral S1 radiculopathy. Other electrophysiological evaluations included standard tibial conduction studies, sural conduction studies and needle electromyography (EMG). The S1 foramen H-reflex had a significantly higher sensitivity (91.0%, 20 of 22) in evaluating S1 radiculopathy than the conventional tibial H-reflex (63.6%, 14 of 22, p < 0.05). Bilateral tibial compound muscle action potential amplitudes were reduced in 3 patients, and sural sensory nerve action potential amplitudes decreased in 7 patients. Needle EMG revealed denervation restricted to the paraspinal muscle and myotomes supplied by the S1 nerve root on the ipsilateral side in 18 patients, and multiple lumbosacral nerve roots were involved bilaterally in the other 4 patients. Our results demonstrate that the S1 foramen H-reflex is a more sensitive assessment of S1 compressive radiculopathy in patients with diabetes mellitus.

The Quantitative Assessment of Imaging Features for the Study of Hirayama Disease Progression

Objective. To evaluate the forward shifting of cervical spinal cords in different segments of patients with Hirayama disease to determine whether the disease is self-limiting. Methods. This study was performed on 11 healthy subjects and 64 patients. According to the duration, the patients were divided into 5 groups (≤1 year, 1-2 years, 2-3 years, 3-4 years, and ≥4 years). Cervical magnetic resonance imaging (MRI) of flexion and conventional position was performed. The distances between the posterior edge of the spinal cord and the cervical spinal canal (X), the anterior and posterior wall of the cervical spinal canal (Y), and the anterior-posterior (A) and the transverse diameter (B) of spinal cord cross sections were measured at different cervical spinal segments (C4 to T1). Results. In cervical flexion position, a significant increase in X/Y of C4-5 segments was found in groups 2–5, the C5-6 and C6-7 segments in groups 1–5, and the C7-T1 segments in group 5 (P < 0.05). The degree of the increased X/Y and cervical flexion X/Y of C5-6 segments were different among the 5 groups (P < 0.05), which was likely due to rapid increases in X/Y during the course of Hirayamas disease. Conclusion. The X/Y change progression indicates that Hirayama disease may not be self-limiting.

Motor unit number estimation in the quantitative assessment of severity and progression of motor unit loss in Hirayama disease

OBJECTIVE To investigate motor unit number estimation (MUNE) as a method to quantitatively evaluate severity and progression of motor unit loss in Hirayama disease (HD). METHODS Multipoint incremental MUNE was performed bilaterally on both abductor digiti minimi and abductor pollicis brevis muscles in 46 patients with HD and 32 controls, along with handgrip strength examination. MUNE was re-evaluated approximately 1year after initial examination in 17 patients with HD. RESULTS The MUNE values were significantly lower in all the tested muscles in the HD group (P<0.05). Despite abnormally low MUNE values, 54.3% (25/46) of patients with HD had normal ipsilateral grip power. There was a significant inverse correlation between MUNE values and disease duration (P<0.05). A longitudinal follow-up MUNE analysis demonstrated slow progression of motor unit loss in patients with HD within approximately 1year (P<0.05), even in patients with an illness duration >4years. CONCLUSIONS A reduction in the functioning motor units was found in patients with HD compared with that in controls, even in the early asymptomatic stages. Moreover, the motor unit loss in HD progresses gradually as the disease advances. SIGNIFICANCE These results have provided evidence for the application of MUNE in estimating the reduction of motor unit in HD and confirming the validity of MUNE for tracking the progression of HD in a clinical setting.

Abnormal flexor carpi radialis H-reflex as a specific indicator of C7 as compared with C6 radiculopathy.

Objective: The H-reflex of the flexor carpi radialis (FCR H-reflex) has not been commonly used for the diagnosis of cervical radiculopathy when compared with the routinely tested soleus H-reflex. Although both S1 and S2 roots innervate the soleus, the H-reflex is selectively related to S1 nerve root function clinically. Flexor carpi radialis is also innervated by two nerve roots which are C6 and C7. Although they are among the most common roots involved in cervical radiculopathy, few studies reported if the attenuation of the FCR H-reflex is caused by lesions affecting C7 or C6 nerve roots, or both. We aimed to identify whether an abnormal FCR H-reflex was attributed to the C7 or C6 nerve root lesion, or both. The sensitivities of needle electromyography, FCR H-reflex, and provocative tests in unilateral C7 or C6 radiculopathy were also compared in this study. Methods: A concentric needle electrode recorded bilateral FCR H-reflexes in 41 normal subjects (control group), 51 patients with C7 radiculopathy, and 54 patients with C6 radiculopathy. Clinical, radiological, and surgical approaches identified the precise single cervical nerve root involved in all patient groups. The H-reflex and M-wave latencies were measured and compared bilaterally. Abnormal FCR H-reflex was defined as the absence of the H-reflex or a side-to-side difference over 1.5 milliseconds which was based on the normal side-to-side difference of the H-reflex latency of 16.9 milliseconds (SD = 1.7 milliseconds) from the control group. We also determined standard median and ulnar conduction and needle electromyography. The provocative tests included bilateral determination of the Shoulder Abduction and Spurlings tests in all radiculopathy group patients. Results: Abnormal FCR H-reflexes were recorded in 45 (88.2%) of C7 radiculopathy group patients, and 2 (3.7%) of C6 radiculopathy group patients (P < 0.05). Needle electromyography was abnormal in 41 (80.4%) of C7 radiculopathy patients and 43 (79.6%) of C6 radiculopathy patients. Provocative tests were positive in 15 (29.4%) of C7 radiculopathy patients and 25 (46.3%) of C6 radiculopathy patients. Conclusions: Flexor carpi radialis H-Reflex provides a sensitive assessment of evaluating the C7 spinal reflex pathway. Clinically, a combination of the FCR H-reflex with needle electromyography may yield the highest level of diagnostic information for evaluating clinical cases of C7 radiculopathy.

Proximally evoked soleus H‐reflex to S1 nerve root stimulation in sensory neuronopathies (ganglionopathies)

Sensory neuronopathy (SNN) mimics distal sensory axonopathy. The conventional H‐reflex elicited by tibial nerve stimulation (tibial H‐reflex) is usually abnormal in both conditions. We evaluated the proximally evoked soleus H‐reflex in response to S1 nerve root stimulation (S1 foramen H‐reflex) in SNN.

Trans-synaptic degeneration of motoneurons distal to chronic cervical spinal cord compression in cervical spondylotic myelopathy

ABSTRACT Objective: To assess the effect of chronic cervical spinal cord compression upon remote motor unit function in patients with cervical spondylotic myelopathy (CSM). Methods: Fifty-three CSM patients and 47 healthy subjects were included. Bilateral motor unit number estimations (MUNEs) were recorded from both abductor digiti minimi and abductor pollicis brevis, and bilateral flexor carpi radialis (FCR) H-reflexes were examined in all subjects along with the nine-hole peg test (NHPT). The main outcome measures included the number of motor units, the average single motor unit potential (SMUP) area, the FCR Hmax/Mmax ratios and the NHPT time. Results: Statistically significant results compared to healthy controls included increased average SMUP area, increased FCR Hmax/Mmax ratio and increased NHPT time (p < 0.05). Abnormal SMUP was observed in 10/53 (18.9%) CSM patients along with reduced motor units in 3 of these 10 patients, while the FCR Hmax/Mmax ratios in the CSM patients with abnormal MUNE were higher than those in others (p < 0.05). There was a positive correlation between the NHPT time and the average SMUP area, and a negative correlation was noted between the NHPT time and the number of motor units (p < 0.05). Conclusion: In CSM patients, the motor units below the level of compression may exhibit dysfunction, which is likely a result of trans-synaptic degeneration. Both cervical spinal cord compressive injury and this trans-synaptic degeneration contribute to the impairment of fine motor ability in CSM patients. Therefore, treatment and rehabilitation efforts should account for these two dysfunctions.

A double determination of central motor conduction time in the assessment of Hirayama disease

OBJECTIVE To investigate central motor conduction time (CMCT) in patients with Hirayama disease (HD) and to analyse the role of motor nerve root lesions in the pathogenesis of HD. METHODS CMCT measured by F-wave (CMCT-F) and by paravertebral magnetic stimulation (CMCT-M) was performed on both abductor pollicis brevis (APB) and abductor digiti minimi (ADM) in 41 HD patients and 22 controls. All patients underwent neck-flexion magnetic resonance imaging evaluation. RESULTS Prolonged CMCT (CMCT-F and/or CMCT-M) was recorded in at least one tested muscle from 7/41 (17.1%) HD patients, and 4 cases presented significant prolonged CMCT-M with normal CMCT-F on the side with significant cervical cord forward-shifting. This asymmetric forward-shifting was identified in 13 HD patients, and forward-shifting on the symptomatic side was more obvious. Compared to the controls (ADM: 0.9±0.3ms; APB: 0.8±0.3ms) and the other 28 HD patients (symptomatic side: ADM: 0.8±0.2ms, APB: 0.8±0.3ms), increased nerve root conduction times were demonstrated in these symptomatic sides (ADM: 1.5±0.7ms; APB: 1.2±0.6ms) (P<0.05). CONCLUSIONS Motor nerve root may be main lesion site in some HD patients, especially on the symptomatic side of patients with asymmetric neck-flexion cervical cord forward-shifting. SIGNIFICANCE Compared to spinal motor neuron lesions, damage to motor nerve root (intra- and/or extra-medullary motor roots) may play an equally important role in the pathogenesis of HD. Abnormally increased forward traction in shorter nerve roots may be the cause for the main damage in motor nerve root.