Hirayama Disease with Proximal Involvement

Kim, Jin-Il; Kim, Yun-Tae; Kim, Sooa; Oh, Ki Young

doi:10.3346/jkms.2016.31.10.1664

Cited by 12 publications

(11 citation statements)

References 10 publications

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“…However, 8 years later, he visited a hospital with relapsed unilateral weakness of proximal upper limb. His neutral position neck MRI findings showed no abnormal cervical vertebrae, and anterior shifting of the spinal cord during cervical vertebrae flexion was not observed 7) . This is consistent with the report which showed that abnormal MRI results are lessened or even disappear naturally 12) .…”

Section: Discussionmentioning

confidence: 87%

“…In Korea, six cases have been reported from 2004 to 2016, but there was no nationwide survey. They were all male between ages from 15 to 29, and some of them showed atypical clinical features 7,[16][17][18][19] . Four of the six patients showed involvement of proximal upper extremities, while the other two showed involvement of distal upper extremities.…”

Section: Discussionmentioning

confidence: 99%

“…Bilateral symmetric involvement occurs in about 10% of cases 6) . Some reports have shown that Hirayama disease can affect the proximal portion as an initial presentation or a secondary progression 7) . Symptoms are related to neck flexion and often exacerbated by cold weather 3) .…”

Section: Introductionmentioning

confidence: 99%

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Two Cases of Hirayama Disease in a Pediatric Clinic

안현지¹,

염미선²,

김현진³

et al. 2018

journalofthekoreanchildneurologysociety

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We report two pediatric cases with Hirayama disease-a 16-year-old boy with a left wrist drop and a 14-year-old-boy with weakness and muscle atrophy of right hand. Motor nerve conduction study revealed decreased motor nerve action potential amplitudes in the ulnar nerve and radial nerve of the affected hands. The former patient showed normal magnetic resonance imaging (MRI) of the cervical spine, but the latter showed mild, asymmetric thinning of the anterior spinal cord at levels C5 to C7. Following active rehabilitation and avoidance of neck flexion, no further progression of neurological findings was noticed. These clinical findings were typical of Hirayama disease. We show that timely and accurate diagnosis for Hirayama disease is possible with awareness of disease history, careful physical examination, and the use of neurophysiological studies and MRI studies.

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Section: Discussionmentioning

confidence: 87%

Section: Discussionmentioning

confidence: 99%

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Two Cases of Hirayama Disease in a Pediatric Clinic

안현지¹,

염미선²,

김현진³

et al. 2018

journalofthekoreanchildneurologysociety

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“…[ 17 ] Ammendola et al ., in a study of three cases, did not show statistically significant differences for F-wave, SSEPs, and motor evoked potentials in standard conditions and during neck flexion both in HD patients and controls, suggesting that some cases of a complex disorder like HD might have a pathogenetic mechanism different from “flexion myelopathy.”[ 2 ] We evaluated the changes in N9–N20 interpeak latencies during neutral in all patients and flexed neck positions in three cases, without significant changes, as published in other reports. [ 1 , 2 , 11 , 17 , 18 ]…”

Section: Discussionmentioning

confidence: 99%

Somatosensory evoked potentials in Hirayama disease: A Brazilian study

Fustes¹,

Kay²,

Lorenzoni³

et al. 2020

Surgical Neurology International

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Background: Hirayama’s disease (HD) is characterized by an insidious onset asymmetric weakness and atrophy of the forearm and hand. Taking as a premise, the etiopathogenesis of the disease is attributed to forward displacement of posterior wall of lower cervical dural canal in neck flexion causing marked compression and flattening of lower spinal cord. This may result in compression of the posterior column of the spinal cord and seems likely to result in somatosensory evoked potentials (SSEPs) abnormalities. In the present study, we studied the possible involvement of the lemniscal dorsal pathway in patients with HD. Methods: SSEPs in upper and lower extremities were prospectively performed in eight patients with HD. All the patients were recruited from the outpatient clinic of a neuromuscular disorder center from South Brazil. SSEPs were obtained by transcutaneous electrical stimulation of the median and posterior tibial nerves, on both sides. We collected the amplitude and the latency of the different components obtained in each channel. The interpretation was based on Brazilian study standards. Results: We evaluated seven men and one woman (mean age 27). The data obtained were compared to a control group consisting of eight patients with spondylotic cervical myelopathy, 6 men and 2 women with mean age of 59 years. The measurements of obtained by the SSEP were also compared between the groups and no significant difference was found for any of them. Conclusion: SSEP did not turn out to be an electrophysiological marker in our HD patients.

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“…Repetitive microtraumas to the anterior horn motor neurons during neck exion are now considered as the possible pathogenesis of the disorder [10,16]. In view of this, cervical collars, which could restrain the neck exion, have been widely used in the treatment of HD and brought out favorable outcomes [4]. Although cervical collars are regarded as the mainstay of the treatment, surgical interventions are well established as an alternative for HD patients who are in advanced conditions, irresponsive to conservative treatment, or intolerant to cervical collars [5,8,12,15].…”

Section: Introductionmentioning

confidence: 99%

Could anterior cervical fixation be an alternative method in the management of Hirayama disease?

Li²,

Zhang³

et al. 2021

Preprint

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The study aimed to evaluate the clinical efficacy and feasibility of surgical treatment for Hirayama disease(HD) by anterior cervical fixation(ACF). From August 2008 to November 2015, 13 patients(12 males and 1 female, average age, 16.3 ± 1.1 years) with HD underwent ACF. Surgery-related indexes, clinical efficacy, and radiographic parameters were used to evaluate the clinical outcomes of surgery. The surgery duration time range from 67 to 128 min(average 89.2 ± 19.9 min)and the blood loss range from 40 to 120 ml(average 68.1 ± 26.3 ml). Four patients got resolved tremor, and the other patients stopped progressing. Grip strength significantly improved from preoperative 15.6 ± 1.7 kilogram to postoperative 18.6 ± 1.9 kilogram(P < 0.001). The cross-sectional areas of the spinal cord at C6 and C7 levels increased from 0.7 ± 0.1 cm2 to 0.8 ± 0.1 cm2(P = 0.018) and from 0.6 ± 0.2 cm2 to 0.7 ± 0.1 cm2(P = 0.049) respectively. The cervical lordosis increased from 2.4 ± 3.4° to 7.0 ± 3.5°(P = 0.001). The range of cervical flexion was significantly decreased from preoperative 31.3 ± 11.5° to postoperative 14.3 ± 7.5°(P < 0.001). No surgical related complications such as hoarseness, cerebral fluid leakage, and surgical site infection were observed after surgery. Two patients, felt abnormal sensation of throat occasionally, showed screw loosening and internal fixators were removed finally. Athough screw loosening was observed, ACF could serve as an effective treatment in the management of the HD.

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Hirayama Disease with Proximal Involvement

Cited by 12 publications

References 10 publications

Two Cases of Hirayama Disease in a Pediatric Clinic

Two Cases of Hirayama Disease in a Pediatric Clinic

Somatosensory evoked potentials in Hirayama disease: A Brazilian study

Could anterior cervical fixation be an alternative method in the management of Hirayama disease?

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