Characteristics of Hirayama Disease in Young South Korean Soldiers
1
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Background and Purpose The purpose of this study was to describe the clinical presentation and features in electrodiagnostic and imaging investigations of young South Korean males diagnosed with Hirayama disease (HD). Methods We reviewed the electronic medical records of South Korean enlisted soldiers who were diagnosed with HD and discharged from military service during 2011–2021. We investigated the clinical characteristics and results of electrodiagnostic and magnetic resonance imaging (MRI) investigations. We analyzed laterality and identified the involved muscles using needle electromyography (EMG). Loss of lordosis, localized cervical cord atrophy, loss of attachment between the posterior dura and subjacent lamina, asymmetric flattening of the cord, crescent-shaped mass in the posterior epidural space, and noncompressive intramedullary T2- weighted high signal intensity were investigated using neutral- or flexion-position MRI. Results Forty-two male patients aged 20.2±0.8 years (mean±standard deviation) were identified. All patients complained of hand weakness, and 10 complained of hand tremor (23.8%). Four patients (9.5%) had symptoms in both upper limbs, and five (11.9%) had sensory disturbances. Needle EMG revealed that muscles in the C7–T1 myotome were commonly involved, and C5–C6 involvement of the deltoid (10.5%) and biceps brachii (12.5%) was also observed. In cervical MRI, localized cord atrophy (90.0%) was the most characteristic finding, and cord atrophy was most severe at the C5–C6 level (58.3%). Conclusions This is the first description of a large number of patients with HD in South Korea. The clinical presentation and features found in electrodiagnostic and imaging investigations will improve the understanding of HD in the young South Korean male population.
Abstract
Background and Purpose The purpose of this study was to describe the clinical presentation and features in electrodiagnostic and imaging investigations of young South Korean males diagnosed with Hirayama disease (HD). Methods We reviewed the electronic medical records of South Korean enlisted soldiers who were diagnosed with HD and discharged from military service during 2011–2021. We investigated the clinical characteristics and results of electrodiagnostic and magnetic resonance imaging (MRI) investigations. We analyzed laterality and identified the involved muscles using needle electromyography (EMG). Loss of lordosis, localized cervical cord atrophy, loss of attachment between the posterior dura and subjacent lamina, asymmetric flattening of the cord, crescent-shaped mass in the posterior epidural space, and noncompressive intramedullary T2- weighted high signal intensity were investigated using neutral- or flexion-position MRI. Results Forty-two male patients aged 20.2±0.8 years (mean±standard deviation) were identified. All patients complained of hand weakness, and 10 complained of hand tremor (23.8%). Four patients (9.5%) had symptoms in both upper limbs, and five (11.9%) had sensory disturbances. Needle EMG revealed that muscles in the C7–T1 myotome were commonly involved, and C5–C6 involvement of the deltoid (10.5%) and biceps brachii (12.5%) was also observed. In cervical MRI, localized cord atrophy (90.0%) was the most characteristic finding, and cord atrophy was most severe at the C5–C6 level (58.3%). Conclusions This is the first description of a large number of patients with HD in South Korea. The clinical presentation and features found in electrodiagnostic and imaging investigations will improve the understanding of HD in the young South Korean male population.
참고문헌 (32)
- Hirayama K, "Juvenile muscular atrophy of unilateral upper extremity", Neurology, 13, pp.373~380 (1963)
- Iacono S, "Hirayama disease : nosological classification and neuroimaging clues for diagnosis", J Neuroimaging, 32, pp.596~603 (2022)
- Wang H, "The radiological and electrophysiological characteristics of Hirayama disease with proximal involvement : a retrospective study", Front Neurol, 13, pp.969484~ (2022)
- Foster E, "Hirayama disease", J Clin Neurosci, 22, pp.951~954 (2015)
- Lyu F, "Establishment of a clinician-led guideline on the diagnosis and treatment of Hirayama disease using a modified Delphi technique", Clin Neurophysiol, 131, pp.1311~1319 (2020)
- Wang H, "Update on the pathogenesis, clinical diagnosis, and treatment of hirayama disease", Front Neurol, 12, pp.811943~ (2022)
- ". In: StatPearls" (2023)[URL]
- Elsheikh B, "Spinal angiography and epidural venography in juvenile muscular atrophy of the distal arm"Hirayama disease"", Muscle Nerve, 40, pp.206~212 (2009)
- Kwon O, "A Korean case of juvenile muscular atrophy of distal upper extremity(Hirayama disease)with dynamic cervical cord compression", J Korean Med Sci, 19, pp.768~771 (2004)
- Jung HJ, "Hirayama disease presenting as isolated triceps atrophy", J Clin Neurosci, 20, pp.1450~1451 (2013)
- Yoo SD, "Monomelic amyotrophy(hirayama disease)with upper motor neuron signs : a case report", Ann Rehabil Med, 39, pp.122~127 (2015)
- Kim J, "Hirayama disease with proximal involvement", J Korean Med Sci, 31, pp.1664~1667 (2016)
- Lee KH, "Clinical experiences of uncommon motor neuron disease : Hirayama disease", Korean J Spine, 13, pp.170~172 (2016)
- Kwon DY, "Progressive myoclonic tremor mimicking functional tremor in Hirayama disease", Acta Neurol Belg, 118, pp.517~518 (2018)
- Park JS, "The reversible effect of neck flexion on the somatosensory evoked potentials in patients with Hirayama disease : a preliminary study", Neurol Sci, 40, pp.181~186 (2019)
- Kim JY, "Polymyoclonus aggravated by neck flexion as the isolated presenting symptom of Hirayama disease : case report", BMC Neurol, 20, pp.325~ (2020)
- Kim KE, "Prevalence of neuromuscular diseases in young South Korean males; a Korean Military Manpower Administration and Medical Command data-based study", J Clin Neurol, 19, pp.565~572 (2023)
- Chen CJ, "Hirayama flexion myelopathy : neutral-position MR imaging findings--importance of loss of attachment", Radiology, 231, pp.39~44 (2004)
- Tashiro K, "Nationwide survey of juvenile muscular atrophy of distal upper extremity(Hirayama disease)in Japan", Amyotroph Lateral Scler, 7, pp.38~45 (2006)
- Nalini A, "Monomelic amyotrophy : clinical profile and natural history of 279 cases seen over 35 years(1976-2010)", Amyotroph Lateral Scler Frontotemporal Degener, 15, pp.457~465 (2014)
- Huang YC, "A clinical study of Hirayama disease in Taiwan", Muscle Nerve, 37, pp.576~582 (2008)
- Gourie-Devi M, "Sympathetic skin response in monomelic amyotrophy", Acta Neurol Scand, 104, pp.162~166 (2001)
- Robberecht W, "Familial juvenile focal amyotrophy of the upper extremity(Hirayama disease). Superoxide dismutase 1 genotype and activity", Arch Neurol, 54, pp.46~50 (1997)
- Gourie-Devi M, "Long-term follow-up of 44 patients with brachial monomelic amyotrophy", Acta Neurol Scand, 107, pp.215~220 (2003)
- Biondi A, "MR Imaging of the cervical cord in juvenile amyotrophy of distal upper extremity", AJNR Am J Neuroradiol, 10, pp.263~268 (1989)
- Raval M, "MRI findings in Hirayama disease", Indian J Radiol Imaging, 20, pp.245~249 (2010)
- Paladi B, "Role of dynamic magnetic resonance imaging in Hirayama disease, a rare motor neuron disease", Cureus, 14, pp.e31099~ (2022)
- Kikuchi S, "A mechanism of juvenile muscular atrophy localized in the hand and forearm (Hirayama’s disease)--flexion myelopathy with tight dural canal in flexion", Rinsho Shinkeigaku, 27, pp.412~419 (1987)
- Schröder R, "MRI findings in Hirayama’s disease : flexion-induced cervical myelopathy or intrinsic motor neuron disease?", J Neurol, 246, pp.1069~1074 (1999)
- Willeit J, "Juvenile asymmetric segmental spinal muscular atrophy(Hirayama’s disease) : three cases without evidence of"flexion myelopathy"", Acta Neurol Scand, 104, pp.320~322 (2001)
- Sonwalkar HA, "Imaging features in Hirayama disease", Neurol India, 56, pp.22~26 (2008)
- Vargas MC, "Magnetic resonance imaging in Hirayama disease", J Radiol Case Rep, 5, pp.17~23 (2011)