Hirayama Disease in an Adolescent Male With Right Hand Weakness and Muscle Wasting

Llano, Julian; Lall, Neil; Davis, Lamar; Steven, Andrew

doi:10.31486/toj.19.0045

Cited by 3 publications

(14 citation statements)

References 22 publications

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“…In approximately 10% of cases, it may manifest bilaterally, either symmetrically or asymmetrically [ 1 ]. Typically, the brachioradialis muscle is spared, leading to the characteristic "Oblique amyotrophy" [ 2 , 6 ]. However, in the case we presented, there was evident involvement of the brachioradialis muscle, in conjunction with forearm muscles, and relative sparing of small hand muscles, rendering our case particularly unusual.…”

Section: Discussionmentioning

confidence: 99%

“…Hirayama’s disease is generally self-limiting, with 95% of patients seeing a plateau in symptoms after five years [ 2 , 6 ]. Most patients, like ours, respond favorably to conservative management using a cervical collar.…”

Section: Discussionmentioning

confidence: 99%

“…Juvenile monomelic amyotrophy (JMA) is a distinctive variant of cervical myelopathy, also known as Hirayama's disease, juvenile non-progressive amyotrophy, and Soube's disease [1]. The condition was first described by K. Hirayama in 1959 [1][2][3][4][5][6]. Predominantly, this disorder affects young Asian males with a maleto-female ratio ranging from 7:1 to 20:1 [2].…”

Section: Introductionmentioning

confidence: 99%

“…The condition was first described by K. Hirayama in 1959 [1][2][3][4][5][6]. Predominantly, this disorder affects young Asian males with a maleto-female ratio ranging from 7:1 to 20:1 [2]. Typically, the onset of the disease is observed between the ages of 10 and 20, with the most pronounced presentation occurring between 20 and 30 years.…”

Section: Introductionmentioning

confidence: 99%

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Brachioradialis Involvement in Hirayama's Disease: An Atypical Presentation of a Rare Cervical Myelopathy

Malode,

Modi,

Seth

et al. 2023

Cureus

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Juvenile monomelic amyotrophy (JMA), also known as Hirayama's disease, is a rare cervical myelopathy that predominantly affects young Asian males. It is characterized by degeneration of anterior horn cells due to compression by the redundant dural sac. This study presents an atypical case of a 23-year-old Indian male who exhibited uncommon symptoms of JMA. The patient displayed progressive weakness and atrophy in the left forearm, including the usually spared brachioradialis muscle. Electrophysiological tests and MRI scans solidified the diagnosis of Hirayama's disease. After wearing a cervical collar for one year, the patient's condition stabilized, reinforcing the diagnosis. Unlike most JMA cases, this instance highlights the involvement of the brachioradialis muscle, underlining the variability in JMA presentations. A precise diagnosis is contingent upon clinical criteria, dynamic MRI, and electrophysiological findings. Recognizing these variations is crucial for early detection and appropriate management of the disease.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Brachioradialis Involvement in Hirayama's Disease: An Atypical Presentation of a Rare Cervical Myelopathy

Malode,

Modi,

Seth

et al. 2023

Cureus

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“…In classic Hirayama disease, a loss of normal cervical lordosis, focal kyphosis, and dorsal epidural fat results in anterior compression of the spinal cord in flexion, 18 resulting in episodic ischemic events that chronically damage cells in the anterior horn. 19 , 20 Over time, this may force the cervical spinal cord into a more anterior position within the spinal canal, causing increased tethering to the ventral vertebral bodies and an increase in dorsal epidural space. 14 This imposes increased mechanical stress on the cervical cord, which propagates further ischemic injury to the anterior region, resulting in progressive lower motor neuron type weakness.…”

Section: Discussionmentioning

confidence: 99%

Management of dynamic cervical kyphosis with dorsal epidural lipomatosis: a Hirayama disease variant? Illustrative case

Kundishora¹,

Reeves²,

Moreno-De-Luca

et al. 2023

Journal of Neurosurgery: Case Lessons

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BACKGROUND Hirayama disease, a cervical myelopathy characterized most commonly by a self-limiting atrophic weakness of the upper extremities, is a rare entity, scarcely reported in the literature. Diagnosis is made by spinal magnetic resonance imaging (MRI), which typically shows loss of normal cervical lordosis, anterior displacement of the cord during flexion, and a large epidural cervical fat pad. Treatment options include observation or cervical immobilization by collar or surgical decompression and fusion. OBSERVATIONS Here, the authors report an unusual case of a Hirayama-like disease in a young White male athlete who presented with rapidly progressive paresthesia in all 4 extremities and no weakness. Imaging showed characteristic findings of Hirayama disease as well as worsened cervical kyphosis and spinal cord compression in cervical neck extension, which has not previously been reported. Two-level anterior cervical discectomy and fusion and posterior spinal fusion improved both cervical kyphosis on extension and symptoms. LESSONS Given the disease’s self-limiting nature, and a lack of current reporting, there remains no consensus on how to manage these patients. Such findings presented here demonstrate the potentially heterogeneous MRI findings that can be observed in Hirayama disease and emphasize the utility of aggressive surgical management in young, active patients whereby a cervical collar may not be tolerated.

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Delayed Diagnosis of Hirayama Disease Due to an Antecedent History of Trauma: A Report of Two Cases

Kumar,

Johnson

et al. 2024

Indian Journal of Physical Medicine and Rehabilitation

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Hirayama illness is a rare neurological condition that first manifests as gradual muscular weakening and wasting of the distal upper limb in young males, followed within a few years by a spontaneous arrest. The spinal cord and cervical dural sac are thought to have been displaced forward by neck flexion, which is thought to be the cause of the condition. In contrast to the Middle East countries, it is more widespread in Asia. We have described here two cases from India, who presented with increasing weakness and wasting of the distal part of their upper limbs after a traumatic event. Although their symptoms were initially thought to be due to trauma, later, they were diagnosed as cases of Hirayama disease (HD), following detailed history and physical examination, radiological investigations and electrophysiological examination. A review of the literature was done which also described electrophysiological and radiographic findings, which supported our diagnosis. For young male patients who present with weakness and wasting of upper limb distal group muscles, HD should be investigated because it is infrequently seen in clinical settings.

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Hirayama Disease in an Adolescent Male With Right Hand Weakness and Muscle Wasting

Cited by 3 publications

References 22 publications

Brachioradialis Involvement in Hirayama's Disease: An Atypical Presentation of a Rare Cervical Myelopathy

Brachioradialis Involvement in Hirayama's Disease: An Atypical Presentation of a Rare Cervical Myelopathy

Management of dynamic cervical kyphosis with dorsal epidural lipomatosis: a Hirayama disease variant? Illustrative case

Delayed Diagnosis of Hirayama Disease Due to an Antecedent History of Trauma: A Report of Two Cases

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