The Importance of Flexion MRI in Hirayama Disease with Special Reference to Laminodural Space Measurements

Boruah, Deb Kumar; Prakash, Arjun; Gogoi, Bidyut B.; Yadav, Rahul; Dhingani, Dhabal D.; Sarma, Binod

doi:10.3174/ajnr.a5577

Cited by 36 publications

(63 citation statements)

References 22 publications

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“…The maximum forward shifting of posterior dura, measured on flexion MR postgadolinium fat-suppressed T1 WI in the mid-sagittal plane, as in Boruah et al [11] , was 6.5 mm.…”

Section: Case Reportmentioning

confidence: 59%

“…On neutral MRI, there was loss of physiological cervical lordosis, which is a common finding in patients with HD, although nonspecific [11] , [12] , [13] . Cervical curvature was assessed as illustrated by Chen et al [13 , 14] , by drawing a line from the inferior aspect of the C2 vertebral body to the inferior aspect of the C7 vertebral body and investigating the relationship of the dorsal aspect of the C3-C6 vertebral bodies to this line.…”

Section: Discussionmentioning

confidence: 97%

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Flexion MRI in a case of Hirayama disease

Lolli

Sârbu

Pezzullo

et al. 2020

Radiology Case Reports

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We report the case of an 18-year old male with a history of asymmetric weakness and amyotrophy of distal upper extremities, suggestive of Hirayama disease. Magnetic resonance imaging (MRI) of the cervical spine was obtained both in flexion and neutral position. Flexion MRI showed forward displacement of the dura and subsequent cord compression, with associated marked enlargement and postcontrast enhancement of posterior epidural plexus. These findings are pathognomonic of the disorder. On neutral MRI abnormalities may be subtle: in our case, they included loss of physiological lordosis, asymmetric atrophy and increased T2 signal intensity of the lower anterior cervical cord. The ability to identify abnormalities on neutral MRI however is even more important in that it allows the radiologist to include a flexion sequence in the MRI examination, if not specifically requested by the referring physician, and in cases in which the suspicion of the disorder has not been raised.

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“…The maximum forward shifting of posterior dura, measured on flexion MR postgadolinium fat-suppressed T1 WI in the mid-sagittal plane, as in Boruah et al [11] , was 6.5 mm.…”

Section: Case Reportmentioning

confidence: 59%

Section: Discussionmentioning

confidence: 97%

Flexion MRI in a case of Hirayama disease

Lolli

Sârbu

Pezzullo

et al. 2020

Radiology Case Reports

View full text Add to dashboard Cite

show abstract

“…This is thought to represent congestion of the posterior internal vertebral venous plexus as it disappears on neutral neck position [ 7 ]. The increase in the laminodural space and the presence of cervical cord flattening during flexion are essential for diagnosis as described by Boruah et al [ 16 ] in 45 patients with clinically definite “Hirayama disease.” He found that the laminodural space at maximum forward shifting of the posterior dural sac ranged from 3 to 9.8 mm, with a mean distance of 5.99 mm.…”

Section: Discussionmentioning

confidence: 99%

Monomelic Amyotrophy (Hirayama Disease): A Rare Case Report and Literature Review

2020

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Hirayama disease is a rare neurological entity that is characterized by initial progressive muscular wasting and weakness of the distal upper limb in young men, followed by a spontaneous arrest within several years. The disease is believed to be a result of forward displacement of the cervical dural sac and spinal cord induced by neck flexion. It is commonly seen in Asia and rarely encountered in the Middle East countries. We report a rare case of a 20-year-old Kuwaiti patient presenting with a 10-month duration of gradual left upper limb weakness and wasting. We describe his electrophysiological and radiological findings that confirmed the diagnosis, and conducted a literature review. Hirayama disease is rarely encountered in clinical settings and should be suspected in male patients presenting with unilateral or asymmetrical bilateral lower motor weakness of hands and forearms. It is a benign entity, and cervical collar is usually the only treatment needed in most cases.

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“…Characteristic findings include local cord atrophy, abnormal cervical spine curvature, and asymmetric cord flattening in the neutral position [ 6 ]. Loss of dural attachment, dorsal dural advancement, enlarged epidural space with flow void, and increase in laminodural space distance are the findings in the flexed position [ 6 , 7 ]. The proposed MRI protocol includes routine cervical spine sequences taken at a neutral position and with cervical spine flexion at 20° to 40° [ 4 , 6 ].…”

Section: Discussionmentioning

confidence: 99%

The diagnostic quandary of magnetic resonance imaging-negative Hirayama disease: a case report

et al. 2020

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Background: Magnetic resonance imaging (MRI) features are typical findings in Hirayama disease (HD) and are useful diagnostic entities but may not be present in all patients. Case presentation: We present the case of a 22-year-old Nepalese man who presented with insidious onset of weakness of his right upper limb of more than 5 years duration. His weakness was progressive for the first 3 years, and then remained static. On examination, weakness of the interossei, thenar, hypothenar, flexor, and extensor muscles were present in his right upper limb, power was normal in his left upper and bilateral lower limbs. Minipolymyoclonus was present in both upper limbs, less prominent on the left side. Electrophysiological findings showed motor axonal neuropathy in his right upper limb, neurogenic discharges and fibrillations, and fasciculations in both upper limbs. Contrast magnetic resonance imaging (MRI) of his cervical spine in flexion was normal. Our patient was diagnosed with HD based on clinical and electrophysiological findings. Our patient was advised to use a cervical collar and regular physiotherapy and was found to have subjective benefit. Conclusion: A normal cervical MRI does not rule out HD and the diagnosis can also be made based on clinical and electrophysiological studies. Progressive distal upper limb weakness or tremor in young patients should be evaluated for HD, because early diagnosis and intervention might halt the progression.

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The Importance of Flexion MRI in Hirayama Disease with Special Reference to Laminodural Space Measurements

Abstract: Flexion cervical MR imaging is a very useful investigation in diagnosing Hirayama disease. The increase in the laminodural space and the presence of cervical cord flattening during flexion are essential for diagnosis.

Cited by 36 publications

References 22 publications

Flexion MRI in a case of Hirayama disease

Flexion MRI in a case of Hirayama disease

Monomelic Amyotrophy (Hirayama Disease): A Rare Case Report and Literature Review

The diagnostic quandary of magnetic resonance imaging-negative Hirayama disease: a case report

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