Cervical cord dysfunction during neck flexion in Hirayama’s disease

Restuccia, Domenico; Rubino, Marco; Valeriani, Massimiliano; Mirabella, Massimiliano; Sabatelli, Mario; Tonali, P.

doi:10.1212/01.wnl.0000068017.75780.44

Cited by 50 publications

(34 citation statements)

References 10 publications

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“…7 9 Significant amplitude reduction of N13 cervical somatosensory evoked potential (SEP) on neck flexion has been attributed to cord compression or microvascular changes during neck flexion. 10 Spinal MRI in cervical flexion showed forward displacement of dural sac and compressive flattening of lower cervical cord. Cinematographic magnetic resonance imaging (MRI) showed signal void in posterior epidural space pulsating synchronously with cardiac beat suggesting passive dilatation of epidural venous plexus.…”

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confidence: 97%

Effect of neck flexion on F wave, somatosensory evoked potentials, and magnetic resonance imaging in Hirayama disease

Misra

2006

Journal of Neurology, Neurosurgery & Psychiatry

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Background: Flexion myelopathy is one of the suggested mechanism for Hirayama disease (HD) but simultaneous radiological and neurophysiological evaluation is lacking. This study therefore evaluates the effect of neck flexion in HD using somatosensory evoked potentials (SEPs), F waves, and magnetic resonance imaging (MRI). Method: Eight HD patients and seven matched controls were subjected to median and ulnar F wave (minimal latency, FM ratio, persistence, and chronodispersion), and SEPs evaluating N9, N13, and N20 potentials in neutral and neck flexion. Spinal MRI was carried out in neutral and neck flexion and evaluated for cord atrophy, signal changes, cord compression, posterior epidural tissue, and loss of dural attachment. Results: The patients were aged 19 to 30 years. Minimal F latency, FM ratio, persistence, and chronodispersion in neutral and neck flexion did not show any change nor was there any change in N13 latency and amplitude on median and ulnar SEPs. The difference in these parameters in neutral and neck flexion were also not significant in HD compared with controls. The change in N13 was also not related to loss of dural attachment and posterior epidural tissue. Conclusion: Neck flexion does not produce significant changes in N13 and F wave parameters and is not related to dynamic MRI changes. The other mechanisms for HD should therefore be explored.

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confidence: 97%

Effect of neck flexion on F wave, somatosensory evoked potentials, and magnetic resonance imaging in Hirayama disease

Misra

2006

Journal of Neurology, Neurosurgery & Psychiatry

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“…As postulated in BMMA, in DBMA also probably the short cervical dural canal is unable to compensate for the flexion related increased length of the vertebral canal (18). However, it is difficult to understand how the mechanical compression of an elliptical spinal cord could induce damage to unilateral or bilateral anterior horn cells, and yet remain in the restricted form for decades.…”

Section: Discussionmentioning

confidence: 99%

Distal bimelic amyotrophy (DBMA): Phenotypically distinct but identical on cervical spine MR imaging with brachial monomelic amyotrophy/Hirayama disease

Preethish‐Kumar

Nalini

Singh

et al. 2015

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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Our objective was to characterize the MR imaging features in a large and distinct series of distal bimelic amyotrophy (DBMA) from India. We utilized a retrospective and prospective study on 26 cases. Results demonstrated that upper limb distal muscle wasting and weakness was predominantly symmetrical in onset. Mean age at onset was 20.9 ± 7.0 years, mean duration 83.0 ± 102.6 months. MRI carried out in 22 patients with flexion studies showed forward displacement of posterior dura in 19 (86.4%). Crescent shaped epidural enhancement on contrast was seen in 20/24 cases (83.3%), and bilateral T2W hyperintensities of cord in17 (65.4%) - symmetrical in15 cases. Maximum hyperintensity was noted at C5-C6, C6-C7 levels. Cord atrophy was noted in 24 (92.3%) cases (most affected: C5-C6, C6-C7) - symmetrical atrophy in 21cases. Cervical spine straightening occurred in six (23.1%) cases and reversal of lordosis in 15 (57.7%). In conclusion, this study confirms that DBMA is phenotypically distinct but pathophysiologically the same as brachial monomelic amyotrophy (BMMA) on MR imaging. Typical MRI features were seen in all. It is important to differentiate this disorder from ALS, which could present at a younger age as often seen among Indians. The clinical and MR imaging features are highly suggestive that DBMA, as with BMMA/Hirayama disease, occurs due to dynamic alterations at the cervical spine level.

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“…Forward migration of the posterior surface of the dura mater with compression of the spinal cord on MRI obtained with flexion of the cervical spine is characteristic 2,3 . Although neck flexion has been implicated in the physiopathology of JADUE (the disorder was termed "flexion myelopathy" by Kikuchi 5 , the underlying mechanism is still controversial 1,5,8,9 . Some authors consider that JADUE is not a flexion-induced cervical myelopathy, but an intrinsic motor neuron disease.…”

Section: Discussionmentioning

confidence: 99%

“…Since neck flexion has been thought to be the cause of the spinal cord damage, cervical orthosis, spinal fusion by an anterior or posterior approach and duraplasty in com- bination with posterior spinal fusion have been proposed as treatment of JADUE 7 . However, the imaging findings observed in dynamic studies have shown, not only the absence of hypermobility in the cervical spine, but also the increase in the rigidity of the dura as a cause of spinal cord compression 2,3,5,7,9 . Histopathological findings have also suggested that alterations in the laxity of the dural tissue are the basic cause leading to a repeated spinal cord compression and, thus, to spinal cord damage 7 .…”

Section: Introductionmentioning

confidence: 99%

A case of Hirayama disease treated with laminectomy and duraplasty without spinal fusion

Arrese

Rivas²,

Esteban

et al. 2009

Neurocirugía

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Presentación de un caso de enfermedad de Hirayama tratado mediante laminectomía y duraplastia sin fusión espinal ResumenIntroducción. La amiotrofia juvenil de extremidad superior distal (AJESD) es una rara enfermedad que afecta a varones jóvenes. Desde que la flexión del cuello fue reconocida como causa de la enfermedad, han sido empleadas como tratamiento las ortosis cervicales, la fusión vertebral y la duraplastia asociada a fusión. Presentamos un caso de AJESD que fue tratado quirúrgicamente mediante duroplastia sin fusión vertebral cervical, de tal forma que la compresión sobre la médula fue eliminada sin limitar la movilidad del cuello.Caso. Varón de 19 años con un cuadro lentamente progresivo de debilidad en antebrazo y mano izquierda. A la exploración se apreció moderada atrofia de los músculos interóseos dorsales izquierdos. El electromiograma mostraba denervación aguda de los músculos interóseos dorsales, así como cambios reinervativos crónicos en otros músculos. La MR objetivó desplazamiento anterior de la duramadre cervical cuando el cuello era flexionado que provocaba compresión medular a dicho nivel. Se realizó laminectomía y duroplastia C3-C7 sin añadir ninguna técnica encaminada a la fusión vertebral.Discusión. El postoperatorio cursó sin incidencias y la progresión sintomática cesó. Dos años tras la cirugía el paciente continua sin cambios.PALABRAS CLAVE: Enfermedad de Hirayama. Amiotrofia juvenil. Laminectomía. Duroplastia.

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Cervical cord dysfunction during neck flexion in Hirayama’s disease

Cited by 50 publications

References 10 publications

Effect of neck flexion on F wave, somatosensory evoked potentials, and magnetic resonance imaging in Hirayama disease

Effect of neck flexion on F wave, somatosensory evoked potentials, and magnetic resonance imaging in Hirayama disease

Distal bimelic amyotrophy (DBMA): Phenotypically distinct but identical on cervical spine MR imaging with brachial monomelic amyotrophy/Hirayama disease

A case of Hirayama disease treated with laminectomy and duraplasty without spinal fusion

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