scholarly journals Hirayama disease. Clinical cases

2021 ◽  
pp. 22-26
Author(s):  
Svitlana Shkrobot ◽  
Maryna Salii ◽  
Zoia Salii ◽  
Yuriy Heryak
Keyword(s):  
Spinal Cord ◽  
Clinical Picture ◽  
Venous Stasis ◽  
Resonance Imaging ◽  
Forward Displacement ◽  
Hirayama Disease ◽  
Dural Sac ◽  
Examination Methods ◽  
Neurological Pathology ◽  
Clinical Cases

Hirayama disease, or monomelic amyotrophy, is a rare neurological pathology manifested by unilateral or bilateral asymmetric paresis with atrophy of the distal upper extremities. The development of this disease is associated with the forward displacement of the posterior dural sac during neck flexion, which leads to compression of the spinal cord and venous stasis. The diagnosis of monomelic amyotrophy is based on the clinical picture and the results of magnetic resonance imaging of the cervical spine with flexion, showing segmental atrophy of the anterior horns of the spinal cord at C7 — Th1, detachment of the posterior dura mater and venous stasis. Most of the cases described in the literature are from India and Japan, with isolated cases diagnosed in North America and Europe. This article presents two clinical cases of progressive hand weakness in young men. The clinical picture of the disease and the results of additional examination methods are presented, which is necessary to establish a diagnosis of Hirayama’s disease.

A severe case of Hirayama disease successfully treated by anterior cervical fusion

2014 ◽  
Vol 20 (2) ◽  
pp. 191-195 ◽  
Author(s):  
Igor Paredes ◽  
Jesus Esteban ◽  
Ana Ramos ◽  
Pedro Gonzalez ◽  
Juan José Rivas
Keyword(s):  
Spinal Cord ◽  
Severe Case ◽  
Cord Compression ◽  
Severe Form ◽  
Cervical Fusion ◽  
Neck Flexion ◽  
Forward Displacement ◽  
Hirayama Disease ◽  
Dural Sac ◽  
Unilateral Weakness

Hirayama disease, or juvenile amyotrophy of distal upper extremity, is a benign, self-limiting cervical myelopathy consisting of selective unilateral weakness of the hand and forearm. The weakness slowly progresses until spontaneous arrest occurs within 5 years of onset. The condition predominantly affects Asian males and is thought to be secondary to spinal cord compression during neck flexion, because of a forward displacement of the posterior dural sac. The authors present what is to their knowledge the first reported case of a Caucasian male with a severe form of Hirayama disease, suffering from weakness of the leg as well as the forearm. An abnormal range of cervical flexion was observed at the C5–6 level. The patient was successfully treated by anterior cervical discectomy and fusion.

scholarly journals Myelopolyradiculoneuritis with lesions predominantly across the spinal cord

2001 ◽  
Vol XXXIII (3-4) ◽  
pp. 52-54
Author(s):  
V. V. Ponomarev ◽  
V. I. Khodulev
Keyword(s):  
Magnetic Resonance Imaging ◽  
Spinal Cord ◽  
Transverse Myelitis ◽  
International Classification Of Diseases ◽  
Resonance Imaging ◽  
Acute Transverse Myelitis ◽  
Classification Of Diseases ◽  
Examination Methods ◽  
Magnetic Resonance Imaging Mri

Acute primary myelitis is an inflammatory disease of the spinal cord. Transverse and multifocal myelitis is distinguished, differing in the size and number of foci of inflammation in individual segments of the spinal cord. Acute transverse myelitis is considered as a separate nosological form in the 10th revision of the International Classification of Diseases. The introduction of such modern paraclinical examination methods as electroneuromyography (ENMG), magnetic resonance imaging (MRI), make it possible to visualize the morphological substrate of the disease, to better understand the nature and mechanisms of its development.

scholarly journals Hirayama Disease: Case report of a rare case in Brazil

2021 ◽  
Author(s):  
Larissa Damian Resende ◽  
Thiago de Abreu Silva Pinto ◽  
Henrique Marreiros Veloso Carneiro ◽  
Fellype Matos do Prado
Keyword(s):  
Case Report ◽  
Upper Limb ◽  
Spinal Canal ◽  
Rare Case ◽  
Clinical History ◽  
Resonance Imaging ◽  
Hirayama Disease ◽  
Dural Sac ◽  
Disease Case ◽  
The Right

Context: Hiravama’s disease (HD) is a rare neurological condition described by keizo Hirayama in 1959, in which growth of the content of the spinal canal displaces the posterior dural sac previously when flexing the neck at the level of C7 and T1. Thus, it generates ischemic damage in the cells of the anterior spine. In this context, we present a case report about the disease. Case report: Male, 13 years old, complaining of difficulty in grasping his right hand for 5 months and atrophy in his right upper limb for 1 month. On physical examination, the neurological changes found were: Decreased strength of the right upper limb distally, the deep hypoactive reflexes in both upper limbs. Magnetic resonance imaging of the flexed cervical spine showed stenosis of the C5 to T1 spinal canal, flow-voids prominence in the posterior epidural space (suggestive of venous engorgement), tapering and alteration of T2 signal in the C5-C6 medulla. These findings and along with the clinical history confirmed the diagnosis of HD. Conclusion: HD is a rare disease that needs to be known. Thus, the present study expands the database about the disease, which if diagnosed early, improves the patient’s prognosis.

scholarly journals Hirayama disease: a rare neurological condition of cervical cord pathology

2017 ◽  
Vol 3 (4) ◽  
pp. 886
Author(s):  
Abhilash Palla ◽  
Vinay Jain K. ◽  
Naveen Kumar L. ◽  
Rangaswamy B. T.
Keyword(s):  
Muscle Weakness ◽  
Upper Limb ◽  
Cervical Cord ◽  
Neck Flexion ◽  
Forward Displacement ◽  
Hirayama Disease ◽  
Progressive Muscle Weakness ◽  
Dural Sac ◽  
Distal Muscle ◽  
High Degree

<p>The disease is characterized by a progressive muscle weakness and wasting of distal upper limb muscles sparing brachioradialis. The pathology is in the lower cervical cord due to forward displacement of dural sac and spinal cord by neck flexion. Repeated neck flexion may result in ischemia of the lower cervical anterior horns. The diagnosis can be confirmed by MRI, EMG. It requires a high degree of suspicion in a young patient presenting with unilateral upper limb distal muscle weakness with no sensory involvement, as early diagnosis will help in preventing the progression of the disease by employing simple measures like usage of a cervical collar. We report a case of a 25 year old man presenting with progressive distal upper limb muscle wasting, on investigating further diagnosed to have Hirayama disease.</p>

scholarly journals Changes of Dorsal Root Ganglion Volume in Dogs with Clinical Signs of Degenerative Myelopathy Detected by Water-Excitation Magnetic Resonance Imaging

Animals ◽  
2021 ◽  
Vol 11 (6) ◽  
pp. 1702
Author(s):  
Eiji Naito ◽  
Kohei Nakata ◽  
Yukiko Nakano ◽  
Yuta Nozue ◽  
Shintaro Kimura ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Spinal Cord ◽  
Magnetic Resonance ◽  
Clinical Signs ◽  
Intervertebral Disc Herniation ◽  
Resonance Imaging ◽  
Diagnostic Potential ◽  
Water Excitation ◽  
Spinal Cord Segment ◽  
Degenerative Myelopathy

Canine degenerative myelopathy (DM) is a progressive and fatal neurodegenerative disease. However, a definitive diagnosis of DM can only be achieved by postmortem histopathological examination of the spinal cord. The purpose of this study was to investigate whether the volumetry of DRG using the ability of water-excitation magnetic resonance imaging (MRI) to visualize the DRG in dogs has premortem diagnostic value for DM. Eight dogs with DM, twenty-four dogs with intervertebral disc herniation (IVDH), and eight control dogs were scanned using a 3.0-tesla MRI system, and water-excitation images were obtained to visualize and measure the volume of DRG, normalized by body surface area. The normalized mean DRG volume between each spinal cord segment and mean volume of all DRG between T8 and L2 in the DM group was significantly lower than that in the control and the IVDH groups (P = 0.011, P = 0.002, respectively). There were no correlations within the normalized mean DRG volume between DM stage 1 and stage 4 (rs = 0.312, P = 0.128, respectively). In conclusion, DRG volumetry by the water-excitation MRI provides a non-invasive and quantitative assessment of neurodegeneration in DRG and may have diagnostic potential for DM.

Multisession CyberKnife Radiosurgery for Intramedullary Spinal Cord Arteriovenous Malformations

Neurosurgery ◽  
2006 ◽  
Vol 58 (6) ◽  
pp. 1081-1089 ◽  
Author(s):  
John Sinclair ◽  
Steven D. Chang ◽  
Iris C. Gibbs ◽  
John R. Adler
Keyword(s):  
Magnetic Resonance Imaging ◽  
Spinal Cord ◽  
Magnetic Resonance ◽  
Arteriovenous Malformations ◽  
Conus Medullaris ◽  
Spinal Angiography ◽  
Resonance Imaging ◽  
Intramedullary Spinal Cord ◽  
Cyberknife Radiosurgery

Abstract OBJECTIVE: Intramedullary spinal cord arteriovenous malformations (AVMs) have an unfavorable natural history that characteristically involves myelopathy secondary to progressive ischemia and/or recurrent hemorrhage. Although some lesions can be managed successfully with embolization and surgery, AVM size, location, and angioarchitecture precludes treatment in many circumstances. Given the poor outlook for such patients, and building on the successful experience with radiosurgical ablation of cerebral AVMs, our group at Stanford University has used CyberKnife (Accuray, Inc., Sunnyvale, CA) stereotactic radiosurgery (SRS) to treat selected spinal cord AVMs since 1997. In this article, we retrospectively analyze our preliminary experience with this technique. METHODS: Fifteen patients with intramedullary spinal cord AVMs (nine cervical, three thoracic, and three conus medullaris) were treated by image-guided SRS between 1997 and 2005. SRS was delivered in two to five sessions with an average marginal dose of 20.5 Gy. The biologically effective dose used in individual patients was escalated gradually over the course of this study. Clinical and magnetic resonance imaging follow-up were carried out annually, and spinal angiography was repeated at 3 years. RESULTS: After a mean follow-up period of 27.9 months (range, 3–59 mo), six of the seven patients who were more than 3 years from SRS had significant reductions in AVM volumes on interim magnetic resonance imaging examinations. In four of the five patients who underwent postoperative spinal angiography, persistent AVM was confirmed, albeit reduced in size. One patient demonstrated complete angiographic obliteration of a conus medullaris AVM 26 months after radiosurgery. There was no evidence of further hemorrhage after CyberKnife treatment or neurological deterioration attributable to SRS. CONCLUSION: This description of CyberKnife radiosurgical ablation demonstrates its feasibility and apparent safety for selected intramedullary spinal cord AVMs. Additional experience is necessary to ascertain the optimal radiosurgical dose and ultimate efficacy of this technique.

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