Hirayama disease causing hand tremor on neck flexion

2019 ◽  
Vol 20 (3) ◽  
pp. 241-242
Author(s):  
Tun Hing Lui
Keyword(s):  
Cervical Spine ◽  
Epidural Space ◽  
Muscle Wasting ◽  
Cervical Cord ◽  
Neutral Position ◽  
Neck Flexion ◽  
Venous Plexus ◽  
Hand Tremor ◽  
Hirayama Disease ◽  
Right Hand

A 21-year-old man presented with right-hand intrinsic muscle wasting and right-hand tremor on neck flexion. MR scan of cervical spine was normal in the neutral position, but on neck flexion showed anterior shift and flattening of the cervical cord, with prominent posterior epidural space and engorged epidural venous plexus. We diagnosed Hirayama disease and his condition stabilised with a neck collar and physiotherapy.

scholarly journals Hirayama disease: the importance of flexion imaging

2021 ◽  
pp. 20210105
Author(s):  
Kieran Kusel ◽  
Richard Warne ◽  
Rahul Lakshmanan ◽  
Michael Mason ◽  
Michael Bynevelt ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cervical Spine ◽  
Neutral Position ◽  
Limb Weakness ◽  
Hirayama Disease ◽  
Intrinsic Signal ◽  
Gadolinium Administration ◽  
Signal Abnormality ◽  
Full Flexion ◽  
Vertebral Bodies

Hirayama disease is a rare cervical myelopathy characterised by asymmetrical upper limb weakness and muscle atrophy in the forearm and hand. MRI of the cervical spine plays an essential role in diagnosis, however, the characteristic findings are often only seen when the patient is imaged with the neck in flexion. We present a case of a 15-year-old male who presented with left forearm and hand weakness with muscle wasting. An MRI of the cervical spine with the neck in a neutral position demonstrated atrophy of the spinal cord with intrinsic signal abnormality between C5 and C7. Further imaging with the patient’s neck in flexion demonstrated the hallmark features of Hirayama disease. There was anterior displacement of the thecal sac and spinal cord, and an enlarged, crescent-shaped dorsal epidural space which enhanced following i.v. gadolinium administration. The atrophic segment of cord contacted the posterior vertebral bodies when the neck was in full flexion. This case highlights the importance of imaging patients suspected of having this entity with the neck in full flexion in order to make a diagnosis.

scholarly journals PROXIMAL BRACHIAL MONOMELIC AMYOTROPHY OR HIRAYAMA DISEASE: NO LONGER AN ALIAS? (case report)

2019 ◽  
Vol 5 (1) ◽  
pp. 5-9
Author(s):  
Akshay Rao
Keyword(s):  
Case Report ◽  
Cervical Spine ◽  
Upper Limb ◽  
Clinical Status ◽  
Supportive Therapy ◽  
Neutral Position ◽  
Mri Findings ◽  
Limb Weakness ◽  
Hirayama Disease ◽  
History Of

Background. Brachial Monomelic Amyotrophy (BMMA) has been called as Hirayama disease (HD) when it is characterized by unilateral distal upper limb weakness and atrophy that shows progression for a limited period and is associated with typical features on MRI of cervical spine in flexion. Objective was to explore the differences when BMMA affects the proximal upper limb muscles with the help of case report. Methods. A case report of BMMA in an adult Indian male is represented. Results. A 30-year-old man presented to us with a history of weakness in the proximal aspect of his left upper limb that began four years ago. The weakness was progressive up until 6 months prior to his presentation since when the weakness had neither worsened nor improved. Cervical spine contrast enhanced MRI revealed mild loss of cervical lordosis, but no features of HD like localized cord atrophy, loss of attachment of dura from subjacent lamina on neutral position axial T2WI MRI, nor any presence of posterior epidural crescentic enhancing mass on flexion contrast sagittal T1WI MRI. The patient was managed with supportive therapy and has been under regular follow up ever since. His clinical status has been stable. Conclusions. We support the suggestion to consider proximal Brachial Monomelic Amyotrophy to be a separate entity and to be distinguished from Hirayama disease that should be reserved for patients with distal upper limb involvement with cervical MRI findings on flexion studies.

Dynamic somatosensory evoked potentials to determine electrophysiological effects on the spinal cord during cervical spine extension

2013 ◽  
Vol 19 (3) ◽  
pp. 288-292 ◽  
Author(s):  
Yuichiro Morishita ◽  
Takeshi Maeda ◽  
Takayoshi Ueta ◽  
Masatoshi Naito ◽  
Keiichiro Shiba
Keyword(s):  
Spinal Cord ◽  
Cervical Spine ◽  
Evoked Potentials ◽  
Somatosensory Evoked Potentials ◽  
Cervical Cord ◽  
Control Group ◽  
Neutral Position ◽  
Healthy Controls ◽  
Median Nerve Stimulation ◽  
Significant Difference

Object The goal of this prospective study was to investigate somatosensory evoked potentials (SSEPs) during dynamic motion of the cervical spine and to evaluate the efficacy of analyzing dynamic SSEPs for predicting dynamic effects on the spinal cord in patients with cervical spondylotic myelopathy (CSM). Methods In total, 40 human subjects (20 CSM patients and 20 healthy volunteers as a control group) were examined prospectively using dynamic SSEPs with median nerve stimulation. The CSM patients showed cervical myelopathy due to cervical cord compression at the C4–5 segment. The SSEPs were examined with the cervical spine in a neutral position and at a 20° extension for 10 and 20 minutes. Changes in the N20 latency and amplitude were determined and analyzed. The authors defined the changes in the N20 latency and N20 amplitude between the neutral and extension positions of the cervical spine as percent latency and amplitude, respectively. Results In the CSM patients, SSEPs tended to deteriorate after cervical spine extension, and a statistically significant deterioration of the N20 amplitude after the extension was observed. Moreover, the percent latency and amplitude progressively increased during cervical spine extension in these patients. In the healthy controls, SSEPs tended to deteriorate with cervical spine extension, but these changes did not result in statistically significant differences. Moreover, in this group the percent latency and amplitude were almost identical during the extension. When the CSM patients and the healthy controls were compared, a significant difference in the percent amplitude was observed between the 2 groups during the cervical spine extension. Conclusions This study suggests the potential of dynamic SSEPs as a useful neurophysiological technique to detect the effect of dynamic factors on the pathogenesis of CSM.

scholarly journals Hirayama Disease: A Rare Disease with Unusual Features

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
S. Anuradha ◽  
Vanlalmalsawmdawngliana Fanai
Keyword(s):  
Motor Neuron ◽  
Rare Disease ◽  
Autonomic Dysfunction ◽  
Cervical Myelopathy ◽  
Cervical Cord ◽  
Neck Flexion ◽  
Forward Displacement ◽  
Hirayama Disease ◽  
Young Males

Hirayama disease, also known as monomelic amyotrophy (MMA), is a rare cervical myelopathy that manifests itself as a self-limited, asymmetrical, slowly progressive atrophic weakness of the forearms and hands predominantly in young males. The forward displacement of the posterior dura of the lower cervical dural canal during neck flexion has been postulated to lead to lower cervical cord atrophy with asymmetric flattening. We report a case of Hirayama disease in a 25-year-old Indian man presenting with gradually progressive asymmetrical weakness and wasting of both hands and forearms along with unusual features of autonomic dysfunction and upper motor neuron lesion.

scholarly journals P.092 Hirayama Disease: a diagnostic and therapeutic challenge

2017 ◽  
Vol 44 (S2) ◽  
pp. S37-S37
Author(s):  
S McGregor ◽  
H Joswig ◽  
N Duggal
Keyword(s):  
Correct Diagnosis ◽  
Dynamic Mri ◽  
Cervical Cord ◽  
Anterior Fusion ◽  
Rare Entity ◽  
Neck Flexion ◽  
Forward Displacement ◽  
Hirayama Disease ◽  
Signal Changes ◽  
Professional Singer

Background: Hirayama disease (HD) is characterized by progressive cervical myelopathy caused by repetive neck flexion leading to forward displacement of the posterior dural sack with compression and injury of the spinal cord. Typically, the C7-T1 myotomes become weak and atrophic, while sparing sensation. Here we present two Canadian cases of this rare entity. Methods: Two cases of HD are presented and literature reviewed, showing the diagnostic and therapeutic challenges of this disease. Results: Case 1 is a 17-year-old male professional singer and musician. He presented with bilateral progressive hand weakness, which was aggrevated while playing the violine. Cervical MRI showed increased T2-weighted signaling at C5-7, but a correct diagnosis could not be identified. Eventually, dynamic cervical MRI showed the compression and he underwent an anterior cervical discectomy and fusion (ACDF) at C5-C6 and C6-C7 without complications. Case 2 is a 19-year-old female with progressive right hand weakness. After numerous investigations, a dynamic cervical MRI diagnosed her with HD with classic findings and she underwent an ACDF at C6-C7 without complications. Conclusions: Hiryama’s disease is rare, but should be kept in mind when cervical cord signal changes cannot be explained by standard MRI. Dynamic MRI is imperative to correct diagnosis and anterior fusion shows good outcomes in its management.

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

2020 ◽  
Vol 20 (2) ◽  
pp. 117-120
Author(s):  
Julian Llano ◽  
Neil U. Lall ◽  
Lamar Davis ◽  
Andrew J. Steven
Keyword(s):  
Muscle Wasting ◽  
Adolescent Male ◽  
Hirayama Disease ◽  
Right Hand

LACK OF EPIDURAL PRESSURE CHANGE WITH NECK FLEXION IN A PATIENT WITH HIRAYAMA DISEASE

Neurosurgery ◽  
2009 ◽  
Vol 64 (6) ◽  
pp. E1196-E1197 ◽  
Author(s):  
Tanmay R. Patel ◽  
E. Antonio Chiocca ◽  
Miriam L. Freimer ◽  
Gregory A. Christoforidis
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Epidural Space ◽  
Venous Pressure ◽  
Pressure Change ◽  
Pathophysiological Mechanism ◽  
Pressure Measurements ◽  
Neck Flexion ◽  
Hirayama Disease ◽  
Cord Injury

Abstract OBJECTIVE Hirayama disease is a nonprogressive cervical myelopathy associated with epidural venous engorgement and spinal canal narrowing. It has been unclear whether dural venous pressure influences spinal cord injury in this illness. CLINICAL PRESENTATION An 18-year-old man presented with profound hand weakness and atrophy that had developed over a period of 1 year. Electromyographic, magnetic resonance imaging, and angiographic findings are presented. INTERVENTION The epidural space was accessed using a microcatheter technique. Pressure measurements were recorded with and without Valsalva maneuver in the inferior vena cava, vertebral veins, and epidural space at C4 and C6 in both the flexion and neutral positions. Cervical epidural venous pressure measurements in flexion and neutral positions are presented. The patient underwent duraplasty with C4 to T1 laminectomies and fusion using lateral mass screws and facet arthrodeses. CONCLUSION Lack of significant pressure change with neck flexion suggested that dural venous engorgement is passive and not the direct cause for spinal cord injury. Data presented herein and review of the literature suggest that surgical treatment targeting the underlying pathophysiological mechanism in Hirayama disease can benefit patients, especially early in the course of the disease.

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 Imaging in Hirayama disease

2016 ◽  
Vol 7 (01) ◽  
pp. 164-167 ◽  
Author(s):  
Keshav Gupta ◽  
Shashank Sood ◽  
Jayesh Modi ◽  
Rajiv Gupta
Keyword(s):  
Magnetic Resonance Imaging ◽  
Muscular Atrophy ◽  
Case Series ◽  
Cervical Cord ◽  
Neutral Position ◽  
Cervical Lordosis ◽  
Resonance Imaging ◽  
Hirayama Disease ◽  
Young Males ◽  
Magnetic Resonance Imaging Mri

ABSTRACTHirayama disease, also known as Sobue disease is a rare nonprogressive spinal muscular atrophy. Here, we report a case series of three young males presenting with atrophy of distal upper limb and Hirayama disease as their clinico-radiological diagnosis. Magnetic resonance imaging (MRI) revealed loss of cervical lordosis with focal areas of lower cervical cord atrophy in a neutral position. MRI in flexion position revealed, anterior displacement of the detached posterior dura from the underlying lamina compressing the thecal sac and widened posterior epidural space with flow voids seen better on 3D-CISS images. All the three patients were managed conservatively.

scholarly journals Morphometrical Study of the Lumbar Segment of the Internal Vertebral Venous Plexus in Dogs: A Contrast CT-Based Study

Animals ◽  
2021 ◽  
Vol 11 (6) ◽  
pp. 1502
Author(s):  
Valeria Ariete ◽  
Natalia Barnert ◽  
Marcelo Gómez ◽  
Marcelo Mieres ◽  
Bárbara Pérez ◽  
...  
Keyword(s):  
Epidural Space ◽  
Ct Images ◽  
Venous Plexus ◽  
Morphological Pattern ◽  
Vertebral Canal ◽  
Dural Sac ◽  
Internal Vertebral Venous Plexus ◽  
Adjacent Structures ◽  
Contrast Ct ◽  
Enhanced Computed Tomography

The internal vertebral venous plexus (IVVP) is a thin-walled, valveless venous network that is located inside the vertebral canal, communicating with the cerebral venous sinuses. The objective of this study was to perform a morphometric analysis of the IVVP, dural sac, epidural space and vertebral canal between the L1 and L7 vertebrae with contrast-enhanced computed tomography (CT). Six clinically healthy adult dogs weighing between 12 kg to 28 kg were used in the study. The CT venographic protocol consisted of a manual injection of 880 mgI/kg of contrast agent (587 mgI/kg in a bolus and 293 mgI/mL by continuous infusion). In all CT images, the dimensions of the IVVP, dural sac, and vertebral canal were collected. Dorsal reconstruction CT images showed a continuous rhomboidal morphological pattern for the IVVP. The dural sac was observed as a rounded isodense structure throughout the vertebral canal. The average area of the IVVP ranged from 0.61 to 0.74 mm2 between L1 and L7 vertebrae (6.3–8.9% of the vertebral canal), and the area of the dural sac was between 1.22 and 7.42 mm2 (13.8–72.2% of the vertebral canal). The area of the epidural space between L1 and L7 ranged from 2.85 to 7.78 mm2 (27.8–86.2% of the vertebral canal). This CT venography protocol is a safe method that allows adequate visualization and morphometric evaluation of the IVVP and adjacent structures.

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