Holocord myelopathy with thoracic stenosis: case report and hypothesis

Ch, Kim; Chung, Chun Kee; Bj, Kwon; Kim, Hyung Jun

doi:10.1038/sj.sc.3101522

Cited by 3 publications

(2 citation statements)

References 7 publications

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“…Furthermore, the narrowed spinal canal may compromise the vascular supply to the spinal cord, particularly the venous outflow, leading to increased venous pressure and exacerbating venous congestion. [8,14] barrier. Or symptom progression could be due to steroidinduced thrombosis and inhibition of thrombolysis.…”

Section: Discussionmentioning

confidence: 99%

Holocord myelopathy misdiagnosed as neuromyelitis optica spectrum disorder (NMOSD): A unique case of dural arteriovenous fistula at the craniocervical junction along first spinal nerve

Iampreechakul,

Sopchokchai,

Wangtanaphat

et al. 2024

Surgical Neurology International

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Background: Dural arteriovenous fistulas (DAVFs) at the craniocervical junction (CCJ) involving the first spinal nerve represent a particularly rare and challenging subtype of DAVFs, with holocord myelopathy secondary to cerebrospinal DAVFs being an exceedingly rare presentation. Case Description: We report the case of a 70-year-old woman who presented with progressive paraparesis over 2 weeks. Initial magnetic resonance imaging (MRI) of the spine showed extensive holocord myelopathy, leading to a misdiagnosis of inflammatory myelopathy and subsequent inappropriate steroid treatment at a local hospital, which exacerbated her neurological symptoms. On transfer to our institution and further evaluation with MRI and magnetic resonance angiography, a lower thoracic DAVF was initially suspected. However, comprehensive spinal angiography failed to localize the fistula, prompting cranial angiography, which ultimately identified a DAVF at the CCJ along the C1 nerve root, supplied by a small radiculomeningeal branch of the left vertebral artery. Successful management involved coagulation of the proximal draining vein, with follow-up imaging confirming complete fistula obliteration and resolution of the holocord edema. Conclusion: This case highlights the diagnostic and therapeutic challenges associated with DAVFs at the CCJ, particularly when presenting with holocord myelopathy. It underscores the importance of a high index of suspicion and the need for timely, accurate diagnosis and intervention to prevent permanent spinal cord damage in such rare and complex cases.

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Section: Discussionmentioning

confidence: 99%

Holocord myelopathy misdiagnosed as neuromyelitis optica spectrum disorder (NMOSD): A unique case of dural arteriovenous fistula at the craniocervical junction along first spinal nerve

Iampreechakul,

Sopchokchai,

Wangtanaphat

et al. 2024

Surgical Neurology International

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“…Although rare, syrinx and holocord have more recently been associated with previously unsuspected conditions such as (1) a dural arteriovenous fistula at the level of the foramen magnum [ 252 ], (2) cervical spondylosis [ 253 ], (3) stenosis of the thoracic spine [ 254 ], (4) a sacral arachnoid cyst [ 255 ], and (5) a tethered cord [ 256 ]. All of these cases were likewise attributed to altered CSF flow and pressure gradients in the spinal canal and cord.…”

Section: Hydrocephalus Hydromyelia and Ccjmentioning

confidence: 99%

The Role of the Craniocervical Junction in Craniospinal Hydrodynamics and Neurodegenerative Conditions

Flanagan

2015

Neurology Research International

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The craniocervical junction (CCJ) is a potential choke point for craniospinal hydrodynamics and may play a causative or contributory role in the pathogenesis and progression of neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, MS, and ALS, as well as many other neurological conditions including hydrocephalus, idiopathic intracranial hypertension, migraines, seizures, silent-strokes, affective disorders, schizophrenia, and psychosis. The purpose of this paper is to provide an overview of the critical role of the CCJ in craniospinal hydrodynamics and to stimulate further research that may lead to new approaches for the prevention and treatment of the above neurodegenerative and neurological conditions.

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