Possible coexistence of MOG-IgG-associated disease and anti-Caspr2 antibody-associated autoimmune encephalitis: a first case report

Myelin oligodendrocyte glycoprotein antibody-associated disease has been proposed as a separate inflammatory demyelinating disease of the central nervous system (CNS) since the discovery of pathogenic antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG). Antibodies targeting contactin-associated protein-like 2 (Caspr2), a component of voltage-gated potassium channel (VGKC) complex, have been documented to be associated with a novel autoimmune synaptic encephalitis with a low incidence. Herein, we reported an adult female with initial presentation of decreased vision in the right eye and subsequent episodes of neuropsychiatric disturbance including hypersomnia, agitation, apatheia, and memory impairment. Magnetic resonance imaging (MRI) revealed multiple lesions scattered in brain, brainstem, and cervical and thoracic spinal cord, showing hypointensity on T1-weighted images, hyperintensity on T2-weighted and fluid attenuated inversion recovery (FLAIR) images. Heterogenous patchy or ring-like enhancement was observed in the majority of lesions. The detection of low-titer MOG-IgG exclusively in cerebrospinal fluid (CSF; titer, 1:1) and Caspr2-IgG in both serum and CSF (titers, 1:100 and 1:1) led to a possible diagnosis of coexisting MOG-IgG-associated disease (MOGAD) and anti-Caspr2 antibody-associated autoimmune encephalitis. The patient was treated with immunosuppressive agents including corticosteroids and immunoglobulin, and achieved a sustained remission. To the best of our knowledge, this is the first report on the possible coexistence of MOGAD and anti-Caspr2 antibody-associated autoimmune encephalitis, which advocates for the recommendation of a broad spectrum screening for antibodies against well-defined CNS antigens in suspected patients with autoimmune-mediated diseases of the CNS.

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Upper thoracic spinal cord herniation after traumatic nerve root avulsion

Neurosurgical FOCUS ◽

10.3171/foc.2004.16.5.20 ◽

2004 ◽

Vol 16 (5) ◽

pp. 306-309

Author(s):

Victor R. DaSilva ◽

Mubarak Al-Gahtany ◽

Rajiv Midha ◽

Dipanka Sarma ◽

Perry Cooper

Keyword(s):

Spinal Cord ◽

Nerve Root ◽

Signs And Symptoms ◽

Root Avulsion ◽

Thoracic Spinal Cord ◽

First Case ◽

Thoracic Spinal ◽

Spinal Cord Herniation ◽

Nerve Root Avulsion ◽

Upper Thoracic

✓ Transdural herniation of the spinal cord, a rare but well-documented entity, has been reported sporadically for more than 25 years as a possible cause for various neurological signs and symptoms ranging from isolated sensory or motor findings to myelopathy and Brown–Séquard syndrome. The authors report, to the best of their knowledge, the first case of upper thoracic spinal cord herniation occurring after traumatic nerve root avulsion.

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Traumatic lumbar spondylolisthesis resulting in complete thoracic spinal cord avulsion: an unusual presentation

Journal of Neurosurgery Spine ◽

10.3171/2018.5.spine17919 ◽

2018 ◽

Vol 29 (6) ◽

pp. 635-638

Author(s):

Jamie Toms ◽

David L. Boyer ◽

Craig R. Kelman ◽

Rafael A. Vega

Keyword(s):

Soft Tissues ◽

Unusual Presentation ◽

Thoracic Spinal Cord ◽

Lumbar Drain ◽

First Case ◽

Thoracic Spinal ◽

Initial Imaging ◽

Lumbar Spondylolisthesis ◽

A Site ◽

Traumatic Spondylolisthesis

Traumatic spondylolisthesis is a known occurrence in trauma, but complete cord transection is relatively rare. Moreover, complete cord transection at a site distant from the traumatic spondylolisthesis without spondyloptosis is exceedingly rare. In this report, authors describe the first case of thoracic cord avulsion following a traumatic grade II lumbar spondylolisthesis. The unusual presentation of this case highlights the importance of further evaluating patients with neurological symptoms out of proportion with the injuries seen on initial imaging. Magnetic resonance imaging performed after initial imaging studies demonstrated T11 cord transection with the distal cord herniating into the lumbar paraspinal soft tissues, thus allowing for preoperative planning to prepare for a more significant intervention including complex dural repair and lumbar drain placement, in addition to instrumented fusion to stabilize the traumatic spondylolisthesis.

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Dorsal root ganglion stimulation for treatment of complex regional pain syndrome of the foot

Neurosurgical Focus: Video ◽

10.3171/2020.7.focvid2034 ◽

2020 ◽

Vol 3 (2) ◽

pp. V8

Author(s):

Kevin Hines ◽

Fadi Al Saiegh ◽

Aria Mahtabfar ◽

Kavantissa M. Keppetipola ◽

Caio M. Matias ◽

...

Keyword(s):

Spinal Cord ◽

Dorsal Root Ganglion ◽

Complex Regional Pain Syndrome ◽

Spinal Cord Stimulation ◽

Dorsal Root ◽

Pain Syndrome ◽

Thoracic Spinal Cord ◽

Plantar Aspect ◽

Thoracic Spinal ◽

The Right

This is a case of a 54-year-old man presenting with complex regional pain syndrome (CRPS) type 1 of the right lower extremity, which was most debilitating in the plantar aspect of the right foot. The patient had prior treatment with thoracic spinal cord stimulation; however, the foot pain remained intractable. Given that his pain was predominantly in his foot and remained debilitating despite thoracic spinal cord stimulation, it was recommended that the patient undergo a trial of dorsal root ganglion (DRG) stimulation. The surgical technique for placement of dorsal root ganglion stimulators is demonstrated in this operative video.The video can be found here: https://youtu.be/_1xMxFZa6tU

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Solitary Fibrous Tumor of the Spinal Cord: Case Report and Review of the Literature

Neurosurgery ◽

10.1227/01.neu.0000130037.45768.84 ◽

2004 ◽

Vol 55 (2) ◽

pp. E433-E438 ◽

Cited By ~ 21

Author(s):

Mitsuhiro Kawamura ◽

Kazutaka Izawa ◽

Noboru Hosono ◽

Hiroshi Hirano

Keyword(s):

Spinal Cord ◽

Solitary Fibrous Tumor ◽

Spinal Cord Tumor ◽

Clinical Manifestations ◽

Thoracic Spinal Cord ◽

Thoracic Spinal ◽

Spindle Cell Proliferation ◽

Long Term Follow Up ◽

Fibrous Tumor ◽

The Right

Abstract OBJECTIVE AND IMPORTANCE: A solitary fibrous tumor (SFT) is a rare neoplasm of probable mesenchymal origin that was first reported in the pleura but can occur in different sites. Only six cases of SFT arising from the spinal cord have been reported. CLINICAL PRESENTATION: We report a case of primary SFT occurring in the thoracic spinal cord in a 64-year-old man with Brown-Séquard syndrome. Magnetic resonance imaging revealed an intradural mass at the level of T2–T3. INTERVENTION: Total T2–T3 laminectomies were performed. The tumor appeared to be adherent to the right lateral aspect of the cord but not attached to the meninges. On histological examination, the tumor exhibited spindle cell proliferation with abundant dense collagen but without a hemangiopericytomatous pattern. Immunohistochemically, the tumor cells were reactive with CD34 and vimentin only. CONCLUSION: We report a rare case of SFT occurring in the thoracic spinal cord. Histologically and immunohistochemically, we confirmed the diagnosis of SFT. Low signal intensity on T1- and T2-weighted images corresponded to the histological findings. When a spinal cord tumor exhibits a signal pattern similar to this, SFT should be included in the differential diagnosis. Because of the rarity of reports on this condition, the clinical manifestations and course of SFT of the spinal cord are unknown, and careful long-term follow-up is recommended.

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Upper thoracic spinal cord herniation after traumatic nerve root avulsion

Journal of Neurosurgery Spine ◽

10.3171/spi.2003.99.3.0306 ◽

2003 ◽

Vol 99 (3) ◽

pp. 306-309 ◽

Cited By ~ 7

Author(s):

Victor R. Dasilva ◽

Mubarak Al-Gahtany ◽

Rajiv Midha ◽

Dipanka Sarma ◽

Perry Cooper

Keyword(s):

Spinal Cord ◽

Nerve Root ◽

Root Avulsion ◽

Thoracic Spinal Cord ◽

Content Type ◽

First Case ◽

Thoracic Spinal ◽

Spinal Cord Herniation ◽

Nerve Root Avulsion ◽

Upper Thoracic

✓ Transdural herniation of the spinal cord, a rare but well-documented entity, has been reported sporadically for more than 25 years as a possible cause for various neurological signs and symptoms ranging from isolated sensory or motor findings to myelopathy and Brown—Séquard syndrome. The authors report, to the best of their knowledge, the first case of upper thoracic spinal cord herniation occurring after traumatic nerve root avulsion.

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Syringomyelia in demyelinating disease of the central nervous system: Report of two cases

Srpski arhiv za celokupno lekarstvo ◽

10.2298/sarh1110657s ◽

2011 ◽

Vol 139 (9-10) ◽

pp. 657-660 ◽

Cited By ~ 1

Author(s):

Dejan Savic ◽

Slobodan Vojinovic ◽

Mirjana Spasic ◽

Zoran Peric ◽

Stevo Lukic

Keyword(s):

Multiple Sclerosis ◽

Spinal Cord ◽

Central Nervous System ◽

Nervous System ◽

Magnetic Resonance ◽

Demyelinating Disease ◽

Clinical Signs ◽

Thoracic Spinal Cord ◽

Thoracic Spinal ◽

The Central Nervous System

Introduction. Syringomyelia is a cavitary extension inside the spinal cord which can be either symptomatic or congenitally-idiopathic. Syringomyelia during the course of the disease in patients presenting with clinically definite multiple sclerosis was described earlier. Syringomyelia in patients presenting with a clinically isolated syndrome suggestive of multiple sclerosis is unusual. Case Outline. We present two patients presenting with demy-elinating disease of the central nervous system with syringomyelia in the cervical and thoracic spinal cord. We did not find classical clinical signs of syringomyelia in our patients, but we disclosed syringomyelia incidentally during magnetic resonance exploration. Magnetic resonance exploration using the gadolinium contrast revealed the signs of active demyelinating lesions in the spinal cord in one patient but not in the other. Conclusion. Syringomyelia in demyelinating disease of the central nervous system opens the question whether it is a coincidental finding or a part of clinical features of the disease. Differentiation of the significance of syringomyelia finding in these patients plays a role in the choice of treatment concept in such patients.

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Fulminant central nervous system demyelination associated with interferon-α therapy and hepatitis C virus infection

Multiple Sclerosis Journal ◽

10.1177/1352458507078684 ◽

2007 ◽

Vol 13 (9) ◽

pp. 1100-1106 ◽

Cited By ~ 11

Author(s):

R. Höftberger ◽

F. Garzuly ◽

H.P. Dienes ◽

J. Grubits ◽

B. Rohonyi ◽

...

Keyword(s):

Multiple Sclerosis ◽

Nervous System ◽

Hepatitis C Virus ◽

Hepatitis C ◽

Demyelinating Disease ◽

Hcv Infection ◽

Interferon Α ◽

Hcv Rna ◽

Thoracic Spinal Cord ◽

Thoracic Spinal

Hepatitis C virus (HCV) infection is common in the general population and may coincide with disease in the central and peripheral nervous system. Interferon-α (IFN-α) is used as treatment for HCV infection. The therapeutic benefit is assumed to result from activation of natural killer cells and CD8+ T cells. Despite its beneficial effects, it has been associated with a number of autoimmune disorders, such as chronic inflammatory demyelinating polyneuropathy and multiple sclerosis. Several clinical reports including magnetic resonance imaging exist, but neuropathological confirmation of MS associated with IFN-α therapy and HCV infection is lacking. We report a case of a female patient with chronic HCV infection who developed `acute MS'-like demyelinating disease after IFN-α administration, with extensive lesions throughout brain and thoracic spinal cord. The patient died after a disease duration of 6 months. Brain autopsy revealed Baló-like demyelinating plaques with positive HCV sequences within florid lesions. The development of fulminant demyelinating disease after administration of IFN-α suggests that autoimmune mechanisms such as T cell mediated tissue damage might be initiated or aggravated by IFN-α therapy. Additionally, the presence of HCV RNA within the demyelinated lesion indicates a possible role in triggering or propagating disease. Multiple Sclerosis 2007; 13: 1100—1106. http://msj.sagepub.com

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Growth of Intramedullary Spinal Cord Dermoid Cyst from a Congenital Thoracic Dermal Sinus Tract after Negative Screening Ultrasound Imaging

Pediatric Neurosurgery ◽

10.1159/000512580 ◽

2021 ◽

pp. 1-6

Author(s):

Liam G. Coulthard ◽

Craig Robert Vonhoff ◽

Ahmad M. Badran ◽

Thomas E. Robertson ◽

Martin J. Wood

Keyword(s):

Spinal Cord ◽

Dermoid Cyst ◽

English Literature ◽

False Negative ◽

Dermal Sinus ◽

Thoracic Spinal Cord ◽

Intramedullary Spinal Cord ◽

First Case ◽

Thoracic Spinal ◽

Screening Ultrasound

Introduction: Intramedullary thoracic dermoid cysts are rare lesions that are associated with dermal sinus tracts (DSTs). Current recommendations advocate for imaging-based screening of suspected DSTs shortly after birth to exclude associated inclusion lesions. Case Presentation: A 6-year-old male child presented with a 2-week history of progressive ataxia, lower limb weakness, and hyperreflexia. He was suspected to have a thoracic DST at birth, though initial screening ultrasound was negative for an inclusion lesion or intradural tract. On representation, MRI demonstrated a 3.9-cm intramedullary thoracic dermoid cyst causing significant spinal cord compression. Intraoperatively, a DST extending intradurally was found. The associated dermoid cyst was removed via intracapsular resection. Conclusions: Whilst dermoid cysts are presumed to progressively develop from DSTs, to our knowledge, this is the first case in English literature documenting a thoracic spinal cord intramedullary dermoid cyst following a negative screening ultrasound for a suspected DST. We use this case to highlight the false-negative rates associated with postnatal screening and advocate for early neurosurgical referral of suspected DSTs, regardless of imaging findings.

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Burst Stimulation of the Thoracic Spinal Cord near a Cardiac Pacemaker in an Elderly Patient with Postherpetic Neuralgia: A Case Report

Medicina ◽

10.3390/medicina57040337 ◽

2021 ◽

Vol 57 (4) ◽

pp. 337

Author(s):

Yeon Joo Lee ◽

Myoung Hoon Kong ◽

Sang Sik Choi ◽

Yong Deok Kwon ◽

Mi Kyoung Lee ◽

...

Keyword(s):

Spinal Cord ◽

Case Report ◽

Postherpetic Neuralgia ◽

Cardiac Pacemaker ◽

Functional Improvement ◽

Spinal Cord Stimulator ◽

Thoracic Spinal Cord ◽

Burst Mode ◽

First Case ◽

Thoracic Spinal

New developments in spinal cord stimulation (SCS) have improved the treatment of patients with chronic pain. Although the overall safety of modern SCS has been established, there are no published reports regarding safety considerations when implanting a burst-mode spinal cord stimulator in patients with permanent cardiac pacemakers (PCPs). An 80-year-old man with a complete atrioventricular block implanted with a PCP was considered as a candidate for burst-mode SCS due to well-established postherpetic neuralgia (>180 days after rash). Cardiac monitoring during the burst-mode spinal cord stimulator trial and insertion did not indicate any interference. After the insertion of the burst-mode spinal cord stimulator, the patient showed functional improvement and significant pain relief. The safety of traditional tonic-mode SCS in patients with PCP has been previously reported. This is the first case report describing the safe and effective use of burst-mode SCS in a patient with PCP.

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Tethered brain: disentangling unintentional brain-mesh interfaces. Illustrative case

Journal of Neurosurgery: Case Lessons ◽

10.3171/case21183 ◽

2021 ◽

Vol 1 (24) ◽

Author(s):

Samantha E. Spellicy ◽

Joseph R. Kilianski ◽

Rachel Poston ◽

Debra Moore-Hill ◽

Fernando L. Vale

Keyword(s):

Primary Motor Cortex ◽

Hand Function ◽

Synthetic Mesh ◽

Brain Parenchyma ◽

Illustrative Case ◽

Left Hand ◽

First Case ◽

Surgical Meshes ◽

Fluid Attenuated Inversion Recovery ◽

The Right

BACKGROUND Surgical meshes have found widespread use in neurosurgical practice. While commonly recognized risks of synthetic mesh include infection, exposure of mesh implants, and foreign body reaction, the risk of mesh tethering to neural structures is often overlooked. OBSERVATIONS The authors presented the first case, to their knowledge, of the disentanglement of mesh interfaced to cortical tissue. The patient, a 68-year-old woman, presented with severe intractable seizure disorder and worsening left hand function and incoordination after meningioma resection and cranioplasty 9 years earlier. Magnetic resonance imaging (MRI) demonstrated interval progression of macrocystic encephalomalacia involving the right supplementary motor area, with fluid-attenuated inversion recovery signal extending posteriorly into the right primary motor cortex. Both computed tomography and MRI suggested potential tethering of the cortex to the overlying cranioplasty mesh. Because of the progressive nature of her condition, the decision was made to surgically remove the tethered mesh. LESSONS De-tethering brain parenchyma from surgical mesh requires careful microdissection and judicious use of electrocautery to minimize further tissue damage and preserve neurological function. This inadvertent complication evinces the importance of using dural substitutes when unable to primarily repair the dura to prevent scarring and tethering of neural tissues to synthetic cranioplasty materials.

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