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Open access

Successful detection of multiple communicating holes in multiple spinal extradural arachnoid cysts by using time-spatial labeling inversion pulse magnetic resonance imaging: illustrative case

Yusuke Kagei, Tatsuya Ishibe, Yusuke Kanba, and Masashi Tanaka

BACKGROUND

Spinal extradural arachnoid cysts (SEACs) communicate with the subarachnoid space through small communicating dural holes. The precise preoperative detection of all communicating holes, followed by minimally invasive dural closure, is the ideal treatment to prevent postoperative spinal deformities, especially in cases of multiple SEACs. However, standard imaging methods often fail to detect communicating hole locations. Although a few cases of successful single-hole detection via cinematic magnetic resonance imaging (MRI) have been reported, this modality’s ability to detect multiple holes has not been demonstrated.

OBSERVATIONS

The authors describe the case of a 14-year-old male with myelopathy due to multiple SEACs at T5–8 and T8–12. Myelography revealed a complete block at the T8 level; no cephalic cyst or communicating holes were identified. Time-spatial labeling inversion pulse (T-SLIP) MRI revealed cerebrospinal fluid flow into the cyst at T10 and T7. A limited laminectomy or hemilaminectomy was performed at T7 and T10, and two dural holes were closed without a total cystectomy. The patient’s gait disturbance and rectal disorder disappeared. The cysts were confirmed to have completely disappeared on conventional MRI at 1 year postoperatively.

LESSONS

T-SLIP MRI, a cinematic MRI, is useful for detecting multiple communicating holes in SEACs.

Open access

Early cerebral venous drainage associated with focal seizures: a correlation of digital subtraction angiography and electroencephalography. Illustrative case

Olivia Holman, Joseph M Bibawy, Lara Wadi, Joel C Morgenlander, and Erik F Hauck

BACKGROUND

Early venous drainage is a critical diagnostic feature of arteriovenous malformations (AVMs) and arteriovenous fistulas (AVFs). However, other conditions associated with early venous drainage can mimic AVMs and AVFs and mislead the treating physician team.

OBSERVATIONS

The authors present a unique case of a 56-year-old man with new left hemianopsia. Workup with magnetic resonance imaging and magnetic resonance angiography had revealed cortical enhancement and early venous drainage, possibly caused by an AVF or AVM. Catheter angiography confirmed the presence of early venous drainage of specifically the right occipital lobe. The early venous drainage occurred as the result of local hyperperfusion limited to the right occipital lobe. Subsequent electroencephalography confirmed focal seizure activity in the right occipital lobe. After seizure control with multiple antiepileptic medications, the patient regained his vision. Repeat digital subtraction angiography revealed restoration of normal cerebral blood flow.

LESSONS

Early venous drainage observed during catheter angiography can be associated with seizure activity. Differentiation from other conditions, such as AVM, AVF, or ischemic stroke, is critical to facilitate prompt and appropriate treatment.

Open access

Incidental durotomy resulting in a postoperative lumbosacral nerve root with eventration into the adjacent facet joint: illustrative cases

Michael J Kelly, Franziska C. S Altorfer, Marco D Burkhard, Russel C Huang, Frank P Cammisa Jr., and J. Levi Chazen

BACKGROUND

Radicular pain after lumbar decompression surgery can result from epidural hematoma/seroma, recurrent disc herniation, incomplete decompression, or other rare complications. A less recognized complication is postoperative nerve root herniation, resulting from an initially unrecognized intraoperative or, more commonly, a spontaneous postoperative durotomy. Rarely, this nerve root herniation can become entrapped within local structures, including the facet joint. The aim of this study was to illustrate our experience with three cases of lumbosacral nerve root eventration into an adjacent facet joint and to describe our diagnostic and surgical approach to this rare complication.

OBSERVATIONS

Three patients who had undergone lumbar decompression surgery with or without fusion experienced postoperative radiculopathy. Exploratory revision surgery revealed all three had a durotomy with nerve root eventration into the facet joint. Significant symptom improvement was achieved in all patients following liberation of the neural elements from the facet joints.

LESSONS

Entrapment of herniated nerve roots into the facet joint may be a previously underappreciated complication and remains quite challenging to diagnose even with the highest-quality advanced imaging. Thus, clinicians must have a high index of suspicion to diagnose this issue and a low threshold for surgical exploration.

Open access

Evaluation of the shrinkage process of a neck remnant after stent-coil treatment of a cerebral aneurysm using silent magnetic resonance angiography and computational fluid dynamics analysis: illustrative case

Toru Satoh, Kenji Sugiu, Masafumi Hiramatsu, Jun Haruma, and Isao Date

BACKGROUND

Silent magnetic resonance angiography (MRA) mitigates metal artifacts, facilitating clear visualization of neck remnants after stent and coil embolization of cerebral aneurysms. This study aims to scrutinize hemodynamics at the neck remnant by employing silent MRA and computational fluid dynamics.

OBSERVATIONS

The authors longitudinally tracked images of a partially thrombosed anterior communicating artery aneurysm’s neck remnant, which had been treated with stent-assisted coil embolization, using silent MRA over a decade. Computational fluid dynamics delineated the neck remnant’s reduction process, evaluating hemodynamic parameters such as flow rate, wall shear stress magnitude and vector, and streamlines. The neck remnant exhibited diminishing surface area, volume, neck size, dome depth, and aspect ratio. Its reduction correlated with a decline in the flow rate ratio of the remnant dome to the inflow parent artery. Analysis delineated regions within the contracting neck remnant characterized by consistently low average wall shear stress magnitude and variation, accompanied by notable variations in wall shear stress vector directionality.

LESSONS

Evaluation of neck remnants after stent-coil embolization is possible through silent MRA and computational fluid dynamics. Predicting the neck remnant reduction may be achievable through hemodynamic parameter analysis.

Open access

A rare case of atypical intradural extramedullary glioblastoma diagnosed utilizing next-generation sequencing and methylation profiling: illustrative case

William J Shelton, Andrew P Mathews, Karrar Aljiboori, J. Stephen Nix, Murat Gokden, and Analiz Rodriguez

BACKGROUND

Primary spinal cord tumors, especially primary spinal cord glioblastoma multiforme (PSC-GBM), are exceptionally rare, accounting for less than 1.5% of all spinal tumors. Their infrequency and aggressive yet atypical presentation make diagnosis challenging. In uncertain cases, a surgical approach for tissue diagnosis is often optimal.

OBSERVATIONS

A 76-year-old male presented with a rapidly progressing clinical history marked by worsening extremity weakness, urinary retention, and periodic fecal incontinence alongside diffuse changes on neuraxis imaging. The patient, in whom subacute polyneuropathy was initially diagnosed, received multiple rounds of steroids and intravenous immunoglobulin without clinical improvement. Histopathological review of the biopsy tissue yielded an initial diagnosis of spindle cell neoplasm. Next-generation sequencing (NGS) is done routinely on all neuropathology specimens at the authors’ institution, and methylation profiling is pursued in difficult cases. Ultimately, NGS and methylation profiling results were essential to an integrated final diagnosis of GBM.

LESSONS

PSC-GBM is a rare but highly aggressive occurrence of this tumor. Prolonged back pain, rapid neurological decline, and imaging changes warrant the consideration of lesional biopsy for precise disease characterization. In inconclusive cases, NGS has proved invaluable for clinical clarification and diagnosis, underscoring its importance for integrated diagnoses in guiding appropriate treatment strategies.

Open access

Immunocompetent isolated cerebral mucormycosis presenting with obstructive hydrocephalus: illustrative case

Khoa N Nguyen, Lindsey M Freeman, Timothy H Ung, Steven Ojemann, and Fabio Grassia

BACKGROUND

Isolated cerebral mucormycosis is rare in immunocompetent adults and is only sparsely reported to be associated with obstructive hydrocephalus.

OBSERVATIONS

Here, the authors report a case of obstructive hydrocephalus secondary to central nervous system mucormycosis without other systems or rhino-orbital involvement and its technical surgical management. A 23-year-old, incarcerated, immunocompetent patient with history of intravenous (IV) drug use presented with syncope. Although clinical and radiographic findings failed to elucidate an infectious pathology, endoscopy revealed an obstructive mass lesion at the level of the third ventricle, which, on microbiological testing, was confirmed to be Rhizopus fungal ventriculitis. Perioperative cerebrospinal fluid diversion, endoscopic third ventriculostomy, endoscopic biopsy technique, patient outcomes, and the literature are reviewed here. The patient received intrathecal and IV amphotericin B followed by a course of oral antifungal treatment and currently remains in remission.

LESSONS

The patient’s unique presentation and diagnosis of isolated cerebral mucormycosis reveal this pathogen as a cause of ventriculitis and obstructive hydrocephalus in immunocompetent adult patients, even in the absence of infectious sequelae on neuroimaging.

Open access

Bifocal germ cell tumor of pineal germinoma and neurohypophyseal embryonal carcinoma: illustrative case

Yu Naruse, Shinya Jinguji, Ryo Hiruta, Ayako Toda, Kenichiro Nagai, Shingo Kudo, Hideki Sano, Rei Sekine, Osamu Suzuki, Mudathir Bakhit, and Masazumi Fujii

BACKGROUND

Bifocal germ cell tumors, with primarily identical tissue composition, occur concurrently in the neurohypophyseal and pineal regions.

OBSERVATIONS

A 16-year-old male patient exhibited increased intracranial pressure symptoms, with concurrent tumors in the pineal and neurohypophyseal regions, causing obstructive hydrocephalus. His serum human chorionic gonadotropin level was elevated, measuring 506.6 mIU/mL. Upon gross endoscopic examination, the pineal tumor appeared white, whereas the neurohypophyseal tumor appeared red and hemorrhagic. Because of the limited sample size of the latter, a frozen section biopsy was feasible only for the pineal lesion, which indicated the presence of a germinoma. Subsequently, carboplatin and etoposide were administered, resulting in the reduction of the pineal tumor, but no effect was observed in the neurohypophyseal tumor. Histopathological analysis confirmed the pineal lesion as a germinoma, whereas the neurohypophyseal lesion was an embryonal carcinoma. Thus, the treatment was altered to ifosfamide, carboplatin, and etoposide (ICE), leading to a response in both tumors. The patient underwent three additional cycles of ICE therapy and high-dose chemotherapy, followed by whole craniospinal irradiation, achieving complete remission.

LESSONS

Although most bifocal germ cell tumors share the same histological tissue, occasional differences may arise, necessitating separate biopsies for accurate assessment.

Open access

Rare variant of large pediatric glioneuronal tumor with novel MYO5A::NTRK3 fusion: illustrative case

David Chenoweth, Hashim Syed, Nahom Teferi, Meron Challa, Jane E Persons, Kathryn L Eschbacher, Maggie Seblani, and Brian J Dlouhy

BACKGROUND

Glioneuronal tumors (GNTs) comprise a rare class of central nervous system (CNS) neoplasms with varying degrees of neuronal and glial differentiation that predominately affect children and young adults. Within the current 2021 World Health Organization (WHO) classification of CNS tumors, GNTs encompass 14 distinct tumor types. Recently, the use of whole-genome DNA methylation profiling has allowed more precise classification of this tumor group.

OBSERVATIONS

A 3-year-old male presented with a 3-month history of increasing head circumference, regression of developmental milestones, and speech delay. Magnetic resonance imaging of the brain was notable for a large left hemispheric multiseptated mass with significant mass effect and midline shift that was treated with near-total resection. Histological and molecular assessment demonstrated a glioneuronal tumor harboring an MYO5A::NTRK3 fusion. By DNA methylation profiling, this tumor matched to a provisional methylation class known as “glioneuronal tumor kinase-fused” (GNT kinase-fused). The patient was later started on targeted therapy with larotrectinib.

LESSONS

This is the first report of an MYO5A::NTRK3 fusion in a pediatric GNT. GNT kinase-fused is a provisional methylation class not currently included in the WHO classification of CNS tumors. This case highlights the impact of thorough molecular characterization of CNS tumors, especially with the increasing availability of novel gene targeting therapies.

Open access

Significance of upper cervical epidural venous engorgement on head computed tomography in the initial diagnosis of spontaneous intracranial hypotension: patient series

Toshihide Takahashi, Kiyoyuki Yanaka, Hitoshi Aiyama, Minami Saura, Michihide Kajita, Nobuyuki Takahashi, Kuniyuki Onuma, and Eiichi Ishikawa

BACKGROUND

Spontaneous intracranial hypotension (SIH) is a rare condition characterized by positional headache, for which contrast-enhanced magnetic resonance imaging (MRI) is the preferred diagnostic method. Although MRI reveals characteristic findings, head computed tomography (CT) is usually the first diagnostic step, but identifying features of SIH on CT is often difficult. This study was specifically designed to evaluate the utility of head CT in detecting upper cervical epidural venous engorgement as a sign of SIH.

OBSERVATIONS

Of 24 patients with SIH diagnosed between March 2011 and May 2023, 10 did not undergo upper cervical CT. In the remaining 14 patients, engorgement of the upper cervical epidural venous plexus was observed. CT detection rates were consistent with MRI for spinal fluid accumulation or dural thickening. After treatment, in 92.9% of patients, the thickness of the epidural venous plexus decreased statistically significantly from 4.8 ± 1.3 mm to 3.6 ± 1.2 mm.

LESSONS

This study suggests that upper cervical spine CT focused on epidural venous engorgement may be helpful in the initial diagnosis of SIH and may complement conventional MRI evaluation. Extending CT imaging to the upper cervical spine will improve the diagnostic accuracy of patients with positional headaches suspected to be SIH.

Open access

Bilateral and asymmetrical localization of language function identified by the superselective infusion of propofol in an epilepsy patient with a mild malformation of cortical development: illustrative case

Mayuko Otomo, Shin-ichiro Osawa, Kyoko Suzuki, Kazuo Kakinuma, Kazushi Ukishiro, Hiroyoshi Suzuki, Kuniyasu Niizuma, Norio Narita, Nobukazu Nakasato, and Teiji Tominaga

BACKGROUND

Atypical localization of language function can result in unexpected postsurgical deficits after cortical resection, but it is difficult to predict the risk in the presurgical evaluation. The authors experienced a rare case of the bilateral and independent existence of different components of language function identified by segmented evaluation of anatomical anterior and posterior language areas using the superselective infusion of propofol.

OBSERVATIONS

A 32-year-old right-handed female presented with drug-resistant epilepsy. Comprehensive epilepsy evaluation suggested that the epileptic foci involved the whole left frontal lobe but provided less evidence of structural abnormality. To estimate the extent of functional deterioration likely to be caused by an extended left frontal lobectomy, the authors evaluated segmented cortical function in the ipsi- and contralateral hemispheres by the superselective infusion of propofol into the branches of the intracranial artery. The results revealed bilateral and asymmetrical localization of language function because the patient presented with different components of aphasia in each hemisphere. Based on the authors’ assessment of her functional tolerance, an extended left frontal lobectomy was performed and resulted in neurological deficits within the anticipated range.

LESSONS

An accurate understanding of the correlations between vascular and functional anatomy and the highly specific evaluation of language function provides more advanced presurgical assessment, allowing more tailored planning of cortical resection.