Vascular Pathologic Conditions in and around the Spinal Cord.

Diagnosing and differentiating spinal vascular pathologic conditions is challenging. Small structures, lengthy imaging examinations, and overlapping imaging features increase the difficulty. Yet, subtle findings and helpful protocols can narrow the differential diagnosis. The authors aim to help radiologists make accurate and timely diagnoses of spinal vascular pathologic conditions in and around the spinal cord by highlighting spinal vascular anatomy, imaging findings, and three broad categories of abnormalities: infarcts, anomalies, and tumors. ©RSNA, 2024.

Comparison of 2 Robotic Systems for Pediatric Stereoelectroencephalography Implantation.

BACKGROUND: Stereoelectroencephalography (SEEG) remains critical in guiding epilepsy surgery. Robot-assisted techniques have shown promise in improving SEEG implantation outcomes but have not been directly compared. In this single-institution series, we compared ROSA and Stealth AutoGuide robots in pediatric SEEG implantation. METHODS: We retrospectively reviewed 21 sequential pediatric SEEG implantations consisting of 6 ROSA and 15 AutoGuide procedures. We determined mean operative time, time per electrode, root mean square (RMS) registration error, and surgical complications. Three-dimensional radial distances were calculated between each electrode's measured entry and target points with respective errors from the planned trajectory line. RESULTS: Mean overall/per electrode operating time was 73.5/7.5 minutes for ROSA and 126.1/10.9 minutes for AutoGuide (P = 0.030 overall, P = 0.082 per electrode). Mean RMS registration error was 0.77 mm (0.55-0.93 mm) for ROSA and 0.6 mm (0.2-1.0 mm) for AutoGuide (P = 0.26). No procedures experienced complications. The mean radial (entry point error was 1.23 ± 0.11 mm for ROSA and 2.65 ± 0.12 mm for AutoGuide (P < 0.001), while the mean radial target point error was 1.86 ± 0.15 mm for ROSA and 3.25 ± 0.16 mm for AutoGuide (P < 0.001). CONCLUSIONS: Overall operative time was greater for AutoGuide procedures, although there was no statistically significant difference in time per electrode. Both systems are highly accurate with no significant RMS error difference. While the ROSA robot yielded significantly lower entry and target point errors, both robots are safe and reliable for deep electrode insertion in pediatric epilepsy.

An Ecchordosis Physaliphora, a Rare Entity, Involving the Central Nervous System: A Systematic Review of the Literature.

Ecchordosis physaliphora (EP) is a benign notochordal remnant, which is often an incidental finding; however, it can rarely present with neurological symptoms. We performed a systematic review of the literature for cases of symptomatic EP published in PubMed, Web of Science and Embase from January 1982 to May 2023. This is the largest review to date and revealed 60 cases including ours. Headache (55%) and CSF rhinorrhea (32%) were the most frequent clinical manifestations. The majority of symptomatic EP lesions were located in the prepontine region (77%) and required surgical resection (75%). EP should be considered in patients with neurologic symptoms in the setting of prepontine or posterior sphenoid sinus lesions. While symptomatic patients often require surgical intervention, rare cases may respond to oral corticosteroids.

Autoimmune and paraneoplastic neurological disorders: A review of relevant neuroimaging findings.

INTRODUCTION: Paraneoplastic neurologic syndromes (PNS) and autoimmune encephalitis (AIE) are immune-mediated disorders. PNS is linked to cancer, while AIE may not Their clinical manifestations and imaging patterns need further elucidation. OBJECTIVE/AIMS: To investigate the clinical profiles, antibody associations, neuroimaging patterns, treatments, and outcomes of PNS and AIE. METHODS: A systematic review of 379 articles published between 2014 and 2023 was conducted. Of the 55 studies screened, 333 patients were diagnosed with either PNS or AIE and tested positive for novel antibodies. Data on demographics, symptoms, imaging, antibodies, cancer associations, treatment, and outcomes were extracted. RESULTS: The study included 333 patients (mean age 54 years, 67% males) with PNS and AIE positive for various novel antibodies. 84% had central nervous system issues like cognitive impairment (53%), rhombencephalitis (17%), and cerebellar disorders (24%). Neuroimaging revealed distinct patterns with high-risk antibodies associated with brainstem lesions in 98%, cerebellar in 91%, hippocampal in 98%, basal ganglia in 75%, and spinal cord in 91%, while low/intermediate-risk antibodies were associated with medial temporal lobe lesions in 71% and other cortical/subcortical lesions in 55%. High-risk antibodies were associated with younger males, deep brain lesions, and increased mortality of 61%, while low/intermediate-risk antibodies were associated with females, cortical/subcortical lesions, and better outcomes with 39% mortality. Associated cancers included seminomas (23%), lung (19%), ovarian (2%), and breast (2%). Treatments included IVIG, chemotherapy, and plasmapheresis. Overall mortality was 25% in this cohort. CONCLUSION: PNS and AIE have distinct clinical and radiological patterns based on antibody profiles. High-risk antibodies are associated with increased mortality while low/intermediate-risk antibodies are associated with improved outcomes. Appropriate imaging and antibody testing are critical for accurate diagnosis.

Histopathologic "Evolution" in Pediatric Primary Intracranial High-Grade Sarcoma - a Key that Unlocked the Correct Diagnosis.

Central nervous system (CNS) tumors are the second most common in the pediatric age group, accounting for 3.5% of overall mortality. The 2021 World Health Organization (WHO) classification of pediatric CNS tumors has given insight into their molecular biology. Correct diagnosis of high-grade intracranial sarcomas is a well-known challenge because of their histopathological variation, presence of heterologous elements, and haphazard pattern of growth. We present a case of a 13-year-old female with a right-sided frontal hemorrhagic mass. Pathological work-up revealed an intra-cranial high-grade sarcoma, not otherwise specified (NOS). Despite receiving chemo-radiation, the lesion recurred after 9 months. This time, the sarcoma had evolved, showing distinct focal rhabdomyoblastic differentiation. Next-generation sequencing (NGS) based assay revealed variants p.E1705V, p.Y1417Ter in DICER1, and other mutations in KRAS and TP53 genes. The lesion was then diagnosed as spindle cell sarcoma with rhabdomyosarcoma-like features, DICER1 mutant. We propose that upfront molecular studies in pediatric undifferentiated high-grade sarcomas are indicated for precise diagnosis and classification.

Idiopathic Hypertrophic Spinal Pachymeningitis.

Hypertrophic pachymeningitis (HP) is a rare presentation with duramater thickening and fibrosis which can result in cranial or spinal compressive disease. Most cases of spinal HP require surgical management. We present an uncommon case of idiopathic hypertrophic spinal pachymeningitis (IHSP) in a 40-year-old male who showed complete improvement to steroids without any further relapses. The patient presented with bilateral upper limb weakness with magnetic resonance imaging (MRI) spine showing diffuse dural thickening of the entire spine with cervical cord compression. He had an extensive workup for underlying etiology and worsening symptoms until he was diagnosed with IHSP. Later, he was started on high-dose steroids with good response and no relapse after 2 years. A descriptive analysis of IHSP cases since 2009 including ours showed that it usually occurs after 50s with female preponderance. Weakness and sensory loss are the most common complaints with 50% patients showing clinical signs of myelopathy like hyperreflexia, clonus, Babinski sign and sensory level. Cerebrospinal fluid (CSF) and inflammatory markers like erythrocytic sedimentation rate (ESR) and C-reactive protein (CRP) can be used to assess disease progression and prognosis. Surgical removal of HP followed by steroids is the best line of management while steroids alone can be tried in cases where clinical signs of myelopathy are absent.

Vascularity Outcomes of Lingual Artery Ligation in Transoral Robotic Base of Tongue Resections.

To explore the effect of lingual artery ligation on tongue vascularity, we performed an analysis of 25 patients who underwent transoral robotic surgery for base of tongue cancers (May 2011 to December 2019). Hounsfield units of the intrinsic muscles (IMs) and genioglossus muscles (GGs) were measured in postoperative imaging (mean 4 months) as a surrogate for vascularity. In ligated patients (n = 15), the values from the ligated/resected side of the tongue were compared with the contralateral side and the nonligated side of resection. Individually, IMs and GGs on the ligated side demonstrated no significant difference to the contralateral side (P = .662 and .618, respectively). Ligation produced a significant decrease in IM measurements but no difference between GG values vs nonligated patients (P = .050 and .818, respectively). No difference was appreciated in mean values for combined IMs and GGs between cohorts (P = .212). No gross tongue atrophy or complications were incurred. Future studies are warranted to delineate long-term effects.

Bone remineralization of lytic lesions in multiple myeloma - The Arkansas experience.

Multiple myeloma (MM) patients frequently present with extensive osteolytic bone lesions. However, the impact of myeloma treatment on focal lytic lesion remineralization has not been extensively studied. In this study, the effect of anti-myeloma treatment on the extent of bone remineralization was examined and potential mediators identified. Newly diagnosed MM patients enrolled in the Total Therapy 4 and 5 (TT4; n = 231, TT5; n = 64) protocols were longitudinally evaluated for changes in radiological parameters for a median of 6.1 years. Bone remineralization was defined as a sclerotic CT change within the lytic lesion and quantified as a percentage of remineralization, using the initial lesion size as a reference. Such changes were correlated to clinical and biochemical parameters, and the gene expression profile of bone marrow biopsy. Overall, remineralization occurred in 72% of patients (213/295). Of those patients that experienced remineralization, 36% (107/295) achieved at least 25% of bone remineralization. Patients with high-risk disease defined by gene expression profile signature (GEP70 ≥ 0.66) experienced significant remineralization compared to low-risk MM. Female patients were also more likely to experience bone remineralization and in a shorter median time (2.0 vs. 3.3 y). Factors such as serum alkaline phosphatase along with high levels of RUNX2 and SOX4 gene expression correlated with increasing extent of bone remineralization. This analysis demonstrated significant remineralization of lytic lesions in MM patients treated on TT clinical trials. While the underlying mechanism remains elusive these findings support the hypothesis that patient baseline bone-related factors play a fundamental role in the skeletal repair of bone lesions in MM that provide new opportunities for improving patient outcomes.