Sacroiliac (SI) joint pathology is a newly appreciated contributor to lower back pain. Sacroiliac joint fusion (SIJF) has grown rapidly in popularity in association with the advent of minimally-invasive surgical techniques. This has led to an explosion of new medical devices used for SIJF. The objective of this article is to outline clinical trends, summarize the current data, and categorize novel devices for SIJF. Trends in SI joint pathology and fusion were obtained via the Healthcare Cost and Utilization Project's (HCUP) National Inpatient Sample (NIS) database and Web of Science. To review literature on devices for SIJF, PubMed was searched using the Boolean phrase "sacroiliac joint AND (fusion OR arthrodesis)" since 2010. To establish a list of SIJF devices not represented in the literature, searches were performed on the FDA 510(k), premarket approval, and de novo databases, as well as Google and LinkedIn. Literature review yielded 11 FDA-approved devices for minimally invasive SIJF. Database query yielded an additional 22 devices for a total of 33 devices. Twenty-one devices used the lateral transiliac approach, six posterior allograft approach, three posterolateral approach, and three combined the lateral transiliac and posterolateral approaches. The evidence for the lateral transiliac approach is the most robust. Many novel devices have been developed for minimally invasive SIJF over the past 10 years. Further randomized comparative trials are warranted to evaluate different surgical approaches and novel devices at this time.

INTRODUCTION: Deep brain stimulation (DBS) is a well-documented therapy for dystonia utilized in many adult and pediatric movement disorders. Pedunculopontine nucleus (PPN) has been investigated as a DBS target primarily in adult patients with dystonia or dyskinesias from Parkinsons disease, showing improvement in postural instability and gait dysfunction. Due to the difficulty in targeting PPN using standard techniques, it is not commonly chosen as a target for adult or pediatric pathology. There is no current literature describing the targeting of PPN in DBS for childhood-onset dystonia. METHODS: Two pediatric and one young adult patient with childhood-onset dystonia who underwent DBS implantation at our institution were identified. Patient 1 has Mitochondrial Enoyl CoA Reductase Protein-Associated Neurodegeneration (MEPAN) syndrome. Patient 2 has Glutaric Aciduria Type 1 (GA1). Patient 3 has atypical pantothenate kinase-associated neurodegeneration (PKAN). PPN was identified as a potential target for these patients due to axial or orofacial dystonia. Pre- and post-operative videos taken as part of routine clinical assessments were evaluated and scored on the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and Barry-Albright Dystonia Scale (BADS). All patients had permanent electrodes placed bilaterally in PPN and globus pallidus internus (GPi). A Likert scale on quality of life was also obtained from the patient/parents as applicable. RESULTS: Significant programming was necessary over the first 3-12 months to optimize patients response to stimulation. All patients experienced at least a 34% improvement in the BFMDRS score. Patients 2 and 3 also experienced an over 30% improvement in BADS score. All patients/parents appreciated improvement in quality of life postoperatively. DISCUSSION: Deep brain stimulation in PPN was safely and successfully used in two pediatric patients and one young adult patient with childhood-onset dystonia. These patients showed clinically significant improvements in BFMDRS scoring post operatively. This represents the first reported DBS targeting of PPN in pediatric patients, and suggests that PPN is a possible target for pediatric-onset dystonia with axial and orofacial symptoms that may be refractory to traditional pallidal stimulation alone.

INTRODUCTION: Mitochondrial Enoyl CoA Reductase Protein-Associated Neurodegeneration (MEPAN) syndrome is a rare inherited metabolic condition caused by MECR gene mutations. This gene encodes a protein essential for fatty acid synthesis, and defects cause progressively worsening childhood-onset dystonia, optic atrophy, and basal ganglia abnormalities. Deep brain stimulation (DBS) has shown mixed improvement in other childhood-onset dystonia conditions. To the best of our knowledge, DBS has not been investigated as a treatment for dystonia in patients with MEPAN syndrome. METHODS: Two children with MEPAN were identified as possible DBS candidates due to severe generalized dystonia unresponsive to pharmacotherapy. Temporary depth electrodes were placed in six locations bilaterally and tested during a 6-day hospitalization to determine the best locations for permanent electrode placement. The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and Barry-Albright Dystonia Scale (BADS) were used for preoperative and postoperative testing to quantitatively assess dystonia severity changes. Patient 1 had permanent electrodes placed at the globus pallidus internus (GPi) and pedunculopontine nucleus (PPN). Patient 2 had permanent electrodes placed at the GPi and ventralis intermedius nucleus of the thalamus (VIM). RESULTS: Both patients successfully underwent DBS placement with no perioperative complications and significant improvement in their BFMDRS score. Patient 2 also demonstrated improvement in the BADS. DISCUSSION: We demonstrated a novel application of DBS in MEPAN syndrome patients with childhood-onset dystonia. These patients showed clinically significant improvements in dystonia following DBS, indicating that DBS can be considered for dystonia in patients with rare metabolic disorders that currently have no other proven treatment options.

Abstract

BACKGROUND

Spindle cell oncocytoma (SCO) of the pituitary gland is an extremely rare non-functional WHO grade 1 tumor. SCO are often misdiagnosed as nonfunctional pituitary adenomas on pre-operative imaging. They are often hypervascular and locally adherent, which increases hemorrhage risk and limits surgical resection, leading to increased risk of recurrence. We report a case of SCO treated at our institution and provide a review of the current literature.

METHODS

A 75-year-old male with a history of hypertension, left thalamic stroke, Parkinson’s disease, and normal pressure hypertension presented to neurosurgery clinic with bitemporal hemianopsia, hyponatremia, and abnormal gait and mobility. Imaging showed an enhancing intra- and suprasellar, hyperdense tumor mass measuring 3.0 cm in diameter. We performed a systemic literature search in the PubMed database to identify previous reports of spindle cell oncocytoma. After exclusion of studies that did not meet criteria, 32 publications were selected for critical reading.

RESULTS

The patient underwent an endoscopic transsphenoidal resection of the tumor via a multi-disciplinary team. The tumor was fibrous and adherent to the intrasellar dura, with gross invasion of the diaphragm sella, necessitating partial resection of the diaphragm. The defect was repaired, and the patient made an uncomplicated recovery. Post-operatively, the patient experienced improved vision. Upon literature review, SCO present in older adults with an average age of 56.2 ± 14.7 with visual deficits (67.9%), headache (33.3%), hypopituitarism (24.7%), and nausea (11.1%). Full resection was achieved in 38.6% of cases leading to recurrence rate of 23.5% with an average time until recurrence of 32.5 months (range 1-120 months).

CONCLUSION

Careful surgical technique is needed due to SCO hypervascularity and strong adherence to minimize risk of injury to surrounding neurovascular structures. Long-term follow up is recommended due risk of recurrence.

We describe a case of a young patient with a recurrent pleomorphic xanthoastrocytoma (PXA) showing unusual cell-in-cell (CiC) phenomena. We observed mostly viable but also necrotic neutrophils engulfed within tumor cells. The recurrent tumor was immunopositive for BRAFV600E mutant protein and showed CDKN2 homozygous deletions typical of PXA. Both genetic alterations were also reported in the original primary tumor. Unlike the original tumor that was GFAP and Olig-2 immunopositive, the recurrent neoplasm was largely negative for GFAP and Olig-2 suggesting dedifferentiation. The large malignant cells that contained the neutrophils were negative for histiocytic and lymphohematopoietic markers. Whereas CDKN2 homozygous deletion is common in PXA, its presence is rare in histiocytic neoplasms. Both reactive astrocytes and glial neoplasms very rarely may engulf neutrophils in a process resembling emperipolesis or cellular cannibalism. Future work may clarify which type of CiC pathway is involved.

Recently, the California Department of Public Health issued an advisory related to the substantial rise in Coccidioidomycosis in California, which has been attributed in part to climate change and rapid housing development. Most cases are self-limiting, but some may spread to the meninges, resulting in coccidioidal meningitis (CM). Many providers mistakenly presume that CM is limited to patients who are immunocompromised. In this case series and literature review, we present 12 cases of CM in immunocompetent individuals seen at a single tertiary academic center between 1 January 2019 and 31 December 2023. All 12 cases developed complications, with 10 requiring ventriculoperitoneal shunting, 6 having spinal cord involvement (5 with cervical spine involvement), 4 having strokes, and 3 dying from complications related to CM. It is important to recognize CM as it may be life-threatening if not promptly diagnosed.

Background

Spindle cell oncocytoma (SCO) of the pituitary gland is an extremely rare nonfunctional World Health Organization grade I tumor. SCOs are often misdiagnosed as nonfunctional pituitary adenomas on the basis of preoperative imaging. They are often hypervascular and locally adherent, which increases hemorrhage risk and limits resection, leading to increased risk of recurrence. The authors report a case of SCO treated at their institution and provide a review of the current literature.

Observations

SCO of the pituitary gland can be a rare cause of progressively growing pituitary tumors that presents similarly to nonfunctional pituitary adenoma. Endoscopic transsphenoidal resection of the tumor by a multidisciplinary team allowed total resection despite local adherence of the tumor. Postoperatively, the patient's visual symptoms improved with persistence of secondary adrenal insufficiency and secondary hypothyroidism.

Lessons

Careful resection is needed due to SCO's characteristic hypervascularity and strong adherence to minimize local structure damage. Long-term follow-up is recommended due to the tendency for recurrence.