Gluten Ataxia and mGluR1 Autoimmune Encephalitis Presenting as Acute Cerebellar Ataxia: A Case Report.

A Caucasian male in his 60s presented with acute onset of dizziness, dysarthria, and gait ataxia. Upon extensive workup, positive findings were cerebrospinal fluid (CSF) showing lymphocytic pleocytosis with oligoclonal bands, positive celiac disease autoantibodies in blood, a duodenal biopsy indicating lymphocytic infiltration, and positive anti-mGluR1 antibody titers in CSF. The patient was started on a strict gluten-free diet and intravenous immunoglobulin therapy for 5 days and showed mild consecutive improvements each day of treatment. He was discharged after 22 days, and was encouraged to continue gluten adherence, physical and speech therapy, and follow up with neuroimmunology. This report demonstrates that autoimmune encephalitis due to anti-mGluR1antibodies and gluten ataxia are both immune-mediated disorders that should be considered in acute cerebellar ataxia cases. By broadening the differential diagnosis and a comprehensive CSF analysis, identification of gluten ataxia and autoimmune encephalitis were beneficial in the management of this particular patient.

Acute Osmotic Central Pontine Demyelination in an Alcoholic Patient With Normal Sodium Levels and a Vitamin B6 Deficiency.

We report a Native American male in his 50s with a complex medical history including alcohol use disorder and seizure disorder who presented with complaints of generalized weakness and multiple falls. The patient was admitted for altered mental status, community acquired pneumonia, sepsis, and bacteremia. On hospital day 23, the patient reported a sudden onset of sensation of food stuck in his upper chest. Brain MRI confirmed osmotic demyelination syndrome (ODS) within the central pons. Further workup revealed this finding was likely due to malnutrition, alcoholism, hypoalbuminemia, and vitamin B6 deficiency. However, the patient presented with normonatremia throughout his entire hospital stay. After acute onset of ODS, the patient was transferred to the ICU where he continued to decline. After 68 days from initial presentation, the patient died in hospice care from myelinolysis complications. This case demonstrates a case of ODS of the central pons in a patient with normonatremia, hypoalbuminemia, and severe vitamin B6 deficiency.

Thoracoabdominal aneurysm repair using the Unitary Manifold Device.

OBJECTIVE: To present a single-center prospective study of 126 consecutively treated patients who underwent endovascular repair of a thoracoabdominal aortic aneurysm with the physician-modified, nonanatomic-based Unitary Manifold (UM) device. METHODS: Data were collected from 126 consecutive all-comer patients treated with the physician-modified, nonanatomic-based UM from 2015 to 2023. Treatment was performed at a single center by a single physician under a Physician Sponsored Investigation Exemption G140207. RESULTS: The UM was indicated for repair of all Crawford extents including juxtarenal, pararenal, and short-neck infrarenal aneurysms (<10 mm) in 126 consecutive patients. Patients were not excluded from the study based on presentation, extent of aneurysm or dissection, or history of a spinal cord event. Patients with a thoracoabdominal aortic aneurysm were categorized by Crawford classification: types I and V (3.3%, n = 4), type II (3.3%, n = 4), type III (1%, n = 1), and type IV (93.3%, n = 117). The type IV classification patients were further categorized with 33 (28.2%) true type IV, 68 (58.1%) pararenal or infrarenal, and 16 (13.7%) with dissection. Technical success was 99.2% (n = 125). The most common major adverse event within both 30 days and 365 days of all patients was respiratory failure (11.9%, n = 15, and 13.5%, n = 17, respectively). One patient (0.8%) experienced persistent paraplegia at 365 days. Reintervention for patients at 365 days was 5.6% (n = 7). Of the 444 branches stented, the primary patency rate was remarkably high as only three patients (2.4%) required reintervention due to loss of limb patency within 365 days. Aneurysm enlargement (≥5 mm) occurred in 1.6% (n = 2) patients, and no patients experienced aneurysm rupture. No patients underwent conversion to open repair. The aneurysm-related mortality at 365 days for all patients was 4.0% (n = 5), whereas all-cause mortality was 16.7% (n = 21). Physician-modified endograft device integrity failure was not observed in any patient. CONCLUSIONS: The UM device demonstrated remarkable technical surgical success, treatment success, and device patency rates with very reasonable major adverse events and reintervention rates. This study is the most representative example of the general population in comparison with other studies of off-the-shelf devices, with 126 consecutive all-comer patients with diverse pathologies.

Chronic Nerve Root Injury in Pediatric Patients With Chronic Lumbar Stress Fractures: A Retrospective Review.

INTRODUCTION: Many orthopedic providers currently treat chronic spondylolysis as self-limited fractures. While the condition has previously been associated with back pain in pediatrics, there has been little attention on the risk of neurologic harm. Electromyography (EMG) is a common study used to evaluate nerve injury, but it has not been previously reported for testing pediatric patients with stress fractures. In this study, pediatric patients with chronic pars fractures and muscle extremity weakness who underwent EMG testing were reviewed to analyze their risk of chronic nerve injury. METHODS: 120 pediatric patients who underwent EMG testing between 2015 and 2021 were analyzed, and 41(21F,20M) patients with a mean age of 16(13-20) met criteria of chronic lumbar pediatric spondylolysis with weakness on ankle dorsiflexion or plantarflexion. No exclusions were made. Initial EMG testing was indicated for the extremity weakness; pain was not the major concern. All exams were completed by JWM. Thin-cut lumbar CT studies were done at the same institution, and EMGs were completed by one of three physiatrists. EMGs were determined as normal, abnormal but not meeting chronic nerve injury threshold, or abnormal and meeting threshold for chronic nerve injury. RESULTS: Of the 41 patients, 33 had bilateral and 8 had unilateral fractures with 95% (39/41) of them located at L4 or L5. 55% (18/33) of the bilateral fractures had abnormal EMGs and demonstrated chronic nerve injury; 1 had an abnormal EMG but did meet chronic injury threshold. 75% (6/8) of the unilateral fractures had abnormal EMGs and demonstrated chronic nerve injury; 1 had an abnormal EMG but did meet chronic injury threshold. Overall, 36% (15/41) had normal EMGs, 5% (2/41) had abnormal results but did not reach chronic injury threshold, and 59% (24/41) met threshold for chronic nerve injury. CONCLUSION: Chronic pars fractures have historically been treated as a benign and self-limited sports injury. However, our analysis showed that 59% of adolescent patients presenting with chronic pars fractures and dorsiflexion or plantarflexion weakness have developed a chronic nerve injury. This study is the first to demonstrate the risk of neurologic harm in unhealed pediatric lumbar stress fractures, and it indicates the importance of EMG testing in young patients presenting with chronic spondylolysis and extremity weakness.