United States

Background
Primary CNS Vasculitis is an extremely rare vasculitis with an incidence of 2.4 cases/million person-years. It can manifest in various ways, including seizures, altered mental state, headaches, and cognitive decline. When different vascular territories are affected across episodes or when headaches are associated with cognitive decline and altered mental status, CNS Vasculitis should be considered. The diagnosis of Primary CNS Vasculitis is challenging due to the potential for
other conditions to mimic it, such as neoplasms like gliomatosis cerebri or intravascular B-cell lymphoma. Our case was complicated by previous treatment for these malignancies.
Case Presentation
In our case, a 35-year-old male with a history of alcohol and cocaine use and seizures presented with transient right hemiparesis, visual deficits, and aphasia. Brain MRI revealed T2 hyperintense lesions in the left cerebral hemisphere. A subsequent neurological workup was negative for encephalitis. Two months later, the patient was admitted again after another seizure, and MRI showed progression of the left-sided brain lesions. A brain biopsy targeting the left superior parietal lobule was inconclusive. The patient underwent chemotherapy and radiation but responded more to steroids, ruling out gliomatosis cerebri. A second biopsy of the left frontal
brain lesion revealed perivascular inflammation with necrosis consistent with vasculitis, although the effects of previous treatment complicated the diagnosis
Discussion
Primary CNS Vasculitis is a rare variant of vasculitis that is still poorly understood, with an unknown etiology and pathogenesis. Immune triggers and genetic predisposition have been identified as potential factors. The disorder primarily affects small and medium-sized vessels in the brain, but spinal cord involvement has also been documented. Clinical presentation varies but commonly includes headache, altered cognition, and hemiparesis. CSF analysis is often abnormal in PCNSV cases. Diagnostic tools like angiography and MRI are helpful but not
sufficient for diagnosis as PCNSV can mimic other disorders like intravascular large B-cell lymphoma and gliomatosis cerebri. Skin biopsy can help rule out the former. Brain biopsy is crucial for a definitive diagnosis, as it has been shown that a significant percentage of cases have an alternate diagnosis following biopsy.Treatment options for PCNSV include a combination of steroids and immunosuppressants, as well as methotrexate, cyclophosphamide, and azathioprine. Prognosis is generally fair, but mortality rates are higher in cases with focal neurological deficits, cognitive impairment, cerebral infarction, and large-vessel involvement. Early detection and treatment can improve the overall
prognosis.

2023 AMA Research Challenge

Primary CNS Vasculitis Disguised as CNS Malignancy 


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Introduction
The external urethral sphincter reflex is a new neurmonitoring modality used to protect bowel, bladder, and sexual function during surgery. EUSR and bulbocavernosus reflex (BCR) assess the sacral segments of the spinal cord, cauda equina, and sacral nerve roots and provides a functional evaluation of the S2-S4 mediated reflex arc.
Objectives
We present a case study where we demonstrate for the first time the EUSR prognosticating postoperative bladder function independently of bowel function.
Methods
A 59-year-old female with hypertension and type 2 diabetes mellitus presented with a tumor causing cord compression at the T10 level. She underwent a T9-T11 laminectomy for tumor resection with a multimodal neuromonitoring paradigm including motor evoked potentials, somatosensory evoked potentials, EMG, BCR, and EUSR. The BCR and EUSR were activated using needle electrodes placed in the clitoris (cathode) and labia majora (anode) to activate the afferent sensory fibers of the dorsal clitoral nerves. Needle electrodes were placed in the left and right external anal hemisphincters to record the downstream motor component of the BCR. A specialized foley catheter embedded with 3D printed electrodes was used to record from the external urethral sphincter muscle for the EUSR.
Results
Baseline neuromonitoring data was acquired in all modalities except the EUSR. The
laminectomy and tumor resection were uneventful, and almost immediately following tumor removal, the EUSR began to reproduce and remained stable throughout the remainder of the procedure. The final pathology was benign schwannoma, and the patient was discharged intact on post-operative day 8. Follow-up at one month revealed cured urinary incontinence.
Conclusion
While this case study has limitations, it suggests that EUSR may not only provide redundancy to BCR monitoring but may discretely assess pathways associated with bladder function and prognosticate post-operative outcomes. More research with a larger cohort is needed to prove efficacy and statistical significance.

Prognostic Value of Intraoperative External Urethral Sphincter Reflexes in
Intradural Spinal Cord Surgery: A Case Report

Introduction: 

Cavernomas are low-flow vascular lesions made up of a collection of dilated capillaries with an adventitia and endothelial lining (1). Cavernous angiomas make up around 10-15% of all vascular abnormalities of the central nervous system (CNS) (2,3). These lesions can be sporadic or familial, and they have a frequency of 0.5% and an incidence of 0.3-0.7%. Females are somewhat more likely to get these lesions than men (3,4). The optic pathway, which is made up of the optic nerve, chiasm, and optic tract, can be affected in some highly unusual scenarios (2,3,5). Of the CNS cavernous malformations, optic pathway cavernomas make up fewer than 1%.(2,6). It was Uihlein et al. who first described anterior visual pathway CM in the literature in 1958(8). Cavernous malformations of the anterior visual pathway exhibit distinctive changes on MRI, with a hypointense to isointense signal on T1-weighted images and a heterogeneous "popcorn" look on T2-weighted images with mixed bright and dark signals. Gradient-echo T2 sequences enhance this dark signal because of a "blooming effect" brought on by the deposition of haemosiderin inside and around the CM (9).
Seizures are the most prevalent sign of symptomatic CMs in general, however, the symptoms of optochiasmatic CMs can vary, and many individuals remain asymptomatic. (7) . Although less common, progressive vision loss may occur, typically accompanied or preceded by headaches, nausea, and focal impairments. (5,7). The conventional therapy is gross total resection (GTR), which has resulted in symptom relief in the vast majority of patients. (1)

Case Presentation:
This 37-year-old male, a known case of Suprasellar Cavernous Angioma, presented with complaints of right-eye blurring for the past few days with no other symptoms. An MRI brain revealed a suprasellar heterogeneous tumor of <2cm. The patient previously had suprasellar cavernous angioma surgery in 2019. Testing for visual acuity showed 6/24 in the right eye and 6/6 in the left. No other investigative findings were found. The patient has no history of diabetes, hypertension, coronary artery disease, or asthma.
Operation:
LEFT PTERIONAL CRANIOTOMY AND TOTAL EXCISION surgery was performed on the patient. Supine ETGA. The head was secured in place by a three-pin Mayfield head rest. The standard procedure for a left-sided pterional craniotomy was used. The Sylvian fissure had been split. The left optic nerve and internal carotid artery were found to have numerous dense adhesions from earlier surgery that were difficult to dislodge. The tumor had bled and increased in size. A yellowish solid component was found embedded in the chiasm, which had been pushed anteriorly and to the left, along with the Right Optic nerve. Microsurgical excision was performed with care. In order to preserve vision, minute pieces adhered to the underside of the optic apparatus had to be left inside. Dura was reinforced with a patch, and the bone flap was secured using miniplates and a screw. The wound was closed in layers. The patient was treated with antibiotics, antidiabetics, analgesics, antiepileptics, anti-inflammatories, anti-emetics, steroids, gastro protectives, and other supportive measures. Histopathological sections showed brain parenchyma with areas of hemorrhage, dilated congested blood vessels, and haemosiderin laden macrophages leading to a diagnosis of cavernous hemangioma.
Discussion:
Observation:
In this case report, we present the case of a 37-year-old male with recurrent suprasellar cavernous angioma who experienced visual loss. Suprasellar cavernous angiomas are uncommon vascular lesions that can lead to neurological symptoms, including visual disturbances. Timely diagnosis and appropriate management are crucial to prevent irreversible neurological complications.
The patient had a previous history of suprasellar cavernous angioma and had undergone surgical intervention in 2019. However, he developed visual loss, which prompted further evaluation. Magnetic resonance imaging (MRI) revealed a recurrent heterogenous mass measuring less than 2 cm in the suprasellar region. Subsequently, the patient underwent a left pterional craniotomy with the intention of completely excising the mass.
During the surgical procedure, the presence of dense adhesions from the previous surgery posed challenges in separating the adhesions around the left optic nerve and internal carotid artery. Intraoperatively, the tumor was found to have bled and increased in size. Microsurgical excision was performed with careful consideration to preserve visual function. However, due to the adherence of tumor fragments to the undersurface of the optic apparatus, complete removal was not feasible, resulting in residual tumor remaining.
Histopathological examination of the excised tissue confirmed the diagnosis of cavernous hemangioma. The surgical intervention successfully alleviated compression on the optic pathways and improved the patient's visual symptoms. Postoperatively, the patient received a comprehensive treatment regimen, including appropriate antibiotics, antidiabetic agents if needed, analgesics, antiepileptics, anti-inflammatory medications, anti-emetics, steroids, and gastroprotective measures. Long-term follow-up is essential to monitor for tumor recurrence and evaluate visual outcomes. Suprasellar cavernous angiomas present management challenges due to their location and the associated risk of intraoperative bleeding. Surgical resection remains the mainstay of treatment, aiming for complete excision while minimizing damage to critical structures. In situations where complete resection is not attainable, partial resection can still provide clinical benefits.
Early diagnosis and prompt intervention are critical in preventing irreversible neurological complications associated with suprasellar cavernous angiomas. Long-term follow-up is necessary to monitor for tumor recurrence and assess visual outcomes. A multidisciplinary approach involving neurosurgeons, ophthalmologists, and other healthcare professionals is essential for comprehensive management.
In conclusion, our case underscores the significance of timely intervention in cases of recurrent suprasellar cavernous angiomas presenting with visual loss. Surgical resection, despite its challenges, can offer symptomatic relief and improve visual function. Further research is warranted to investigate optimal treatment strategies and long-term outcomes for these rare vascular lesions.
Lessons:
Cavernous hemangiomas of the optic pathway are extremely rare (<1%), and the involvement of the optic nerve in the optic canal is even rarer. Diagnosing cavernous malformations (CMs) of the optic pathway preoperatively is challenging and often can be misdiagnosed. The relevance of this case, therefore, is that in a patient with loss of visual acuity and visual field defects, it is extremely important for clinicians to keep cerebral cavernous malformations in their differential diagnosis. [3]
MRI is the most specific and sensitive imaging modality to diagnose CMs, the mainstay of treatment for which is radical resection. It is a challenging procedure, but usually curative and associated with favorable outcomes. [7]
Surgery for radical resection of the CM is a technically challenging procedure that has a high risk of morbidity and death. The hazards, which include pre- and/or post-operative bleeding, hormone imbalances, and transient or permanent nerve palsies are principally brought on by the complex neuroanatomy of the CS-sellar area. The literature only contains a small number of recorded cases claiming effective total eradication as evident in our case, wherein some minute pieces of the lesion had to be left behind in order to prevent vision loss.
In instances when complete removal implies excessive risks during the perioperative period, surgical reduction with cranial nerve decompression, followed by stereotactic radiosurgery, is currently regarded as the best choice. []
In all cases, the surgeon's experience, anatomical knowledge, and microsurgical skills are important factors that influence the final outcome. [7] With our case, we aim to highlight the importance of early surgical intervention in order to prevent regrowth and fatal complications.

Optic Pathway Cavernous Malformations - A Rare Cause Of Vision Loss

**Scoring is only possible in the semifinals hall**

**Abstract:**



Incidental Finding of Fahr’s Disease in Patient with Stroke-Like Symptoms Without Acute Infarction

Acute Liver Injury from High Dose Pulsed Steroid Therapy in a Patient with Multiple Sclerosis: A Case Report

Learning Objective:
Identify Wernicke encephalopathy and copper deficiency as potential causes of neurologic abnormalities.
Case:
Wernicke encephalopathy is a potential consequence of heavy alcohol use. It classically presents with disorientation, ocular motor dysfunction, and ataxia. However, other medical complications may muddle this clinical picture. A 26-year-old previously healthy female presented with several months of worsening bilateral lower extremity weakness. She had multiple shots of hard liquor daily for several years and a low appetite for the past month following a friend’s death. Her last drink was three days before admission. On admission, vital signs were stable except for mild tachycardia. The patient was completely disoriented and unable to ambulate due to unsteadiness. She had an abnormal finger-to-nose test, bilateral vertical nystagmus, and stocking-glove pattern neuropathy. She also had leukopenia, normocytic anemia, and low serum thiamine, folate, and copper. HIV, RPR, vitamin B12 level, methylmalonic acid, urine drug screen, and lumbar puncture were all unremarkable. MRI brain was negative for mamillary body or thalamic changes. She was empirically treated for Wernicke encephalopathy using IV thiamine 500 mg TID for 2 days, then 200 mg daily for 5 days. After completing this, she was discharged with oral thiamine and folate supplementation for alcohol use disorder and gabapentin for neuropathy. She was instructed to purchase over-the-counter copper supplements for her copper deficiency. The patient made a full recovery other than she did not remember being hospitalized during a follow-up phone visit.
Discussion:
This patient’s neurological status was initially attributed to Wernicke encephalopathy given her disorientation, vertical nystagmus, and positive finger-to-nose test. However, she incidentally had low serum copper, which can be treated with oral supplements or cocoa. Copper deficiency is not classically associated with heavy alcohol use, though there has been literature hinting towards some association. Low copper can lead to leukopenia, normocytic anemia, and stocking-glove pattern neuropathy, all of which were present in this patient. She also lacked the contrast enhancement of the thalamus and mamillary bodies that is present in patients with Wernicke encephalopathy, which suggests that her clinical presentation was not solely from Wernicke encephalopathy. This case illustrates that although Wernicke encephalopathy is usually in the differential diagnosis of patients with neurologic abnormalities in the setting of heavy alcohol use, copper deficiency should also be included. Properly managing patients with such a complex clinical picture is essential to improve their neurologic status, avoid irreversible complications such as Korsakoff psychosis, and treat reversible vitamin and mineral deficiencies.

Mineral Mining: A Confounding Case of Wernicke Encephalopathy

Background:
Creutzfeldt-Jakob Disease (CJD) is a neurodegenerative process that manifests as a rapidly progressive dementia with a usual poor prognosis within 12 months. CJD can be further grouped into three main categories: sporadic (sCJD), genetic (gCJD), and acquired (aCJD). The worldwide incidence is around 1.5 to 2.0 per million person-years and about 85-95% of CJD can be attributed to sporadic CJD. The presentation of this disease can vary and therefore requires a criterion to make this consideration carefully. For sporadic CJD to meet the criteria for “probable,” it must have 3 main criteria: (1) Clinical findings of a neuropsychiatric disorder with a positive positive Real Time Quaking Induced Conversion (RT-QuiC) test or rapid dementia plus at least two of the following symptoms: myoclonus, visual or cerebellar abnormalities, pyramidal/extrapyramidal signs, and akinetic mutism, (2) a typical EEG findings of periodic sharp wave complexes, positive 14-3-3 CSF assay in disease duration of less than 2 years, or high signal in caudate/putamen regions on MRI brain scan or at least two cortical regions either on DWI or FLAIR, and (3) exclusion of other causes in diagnostic workup.

Case Presentation:
We report the case of a 61-year-old woman with a past medical history of hypothyroidism, psoriasis, and hypertension that presented for acute onset, rapidly progressive cognitive decline that was 1 month in duration. The patient’s clinical presentation of rapid cognitive decline, subtle cerebellar and upper motor neuron findings (e.g. pseudobulbar affect, slow speech), and MRI brain findings of cortical ribboning and restricted diffusion in the caudate nucleus was most consistent with probable sporadic CJD. Cerebral spinal fluid analysis revealed a positive Real Time Quaking Induced Conversion (RT-QuiC) test, t-tau protein of 18531 pg/mL (0-1149 pg/mL), and 14-3-3 gamma protein of 61440 Au/mL (<30-1999 Au/mL). An EEG revealed pseudo periodic triphasic sharp wave complexes over the left frontotemporal regions. Additional laboratory findings were obtained to rule out mimickers such as autoimmune encephalitis, Hashimoto’s encephalopathy, and CNS vasculitis. Upon receiving diagnosis, the patient’s condition rapidly worsened and died after 1 month possibly due to respiratory arrest.

Discussion:
This patient had an “atypical” presentation due to absence of hallmark symptoms such as myoclonus, however, the rapid course of the disease was consistent with the nature and course of sCJD. Definitive diagnosis of CJD requires neuropathological evaluation of brain tissue via immunohistochemistry or protease-resistant PrP/scrapie-associated proteins on Western blot via autopsy post-mortem. The patient presented in this report did not have an autopsy, however, with information gathered based on criteria from Center of Disease and Control, we have high clinical suspicion that this patient had probable sporadic CJD. There is no current FDA approved treatment that is available to treat this disease.

A Rare Case of Probable Sporadic Creutzfeldt-Jakob Disease

Background
Myasthenia gravis is classically described in the literature as an autoimmune disorder with antibodies directed at the acetylcholine receptor on the motor end plate. This autoimmune destruction of these key areas of the interface between the nervous system and the musculoskeletal system leads to weakness and fatigue in patients. Rarely in the literature has this been described as a molecular mimicry event secondary to viral illnesses like that of COVID-19. 

Case Report
Herein the authors describe the case of a 71-year-old right-handed female who presented with key symptoms of dyspnea, drooping eye lids, difficulty lifting her neck, dysphagia, slurring of speech, bilateral shoulder weakness, and needing to change eating positions due to discomfort of her shoulders and arms. The patient endorsed her eyelid drooping worsened in the later part of the day such that she had to tape her eyelids open in order to see. The patient underwent ice testing, trial of pyridostigmine, was discharged with a prescription for prednisone and pyridostigmine, set up with outpatient follow up, and was given a working diagnosis of COVID-19 induced myasthenia gravis.

Discussion
This case is important as it highlights three key lessons of importance within the literature on myasthenia gravis. First is the transient worsening of a patient when given corticosteroids. In high doses, steroids can worsen or send a person into myasthenic crisis which is the reason that they should not be utilized as primary treatment in crisis. Second is the etiology of myasthenia gravis, often there is not found an antecedent event in patients, however, the history of this case made it clear that this was likely a molecular mimicry event secondary to a COVID-19 infection. Thus, the authors desire to remind the readership of the importance of a thorough history and physical examination. Lastly, as a part of the diagnostic work up, the clinicians utilized an older physical examination maneuver known as the ice test. When utilized, this test has a high negative predictive value and therefore still has a place in bedside diagnostics when ruling out myasthenia gravis.

"It's been downhill since COVID": Myasthenia Gravis secondary to COVID Infection

Background:
Brainstem abscesses are collections of pus within the brainstem, which can cause inflammation, tissue damage, and compression of adjacent structures, leading to headache, fever, and focal neurological deficits. Brainstem abscesses are considered rare and potentially life-threatening, and pontine abscesses are even rarer. Both are often caused by the spread of infection from nearby structures like the middle ear, sinuses, and mastoid air cells but can also result from hematogenous spread. 

Case Presentation:
A 54-year-old male presented with numbness and clumsiness in his left hand and leg over a three-day period. These symptoms were preceded by a headache that awoke him the night before without accompanying fever. An initial brain magnetic resonance imaging (MRI) showed a 1.5 cm ring-enhancing lesion in the right pons. The patient’s blood cultures were negative as were screenings for tuberculosis, methicillin-resistant Staphylococcus aureus, human immunodeficiency virus, and hepatitis B virus; cysticercosis antibody testing was equivocal. The patient was started on ceftriaxone, vancomycin, and metronidazole.

Three days later, the patient showed worsening weakness in his left arm and leg. A repeat MRI showed thickening of the enhancing wall and formation of another lesion in the right dorsal paramedian pons. A lumbar puncture revealed 11 WBCs, two RBCs, a glucose level of 76 mg/dL, and a protein level of 62 mg/dL. Cerebrospinal fluid was clear, colorless, and negative for cultures, toxoplasma IgG, cryptococcal antigen, human simplex virus, and acid-fast bacteria. 

The patient underwent transfrontal stereotactic drainage of the lesion which revealed 1.3 cc of a purulent, foul-smelling material and the cultures of which grew Streptococcus pyogenes. A postoperative MRI showed a 0.85 cm abscess without hemorrhage. The patient stated improvements in strength and functionality but residual hemiparesis. After eight weeks, all antibiotics were stopped after verifying decreasing abscess size on an MRI.

Discussion:
The patient presented with a pontine abscess of both undetermined causal origins and unclear infections signs, namely the lack of fever and nausea which are commonly associated with brainstem abscess. Additionally, the stereotactic aspiration cultures grew Streptococcus pyogenes, which can exist in the oropharynx, yet our patient denied recent upper respiratory illness. 
This case reveals several learning points on cytogenic brainstem abscesses: the importance of early neuroimaging by MRI for a definitive diagnosis of brain abscesses and the value and utility of stereotactic aspiration, a minimally invasive procedure, which can treat nearly all brain abscesses measuring at least 1 cm, regardless of the location.


Cryptogenic Pontine Abscess Treated With Stereotactic Aspiration: A Case Report

Psychogenic Non-Epileptic seizures: Complex diagnosis and Interdisciplinary Management

Background: Vaginal Birth After Cesarean (VBAC) plays an essential role in current strategies to lower cesarean rates. Despite the American College of Obstetrics and Gynecology (ACOG) endorsement, Trial of Labor after Cesarean (TOLAC) attempt rates remain low. The Zavanelli maneuver is considered a last resort procedure used in the management of severe shoulder dystocia unable to be relieved by other techniques. It is the cephalic replacement of the fetal head back into the pelvis immediately followed by cesarean delivery. We discuss a case of a woman determined to have a vaginal birth after her prior birth required this heroic measure to facilitate delivery.

Case Presentation: A 34-year-old G4P1021 presented seeking care with a history of a complicated delivery requiring Zavanelli maneuver of a 4.4kg female infant. She was determined to avoid a repeat cesarean delivery and desired to pursue a trial of labor after cesarean section (TOLAC) this pregnancy. She addressed her modifiable risk factors and decreased her pre-pregnancy body mass index (BMI) to 26 from 39 kg/m2. She avoided excess weight gain during pregnancy (with a 25 pound gain versus 50 in her prior pregnancy). Her lifestyle changes influenced improvement in fetal growth with a current estimated fetal weight in the 50th percentile, compared to 95th percentile in her prior and her avoidance of gestational diabetes; whereas she was previously diagnosed. Consultation with three different perinatologists, all agreed her pursuit of TOLAC was reasonable. Nonetheless, she had to travel across state lines to find a provider willing to permit a trial of labor. She then presented in spontaneous active labor at 41 weeks gestation and went on to deliver a 3350g female infant in an uncomplicated vaginal delivery. She described this birth experience as euphoric.

Discussion: Practitioner fear was demonstrated as a barrier to this patient’s autonomy in delivery route decision. Risk factors for shoulder dystocia may vary in each pregnancy. Improvement in the overall risk profile should not be ignored when counseling patients and deciding on the best delivery route for that and future pregnancies. Decisions must be based on a thorough analysis of risk factors, past and present, and individualized to meet the goals of a woman's reproductive career.


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Prognostic Value of Intraoperative External Urethral Sphincter Reflexes in Intradural Spinal Cord Surgery: A Case Report

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