Severe Headache Research Articles

Abstract 130: Endovascular Therapy for Cerebral Venous Sinus Thrombosis: A Systematic Review of 267 patients

Introduction Cerebral venous sinus thrombosis (CVST) is a rare form of stroke that usually affects young females especially during pregnancy and the postpartum period. Several risk factors can contribute to the development of cerebral venous sinus thrombosis including hypercoagulable states, dehydration, infections, head or neck injuries, cancer, and some rheumatological diseases (1). The symptoms of cerebral venous sinus thrombosis can vary depending on the location and extent of the clot. Common signs and symptoms may include severe headaches, visual disturbances, seizures, focal neurological deficits, altered mental status, and in severe cases, coma. The initial treatment for CVST is systemic anticoagulation (2‐3). However, endovascular treatment (EVT) of CVST is typically reserved for cases associated with significant thrombus burden and lack of clinical improvement or worsening of symptoms despite systemic anticoagulation therapy without current conclusive evidence of its benefit. The aim of this study is to evaluate the evidence of EVT in patients with CVST. Methods A systemic literature search was conducted via PubMed to identify relevant studies. Only studies with sample sizes larger than 10 were included. We excluded small case series, meta‐analyses, systematic reviews, and studies that lack the main outcomes of interest. Our main outcomes included functional outcome defined as modified Rankin Scale score (mRS 0‐3), mortality, new or expanded ICH and recanalization rates. Results We finally included 10 studies (4‐13) in our qualitative synthesis. Only one study was a small, randomized clinical trial while the rest were observational studies. In our included studies, 267 patients underwent EVT for CVST. Among them, the median age was ranging from 12 to 75 and 162 (60 %) of patients were females. Patients were followed up for a mean ranging from 3 to 12 months. Recanalization was observed in 161 patients, out of which 111 were complete recanalization (68.9%) and 50 were partial recanalization (31.1%). Good functional outcome defined as mRS 0‐3 was achieved in a total of 181 patients. Additionally, 26 (9.7%) patients had a new onset or expanded ICH while 26 patients had died (9.7%). Conclusion EVT for CVST was associated with improved clinical outcomes with acceptable safety profile in severe cases. There is a lack of evidence from large randomized clinical trials so more prospective studies are needed to confirm these results.

Mast-cell-specific receptor mediates alcohol-withdrawal-associated headache in male mice

Rehabilitation from alcohol addiction or abuse is hampered by withdrawal symptoms including severe headaches, which often lead to rehabilitation failure. There is no appropriate therapeutic option available for alcohol-withdrawal-induced headaches. Here, we show the role of the mast-cell-specific receptor MrgprB2 in the development of alcohol-withdrawal-induced headache. Withdrawing alcohol from alcohol-acclimated mice induces headache behaviors, including facial allodynia, facial pain expressions, and reduced movement, which are symptoms often observed in humans. Those behaviors were absent in MrgprB2-deficient mice during alcohol withdrawal. We observed invivo spontaneous activation and hypersensitization of trigeminal ganglia (TG) neurons in alcohol-withdrawal WT mice, but not in alcohol-withdrawal MrgprB2-deficient mice. Increased mast cell degranulation by alcohol withdrawal in dura mater was dependent on the presence of MrgprB2. The results indicate that alcohol withdrawal causes headache via MrgprB2 of mast cells in dura mater, suggesting that MrgprB2 is a potential target for treating alcohol-withdrawal-related headaches.

Investigation of the Differences in the Pharmacokinetics of CYP2D6 Substrates, Desipramine, and Dextromethorphan in Healthy African Subjects Carrying the Allelic Variants CYP2D6*17 and CYP2D6*29, When Compared with Normal Metabolizers.

This study investigated the differences in the pharmacokinetics (PK) of dextromethorphan and desipramine in healthy African volunteers to understand the effect of allelic variants of the human cytochrome P450 2D6 (CYP2D6) enzyme, namely the diplotypes of CYP2D6*1/*2 (*1*1, *1*2, *2*2) and the genotypes of CYP2D6*17*17 and CYP2D6*29*29. Overall, 28 adults were included and split into 3 cohorts after genotype screening: CYP2D6*1/*2 (n=12), CYP2D6*17*17 (n=12), and CYP2D6*29*29 (n=4). Each subject received a single oral dose of dextromethorphan 30mg syrup on day1 and desipramine 50mg tablet on day8. The PK parameters of area under the plasma concentration-time curve from time of dosing to time of last quantifiable concentration (AUClast), and extrapolated to infinity (AUCinf), and the maximum plasma concentration (Cmax) were determined. For both dextromethorphan and desipramine, AUCinf and Cmax were higher in subjects of the CYP2D6*29*29 and CYP2D6*17*17 cohorts, as compared with subjects in the CYP2D6*1/*2 diplotype cohort and with normal metabolizers from the literature. All PK parameters, including AUCinf, Cmax, and the elimination half-life, followed a similar trend: CYP2D6*17*17>CYP2D6*29*29>CYP2D6*1/*2. The plasma and urinary drug/metabolite exposure ratios of both drugs were higher in subjects of the CYP2D6*17*17 and CYP2D6*29*29 cohorts, when compared with subjects in the CYP2D6*1/*2 diplotype cohort. All adverse events were mild, except in 1 subject with CYP2D6*17*17 who had moderately severe headache with desipramine. These results indicate that subjects with CYP2D6*17*17 and CYP2D6*29*29 genotypes were 5-10 times slower metabolizers than those with CYP2D6*1/*2 diplotypes. These findings suggest that dose optimization may be required when administering CYP2D6 substrate drugs in African patients. Larger studies can further validate these findings.

Chiari Syndrome: Advances in Epidemiology and Pathogenesis: A Systematic Review.

Arnold Chiari syndrome is a rare congenital disease of unknown prevalence and whose origin is still under study. It is encompassed within the posterior cranial malformations, showing a wide spectrum of symptomatology that can range from severe headache, dizziness, and paresthesia to complete asymptomatology. It is for this reason that early diagnosis of the disease is difficult, and it is usually diagnosed in adolescence. Treatment is based on remodeling and decompression of the malformed posterior cranial fossa, although the risk of residual symptoms after surgery is high. The aim of this review is to update all the existing information on this pathology by means of an exhaustive analysis covering all the scientific literature produced in the last 5 years. In addition, it has been carried out following the PRISMA model and registered in PROSPERO with code CRD42023394490. One of the main conclusions based on the results obtained in this review is that the origin of the syndrome could have a genetic basis and that the treatment of choice is the decompression of the posterior cerebral fossa.

Stereotactic Intracavitary Irradiation for Cystic Craniopharyngiomas with Rhenium-186

Abstract Objective The intracavitary irradiation of cystic tumors has been used as a therapeutic alternative modality in the management of cystic craniopharyngiomas. In the present study, we review our experience, considering the technical issues, outcomes, and complications associated with the use of stereotactic intracavitary irradiation (SICI) with colloidal rhenium-186 (186Re) for cystic craniopharyngioma. Material and Methods The records of 33 patients with cystic craniopharyngiomas treated by SICI with colloidal 186Re were retrospectively reviewed. The median radiation dose to the cyst wall was of 408 Gy (range: 175 Gy to 500 Gy). All tumors were composed of a large cyst cavity, and 9 (27.3%) also had a solid component. The mean follow-up period was of 3.7 years. Results After SICI, 31 (93.9%) patients showed radiological evidence of cyst regression, and, in 2 (6.1%), no response was observed. An improvement in the visual deficits was observed in 8 cases (24.2%), and an improvement in endocrinogical disturbances, in 2 cases (6.1%). We observed complications in 3 patients (9.1%): diabetes insupidus in 1 case (3%), aggravation of visual acuity in 1 case (3%), and severe headache after infusion of the colloid in 1 case (3%); and 1 patient (3%) died after meningitis. Conclusion Stereotactic intracavitary irradiation with colloidal 186Re is a safe procedure, with satisfactory results in the present series, and should be considered, in the management of cystic craniopharyngiomas, the first-intention therapy or as an adjuvant to other therapeutical modalities, with acceptable morbidity and mortality rates.

Management of Paediatric Migraine - A Brief Review

Purpose: Paediatric migraine is a common and debilitating neurological condition that affects a significant number of children worldwide. Episodes of moderate to severely severe headaches, frequently accompanied by nausea, photophobia, and phonophobia, are the most typical indications and symptoms. Effective management of Paediatric migraines requires a comprehensive approach that includes acute treatment of individual attacks, preventive strategies, and lifestyle modifications. Acute treatment options for paediatric migraines primarily involve the consumption of nonsteroidal anti-inflammatory drugs (NSAIDs) as initial medication treating episodes that are mild to severe. In cases of severe or refractory migraines, triptans may be considered. Preventive strategies occupy a crucial part in reducing the frequency and the degree of intensity of paediatric migraines. These strategies include the use of medications such as antiepileptic drugs, beta-blockers, and tricyclic antidepressants. Lifestyle modifications are integral to the management of Paediatric migraines. Identifying and avoiding triggers, maintaining regular sleep patterns, promoting a healthy diet, and managing stress are key elements in preventing migraine attacks. Design/Methodology/Approach: All pertinent standard papers were briefly reviewed and relevant data was extracted. Findings/Result: The management of paediatric migraines requires a multidimensional approach that encompasses acute treatment, preventive strategies, lifestyle modifications, and healthcare professional involvement. By implementing evidence-based practices tailored to each child, healthcare providers can significantly improve the quality of life for paediatric headache migraine sufferers and minimize the impact of this condition on their overall well-being. Originality/Value: This review article focuses on a thorough summary of the existing research regarding the management of paediatric migraine Paper Type: Review Article

Legislative and regulatory barriers to pharmacies dispensing buprenorphine for OUD.

Buprenorphine (BUP) is increasingly recognized and utilized as a valuable medication for the treatment of opioid use disorder. This article focuses on the problem of regulatory restrictions on access to buprenorphine products without naloxone (mono-product), involving patients in one geographic area, but which may represent a more general access problem in the United States. In response to an audit by the Tennessee Board of Pharmacy, a pharmacy in northeast Tennessee designed a questionnaire to survey patient motivation for traveling long distances to fill their prescriptions for BUP, rather than buprenorphine/naloxone (BNx, combo-product), and to document their satisfaction with treatment with the mono-product. Questionnaires were submitted by 194 patients, living in northeast Tennessee, southwest Virginia, and southeast Kentucky. Significant, intolerable, side effects were reported by all patients in the survey prescribed BNx, but because of legislative and regulatory restrictions in their respective states, they were unable to obtain BUP closer to home. Consequently, they were required to drive significant distances from their homes to fill their prescriptions, a median distance of 52 miles, and in some cases as far as 216 miles round trip. Intolerable reactions included severe headaches, nausea and vomiting, allergies, and severe dysphoria. All patients tolerated BUP and were clinically well maintained on the mono-product. Severe, intolerable reactions/side effects from the naloxone component of BNx are not uncommon, but legislative and regulatory restrictions on the mono-product prohibit providers and pharmacies in some states from prescribing and dispensing BUP. The participants in this qualitative study found it necessary to travel significant distances to obtain their prescribed medication, thereby potentially limiting access to this life-saving therapy.

Glucagon-Like Peptide-1 Analogue In The Management Of Rebound Intracranial Hypertension: A Case Report

Background: Rebound intracranial hypertension (RIH) is a possible complication of epidural blood patching used in the management of spontaneous intracranial hypotension (SIH). RIH has been reported to occur in up to 27% of patients after SIH treatment and is characterized by elevated intracranial pressure, resulting in severe headache, nausea, and vomiting. Although it typically resolves spontaneously, treatment may be warranted for symptom control. While acetazolamide is the most commonly used agent for RIH, there are side-effects, and its efficacy remains inconclusive. Recently, novel glucagon-like peptide-1 (GLP-1) analogues have been found to modulate CSF secretion and consequently reduce intracranial pressure. However, there are no studies that have evaluated the use of these agents in the treatment of RIH. We report of a case of a 46 year old female patient with persistent RIH after CSF leak repair that was refractory to pharmacologic and interventional treatments. The patient was treated with Semaglutide, an oral GLP-1 analogue and experienced immediate and sustained benefit of her symptoms. Conclusion: RIH is a common complication after successful patching of a CSF leak, a proportion of patients suffer from persistent and debilitating headaches. Our case report showed that GLP-1 agonist Semaglutide appeared to reduce symptoms in this one patient with refractory RIH symptoms. The use of GLP-1 agonists in the treatment of RIH should be evaluated in controlled studies to establish its safety and efficacy in this population.

Quality of Life in Patients With Confirmed and Suspected Spinal CSF Leaks.

Spontaneous intracranial hypotension (SIH) is a debilitating condition typically producing orthostatic headache limiting upright time. SIH is often difficult to diagnose and treat, negatively affecting quality of life (QoL) in patients with the disorder. We studied QoL in patients with confirmed and suspected SIH using standardized instruments, including suicidality. We performed a cross-sectional survey of adult patients with confirmed and clinically suspected SIH evaluated in our Headache and Facial Pain Program from 2016 to 2022. Using an online data collection tool (REDCap V 11.2.2), participants completed validated questionnaires assessing general well-being (SF-36), depression (PHQ-9), generalized anxiety disorder-7 (GAD-7), spiritual well-being during chronic illness therapy (FACIT-Sp-12), and headache impact (HIT-6). Subsequently, we interviewed willing participants to administer the Columbia-Suicide Severity Rating Scale (C-SSRS) assessing suicidal behavior and ideation. A total of 234 patients met inclusion criteria and were invited to participate in the study, and 95 patients (59 confirmed and 36 clinically suspected) completed the questionnaires. The average age of the cohort was 51.1 years (SD: 15.5), predominantly female (69.5%), White (91.6%), and married (69.5%). Three-quarters (74.5%) scored within the most severe headache category (HIT-6). SF-36 scores were significantly inferior (p < 0.0001) to the general population and lower than reported values for patients with multiple sclerosis and idiopathic intracranial hypertension. Almost half (49.1%) of respondents scored in the moderate depression range or worse (>10), and 25.4% scored with moderate anxiety or worse (>10). FACIT-Sp-12 scores were significantly worse (p < 0.0001) in symptomatic participants than in the validation cohorts of patients with AIDS and cancer. Of the 67 respondents who completed the C-SSRS, more than half (64.2%) endorsed a wish to be dead, and 22.4% had demonstrated suicidal behavior. Patients with symptom-free SIH (n = 22) scored significantly better than symptomatic patients, comparable with the general population. Based on our single-center cohort, SIH is associated with severe headache pain and high rates of depression, anxiety, and disability, affecting basic activities of daily living. Individuals with confirmed and suspected spinal CSF leaks scored similarly on these measures including suicidality. Outcomes were comparable with the general population after successful treatment or spontaneous remission. Improved identification and treatment of SIH are imperative to improve patients' QoL.

Low pressure headache and cerebral fat embolism from a sacral fracture through a Tarlov cyst: a case report

BackgroundHere we report the only formally documented case in the United Kingdom, to our knowledge, of a cerebral fat embolism secondary to non-iatrogenic trauma through a Tarlov cyst. This case demonstrates the pathology clearly giving an excellent opportunity to demonstrate a rarely seen pathology as well as illustrating the importance of the patient history to guiding further management.Case presentationA middle-aged patient was admitted on the acute medical take complaining of severe headache with photophobia, having just returned after a skiing holiday. Computerised tomography scan of the head showed fat within the anterior horn of both lateral ventricles, and within the subarachnoid space. Re-discussion with the patient and subsequent MRI (Magnetic Resonance Imaging) of the spine identified the pathogenesis of her symptoms: a sacral insufficiency fracture through a Tarlov cyst, causing subarachnoid fat embolism and symptoms of a low-pressure headaches due to a dural leak. Patient was medically managed and discharged with planned follow-up. Due to the Coronavirus pandemic and resolution of the patient's symptoms, they declined further follow up imaging.ConclusionsThe case demonstrates a rarely seen pathology as cause of a common presenting problem, headache. Emphasizing the importance of history taking and appropriate investigations in medical cases that do not conform to the usual diagnosis.

FRI351 Pituitary Stalk Epidermoid Cyst: A Rare Cause Of Diabetes Insipidus

Abstract Disclosure: J.T. Batch: None. F. Warda: None. P. Natter: None. R. Makary: None. G.Y. Gandhi: None. Introduction: Epidermoid cysts are benign squamous epithelial cysts filled with keratin. They are rarely found in the central nervous system, constituting 0.2-1.8% of intracranial masses, with the majority located in the cerebellopontine angle. Case Report: A 34-year-old female presented with blurred vision, debilitating headaches, milky breast discharge, and no menstrual periods for three years. Prolactin was mildly high on two occasions at 40.3 and 52.8 ng/mL (3.0-30.0 ng/mL). Estradiol was low at 15 pg/ml in the setting of inappropriately normal FSH (10.1 mIU/mL) and LH (5.8 mIU/mL). ACTH, cortisol, TSH, free thyroxine, and IGF-1 were in the normal range. A pregnancy test was negative. A 1:100 diluted prolactin did not detect the hook effect. MRI revealed a 1.8 x 1.4 x 1.4 cm suprasellar mass centered along the pituitary stalk with peripheral enhancement and central cystic vs. necrotic components. It caused a mass effect on the optic chiasm. Formal visual field testing revealed a left inferior temporal defect. Subsequently, the patient reported increased thirst (consuming 4-5 L of water daily) and urination (confirmed with a 24-hour urine output of 4 L). Following 12 hours of overnight water deprivation, morning labs confirmed diabetes insipidus with high serum sodium of 146 mmol/L, high serum osmolality of 306 mOsm/kg, and low urine osmolality of 204 mOsm/Kg. Initiation of oral DDAVP 0.1 mg nightly resulted in a resolution of nocturia and dramatic improvement in thirst. The patient underwent transsphenoidal resection, during which the neurosurgeon identified a suprasellar cystic lesion with classic epidermoid pearl white material. The lesion and capsule were resected, and patient started on dexamethasone 6 mg IV every 6 hours to prevent chemical meningitis. Pathology was consistent with an epidermoid cyst. Prolactin stained several cells, possibly from compression of the pituitary stalk by the epidermoid cyst. Postoperatively, the patient had severe headaches, and photophobia thought to be associated with chemical meningitis. Lumbar puncture revealed elevated CSF neutrophils and aseptic culture. The patient was continued on dexamethasone with slow taper upon discharge. DDAVP was increased to 0.1 mg twice daily as polyuria and polydipsia worsened. Discussion: There have been six reported cases of epidermoid cysts involving the pituitary stalk. Visual disturbance and headaches are common, while galactorrhea and amenorrhea are uncommon. Preoperative polyuria can be a frequent presenting symptom that can evolve into permanent postoperative diabetes insipidus. Surgical resection is the treatment of choice. It may be difficult to achieve complete excision due to the adherence of the cyst wall to the adjacent structures. The most common complications after resection include chemical meningitis (noted in our patient), hydrocephalus, infectious meningitis, and cranial nerve palsies. Presentation: Friday, June 16, 2023

FRI086 Statin Use After Lobar Intracerebral Hemorrhage- Is It Safe?

Abstract Disclosure: D. Vedamurthy: None. M. Ambrosino: None. K. Suri: None. D. Soffer: None. D. Jacoby: None. Background: Observational studies and randomized clinical trials have yielded conflicting data on the role of statins in causing intracerebral hemorrhage. It is unclear whether it is safe to continue statins in patients who have atherosclerotic cardiovascular disease (ASCVD) when they sustain an intracerebral hemorrhage (ICH). It is also not clear whether this risk of ICH (if present) relates to statin use or “too low” levels of LDL-C. Case: 75-year-old female with history of hypertension, hyperlipidemia, and a coronary artery calcium score of 450, presented to the hospital with severe headache and was diagnosed to acute right ICH. She did not smoke, use street drugs or drink excess alcohol. She did not have a family history of premature ASCVD. Her other medication included amlodipine, rosuvastatin and aspirin. Her lipid panel at hospitalization was TC 130 mg/dl, HDL 38 mg/dl, TG 137 mg/dl and LDL-C 70 mg/dl. Imaging studies were negative for cortical venous thrombosis, reversible cerebrovascular constriction, vasculitis, aneurysm, or AV fistula as etiologies. TEE was negative for cardioembolic source. Her hypertension medications were optimized, and she was discharged without any statin therapy. She fortunately recovered with mild deficits. She enrolled in an open-label randomized study- SATURN- which aims to assess safety of statin use in recurrent ICH. Her lipid panel while off statins showed a TC 274 mg/dl, TG 159 mg/dl, markedly elevated LDL-C of 197 mg/dl, and HDL-C 47 mg/dl. She presents to the lipid clinic for risk reduction. Early studies suggested increased ICH related to very low LDL-C levels, especially in the East Asian population. Concerns about statin use and ICH sparked after post-hoc analyses of SPARCL. Data from mendelian randomization studies and meta-analysis have failed to show convincing associations between statin use and ICH. Certain genetic variants, especially related to Apo E, have been shown to enhance this risk for ICH. There is a diversity in management of patients who have sustained an ICH (especially the lobar type as opposed to deep). An open label randomized trial (SATURN) is currently recruiting patients to answer this unresolved question. The dilemma is compounded in patients who have comorbid ASCVD. There are signals that PCSK9 inhibitors may be safer in terms of less propensity to cause intracerebral hemorrhage, but more data is needed. Moreover, the very low LDL-C levels achieved with use of PCSK-9 inhibitors may be concerning. Conclusion: There are no clear major society guidelines to advice clinicians on the best way to manage patients who have sustained a lobar intracerebral hemorrhage and have a compelling reason to be on statins for lipid lowering therapy to treat ASCVD. There are no clear guidelines on how low is “too low” as far as LDL-C targets are concerned. We present this case study as a call for more robust and expedient research in this field. Presentation: Friday, June 16, 2023

SAT437 Immunotherapy Induced Pituitary Apoplexy

Abstract Disclosure: A. Uddin: None. M.J. Gardner: None. A. Akofu: None. N. Sheung: None. S. Dejhansathit: None. N. Akabogu: None. F. Marium: None. We present a case of a 42 year old male with past history of metastatic melanoma s/p 3 cycles of ipilimumab and nivolumab immunotherapy that originally was referred to endocrinology due to elevated prolactin of 40 and MRI finding of questionable 6 mm pituitary adenoma. There was not a clear other cause of prolactin elevation. Other notable labs in pituitary hormonal evaluation were elevated FSH of 24 and LH 16 with normal testosterone 663. TSH of 4.3 and FT4 of 1.22. After the 4th cycle of ipilimumab and nivolumab he had a severe headache that was unremitting, he called his oncology nurse line which instructed him to hydrate. He presented for admission to the hospital after coming confused, unsteady on his feet and his headache had already improved. Labs demonstrated severe hyponatremia at 111 with serum osm of 238 and urine osmolality of 504 and specific gravity of 1.012. Brain MRI demonstrated a 1.2 cm pituitary lesion compared to less than 1 month prior, consistent with hypophysitis. Repeat pituitary labs showed hypogonadotropic hypogonadism with total testosterone of 39 and inappropriately normal FSH of 7 and LH of 2, central hypothyroidism with TSH 0.232, FT4 0.70, FT3 1.8, secondary adrenal insufficiency with cosyntropin stimulation test up to cortisol of 16 for partial response and ACTH of 17. Hyponatremia was managed inpatient and he was discharged on appropriate hormone replacement. Subsequent brain MRI with normal pituitary finding without enhancing lesions. Repeat hormonal testing showed persistent hormonal deficiencies with undetectable cortisol and ACTH despite holding hydrocortisone therapies. Prolactin was also tested in the 4-10 range on repeat testing. He continued on nivolumab monotherapy with oncology. This case report details a clear case of immunotherapy treatment leading to destruction of prolactinoma and presentation of pituitary apoplexy given the short time line of 1 month and cycle of therapy between radiographic and hormonal assessment. We hope this case helps bring awareness to the evolving list of conditions to survey for with immunotherapy and suggests a role of identifying prolactinomas biochemically and or radiographically to affect management. It may even suggest a role in treatment for medically refractory pituitary adenomas. Presentation: Saturday, June 17, 2023

FRI320 The Various Clinical Outcomes Of Giant Prolactinomas In Men

Abstract Disclosure: A. Gilis-Januszewska: None. A. Bogusławska: None. M. Godlewska: None. &amp;. Kluczyński: None. E. Rzepka: None. A. Hubalewska-D: None. Giant prolactinomas constitute 2-3% of all lactotroph PitNETs with male preponderance. We present a case series of giant prolactinomas with various clinical presentation. Case 1: A 66-year-old male hospitalized due to left peripheral facial palsy. In computer tomography (CT) pituitary mass (41x43x64mm) invading cavernous/sphenoid sinuses/optic chiasm was visualized. Bitemporal hemianopia/headaches were observed. Prolactin level (PRL) was 22083 uIU/ml(N: 86-324uIU/ml). Cabergoline (1mg/week) was implemented. After 3 months, regression of PitNET by 14mm and normoprolactinemia were observed. Milder headaches and improvement of visual field were reported. Case 2: A 62-year-old male presented with panhypopituitarism/diabetes insipidus at the age of 52. In MRI PitNET 36x62x35mm with extrasellar extension/ invading third ventricle was found. PRL was 223549uIU/ml. Despite dopamine agonist treatment progression of pituitary tumor/high prolactin level were observed-patient did not consent to neurosurgery. In 2019, significant visual filed deterioration was observed- patient consent to craniotomy. Histopathology revealed lactotroph-PitNET with Ki67&amp;gt;3%. After 6 months, tumor progression was noted. Patient was disqualified from radiotherapy. Temozolomide was introduced. After 9 cycles, regression of PitNET was observed and decrease of PRL by 2600%. Case 3: A 56-year-old male was hospitalized due to syncope. In CT pituitary tumor 40x30mm was diagnosed with bitemporal hemianopsia. PRL was 10446uIU/ml. Cabergoline(1mg/week) was implemented. After 3 months, regression of pituitary tumor/normal prolactin level/improvement of vision were noted. Case 4: A 23-year-old male presented with severe headaches and visual impairment at the age of 21. In MRI pituitary mass 52x52x41mm with extrasellar extension was found. PRL was 21522uIU/ml. Insufficiency of thyroid and gonadal axis was diagnosed. Cabergoline was implemented (4mg/week) with regression of the tumor, decrease of PRL (8400uIU/ml) and complete remission of headaches. Case 5: A 67-year-old male diagnosed with a PitNET (65x35x40mm) at the age of 50 years. Due to hyperprolactinemia, cabergoline was implemented (7mg/week). After few weeks, pituitary apoplexy occurred. Patient underwent emergency neurosurgery. MRI over next 20 years demonstrated stable residual tumor (22x28x11mm). Case 6: A 47-year-old male admitted to Emergency Unit due to severe headache. MRI showed PitNET (56x70x67mm). PRL was 22083 uIU/ml and plurihormonal pituitary insufficiency was observed. Cabergoline 2mg/week was implemented. After 3 months of treatment tumors regression was observed (56x68x50 mm) with normoprolactinemia. The management of giant prolactionomas in men is challenging. Further multicenter studies to understand the pathophysiology and prognostic factors of giant prolactionomas in men are needed. Presentation: Friday, June 16, 2023

THU106 Malignant Epithelioid Neoplasm As The Cause Of A Rapidly Growing Pituitary Mass

Abstract Disclosure: S. Riahi: None. S.S. Chaidarun: None. Background: Malignant epithelioid neoplasm is a rare cause of a pituitary mass, and should be kept on the differential whenever evaluating patients with evidence of rapid pituitary enlargement. Clinical Case: A 43 year-old man presents with 3-month history of worsening severe headaches, decreased libido, erectile dysfunction, polydipsia, polyuria (6L/day) with nocturia every hour, and fatigue. He also noted some blurry vision without loss of his peripheral vision. MRI of the brain showed a 1.5cm mass in the sellar and suprasellar region abutting the optic chiasm. Of note, he had a brain MRI performed 18 months prior for vertigo which did not demonstrate any mass in the pituitary. He presented to the endocrinology office for evaluation. His laboratory tests demonstrated mildly elevated prolactin 31.2 ng/mL (n 4.0-15.2), and findings consistent with panhypopituitarism and central diabetes insipidus, including low FT4 of 0.46 ng/dL (n 0.93-1.70), FSH 0.7 mIU/mL (n 1.5-12.4), an undetectable LH (n 1.7-8.6mIU/mL), random cortisol of 1mcg/dL (n 4.8-19.5) ACTH 6.2 pg/mL (n 7.2-63), IGF-1 of 139 ng/mL (n 44-275), total testosterone undetectable (n 240-950 ng/dL), sodium 144mmol/L (n 135-145) with low urine specific gravity 1.008 and urine osmolality of 143mOsm/Kg (n 60-1200). HCG was undetectable (n 0-2mIU/mL) but LDH was elevated at 313 unit/L (n 110-220). He was started on corticosteroids, levothyroxine, DDAVP, and referred to neurosurgery. Specimens from his transphenoidal biopsy demonstrated findings consistent with a malignant epithelioid neoplasm. Further analysis and genetic testing is still pending. He has been referred to oncology service for further management and care. Conclusion: A rapidly growing symptomatic pituitary mass with central diabetes insipidus should raise suspicion for malignant etiology beyond the more prevalent pituitary adenoma. Malignant epithelioid neoplasm are uncommon tumors of the brain, however they are known to be aggressive tumors with high mortality. Treatment commonly includes some combination of surgery, radiotherapy, and chemotherapy1. Reference: (1) Jog AP, Ronderos DM, Ali A, Niazi M, Diaz-Fuentes G. Malignant Epithelioid Neoplasm of the Brain. Cureus. 2020 Aug 27;12(8):e10079. doi: 10.7759/cureus.10079. PMID: 33005505; PMCID: PMC7523542. Presentation: Thursday, June 15, 2023

FRI349 Atypical Timing: Transient Diabetes Insipidus After Pituitary Apoplexy

Abstract Disclosure: I.A. Rivera Nazario: None. A.M. Santiago Carrion: None. M.T. Torres: None. A. Figueroa Cruz: None. Y. Ortiz: None. A. Aponte Velez: None. A. Vazquez: None. E.J. Sola Sanchez: None. M.M. Mangual Garcia: None. Pituitary apoplexy results from acute hemorrhage or infarction of the pituitary gland. Also, an adenoma that can develop transient or permanent pituitary hormone deficiency. Posterior pituitary function is nearly always preserved due to alternate blood supply. Most cases presenting among the fifth or sixth decade. Few cases of transient Diabetes Insipidus (DI) have been described in literature, approximately 4% of patients, making this an uncommon complication of this condition. Hereby, we describe a young male with sudden onset headache due to pituitary apoplexy with transient DI. A 33-year-old man without past medical history, presented to the emergency room with the chief complain of sudden severe headache. Physical examination without altered consciousness, visual impairment or meningeal irritation. Initial head CT was negative, except for findings of muscle spasms. Patient was discharged home with muscle relaxant and NSAIDs. Ten days later, patient continued with worsening headache, not responding to medical therapy. On reevaluation, brain MRI identified a pituitary macroadenoma, measuring 2.2 cm, with mass effect, suprasellar extension impinging the pituitary stalk, prechiasmatic optic nerves and cavernous sinus extension. Blood tests showed Na 139 mEq/L, K 4.1 mmol/L and adequate renal function. Pituitary function test consistent for hypopituitarism with free T4 0.39 ng/dL, TSH 0.084 mIU/mL, cortisol 0.19 mcg/dL, ACTH 9.39 ng/mL, GH &amp;lt;0.1 ng/mL, IGF-1 22.8 ng/mL and prolactin 1.52 ng/mL; except normal FSH, LH and testosterone levels. After four months of apoplectic episode, he developed polyuria and polydipsia. Brain MRI with residual sellar mass of 1.2 cm with resolution of mass effect. Visual field charting was normal. Blood tests showed Na 139 mEq/L, K 4.1 mmol/L and adequate renal function. Started on hydrocortisone and levothyroxine replacement. Diagnosis of DI was suspected, but he refused water deprivation test or DDAVP therapy. During follow-up, patient was found with serum osmolality 288 mOsm/kg, urine osmolality 202 mOsm/kg and 4.6 L 24-hour urine collection. Three months later serum osmolality was 294 mOsm/kg, urine osmolality of 383 mOsm/kg with resolution of polyuria and polydipsia. This case presents a delayed onset of transient DI treated in a conservative way. In documented cases average onset was a period of 2 weeks with resolution in 2 months. Etiology most likely due to compression or destruction of the pituitary gland or stalk impeding transit of ADH from hypothalamus to the neurohypophysis. Transient impairment of the ADH secretion is rare in pituitary apoplexy deserving evaluation in this clinical scenario. When seen, it has been related to glucocorticoid use. This is an example of an atypical pituitary disorder requiring prompt recognition to prevent further complications. Presentation: Friday, June 16, 2023

FRI321 Aggressive Prolactinoma Empirically Treated With Cabergoline Leading To Rapid CSF Leak

Abstract Disclosure: A. Yau: None. T. Reisman: None. Over two-thirds of pituitary tumors are prolactinomas. These are discovered as a result of symptoms of hypogonadism, menstrual irregularities, infertility or galactorrhea, as well as symptoms of mass effects including visual field deficit or acute severe headaches.1 A more rare subgroup, aggressive prolactinomas, in which the tumors are radiographically invasive and show resistance to dopamine agonist treatment as well as surgery, account for 10% of all hypophyseal neoplasia. 2 During medical treatment of prolactinomas, there is a known rare complication of CSF rhinorrhea; recorded time of onset of leak following treatment ranges from 14 days to 5 years.3 We present a case in which a patient with an aggressive prolactinoma rapidly developed a CSF leak within 48 hours of starting cabergoline treatment. Case: A 36-year-old male with type 2 diabetes, erectile dysfunction and infertility presents to Endocrine clinic after initial evaluation by Urology revealed elevated prolactin. He reported headaches, gynecomastia associated with liquid discharge from nipples, and minor issues with peripheral vision. He was started empirically on cabergoline for probable prolactinoma and within 48 hours developed worsening frontal headache, cough and clear fluid leakage from his right nostril. Labs revealed prolactin over 2600 ng/ml. MRI pituitary showed evidence of pituitary adenoma as well as likely CSF leak. Cabergoline was stopped and the patient was sent to the emergency room. The patient was underwent urgent neurosurgical transsphenoidal endoscopic surgery and CSF leak repair with resolution of rhinorrhea and cough. Post-operative MRI was consistent with minimal residual tumor. He was monitored in the NICU with no obvious post-operative pituitary hormone imbalance, diabetes insipidus, adrenal insufficiency or syndrome of inappropriate ADH. Months after discharge, the patient’s prolactin remained elevated to the 600’s ng/ml and repeat MRI showed a mass occupying the entire sella and suprasellar cistern, compressing the optic chiasm. Conclusion: In this case of a patient with the subtype of aggressive prolactinoma, there seems to be a more rapid presentation of complications. When treating these rare subtypes, physicians need to stay vigilant of developing complications or even consider nonstandard therapies as first-line management. 1. Molitch ME, Drummond J, Korbonits M. Prolactinoma Management. In: Feingold KR, Anawalt B, Boyce A, et al., eds. Endotext. South Dartmouth (MA): MDText.com, Inc.; January 6, 2022.2. Lasolle H, Ilie MD, Raverot G. Aggressive prolactinomas: how to manage?. Pituitary. 2020;23(1):70-77. doi:10.1007/s11102-019-01000-73. Arimappamagan A, Sadashiva N, Kandregula S, Shukla D, Somanna S. CSF Rhinorrhea Following Medical Treatment for Prolactinoma: Management and Challenges. J Neurol Surg B Skull Base. 2019;80(6):620-625. doi:10.1055/s-0039-1677686 Presentation: Friday, June 16, 2023

SAT621 Hemorrhage After Treatment: A Case Of Pituitary Apoplexy In Metastatic Prostate Cancer After Leuprolide

Abstract Disclosure: M. Dillon: None. S. Shteyman: None. W. Rashid: None. B.P. Ramchandani: None. A.G. Samat: None. Introduction: Hormone-sensitive cancers, including prostate and breast cancer, and some gynecological conditions can be treated with GnRH agonists, including leuprolide. However, pituitary apoplexy is a rare potential complication after administration. Case Description: 71-year-old gentleman with metastatic hormone-sensitive prostate cancer presented with an acute headache after receiving a dose of leuprolide. This was his first GnRH agonist dose after prior Degarelix treatment. After two hours, the patient developed a constant throbbing headache, palpitations, left eye tearing and non-bilious non-bloody vomiting. Vitals were within normal ranges, and initial lab work showed low serum cortisol of 4.6. CT scan of the head showed exophthalmos and a mildly enlarged sella. Subsequent MRI demonstrated pituitary hemorrhage and optic chiasm impingement from resulting swelling. Hormone levels remained normal. Since the patient did not develop visual field defects or other ocular complaints, the decision was to manage the patient medically rather than with surgery. The day after admission, he developed hypotension requiring high dose steroids. After discharge, outpatient repeat MRI showed interval resolution of hemorrhage into a predominantly cystic pituitary macroadenoma. Discussion: With continued stimulation, GnRH agonists desensitize the pituitary and cause a downregulation of hormonal induced cancer growth. However, there is an initial transient burst of stimulation that can induce pituitary apoplexy in patients who already have a large adenoma. The hemorrhage and subsequent swelling cause a sudden severe headache and possible visual loss or hypopituitarism. This is life-threatening due to hypotension from catecholamine insensitive blood vessels in the absence of cortisol. Treatment involves high dose steroids, frequent sodium checks and possible emergency neurosurgery for decompression. It is important with GnRH agonists to watch out for this rare complication, whether the hormonal axis is being suppressed for metastatic cancer treatment or for hormonally driven gynecological conditions. Sources: Briet C, Salenave S, Bonneville JF, Laws ER, Chanson P. Pituitary Apoplexy. Endocr Rev. 2015 Dec;36(6):622-45. doi: 10.1210/er.2015-1042. Epub 2015 Sep 28. PMID: 26414232. Tanios G, Mungo NA, Kapila A, Bajaj K. Pituitary apoplexy: a rare complication of leuprolide therapy in prostate cancer treatment. BMJ Case Rep. 2017 Jul 14; 2017:bcr2016218514. doi: 10.1136/bcr-2016-218514. PMID: 28710301; PMCID: PMC5534692. Presentation: Saturday, June 17, 2023

SAT620 Dopamine Challenge: Treatment Of Pituitary Macroadenoma/Prolactinoma In Patient On Antipsychotic Medication

Abstract Disclosure: A. Bazhenov: None. I. Alpertunga: None. Background When hyperprolactinemia is a well-known side effect of antipsychotic medications1, the link between prolonged treatment with dopamine antagonist and the development of lactotroph adenomas is still unclear4. Treating a patient with prolactinoma and psychotic disorder may pose a challenge. Antipsychotic agents can cause increases in prolactin levels. Additionally, treatment of prolactinoma with dopamine agonists may worsen psychotic symptoms. Case presentation We present a case of 56 yo male with a PMH of bipolar disorder with psychotic features who was found to have a macroprolactinoma after 18 years of treatment with antipsychotic medications. At age 30, the patient was diagnosed with Bipolar Disorder with psychotic features. The course of the disease was complicated by numerous hospitalizations and frequent changes to the treatment plan, with different combination of second-generation antipsychotic, haloperidol and antiseizure medications. The patient’s prolactin level was 50 ng/ml at the time. 18 years after the diagnosis, the patient developed severe headaches. An MRI revealed a pituitary macroadenoma measuring 1.3 cm, and a repeat prolactin level resulted in 729 ng/ml. He was started on cabergoline 0.5 mg twice a week. As a result, the prolactin level dropped to 32 ng/ml and the macroprolactinoma size decreased to 1.1 cm. Unfortunately, the patient was lost to follow up. Once he re-established his care, he was started on Olanzapine 15 mg daily, Lamotrigine 200 mg daily and cabergoline 0.5 mg twice a week. After reinitiating cabergoline, the patient developed three maniac episodes, two of which required hospitalization. The dose of the cabergoline was decreased to avoid future maniac episodes. Neurosurgery will be consulted for possible surgery treatment. Discussion The treatment of a patient with prolactinoma and a psychotic disorder poses a particular challenge. We believe that care of these patients requires a multidisciplinary approach, and surgical treatment should be considered for those who cannot tolerate dopamine-agonist therapy. Literature 1. Bostwick JR, et al. Antipsychotic-induced hyperprolactinemia. Pharmacotherapy. 2009;29(1):64-73. 2. Lertxundi U et al. Antipsychotics and pituitary tumors. Int Clin Psychopharmacol. 2019;34(2)89. 3. Andrade EH, et al. Insights in the management of antipsychotics. Case Rep Med. 2010;2010. 4. McCarren M,et al. Follow-up study of a pharmacovigilance signal. J Clin Psychopharmacol. 2012;32(6)743. 5. Bakker IC, et al. Successful treatment of a prolactinoma on antipsychotic drug. Endocrinol Diabetes Metab Case Rep. 2016. 6. The challenges in managing prolactinomas SFEBES2009 Accessed April 11 2023. https://endocrine-abstracts.org 7. Howes OD et al. The role of dopamine in schizophrenia. Biol Psychiatry. 2017;81(1)9. 8.Bamarinejad A, et al. Schizophrenia and Macroprolactinoma: Adv Biomed Res. 2020;9(1)38. Presentation: Saturday, June 17, 2023

SAT392 A Case Of Idiopathic Intracranial Hypertension in Gender Dysphoria: Is Testosterone The Culprit?

Abstract Disclosure: S. Saad-Omer: None. D. John: None. M. Matos: None. C. Botero Suarez: None. S.K. Suryanarayanan: None. Background: The role of gender affirming Testosterone therapy in female-to-male patients with idiopathic intracranial hypertension (IIH) remains controversial. Case: A 32-year-old transgender male (Female-to-Male) was admitted after ophthalmologic evaluation for difficulties with night vision revealed papilledema. On admission, the patient reported a 2-month history of worsening bifrontal headaches accompanied by nausea, photophobia, and hyperacusis. Physical examination revealed papilledema and photophobia with BMI (34) and BP (132/86mmHg). CT Head was normal. Lumbar puncture showed an opening pressure of 22 cm water consistent with elevated intracranial pressure. Cerebrospinal fluid (CSF) lab results were grossly unremarkable. He reported being on 4 pumps daily of testosterone gel 1% (50mg) which was started approximately 3 months prior to admission with the last dose administered the day prior to admission. Initial labs showed bioavailable testosterone of 48.7 ng/dl, sex hormone binding globulin 29 nmol/l, free testosterone 24.2ng/dl, albumin 4.4g/dl, total testosterone 178ng/dl, estradiol 67pg/ml, FSH 6.7 mIU/ml, and LH 3.66 IU/ml. MRI Brain and Orbits revealed signs consistent with intracranial hypertension with no masses or bleeding to suggest an alternative cause. He was treated with Solumedrol, Acetazolamide, a brief course of Topiramate. Due to the suspected role of testosterone therapy in development of IIH, it was held during hospitalization. The risks and benefits of testosterone were discussed with the patient and he elected to restart it for gender affirmation at the time of discharge. However, his severe headache recurred after one week and he was asked to hold therapy for 30 days. He had no significant symptomatic improvement while off testosterone and therapy was resumed. Discussion: Several theories have been proposed to explain the occurrence of IIH in transgender patients with the leading hypothesis suggesting a “physiological window” of abnormal hormone levels, shared by women with elevated androgen levels and men with low androgen levels, where the risk of developing IIH is increased. Testosterone levels in women who have hyperandrogenism is similar to those in men with hypoandrogenism and these levels are likely contributing to the increase in volume of visceral fat and decrease in the size of muscles. It is therefore feasible that IIH is a distinct neuro-metabolic complication of circulating testosterone levels within this range. Conclusion: Our patient reported no symptomatic improvement despite withholding testosterone therapy for 30 days. Given the scarcity of evidence, it is important to discuss the potential risk of IIH with transgender patients, but not to withhold gender affirming hormone therapy for these patients. More research is required to better explore the mechanism of this association and possible methods to prevent its occurrence. Presentation: Saturday, June 17, 2023