United States

Parkinsonism-hyperpyrexia syndrome is defined as a hypo-dopaminergic state due to sudden withdrawal of dopaminergic therapies in those with Parkinson’s disease. Presentation mimics medical emergencies like neuroleptic malignant syndrome, anticholinergic toxicity, and serotonin syndrome. Differentiating the conditions can be limited by the patient’s cognitive and verbal status. A detailed history is vital to identifying the most likely etiology. Treatment is directed at restoring the patient’s central dopamine levels and minimizing physical and psychiatric distress. In this report, we discuss a case of Parkinson’s hyperpyrexia that highlighted the importance of detailed history investigation, pharmacological reconciliation, and prompt inclusion of subspecialists.
A 79-year-old female with a history of Parkinson’s disease presented to the emergency department after multiple falls, and new onset of decreased articulation, ability to perform activities of daily living (ADL), fixed rigidity of the lower extremities, and an inability to move arms from the flexed position. Physical exam revealed incomprehensible speech without slurring, masked facies, restricted range of motion in the upper extremities, and lower extremities fixed in the flexed position at the hips and knees. Stroke evaluation was limited due to the patient’s condition. CT head demonstrated no acute process, volume loss, and multifocal white matter disease. Through neurology consult and survey of medical records, it was discovered that medications had been changed in the outpatient setting two weeks prior. Specifically, the patient was advised to discontinue Carbidopa-Levodopa (Sinemet) due to patient reporting no significant benefit. Sinimet had been replaced with the dopamine potentiator Amantadine.
In the hospital, Amantadine was discontinued and Sinemet as started at prior dosing of 25/100 mg. Physical and occupational therapy were also consulted. Within 24 hours the patient could dorsiflex her feet and abduct her shoulders up to 30 degrees. By day 3, the patient stood independently, pronate and supinate her forearms, and speak at an audible volume. Over five days, the patient was once again able to demonstrate facial expressions, ambulate, and feed herself independently without aspiration.
This patient encounter emphasized the importance of obtaining a detailed medical history including reconciliation of home medications and recent changes to medication. In this patient, the abrupt withdrawal of a basal dopamine supply precipitated an exaggerated parkinsonism which mirrored other debilitating and life-threatening conditions. Although the patient believed that the benefits of therapy diminished it became clear that maintenance therapy was necessary for the conservation of ADLs. Similarly to stroke patients, early rehabilitative therapy accelerated the patient’s recovery to baseline and beyond. Future providers should consider the pros and cons of medication changes for chronically ill patients, investigate the patient&#39;s history in detail, and form a multidisciplinary care team early in the care process.


2022 AMA Research Challenge

Parkinsonism-hyperpyrexia Syndrome: a Rare Case

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Dear IPSO Virtual Conference Attendees:

Welcome to IPSO’s first-ever conference! We are delighted to bring together experts from multiple disciplines and six continents to provide a diverse perspective on pediatric cerebrovascular disease. 

A few quick words about what to expect. Two important goals of this conference are to (1) foster a sense of community and (2) identify global priorities for our field. Hence, the program includes short presentations on an array of clinical controversies and dedicates more time to panel discussions with a live chat function so that everyone can participate. We are excited to announce IPSO is able to offer 8.75 CME units for the virtual conference. If you would like to claim CME credits, please email <membership@internationalpediatricstroke.org>. 

We want to hear from you! Planning for the [IPSO Founding Members’ Congress](https://internationalpediatricstroke.org/2022-congress/) in Vienna, April 24-26, 2022, will commence as soon as the Virtual Conference ends. Let us know your priorities through the live chat function, via email, or by submitting a symposium idea. Based on your input, the Scientific Program Committee will select the topics that we will address in greater depth when we meet again in the spring.

We hope you find the Virtual Conference both interesting and educational, and hope to see you in person in Vienna, or at another IPSO meeting in the future. 

Warm regards,

Heather Fullerton, IPSO President

Adam Kirton, IPSO Vice-President

Lori Jordan, IPSO Secretary & Treasurer


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*Header Photo Credit: Helen Carlson, Ph.D, Alberta Children's Hospital*

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IPSO 2021

Welcome to the first IPSO Virtual Conference. The mission of the virtual conference is to unify the global community of professionals engaged in research and clinical care for children with cerebrovascular diseases, and chart a path forward for multinational, multidisciplinary efforts. Please go here to access the virtual conference platform- <a href="https://underline.io/events/157/reception">Reception page</a>.

Parkinsonism-hyperpyrexia syndrome is defined as a hypo-dopaminergic state due to sudden withdrawal of dopaminergic therapies in those with Parkinson’s disease. Presentation mimics medical emergencies like neuroleptic malignant syndrome, anticholinergic toxicity, and serotonin syndrome. Differentiating the conditions can be limited by the patient’s cognitive and verbal status. A detailed history is vital to identifying the most likely etiology. Treatment is directed at restoring the patient’s central dopamine levels and minimizing physical and psychiatric distress. In this report, we discuss a case of Parkinson’s hyperpyrexia that highlighted the importance of detailed history investigation, pharmacological reconciliation, and prompt inclusion of subspecialists.
A 79-year-old female with a history of Parkinson’s disease presented to the emergency department after multiple falls, and new onset of decreased articulation, ability to perform activities of daily living (ADL), fixed rigidity of the lower extremities, and an inability to move arms from the flexed position. Physical exam revealed incomprehensible speech without slurring, masked facies, restricted range of motion in the upper extremities, and lower extremities fixed in the flexed position at the hips and knees. Stroke evaluation was limited due to the patient’s condition. CT head demonstrated no acute process, volume loss, and multifocal white matter disease. Through neurology consult and survey of medical records, it was discovered that medications had been changed in the outpatient setting two weeks prior. Specifically, the patient was advised to discontinue Carbidopa-Levodopa (Sinemet) due to patient reporting no significant benefit. Sinimet had been replaced with the dopamine potentiator Amantadine.
In the hospital, Amantadine was discontinued and Sinemet as started at prior dosing of 25/100 mg. Physical and occupational therapy were also consulted. Within 24 hours the patient could dorsiflex her feet and abduct her shoulders up to 30 degrees. By day 3, the patient stood independently, pronate and supinate her forearms, and speak at an audible volume. Over five days, the patient was once again able to demonstrate facial expressions, ambulate, and feed herself independently without aspiration.
This patient encounter emphasized the importance of obtaining a detailed medical history including reconciliation of home medications and recent changes to medication. In this patient, the abrupt withdrawal of a basal dopamine supply precipitated an exaggerated parkinsonism which mirrored other debilitating and life-threatening conditions. Although the patient believed that the benefits of therapy diminished it became clear that maintenance therapy was necessary for the conservation of ADLs. Similarly to stroke patients, early rehabilitative therapy accelerated the patient’s recovery to baseline and beyond. Future providers should consider the pros and cons of medication changes for chronically ill patients, investigate the patient's history in detail, and form a multidisciplinary care team early in the care process.


poster

Welcome to the largest national, multi-specialty research event **featuring research from 750 medical students, residents and fellows, and international medical graduates.**


#### Event Details
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At the conclusion of this event, the AMA will announce the top five finalists who will advance to the AMA Research Challenge finals, present their research to an elite panel of judges and compete to win a $10,000 grand prize sponsored by Laurel Road.
 

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 **Speakers**
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Ricardo Correa, MD, EdD
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Stuart M. Greenstein, MD
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Stephanie Strohbeen, MD
 
 **Oct. 22, 11 a.m. — Noon Central**
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Jessica Li, MD/PhD Candidate
![](https://assets.underline.io/uploads/markdown_image/1/image/9950ef2e0237f54cb3d8280334d42491.jpg)
Charles Lopresto, DO

### Sponsor
AMA would like to thank Laurel Road for sponsoring a $10,000 grand prize for the winner of the AMA Research Challenge. Laurel Road is not involved in the judging or scoring process to select the winner. The winner will be announced on Dec. 7 during the AMA Research Challenge finals. Payment of the grand prize will be directly from Laurel Road. See the AMA Research Challenge Official Rules for terms and conditions.

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Registration Is Required

Welcome to the largest national, multi-specialty research event featuring research from 750 medical students, residents and fellows, and international medical graduates.


Latent reactivation of varicella zoster virus (VZV) has been increasingly recognized as a cause of ischemic stroke in adults, a poorly documented VZV complication in the United States. VZV vasculitis should be suspected if a patient: is immunocompromised, has a recent history of varicella or zoster infection or reactivation, lacks vascular risk factors, has recurrent strokes despite therapy, or has arterial narrowing inconsistent with atherosclerosis. We present a case where an immunocompetent adult, with no history or risk factors for stroke, was diagnosed with VZV vasculopathy and experienced recurrent ischemic strokes despite antiviral therapy. Further research into causes and management of VZV-related strokes refractory to acyclovir and steroids is needed to improve patient management.

Recurrent ischemic strokes caused by varicella zoster vasculopathy in an immunocompetent patient

Background. Carotid artery dissection is a major cause of stroke in patients younger than 40 years old, accounting for up to 25% of ischemic cerebral events that lead to a long-term disability and a large economic burden. The clinical picture of this condition varies widely, thus, the disease may be easily misdiagnosed. We present a case of spontaneous left carotid artery dissection in a previously healthy 29-year-old female. Our aim is to stress on the presentation and a diagnostic approach of this condition, as prompt diagnosis and, thus, early treatment decrease the rate of a stroke and its consequences in this age population.
Case Presentation. A 29-year-old female presented to our clinic with the presumptive diagnosis of first manifestation of migraine, complaining of moderate unilateral headache, involving the left half of the head and neck, including periorbital region, exacerbated by physical activity. However, the clinical presentation of migraine was atypical, including the absence of aura, persistent character of pain, inadequate response to therapy. A decision to perform carotid artery ultrasound was made, revealing a hypoechoic thrombus, completely occluding the lumen of the left internal carotid artery, arising from posterior aspect of the left common carotid artery bifurcation. On color doppler, blood flow in the internal carotid artery was not visualized. MRA of the brain revealed dissection of the left carotid artery with the false lumen that has been completely clotted. A true lumen, 2 mm in diameter, also contained thrombus. The patient started anticoagulant therapy that lasted for 8 weeks. Our patient had a favorable outcome: symptoms were resolved and the blood flow was restored due to complete recanalization of the thrombus.
Discussion. In young adults, carotid artery dissection may occur spontaneously, thus, may be missed or misdiagnosed as other more common conditions, such as migraine, cluster or tension headache. Carotid artery dissection may cause serious complications (TIA, stroke, or one-sided blindness), so it should be included into the differential diagnosis of headache. Early diagnosis is imperative to initiate appropriate therapy that improves patients’ prognosis. More studies are needed to improve diagnostic approach and outcomes in this group of patients.


Spontaneous carotid artery dissection presenting with migraine-like headache in a young female: a case report.

Background
Fetal tachyarrhythmia presents in 0.4%-0.6% of pregnancies. Possible causes include cardiac structural abnormalities, atrioventricular node dysfunction, and atrioventricular re-entry tachycardia (AVRT). It is believed that around 41% of fetal supraventricular tachycardia (SVT) cases will progress to hydrops fetalis, making early identification and treatment imperative. This case illustrates challenges of managing fetal SVT as well as various avenues of treatment to consider in refractory cases.
Case Presentation
This patient is a 27-year-old G4P2012 female with a history of epilepsy controlled on Lamictal. After 32 weeks of uncomplicated prenatal exams, fetal tachycardia at 220-240 bpm and fetal pericardial effusion were found incidentally. Initial treatments with sotalol for 2 days, followed by digoxin and sotalol for 4 days were unsuccessful. On day 7, cordocentesis with adenosine was attempted unsuccessfully. Finally, flecainide in combination with sotalol led to spontaneous cardioversion. On day 10, prolonged PR and QRS intervals were noted. Sotalol was discontinued and by hospital day 14 the PR interval normalized. Three weeks later the patient was readmitted for scheduled induction of labor using Pitocin, and vaginal delivery at 36 weeks and 6 days was uncomplicated. The neonate required 5 days of NICU evaluation with escalating doses of propranolol from 1mg to 5.85mg daily in order to suppress SVT recurrences. The infant was discharged on 2mg propranolol twice daily with continuous fetal heart rate monitoring. On posthospital day 7 the baby was evaluated by pediatric cardiology and was diagnosed with Wolff- Parkinson-White (WPW) syndrome. Propranolol was continued with a plan for eventual ablation.
Discussion
SVT with ventricular preexcitation, specifically AVRT, is typically the presenting arrhythmia of WPW. Clinical manifestations can be recognized in utero, though most cases are not diagnosed until early childhood. Hydrops fetalis may develop if the tachyarrhythmia persists for greater than 6-12 hours. Management strategies include pharmacologic therapy, vagal stimulation, and ablation. Many options exist within the pharmacologic treatment category alone, and various combinations may be considered. Due to the critical nature of these cases, there should be strong familiarity with treatment pathways for refractory cases to ensure patients are managed appropriately and quickly enough to avoid morbidity and


A Rare and Challenging Case of Refractory Fetal Supraventricular Tachycardia

Background:
Local anesthetics such as lidocaine and other sodium channel blockers are commonly used in obstetrics for laceration repair, epidurals, and spinal blocks. Although rare, the most common adverse effects from these medications result from drug toxicity or allergic reactions. Numerous studies in dentistry, dermatology, and emergency medicine have shown that a 1% diphenhydramine solution is comparable in anesthetic effects to lidocaine for minor laceration repairs, however no cases have been described in obstetrical literature. 

Case Presentation:
A 23-year-old gravida 1 with a reported history of an anaphylactic reaction to lidocaine and confirmatory allergy testing in Venezuela presented to Labor and Delivery for induction of labor. She strongly desired an epidural for pain control; however, our hospital was unable to offer a benzoic acid ester sodium channel blocker as an alternative to lidocaine. After fully discussing options, she agreed to an induction of labor with IV pain meds and potential use of 1% diphenhydramine local injection for laceration repair after delivery. She had a spontaneous vaginal delivery with a 2nd degree perineal laceration. The physician created a 1% diphenhydramine solution using 2mL of 5% diphenhydramine (50mg/mL) provided by the pharmacy combined with 8mL of normal saline. She then injected the solution into the vaginal tissue and perineal skin along the torn edges. The repair was done in the usual fashion. She declined any IV pain meds before or during the laceration repair and tolerated the procedure well. She reported satisfactory pain control with diphenhydramine.

Discussion
Lacerations are commonly encountered during vaginal deliveries, with 53-79% of women experiencing some form. Adequate analgesia is an essential repair component, whether via epidural or local anesthetic injection. Diphenhydramine is primarily an H1 receptor antagonist with sodium channel blocking properties similar to the amide and benzoic acid ester local anesthetics, thus giving comparable anesthesia. Although rare, lidocaine allergies pose a significant problem for obstetric patients requiring laceration repair following a delivery. Currently, there are no clear guidelines on how to proceed with a laceration repair for a patient with a true lidocaine allergy. Therefore, having an available alternative option for these patients is vital. 



Analgesia for obstetric lacerations in patients with known lidocaine allergy; local injection with 1% diphenhydramine.

Background 
Cervical cancer (CC) is one of the most frequently diagnosed antepartum gynecologic malignancies. Treatment of antepartum CC varies based on stage, gestational age at diagnosis, and patient preference for continuation or termination of pregnancy. The purpose of this case report is to demonstrate an aberrant presentation of CC and describe clinical reasoning for determining medical management. 
Case Presentation 
A 31-year-old G5P5 presented to the Emergency Department with acute profuse vaginal bleeding. Her last menstrual period was unknown, and she denied using contraception. Quantitative HCG was 8,197 mlU/mL and bedside ultrasound showed a 21w5d fetus with cardiac activity. Patient was unaware of the pregnancy. 
Initial pelvic exam showed unknown tissue that easily detached from cervix. This tissue was confirmed to be stage IIIC1 squamous cell carcinoma of the cervix. Patient desired treatment that would prioritize neonatal survival and was started on weekly chemotherapy with the intent of delaying delivery until viability. 
The patient delivered at 32w3d, and postpartum lymphatic pathology was negative for metastases. Patient had uncomplicated postpartum care and resumed chemotherapy shortly after. 
Discussion 
Medical management for antepartum CC aims to balance treatment of the patient while minimizing harm to the fetus. This patient prioritized fetal survival, but due to the advanced stage of the cancer, it was crucial for her to receive prompt oncologic treatment. Due to later gestational age, there was decreased risk for chemotherapy related fetotoxicity. Future research can attempt to determine long term side effects of second and third trimester antepartum chemotherapy. 


Cervical cancer presenting as an inevitable abortion: determining treatment for new onset cervical cancer in a previously unknown second trimester pregnancy

Uterine rupture, caused by separation of the uterine myometrium, is a rare but life-threatening complication of pregnancy that has increased in incidence over the past two decades alongside the increased rate of cesarean delivery. Uterine rupture is most often correlated with a prior cesarean delivery, prior uterine surgery, or abnormal placentation such as placenta increta, placenta percreta, or placenta accreta. It can also be associated with a short interval pregnancy of less than 6 months, prior uterine rupture, or induction of labor. A thirty-four-year-old G7P3123 African American female with a history of four prior cesarean sections and a current a viable intrauterine pregnancy at 16 weeks and 2 days by bedside ultrasound presented in critical condition to the Emergency Department at the University of Louisville with sudden onset abdominal pain following intercourse. On presentation, she was hypotensive with a blood pressure of 68/30 mmHg. Pulse was 88 beats/minute with lack of tachycardia secondarily to use of a beta blocker for chronic hypertension. Respiratory rate was 24 times per minute, and oxygen saturation by pulse oximetry (Sp02) was 100% on room air. The patient on physical exam looked pale and abdomen was distended showing clinical signs of peritonism, rebound tenderness, and guarding. She was conscious but disorientated. Early diagnosis and appropriate management are essential to decrease the high maternal and fetal morbidity and mortality rates associated with uterine rupture. High clinical suspicion and early surgical intervention with a low threshold to perform a laparotomy is fundamental to successful management. The decision for method of surgical intervention should be made on a case-by-case basis depending upon patient wishes, hemodynamic stability, as well as hospital resources for mother and a potentially premature infant. For uncontrollable, life-threatening bleeding hysterectomy should be considered. In other circumstances, decision for uterine evacuation and repair versus uterine repair alone should weigh the risk and benefits of each intervention on patient outcomes.

High Index of Clinical Suspicion for a Spontaneous Uterine Rupture in the Early Second Trimester

Protein S Deficiency in Pregnancy

Background: Herpes infections can be dormant and recurrent in patients and in the eye, can cause corneal scarring. This coupled with cataracts in patients leaves them blinded with no treatment options since the herpetic scars will reoccur if removed. 
 
Case Presentation: A 72 year old Caucasian female was referred with a Herpetic corneal scar post a prior LASIK surgery. Her best corrected vision was a distorted 20/60 with an irregular cornea. Using Excimer Laser Corneoplastique™, her cornea was brought to a measurable outcome which then allowed her to undergo accurate premium cataract surgery. 
 
Discussion: By using a two step surgical method, the patient was able to achieve emmetropia, a feat normally not possible for most herpes patients. Each problem was separately addressed: first the corneal scar secondary to herpes, and next the cataracts with a Corneoplastique technique.

Converting Irregular Herpes Cornea to Regular for Accurate Premium Cataract Surgery to 20/20

Abstract Title: Impact of Pregnancy on Idiopathic Intracranial Hypertension: A Case Series of 13 Patients
Richard Pellizzari BS, Nathan Lambert-Cheatham DO, Nathaniel Pasmanter DO, PhD, Beom Lee BS, Neysa Miller BS, Lina Nagia DO, David Kaufman DO

Background
Idiopathic Intracranial Hypertension (IIH) is a syndrome in which intracranial pressure is elevated due to unknown causes leading to papilledema and potentially permanent vision loss. Due to the association of weight gain to worsening papilledema, it has been believed that pregnancy induced weight gain could worsen IIH exacerbations. Current literature has challenged this assertion. These findings have raised questions about the impact of pregnancy in IIH and how this differs from weight gain outside the context of pregnancy. To explore these questions, our study reviews the changes in papilledema and symptoms in pregnant IIH patients at Michigan State University.

Methods
Charts were reviewed for patients who had diagnoses of IIH, had at least two clinic visits with neuro-ophthalmology, were seen during pregnancy, and did not have prior surgery for IIH. Data collected included demographics, weight/BMI, gestation, diagnostic studies (OCTs, Humphrey visual fields, head imaging, lumbar punctures), clinical tests (visual acuity, color vision, alignments, disc edema grade), and treatments. For analysis, data collected during pregnancy was compared to data from their initial IIH diagnosis, their baseline exam prior to pregnancy, and their first clinical visit after delivery.

Results
13 patients were identified who met selection criteria. 11/13 patients gained more than their previous exacerbation weight. 8/13 patients gained more weight than their goal during pregnancy. 6/13 had pharmacologic therapy for IIH discontinued during pregnancy. 3/13 patients developed a clinically significant increase in disc edema during pregnancy. 2/3 of the patients developed optic nerve atrophy and new visual field defects post pregnancy.

Conclusion
In our case series, most patients had risk factors for worsening papilledema including increased weight from initial exacerbation, weight gains over goal for pregnancy, and discontinuation of pharmacologic therapy. With these risk factors, we would expect most patients to have worsening papilledema. Despite this, only 3 patients had worsening papilledema. This finding suggest that weight gain during pregnancy likely has less impact on papilledema than traditional weight gain in IIH.

Impact of Pregnancy Weight Gain on Papilledema and Vision in IIH Patients: A Case Series


Abstract Title: MOG-Positive Optic Neuritis In A Child Presenting With Bilateral Disc Edema And Pre-Retinal Hemorrhage

Rasika Sudharshan1, Mark S. Borchert2, Melinda Y. Chang2
1USC Keck School of Medicine, 2Children’s Hospital Los Angeles, Department of Ophthalmology 

Background
Our case discusses an 8-year-old male with no relevant past medical history or trauma, who presented with two weeks of progressive bilateral vision loss that acutely worsened over 3 days. This case demonstrates typical features of optic neuritis in children, including bilaterality, severe vision loss, disc edema, and the atypical finding of extensive retinal and preretinal hemorrhages in the macula. 

Case Presentation
On presentation, his visual acuity was light perception with dilated and poorly reactive pupils. Fundus exam showed bilateral optic disc edema (with pallor) and retinal venous tortuosity without cotton wool spots. Pupil exam showed a right RAPD. His left fundus exam was also significant for preretinal and intraretinal hemorrhages. Lumbar puncture showed an elevated opening pressure (36mmH2O) with normal glucose, abnormal protein at 77, and mildly elevated pleocytosis at 12. Brain MRI showed an incidental small posterior fossa arachnoid cyst but no white matter lesions, and orbit MRI showed bilateral optic nerve enhancement consistent with optic neuritis, right greater than left. Lab tests showed a normal CBC and a MOG-Ab positive titer level of 1:100. 

After six doses of IV methylprednisolone and completion of five days of plasmapheresis, visual acuity improved to 10/200 OD and 20/100 OS. Then, after three rounds of IVIG and prednisone taper, his visual acuity improved to 20/80 and 20/30. Forty-eight days after treatment, bilateral optic disc edema and retinal hemorrhages resolved. His final examination showed bilateral optic atrophy OU and visual acuity of 20/40 OD and 20/20 OS. 

Discussion
Due to increased intracranial pressure (ICP) on lumbar puncture and intra- and preretinal hemorrhages, papilledema or leukemia were considered. Increased ICP has not been consistently reported in patients with MOG-Ab ON, and ICP was believed to be falsely elevated in this case due to excessive hypercapnia associated with sedation. Retinal hemorrhages are rare in optic neuritis, which typically occurs without disc edema in adults. In children, optic neuritis may be associated with disc edema and peripapillary hemorrhages in severe cases, especially in those with MOG-Ab positivity. This is the first case, to our knowledge, of preretinal hemorrhage in the macula associated with MOG-Ab positive optic neuritis.


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Parkinsonism-hyperpyrexia Syndrome: a Rare Case

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Recurrent ischemic strokes caused by varicella zoster vasculopathy in an immunocompetent patient

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