United States

Background
Left-right disorientation is most often associated with Gerstmann’s syndrome, a syndrome caused by damage to the dominant parietal lobe which presents with a tetrad of symptoms including left-right disorientation, acalculia, agraphia, and finger agnosia. This disorder is most often caused by a stroke but is also rarely associated with brain tumors and subdural hematomas. There are currently only 4 cases reported in the literature of seizures presenting with Gerstmann’s symptoms.

Case Presentation
A 60-year-old man presented to our neurology office with frequent episodes of decreased awareness and shock-like sensations in his hands and feet. His evaluation showed no major abnormalities including a normal electroencephalogram and magnetic resonance imaging of the brain. He was started on levetiracetam for presumed complex partial seizures. He experienced no further seizure episodes thereafter. During a follow-up visit, he reported new historical details not previously disclosed: since childhood, he had difficulty differentiating between left and right. These symptoms had caused significant difficulties for him throughout his life, leading to special tutoring in elementary school, and trouble learning to drive. He often had to use external cues such as looking at the watch on his left hand to determine left and right. He reported that after starting levetiracetam he became hyperaware of directionality and even felt that his visual field was labeled with “left” and “right”. His left-right confusion had seemed to completely and permanently abate. 

Discussion
The resolution of this patient’s left-right confusion with levetiracetam was dramatic and unexpected. We question whether his left-right disorientation may have been related to underlying seizure activity in the parietal lobe as has been described rarely in the literature. However, the persistence of his left-right disorientation throughout life, even when not actively seizing, is puzzling and would represent a presentation unique from anything currently in the literature. 


AMA Research Challenge 2022

Lifelong Left-Right Disorientation that Resolved with Anti-Epileptic Therapy

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Charles Lopresto, DO

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poster

Background:
Miller-Fisher Syndrome (MFS), a rare subtype of Guillain-Barre Syndrome (GBS), is an autoimmune disease characterized by inflammatory demyelination. The classic presentation is a triad of ophthalmoplegia, ataxia, and areflexia. Rare cases of MFS have been documented following SARS-CoV-2 infection and some SARS-CoV-2 vaccines.
Case Presentation:
A 64-year-old male received the J&J SARS-CoV-2 vaccine on 04/04/2021. He developed gait ataxia, facial weakness, dysarthria, dysphagia, diplopia, and extremity paresis over the following 2 weeks. On 04/23/21 he was diagnosed with GBS based on clinical presentation and albuminocytologic dissociation appreciated in CSF at an outside hospital. He received a 5-day course of IVIG and was discharged home with minimal improvement. 
The patient was re-evaluated on 06/18/2021. Neurological examination was significant for bilateral facial weakness worse on the left compared to the right, patellar and ankle areflexia, and absent pallesthesia below the knees. Gait was ataxic with positive Romberg. Laboratory results showed elevated titers of IgG GQ1b (15000), supporting the diagnosis of MFS. 6 months later, he returned to work and resumed a fully functional life. His dysarthria was minimal; paresis on the right face resolved and was minimal on the left; diplopia completely disappeared.
Discussion:
MFS is a rare GBS variant that is associated with ophthalmoplegia, ataxia, and areflexia. It has been described as a rare complication of COVID-19 infection and Pfizer and Oxford-AstraZeneca vaccines but has not been previously documented in association with the J&J vaccine.
The documented cases of MFS after SARS-CoV-2 infection and vaccination can be anti-GQ1b negative or positive. The only published MFS case with positive anti-GQ1b has limited presentation without ataxia or neuropathy. We present a unique case of MFS with positive anti-GQ1b antibody, suggesting GQ1b molecular mimicry as the possible immunopathogenic etiology.
Conclusion:
This case offers evidence that physicians should be vigilant for neurological complications after SARS-CoV-2 vaccination. MFS is a rare complication but has a good prognosis with early identification and treatment. There is limited evidence regarding factors influencing MFS development post SARS-CoV-2 vaccination, and we hope this case contributes to a better understanding of it.


Miller-Fisher Syndrome in a 64-year-old male following J&J SARS-CoV-2 vaccine

Background
Neuroblastoma is a pediatric neuroendocrine tumor of the sympathetic nervous system that can present distinctively in terms of location, histopathologic appearance, and biologic characteristics. The clinical presentation is reflective of the location of the primary tumor, tumor metastasis, and presence of neoplastic syndrome. We report a rare case of neuroblastoma in a 3-year-old female with clinical presentation of multiple cranial nerve involvement. 

Case Presentation
A 3-year-old afebrile, lethargic female presented with bilateral eyelid droop, right head tilt, slurred speech, gagging, abnormal walking and no bowel movement. Neurological examination noted bilateral ptosis, dysarthria, left tongue deviation, proximal weakness in upper and lower extremities, areflexia in biceps and patellar tendons, dysmetria, and wide-based gait. 

MRI of the brain showed heterogeneous appearance of the clivus and MRI of the spine showed right adrenal mass and heterogeneous enhancement of multiple vertebrae, suggesting possible metastatic disease. Serum and cerebrospinal studies were unremarkable. Patient was treated with intravenous methylprednisolone and plasmapheresis for suspected paraneoplastic syndrome; however, she continued to clinically progress. Adrenal mass biopsy results and elevated urine VMA and HVA levels were consistent with the diagnosis of neuroblastoma. Nuclear imaging and meta-iodobenzylguanidine scan were negative. Paraneoplastic panels and Lambert Eaton panel were negative for autoantibodies. Total resection of the abdominal mass and right adrenal gland with continued steroid taper, resulted in reported near total symptom resolution. 

Discussion
Our patient presented with antibody negative paraneoplastic polyneuropathy due to neuroblastoma. Cranial nerve involvement in neuroblastoma can result from tumor involvement of the sympathetic chain, paraneoplastic syndromes, or metastasis to the skull. Our patient's initial imaging suggested potential metastatic or inflammatory involvement of the clivus, suspected of causing her cranial nerve symptoms. However, nuclear studies were negative for metastatic disease and patient’s symptoms resolved after resection of the main mass suggesting a paraneoplastic etiology. Although her paraneoplastic panel and Lambert-Eaton panel were negative for common associated antibodies, it is likely an unmeasured autoantibody, cytokine, hormones, or peptide was at play. 

Neuroblastoma should be considered as a differential for a neurological presentation involving multiple cranial nerves in a child. 




Neuroblastoma Presentation with Multiple Cranial Nerve Involvement

Occult vascular structures in gliomas visible on routine MRI are associated with risk of intracranial hemorrhage 
Benson JW, Bonm AN, Ko AL, Graber JJ
University of Washington Medical Center
Seattle, WA
Purpose
This study aimed to assess the clinical significance of types of occult vascular
structures seen within gliomas on routine MRI.
Methods
Three types of vascular structures (angioarchitecture) seen within
gliomas on routine magnetic resonance imaging (MRI) have been described that were not common in brain metastases. These occult vascular structures are classified as intratumoral, peritumoral or periventricular. A retrospective review analyzed 52 consecutive grade II-IV gliomas and 70 additional gliomas from
a retrospective study assessing post radiation changes were assessed. The initial 52
patients were evaluated by two blinded neuroradiologists. The second 70 patients were evaluated by a neurooncologist who is board certified in neuroimaging and a second year medical student after training on the initial dataset in a blinded fashion. Multivariate analysis was performed to develop predictive models of the vascular-related parameters. 
Results
Presence of peritumoral vessels or peritumoral and intratumoral vessels
were significantly associated with intracranial hemorrhage.
Conclusion
Occult microvascular angioarchitectural structures detectable on routine clinical MRI may aid in determining risk of intracranial hemorrhage for patients with gliomas. 

Occult Vascular Structures in Gliomas Visible on Routine MRI are Associated with Risk of Intracranial Hemorrhage

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.


Parkinsonism-hyperpyrexia Syndrome: a Rare Case

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.

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Lifelong Left-Right Disorientation that Resolved with Anti-Epileptic Therapy

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