High-dose Methylprednisolone Research Articles

A Case of Neuropsychiatric Systemic Lupus Erythematosus as A Sequela of Kikuchi’s Disease

The aetiology of Kikuchi’s Disease (KD), a benign condition associated with multiple lymph node enlargements, remains unknown but several reported cases associate it with systemic lupus erythematosus (SLE). A 34-year-old Indonesian woman presented with multiple painless lymph node enlargements around the neck with three weeks of fever, myalgia, arthralgia, night sweats, and weight loss. Laboratory examination showed pancytopenia and elevated ESR. HIV and tuberculin skin testing were negative. Lymph node biopsy confirmed the diagnosis of KD. One week later, despite improvement in lymphadenopathy, she developed fever. Her ANA, anti-Smith antibodies, and anti-Ribosomal-P protein antibodies were positive. She was diagnosed with SLE and managed as an inpatient with high dose methylprednisolone. One week later, the patient developed psychotic symptoms and fever. The results of Laboratory examinations with lumbar puncture were unremarkable. She was diagnosed with neuropsychiatric SLE (NPSLE) and managed with methylprednisolone, paracetamol, folic acid, alprazolam, and amitriptyline. One month later, the patient returned with three days of fever and dyspnea. Despite improvement in neuropsychiatric symptoms, pneumonia and tonsillopharyngitis were diagnosed based on physical examination and chest X- rays. Her condition deteriorated into septic shock. She suffered cardiac arrest and was pronounced dead, despite attempted resuscitation.While KD is a benign condition, diagnosis is challenging due to its rarity and similarity to other lymphadenopathy diseases. Its association with SLE and NPSLE carries poor prognosis with higher mortality rate from the disease progression and adverse medication effects. Thus, early intervention and prevention of complications are crucial in managing patientswith KD.

Resistant dermatomyositis as a manifestation of post COVID-19 infection: A case report

Many autoimmune diseases were reported as a sequel of SARS CoV-2 infection. We report here a 51-year-old female who presented with myalgia, progressive quadriparesis, and skin rash 2 weeks after COVID-19 infection. She improved with high-dose methylprednisolone. She was given oral methotrexate and mycophenolic acid during follow up. However, her condition deteriorated until she passed away half a year after the initial illness. This case showed that dermatomyositis can be associated with COVID-19 infection.

Autopsy evidence of central and peripheral demyelination in a case of Guillain-Barré syndrome/ Bickerstaff brainstem encephalitis overlap syndrome

We report a case of a 59-year-old male who had acute, severe, rapidly progressive ascending weakness, which progressed to difficulty of breathing in a span of twelve hours. Neurophysiologic studies showed acute denervation compatible with acute motor axonal neuropathy. He was started on intravenous immunoglobulin therapy on the first day of hospitalization and completed five days of treatment but still developed decrease in sensorium and bilateral ophthalmoplegia. Imaging studies of the brain and cervical spinal cord showed findings that are non-contributory to the clinical presentation, leading to the consideration of Bickerstaff’s brainstem encephalitis. Pulse therapy with high dose methylprednisolone for five days was given however, despite maximal treatment, he expired on the 12th day of illness. Post-mortem immunohistochemical studies of the pons and sural nerve showed areas of inflammation and demyelination in both areas, suggesting combined central and peripheral demyelination in a single patient. Literature review shows that our patient presents with atypical clinical and diagnostic features, different from Bickerstaff’s brainstem encephalitis and combined central and peripheral demyelination, suggesting another disease entity presenting as acute fulminant neuropathy.

Clinical characteristics of radiation-induced optic neuropathy: A single-center retrospective study

PurposeTo observe the clinical and imaging characteristics of radiation-induced optic neuropathy (RION). MethodsWe retrospectively reviewed the clinical data of 43 patients (69 eyes) who were diagnosed with RION at the Chinese PLA General Hospital from 2010 to 2021. ResultsThe latency from radiotherapy to onset of visual loss ranged from 1 to 132 (36.33 ​± ​30.48) months. Optic disc pallor and optic disc edema were found in 27.0% (10/37) and 8.1% (3/37) of the eyes, respectively, within 2 months. After treatment, the best corrected visual acuity (BCVA) was restored in 24.6% (17/69) of the eyes and the final BCVA improved in 13.0% (9/69) of the eyes. An 82.5% (33/40) of the eyes with magnetic resonance imaging (MRI) showed enhancement of the affected optic nerve, mostly (69.7%) in the intracranial segment, and 36.4% (12/33) of the eyes with expansion and T2-high signals also showed enhancement of the affected optic nerve. The superior retinal nerve fiber layer (RNFL) and the outer circle superior quadrant (OS) of the inner limiting membrane to retinal pigment epithelium (ILM-RPE) layer thinned significantly during the first month. The center of the ILM-RPE layer thickened significantly during the first two months and the inner circle temporal quadrant (IT) of the ILM-RPE layer thickened significantly from the third to sixth month. The RNFL thinned significantly after 6 months except for the temporal quadrant, and the average inner circle superior quadrant (IS) and outer circle of the ILM-RPE layer thinned significantly after 6 months. There was no significant difference between hyperbaric oxygen therapy (HBOT) and high-dose intravenous methylprednisolone (IVMP) therapy in improving BCVA recovery or final BCVA (P ​> ​0.05). ConclusionsThe structural damage of the RNFL and ILM-RPE layer occurred during the first month, the RNFL showed progressive thinning during the follow-up period, while the ILM-RPE layer showed thinning during the first month, thickening from the third to sixth month, and thinning after 6 months. There was a discrete region of enhancement of the optic nerve, often with expansion and high-T2 signals on MRI. HBOT and high-dose IVMP therapy were hardly effective for treating RION in the non-acute stage.

A confirmed case of macrophage activation syndrome secondary to spotted fever rickettsiosis: Response to immunomodulation in a pediatric patient in northeastern, Mexico

An 8-year-old girl with a rash and high-grade fever for 6 days arrived at the emergency room. She had an erythematous macular rash on the face, trunk, arms, and legs. Further interrogation called attention to the presence of close contact with stray dogs. Her town had been recognized as a site of a rickettsiosis outbreak in the past year. Spotted fever rickettsiosis was suspected, and doxycycline treatment was initiated. Macrophage activation syndrome (MAS) secondary to Rickettsia rickettsii infection was diagnosed according to the Hemophagocytic lymphohistiocytosis and EULAR/PRINTO/PRES 2016 criteria. As there are no clear guidelines on the treatment of MAS secondary to R. rickettsii. the course of action taken by the pediatric intensive care unit team was to avoid disseminated intravascular coagulopathy and treat MAS, both life-threatening conditions. Directed therapy with high doses of methylprednisolone and intravenous immunoglobulin therapy was initiated. The patient recovered, regaining her functional state before the illness. Few articles have described the association between MAS and rickettsiosis, an illness with high mortality, which makes it paramount to detect and treat promptly.

Abstract #1401671: Hashimoto’s Disease with Thyroid Eye Disease, Negative TRAB and Positive TPO Antibodies Treated with Teprotumumab

Thyroid Eye Disease (TED) is most commonly associated with hyperthyroidism secondary to Graves’ disease (GD). The disease activity of TED is positively correlated with the presence of thyrotropin receptor antibodies (TRAB). Some with TED are euthyroid at presentation but have positive TRAB. Less frequently, patients with Hashimoto’s disease have TED, the majority of which are TRAB positive. We present a unique case of euthyroid TED with negative TRAB but positive thyroid peroxidase antibodies (TPO Ab) treated with teprotumumab. A 65-year-old African-American male presented with a 9-day history of gradually worsening vision, ocular pain, and bilateral eye bulging. His best-corrected visual acuity at near was 20/200 right eye and 20/400 left eye with full peripheral vision by confrontation. Pupils were equal and reactive to light with normal intraocular pressure. There was bilateral upper eyelid retraction, diffuse conjunctival injection, proptosis, and diffuse external ophthalmoplegia. His clinical activity score was 7. The thyroid exam was normal. Computed Tomography (CT) scan of the orbits showed significant, tendon-sparing enlargement of all extraocular muscles along with bilateral orbital apex crowding. Bilateral compressive optic neuropathy from severe TED was suspected. TSH, free T4, and T3 were normal. TPO Ab was positive but TRAB was negative by both binding inhibitory and Thyroid Stimulating Immunoglobulin (TSI) assays. He was treated with high-dose intravenous methylprednisolone for 5 days followed by 2 weeks of oral prednisone. One month after presentation treatment was initiated with 8 infusions of teprotumumab with complete resolution of all ocular symptoms and restoration of normal vision. Six months after the last dose of teprotumumab he remains euthyroid with negative TRAB and positive TPO Ab. TED is uncommon in euthyroid individuals. Most have positive TRAB and many ultimately develop hyperthyroidism. Even less common are those with Hashimoto’s thyroiditis (HT) presenting with TED. Teprotumumab is the only Food and Drug Administration-approved medication to treat TED. Our patient with vision-threatening TED had a remarkable response to teprotumumab with the restoration of normal vision and resolution of TED. We could not identify any other reports of a patient with euthyroid HT with positive TPO Ab but negative TRAB treated with teprotumumab. Physicians should be mindful of this unusual presentation to detect cases early and institute appropriate treatment to prevent visual complications.

Unique ulcerative autoinflammatory disease mistaken for factitious disorder

Autoinflammatory diseases (AID) are disorders of inappropriate innate immune system activation. Twenty-four percent of patients with AID do not have a well-defined clinical picture or pathogenic mutation and are characterized as having undefined AID. Cutaneous ulcers have been described but not outside of distinct histologic and clinical disorders. We present a unique case of undefined adult-onset AID with ulcers mistaken for a factitious disorder.A 32 year-old female with borderline personality disorder, depression, and anxiety developed recurrent cutaneous MRSA abscesses and recurrent ulcers of the extremities and scalp associated with alopecia. The ulcers were minimally-responsive to IV antibiotics, isotretinoin, oral dapsone, intralesional triamcinolone, and adalimumab. Flares of the skin disease were associated with fevers and elevated inflammatory markers.Scalp ulcer biopsies revealed numerous pigmented hair casts with trichomalacia and perifollicular lymphocytic infiltrate. One hair follicle contained bacterial cocci associated with a perifollicular neutrophilic infiltrate. These findings along with the patient's psychiatric history led to a diagnosis of trichotillomania from multiple dermatology and psychiatry consultations. The patient denied that she was inducing the lesions and sought another psychiatric consultation, who felt this was not a factitious disease. She was referred to immunology for evaluation of a possible IEI. Laboratory evaluation revealed mild hypogammaglobulinemia with IgG of 663 (768–1632) mg/dL, normal antibody response to recall antigens, but absent response to neo-antigen (Salmonella typhi). Peripheral lymphocyte phenotyping was normal except for a relative decrease in the number of memory B-cells with CD27+IgD+% of 6.9 (13.4–21.4) and CD27+IgD-% of 2.5 (9.2–18.9). Neutrophil oxidative burst was normal. Whole exome sequencing did not identify variants in genes associated with her phenotype.The patient was partially responsive to IVIG but highly responsive to high-dose intravenous methylprednisolone. She was subsequently diagnosed with undefined AID based on Eurofever registry criteria matching her clinical course and had near complete resolution of skin lesions and alopecia with the addition of anakinra.Clinicians must be vigilant for atypical presentations of AID. Our patient had signs of undefined AID but had a delayed diagnosis due to her psychiatric disorders, to which her symptoms were attributed, and a previously undescribed presentation of AID.

Case report of extranodal natural killer/T-cell lymphoma that induced secondary hemophagocytic syndrome-related histiocytic glomerulopathy

Hemophagocytic syndrome (HPS) is a proliferative disease of the mononuclear macrophage system involving multiple organs and systems. We report a 50-year-old Asian woman who presented with unexplained fever and proteinuria. Laboratory tests showed cytopenia, considerably elevated serum ferritin and IL-2 receptor concentrations, and evidence of hemophagocytosis in the bone marrow. A renal biopsy showed macrophage infiltration into the glomerulus, resulting in podocyte and endothelial cell damage. We finally diagnosed the patient with extranodal natural killer/T-cell lymphoma, nasal type that induced HPS-related histiocytic glomerulopathy. Proteinuria and inflammation responded to treatment with high-dose pulsed methylprednisolone combined with VP-16 and cyclosporine. To the best of our knowledge, this is the first documented case of HPS-related histiocytic glomerulopathy triggered by a malignant tumor.

Impact of immunosuppressive regimen on ICU acquired pneumonia in critically ill COVID-19.

Immunosuppressors (IS) such as Dexamethasone (DXM), Tocilizumab, and high-dose methylprednisolone boli (HDMB), are used in COVID-19-related acute respiratory distress syndrome (ARDS). This study aimed to determine whether COVID-19 ARDS-related combined IS therapy was associated with an increased incidence of ICU-acquired pneumonia (IAP). We retrospectively analyzed COVID-19-ARDS admitted to ICU from March 2020 to April 2022. Patients' and IAP characteristics were analyzed according to five IS regimens: No IS, DXM alone, DXM+HDMB, DXM+tocilizumab, and DXM+tocilizumab+HDMB. To investigate the role of IS on IAP incidence, we performed a multivariate logistic regression and built a propensity score. Ultimately, we used a conditional logistic regression after pairing on the propensity score. The study included 496 COVID-19-ARDS. Regarding the IS therapy, 12.7% received no IS, 43% DXM alone, 21.6% DXM+HDMB, 15.5% DXM+tocilizumab and 5.4% DXM+tocilizumab+HDMB. 37% presented at least one IAP, and the IAP incidence was higher with DXM+HDMB (66.4%) compared to no IS (P<0.0001), DXM (P<0.0001) and DXM+tocilizumab (P<0.0001). HDMB and probabilistic antibiotherapy at admission were independent IAP predictors after adjustment on the propensity score (respectively OR:2.44; P<0.0001 and OR:2.85; P<0.001). In critically ill COVID-19, HDMB significantly increases the risk of IAP whereas DXM alone, nor in combination with tocilizumab, did not.

Chronic granulomatous disease identified in the evaluation of atypical Kawasaki disease in an infant

An 8-week-old full-term male was admitted after 5 days of fever and cough. He was previously admitted at 3 weeks of age for a Klebsiella pneumoniae urinary tract infection (UTI) and treated with 10 days of antibiotics. Blood and cerebrospinal fluid cultures, serum inflammatory markers, and respiratory viral testing obtained at that time were negative. During the current admission, urine cultures grew Klebsiella oxytoca and he was again treated for a UTI.His fevers persisted and on day 8 of illness he met criteria for atypical Kawasaki disease with elevated inflammatory markers, elevated liver enzymes, thrombocytosis, leukocytosis, and anemia. Prior to initiating IVIG, the patient underwent abdominal ultrasound to rule out occult infection, revealing multiple liver and spleen abscesses. Aspirated fluid grew Serratia marcescens and he was started on cefepime. Additional imaging of the chest, abdomen, and pelvis revealed pulmonary nodules. Evaluation of these nodules demonstrated no additional mycobacterial, fungal, or bacterial infections.Chronic granulomatous disease (CGD) was suspected based on the patient’s age, multi-organ abscesses with Serratia marcescens, and parental consanguinity (first cousins). Neutrophil oxidative burst testing showed absent ROS production. Genetic testing demonstrated a homozygous pathogenic variant in NCF2 (exon 3 deletion), confirming the diagnosis of autosomal-recessive CGD. He received high-dose methylprednisolone with a prolonged taper to promote antibiotic penetration of his CGD-associated liver abscesses. Repeat imaging showed interval improvement and the patient began prophylactic itraconazole and trimethoprim-sulfamethoxazole.Familial testing revealed heterozygous carrier status of the same NCF2 variant in both parents and the patient’s two sisters. At 10 months of age, the patient underwent a 10/10 matched sibling bone marrow transplant. Chimerism studies showed rapid neutrophil engraftment with delayed T cell engraftment. As of follow up at 12 months of age, he has appropriate post-transplant weight gain, no infectious complications, and no evidence of GVHD.Initial presentation in patients with CGD and liver/spleen abscesses can be insidious with isolated fever and elevated inflammatory markers. Such patients can readily meet criteria for atypical Kawasaki disease, highlighting the importance of maintaining a broad differential diagnosis for fever of unknown origin with a high index of suspicion for primary immunodeficiency.

Characterising approaches to steroid therapy in paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2.

We describe approaches to steroid therapy use in paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2 (PIMS-TS) and examine the association between steroid therapy and key clinical markers of severity. We conducted a retrospective review of children (<18 years) admitted to a tertiary paediatric hospital in the UK with PIMS-TS. We collected data on if and why steroid therapy was used; the duration, type and dosing of steroids prescribed; and approaches to hypothalamo-pituitary-adrenal (HPA) axis monitoring, if performed. We examined associations between steroid exposure/total steroid dose (mg/m2 /day) and paediatric intensive care unit admission, mechanical ventilation and inotropic support. Steroid therapy was commenced in most children (84.9%, n = 104) with a median total daily steroid dose (hydrocortisone equivalent) of 271.0 mg/m2 /day (interquartile range 232.5-355.5) and treatment length of 26.0 days (interquartile range 19.0-32.0). Dosing regimens predominantly involved a short course of high-dose methylprednisolone followed by tapering oral prednisolone. Basal and/or dynamic testing of the HPA axis was conducted in a minority (11.8%, n = 15) and was normal. Duration of steroid therapy correlated positively with durations of paediatric intensive care unit admission (r = 0.407, P < 0.001) and mechanical ventilation (r = 0.797, P < 0.001). A greater proportion of children receiving steroid therapy also received inotropic support compared to those that did not receive steroid therapy (71.4% vs. 45.5%, P = 0.025). Prolonged, high-dose steroid therapy is often used in the management of severe PIMS-TS with the potential for HPA axis suppression and should be withdrawn carefully.

A Rare Case of Thymoma-Associated Idiopathic Inflammatory Myopathy and Myasthenia Gravis (P1-5.009)

<h3>Objective:</h3> To describe the phenotype of and to support further research into the etiology and appropriate therapeutic management of thymoma-associated myasthenia gravis (MG)-inflammatory myopathy (IM) overlap syndrome. <h3>Background:</h3> While rare, the combination of MG and idiopathic IM has been previously documented. The prevalence of thymoma in patients with both IM and MG (70%) is significantly higher than in those with MG alone (10%). It has been postulated that thymoma-related immunopathogenic mechanisms, including dysregulation of the immune checkpoint pathway, may increase the risk of idiopathic IM and MG. <h3>Design/Methods:</h3> A 47-year-old male with a history of thymoma complicated by recent tumor recurrence presented to our tertiary referral center with rapid deterioration in bulbar and appendicular motor strength and a presumed diagnosis of amyotrophic lateral sclerosis. Prednisone, pyridostigmine, and IVIG previously provided no relief. Further workup was undertaken to elucidate the true etiology of his symptoms. <h3>Results:</h3> Electromyography/nerve conduction study was unremarkable. CSF studies revealed a normal protein and cell count. Creatine kinase was elevated at 2578 units/L. MRI of his cervical spine was remarkable for inflammatory changes along the myofascial planes of the posterior paraspinal muscles, concerning for myositis. Neural autoantibody testing, albeit after he had received IVIG, revealed multiple positive findings including acetylcholine receptor (AChR) blocking and modulating, smooth muscle, F-Actin, fibrillarin, and voltage-gated potassium channel antibodies. High dose methylprednisolone and plasmapheresis were initiated with marked clinical improvement. He has since been maintained on rituximab. On follow-up testing, continued positive antibodies include AChR binding, AChR blocking, and elevated striated muscle antibody. <h3>Conclusions:</h3> When presented with a patient with a history of recurrent thymoma and weakness of unclear etiology, MG-IM overlap syndrome should be considered in the differential diagnosis. Further study into the mechanism and appropriate management of this autoimmune dysregulation syndrome should be undertaken. <b>Disclosure:</b> Dr. Schmoll has nothing to disclose. Dr. Rodenbeck has nothing to disclose.

Diaphragmatic paralysis associated with IgLON5 autoimmunity (P5-5.022)

<h3>Objective:</h3> To describe a rare presentation of IgLON5 IgG mediated neurological disorder <h3>Background:</h3> IgLON5 autoimmunity was previously described with sleep disorder, rhombencephalopathy, vocal cord paresis, progressive-supranuclear-palsy-like syndrome, and recently motor-neuron-disease (MND)-like syndrome. Although diaphragmatic paralysis has been known as a presenting symptom of degenerative (MND) but has never been reported with IgLON5 autoimmunity. <h3>Design/Methods:</h3> Case report <h3>Results:</h3> A 67-year-old man with obstructive sleep apnea and history of vocal cord paralysis 11 years ago, presented with 2-year history of dream enactment behavior, progressive dyspnea and orthopnea. The symptoms became more noticeable and rapidly progressive in the past 10 months. Initially, he felt shortness of breath during the daytime when he bent over. Within a few months, he had to sleep on the recliner with a minimal tilt back at night. Neurological examination demonstrated moderate right facial weakness, mild weakness of interossei muscles of right hand, and triceps. Deep tendon reflexes were marginally brisk without Babinski sign. Sensory examination was normal. Occasional fasciculation was noted in deltoid region. Right upper extremity electromyography was normal. Diaphragmatic ultrasound showed no diaphragmatic expansion during inspiration. Ultrasound-guided electromyography of diaphragm and phrenic nerve conduction study showed no phrenic nerve response on the right, and markedly reduced amplitude of diaphragmatic compound motor action potential on the left side. Brain MRI and cervical spine was essentially unremarkable. Blood work showed normal vitamin B12, creatinine kinase and monoclonal gammopathy screen. Serological evaluation of neural specific antibodies was remarkable for IgLON5-IgG (titer 1:480). The patient was treated with high-dose methylprednisolone and is scheduled to follow up in autoimmune neurology clinic for outcome assessment. <h3>Conclusions:</h3> IgLON5 autoimmunity should be considered in patients with diaphragmatic paralysis, especially ones with sleep disorder, vocal cord paresis, and episodic respiratory disturbances. <b>Disclosure:</b> Dr. Thakolwiboon has nothing to disclose. Dr. Klein has a non-compensated relationship as a Klein with Neurology Journal that is relevant to AAN interests or activities. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. The institution of Dr. Dubey has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Astellas. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. Dr. Dubey has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for AGRIMS. Dr. Dubey has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Advances in Neurology . Dr. Dubey has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Moffit Cancer Center . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care.

Wernicke’s Encephalopathy Preceded by COVID-19 Infection: A Case Report (P2-7.006)

<h3>Objective:</h3> To describe a case of post-infectious encephalopathy following COVID-19 with MRI brain changes suggestive of Wernicke’s encephalopathy and a transient presence of anti-GAD65 autoantibodies in the cerebrospinal fluid (CSF). <h3>Background:</h3> Chronic malnutrition and alcoholism are known risk factors for developing Wernicke’s encephalopathy. However, it is not typically associated with thiamine deficiency resulting from acute starvation as in this case of severe respiratory syndrome due to coronavirus-2 (SARS-CoV-2). <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 59 year-old female presented to the neurointensive care unit with subacute disorientation, decreased verbal fluency, memory deficits, gait dysfunction, confusion, and rapid weight loss that progressed to coma over two months following COVID-19 infection. Initial lumbar puncture (LP) one month following infection was notable for positive anti-GAD65 autoantibodies and elevated protein which was suggestive of post-infectious encephalitis. MRI brain at the time of initial evaluation was unremarkable for abnormalities and the patient’s symptoms continued to progress to include depressed mentation. Subsequent MRIs of the brain with and without contrast showed T2 FLAIR abnormalities in the periaqueductal grey, lateral walls of the third ventricle, splenium, and mammillary bodies. Repeat LP showed similar CSF profile and absence of anti-GAD65 autoantibodies. Electroencephalogram showed slowing with frequent generalized periodic discharges. Patient’s treatment course included high dose thiamine, methylprednisolone, intravenous immunoglobulin, and plasmapheresis without clinical improvement. The patient did not show meaningful cognitive recovery and was transitioned to comfort care. <h3>Conclusions:</h3> The patient’s progression to coma was suspected to be due to development of COVID-19 encephalopathy precipitating Wernicke’s encephalopathy in the setting of severely diminished oral intake. Because Wernicke’s encephalopathy is potentially reversible with thiamine administration, we propose that early empiric treatment should be considered in all patients suffering from persistent encephalopathy following COVID-19 infection. <b>Disclosure:</b> Dr. Volski has nothing to disclose. Dr. Wang has nothing to disclose. Dr. Chakrabarti Pulakanti has nothing to disclose. Dr. Dolla has nothing to disclose.

Rapidly Progressive, Lethal Microangiopathy of the Brain: Two Cases of Malignant Susac Syndrome? (P9-5.015)

<h3>Objective:</h3> To present two fulminant cases of suspected Susac syndrome refractory to immunomodulatory therapy. <h3>Background:</h3> Susac syndrome (SS) is a presumed immune-mediated endotheliopathy characterized by a triad of encephalopathy, hearing loss, and branch retinal artery occlusions (BRAO). SS may vary in clinical presentation and course and yet typically responds to immunosuppressive therapy. Fatal cases of SS have been rarely reported. <h3>Design/Methods:</h3> NA <h3>Results:</h3> We describe two cases, 15 years apart, of suspected malignant SS. Our patients, a 36-year-old man and a 35-year-old woman presented with several symptoms including encephalopathy, hearing loss, and, in one case, blurred vision. Neither demonstrated BRAO. The first patient had a positive toxicology screen for methamphetamines. The second patient had a history of hypertension and morbid obesity. Serial MRIs demonstrated progressive scattered punctate foci of restricted diffusion throughout the brain, including the cerebrum, corpus callosum, brainstem, and cerebellum. An area of leptomeningeal enhancement was appreciated in our second patient. Extensive workup in both cases failed to yield an alternative diagnosis. Our patients were treated with high-dose intravenous methylprednisolone and intravenous immunoglobulin without improvement. The second patient also received intravenous cyclophosphamide, aspirin, clopidogrel, therapeutic heparin, and rituximab. Both declined in the hospital despite treatment and died shortly following withdrawal of life-sustaining treatments: the man on hospital day 25 and woman on hospital day 42. Autopsy in both cases demonstrated innumerable microscopic and lacunar infarcts of varying ages throughout the brain, interpreted as most consistent with SS. <h3>Conclusions:</h3> These cases highlight an uncommon aggressive variant of suspected SS leading to death within weeks of symptom onset despite intensive therapies. SS poses a diagnostic and treatment dilemma particularly in patients with a fulminant course. <b>Disclosure:</b> Dr. Tierney has nothing to disclose. Dr. Kazanci has nothing to disclose. Dr. Weinstein has received intellectual property interests from a discovery or technology relating to health care. The institution of Dr. Longstreth has received research support from NIH. Dr. Davis has nothing to disclose. Desiree A. Marshall, 431 has nothing to disclose. Dr. Yoda has nothing to disclose.

Neurological Manifestations of Human Monkeypox: A Systematic Review (P11-10.003)

<h3>Objective:</h3> To characterize the neurological manifestations and adverse events related to the monkeypox virus. In an effort to better inform clinicians on the rare but devastating neurological manifestations in human monkeypox. <h3>Background:</h3> The monkeypox virus (MPXV) belongs to the family of orthopoxviruses. MPXV is the causative agent of human monkeypox. Traditionally human monkeypox has been confined to the African continent however a growing number of monkeypox cases have been reported by the Centers of Disease Control (CDC) starting in May 2022. This systematic review aims to characterize the neurological manifestation of human monkeypox. <h3>Design/Methods:</h3> A systematic review was conducted across three databases PubMed/PubMedCentral/MEDLINE, ScienceDirect, and Scopus. The review was conducted in accordance with PRISMA guidelines and was prospectively registered on PROSPERO (Registration ID: CRD42022336088). <h3>Results:</h3> A total of 8 cases are identified describing neurological manifestations secondary to human monkeypox. Five cases of encephalitis are identified and one of conjunctivitis leading to severe vision impairment. Notably, during the current 2022 outbreak two cases of encephalomyelitis have been identified within the United States. Cases involving children are believed to be of greater severity and two deaths were identified. Most cases were managed with high-dose intravenous methylprednisolone and immunomodulators such as IVIG. <h3>Conclusions:</h3> Although rare, neurological manifestations and secondary neuroinflammatory conditions are possible secondary to human monkeypox. Cases in children are likely to carry greater severity with increased morbidity and mortality. As cases continue to emerge through the 2022 global outbreak clinicians must remain vigilant in monitoring for neurological complications from the monkeypox virus. <b>Disclosure:</b> Dr. Srichawla has nothing to disclose. Dr. Garcia-Dominguez has nothing to disclose.

Cerebral amyloid angiopathy-related inflammation (CAARI) and HHV-6 positivity: A case report of a treatment dilemma and a possible relationship (P1-12.003)

<h3>Objective:</h3> To examine the treatment dilemma utilizing high-dose steroids in a patient with cerebral amyloid angiopathy-related inflammation (CAARI) and HHV-6 positivity. Additionally, to question a possible relationship between HHV-6 and CAARI. <h3>Background:</h3> Cerebral amyloid angiopathy-related inflammation (CAARI) has been described as a distinct syndrome with subacute cognitive decline or seizure symptoms. Previous studies have established diagnostic criteria for this entity and proposed a treatment strategy. However, the precipitating factors behind developing CAARI in a subset of patients with CAA are unclear. We present a case of CAARI in a patient with no underlying diagnosis of dementia that was found to be positive for HHV-6 in serum and CSF and treated successfully with steroids. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> An 83-year-old woman presented with a first-time generalized tonic-clonic seizure with postictal confusion. On presentation, her systolic blood pressure was elevated at 150–170, and she was tachycardic to 160 with a temperature of 38.4. A contrasted MRI brain showed multifocal vasogenic edema, sulcal FLAIR hyperintensity, and innumerable lobar microhemorrhages. Black blood contrast MRI brain angiogram was unremarkable, with no evidence of vasculitis. At this point, the patient had clinically improved but remained slightly encephalopathic with word-finding difficulties. Continuous EEG monitoring showed diffuse background slowing. Initial CSF studies revealed an isolated mildly elevated protein of 49 and negative cytology. Therefore, based on the clinical and radiographic evidence, a decision was made to undergo five days of high-dose methylprednisolone for probable CAARI. However, days later, HHV-6 in CSF resulted positive with 249 copies/ml, and a serum sample showed 1100 copies/ml. <h3>Conclusions:</h3> The concurrence of cerebral amyloid angiopathy-related inflammation (CAARI) and HHV-6 in serum and CSF might yield a diagnostic and therapeutic challenge. However, the treatment with high-dose pulsatile steroids with taper did improve the overall clinical picture. Further studies are needed to investigate a potential correlation. <b>Disclosure:</b> Dr. Alshawa has nothing to disclose. Dr. Makkieh has nothing to disclose. Miss Alsakka has nothing to disclose. Dr. Torres-Melendez has nothing to disclose.

Delayed reversal of methanol-induced blindness in an adult by a combination of erythropoietin and a high dose of methylprednisolone: a case report

BackgroundThe clinical manifestation following methanol toxicity accounts for a life-threatening problem that contributes to metabolic disorders, neurological complications, blindness, and even death. There is no completely effective treatment to retain the patient’s vision. Herein, we apply a new therapeutic strategy for the recovery of bilateral blindness in a patient who had ingested methanol.Case presentationA 27-year-old Iranian man with complete bilateral blindness was referred 3 days after accidental ingestion of methanol to the poisoning center at Jalil Hospital, Yasuj, Iran, in 2022. After taking his medical history, performing neurologic and ophthalmologic examinations, and routine laboratory tests, ordinary management was undertaken and counterpoisons were given for 4–5 days; however, the blindness did not reverse. Following the 4–5 days of unsuccessful standard management, he was given ten doses of subcutaneous erythropoietin 10,000 IU/12 hours twice daily, folinic acid 50 mg/12 hours, and methylprednisolone 250 mg/6 hours for 5 days. After five days, vision of both eyes recovered, reaching 1/10 in the left and 7/10 in the right eye. He remained under daily supervision until his release from the hospital, and he was discharged from the hospital 15 days post admission. In outpatient follow-up, his visual acuity was improved without having any side effects at 2 weeks after discharge.ConclusionA combination of erythropoietin and a high dose of methylprednisolone were useful for relieving the critical optic neuropathy and improved the optical neurological disorder following methanol toxicity.

Teaching NeuroImages: Cortical damage with leptomeningeal enhancement in neuromyelitis optica spectrum disorder

A 27-year-old woman presented with headache, weakness, and numbness in both lower extremities for 2 weeks. Two years ago, she had refractory hiccups and vomiting with associated area postrema lesions on MRI (figure, A–C). Workup for infectious disease was negative. Serum aquaporin-4–immunoglobulin G was positive. Recent MRI showed cortical damage adjacent to the cerebral falx with leptomeningeal enhancement (figure, D–F). Treatment with immunoglobulin and high-dose methylprednisolone produced a significant improvement of the symptoms and the follow-up MRI (figure, G–I). This patient's case is unusual, as neuromyelitis optica spectrum disorders rarely involve the cortex and pia mater.1,2

Systemic lupus erythematosus with diffuse alveolar hemorrhage.

Systemic lupus erythematosus with diffuse alveolar hemorrhage.