Course Of Intravenous Methylprednisolone Research Articles

Abstract 408: Spontaneous intracerebral hemorrhage in a patient with multiple sclerosis : A case of vascular complication of a demyelinating disorder

Introduction Intracerebral hemorrhage [ICH] accounts for only 6.5% to 19.6% of strokes, yet with a 40% to 50% 30‐day mortality rate [twice that of ischemic strokes]. It presents as acute onset focal neurological deficits that localize to the area of hemorrhage. Most common causes of spontaneous ICH include hypertension [HTN] and cerebral amyloid angiopathy [CAA]. Multiple sclerosis [MS] is the most prevalent non‐traumatic disabling demyelinating disease that affects young adults. Most commonly, MS presents with optic neuritis or syndromes involving the brainstem or spinal cord. This case aims to bolster the assertion that demyelinating lesions “set the stage” for ICH through a variety of pathophysiologic mechanisms. Methods [Case Presentation] A 40 year old woman with a history of chronic well controlled HTN without medications, hypothyroidism, active tobacco use, not on anticoagulation or antiplatelet agents was hospitalized for progressively worsening blurry vision of her left eye. She underwent MRI brain/orbits +/‐ contrast and lumbar puncture and was diagnosed with MS per McDonald's criteria. She completed a five day course of intravenous methylprednisolone. Six days into her hospital stay, just after completing the steroid course, she developed sudden onset of altered mental status and left sided hemiparesis. Imaging revealed a right frontal intraparenchymal hematoma measuring 3.7 times 5.4 times 4.1 cm with surrounding edema causing effacement of the right lateral ventricle and a 5 mm leftward midline shift. Patient required emergent intubation and decompressive craniectomy. She showed no evidence of elevated blood pressures prior to ICH [systolic blood pressure range 95‐146], hypercoagulability or other autoimmune/rheumatological disorders. Digital subtraction angiography did not reveal any vascular malformations. No clear etiology of ICH could be determined and she was discharged to inpatient rehabilitation unit. Conclusion This case represents a rare instance of spontaneous ICH in MS patients, in which the pathophysiologic processes are not yet known. One potential hypothesis is the rupture of ‘cryptic’ AVMs that remain undetected on angiography. These aneurysms are prone to rupture in the setting of active ongoing inflammation from demyelination during MS exacerbations. Another possibility is localized vascular inflammation rendering damage to vessel wall structures. A large retrospective cohort study has also shown that age, smoking and anticoagulant use increase the risk of ICH in MS patients. One paper posits that demyelinating lesions set the stage for focal hemorrhage through various mechanisms, including blood brain barrier breakdown at the location of the demyelinating plaques, as well as increased endothelial fragility, transient thrombocytopenia, close proximity of the lesion to blood vessels, and increased fibrinolytic activity. Though theoretical, this may explain the rare hemorrhagic complications in MS patients. While there have been reports of hemorrhagic lesions in tumefactive MS, our case did not reveal any lesions suggestive of the same. The association between ICH and MS remains unclear, and larger studies are needed to investigate such hemorrhagic complications of MS.

Prognostic factors for thyroid-stimulating immunoglobulin normalization in moderate-to-severe Graves’ orbitopathy: a 36-month longitudinal study

PurposeThe primary objective of this study was to identify predictive factors linked to the normalization of thyroid-stimulating immunoglobulin (TSI) levels in patients diagnosed with active, moderate-to-severe Graves’ orbitopathy (GO). The study also tracked the longitudinal changes in TSI levels over a 36-month period following treatment.MethodsThe study population consisted of individuals who were recently diagnosed with active, moderate-to-severe GO and received a 12-week course of intravenous methylprednisolone (IVMP) treatment. A subgroup of patients who did not respond to the initial treatment received an additional 20 Gy of radiation therapy (RTx). TSI levels were monitored at the time of diagnosis, after treatment, and subsequently every 6 months for 36 months. Normalization was defined as a TSI level below 140%. Patients were divdied into two groups with success and failure group depending on whether TSI became normal or not.ResultsOut of 83 patients, 36 (43.4%) achieved normalized TSI levels within two years post-IVMP treatment. Lower initial TSI levels (< 425%), absence of additional RTx, and early treatment initiation were associated with a higher likelihood of TSI normalization (P = 0.035, P = 0.028, P < 0.001, respectively). Notably, significant differences in TSI level reduction were observed from 18 months post-treatment between the two groups (P = 0.031). A TSI cutoff value of 413% was identified as predictive for normalization at 24 months (P = 0.002).ConclusionThis study is the first to identify key factors that influence normalization of TSI levels in moderate-to-severe Graves’ Orbitopathy. It highlights the importance of early treatment decisions, particularly for patients with initial TSI levels above 425%. Despite the treatment, less than half of the patients achieved TSI normalization within 24 months, underscoring the need for additional research to explore the relationship between TSI levels and the clinical manifestations of chronic GO.

Epidemiology of thyroid-stimulating immunoglobulin in recent-onset symptomatic thyroid eye disease.

This study aims to report correlations between thyroid-stimulating immunoglobulin (TSI) and both clinical and radiological parameters in recent-onset symptomatic thyroid eye disease (TED) patients. A prospective cohort study of TED patients managed at the Chinese University of Hong Kong from January 2014 to May 2022. Serum TSI levels were determined with the functional assay. Outcomes included the Clinical Activity Score (CAS), marginal reflex distance1 (MRD1), extraocular muscle motility restriction (EOMy), exophthalmos, and diplopia. The radiological assessment included cross-sectional areas and signal of extraocular muscles on STIR-sequence MRI. A total of 255 (197 female) treatment-naive patients, with an average onset age of 50 ± 14 years (mean ± s.d.), were included. Elevated pre-treatment TSI level was observed in 223 (88%) patients. There was a weak positive correlation between TSI and CAS (r = 0.28, P = 0.000031), MRD1 (r = 0.17, P = 0.0080), and the size of the levator palpebrae superioris/superior rectus complex (r = 0.25, P = 0.018). No significant correlation existed between TSI and STIR signals. The AUC and optimal cut-off value for clinical active TED were 0.67 (95% CI: 0.60-0.75) and 284% (specificity: 50%, sensitivity: 85%). In total, 64 patients received intravenous methylprednisolone (IVMP) during the study interval, and they had a higher baseline TSI level than those who did not have IVMP (P = 0.000044). Serial post-IVMP TSI among the 62 patients showed a significant reduction compared to the baseline level (P < 0.001). Both the baseline and post-IVMP TSI levels, and percentages of TSI changes were comparable between patients who responded and did not respond to the first course of IVMP. TSI can be a serum biomarker for the diagnosis, prognosis, and treatment response of TED. Further validation should be warranted.

Tubercular Meningitis Mimicking Acute Disseminated Encephalomyelitis (ADEM)- A Case Report

Tuberculosis remains endemic in India, and its manifestation in the central nervous system (CNS) poses significant challenges in diagnosis and management. We present a rare case of a 65-year-old male with tubercular meningitis exhibiting atypical features of acute disseminated encephalomyelitis (ADEM). The patient presented with a month-long history of fever, severe headache, altered sensorium, and neurological signs including brisk reflexes, neck rigidity, and bilateral motor weakness. Initial investigations revealed elevated inflammatory markers and abnormal cerebrospinal fluid (CSF) findings consistent with tubercular meningitis. Despite negative CSF culture and nucleic acid amplification tests (NAATs) for Mycobacterium tuberculosis, the patient displayed characteristic asymmetrical bilateral cerebral lesions on MRI suggestive of ADEM. Given the diagnostic ambiguity and a high suspicion of CNS tuberculosis with concurrent ADEM, the patient was treated with anti-tubercular therapy (ATT) and a course of intravenous methylprednisolone followed by oral prednisolone. Subsequent clinical improvement prompted gradual tapering of prednisolone, leading to a full recovery at discharge. This case highlights the complexity of diagnosing CNS tuberculosis coexisting with ADEM, emphasizing the importance of a comprehensive clinical assessment and tailored therapeutic interventions in managing such intricate neurological presentations.

Pembrolizumab-associated Neuronopathy with Improved Lower Limb Somatosensory Evoked Potential After Intravenous Methylprednisolone Treatment: A Case Report.

Cases of neuronopathy associated with immune checkpoint inhibitors (ICIs) have rarely been reported. We herein report a case of ICI-associated neuronopathy. A 54-year-old man underwent chemotherapy for right maxillary sinus cancer. Two months after pembrolizumab treatment, diarrhea, worsening of abnormal sensations, and severe ataxia of the lower limbs were observed. Somatosensory evoked potentials (SEPs) with tibial nerve stimulation showed disappearance of the N21 waveform. A colonic biopsy suggested ICI-associated colitis. Based on these findings, the patient was diagnosed with ICI-associated neuronopathy. Clinical symptoms and SEP findings improved markedly after two courses of intravenous methylprednisolone.

Elevated serum thyroid stimulating immunoglobulin linked to failure of first-line intravenous methylprednisolone monotherapy in moderate-to-severe thyroid eye disease.

To assess factors associated with failure of intravenous methylprednisolone (IVMP) monotherapy as the first-line treatment for thyroid eye disease (TED) and to identify patients who might benefit from supplementing mycophenolate mofetil (MMF) to IVMP. Data for all patients with TED treated with IVMP according to the EUGOGO protocol in our center between 2016-2021 were retrospectively analysed. Forty-seven patients (mean age 51.32 ± 14 years, 27 females) were enrolled. The mean time from first reported symptoms to first IVMP treatment was 12.1 ± 5.59 months (range 0-120). The mean clinical activity score (CAS) before treatment and at a mean of 5 and 12.2 weeks after treatment initiation was 6.00, 2.96, and 1.81, respectively (P < 0.01). Twenty-one patients (44.68%) were recommended second-line treatment: nine due to no response or worsening of CAS, six due to partial response, four with good response but early relapse after completion of treatment, and one due to late relapse. Eighteen of those 21 patients received second-line treatment which included rituximab (n = 7), MMF (n = 6), a second course of IVMP (n = 4), and tocilizumab (n = 1). Serum thyroid-stimulating immunoglobulin (TSI) levels were higher in patients who received second-line treatment compared with patients who responded well to first-line IVMP monotherapy at presentation (2135% vs 1159%, P = 0.05) and after completion of first-line treatment (2201% vs. 986%, P = 0.043). TED patients requiring second-line treatment after failed IVMP monotherapy had higher baseline and post-first-line treatment serum TSI levels. Those with elevated TSI may benefit from dual therapy (IVMP and MMF) and require closer monitoring.

Treatment-Refractory Autoimmune Glial Fibrillary Acidic Protein Meningoencephalomyelitis in a Young Adult Female

Objective To describe a case of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy refractory to immunotherapy without evidence of malignancy or coexisting autoimmunity. Background Autoimmune GFAP astrocytopathy is an autoimmune disease of the central nervous system associated with the presence of GFAP-IgG in the CSF. Patients may present with acute or subacute onset of headache, encephalopathy, seizures, abnormal vision, weakness or numbness, postural tremor and cerebellar ataxia. GFAP astrocytopathy is usually corticocorticosteroid-responsive in the acute setting but may rarely require maintenance immunotherapy to prevent relapse. Treatment refractory cases should trigger work up for coexisting autoimmunity or malignancy. Design/Methods A 35-year-old female patient presented with subacute meningoencephalomyelitis with prodromal symptoms. Results Her cerebrospinal fluid revealed lymphocytic pleocytosis and elevated protein. Brain magnetic resonance imaging (MRI) with and without contrast showed perivascular radial enhancement and periventricular T2 FLAIR hyperintensity. Spinal MRI with and without contrast demonstrated longitudinal T2 FLAIR hyperintensity from T1-T2 to T7-T8. Despite high dose steroid treatment, her disease progressed with an enlarging periventricular lesion and worsening visual acuity. Biopsy of the enhancing periventricular lesion showed perivascular inflammation. After five cycles of plasma exchange along with a five-day course of intravenous methylprednisolone 1 gram daily, her symptoms stabilized. The CSF autoimmune encephalopathy panel (Mayo Clinic Laboratories) came back positive for GFAP-IgG antibody on tissue immunofluorescence assay, and was confirmed positive by GFAP cell-based assay. No neoplastic disease was identified using high resolution PET/CT scans. Based on the aggressiveness of her disease, she received one cycle of cyclophosphamide, and was discharged home on an oral corticosteroid taper. Even one year after addition of both mycophenolate mofetil and rituximab, MRI imaging continued to reveal new enhancing lesions. Conclusions Autoimmune GFAP astrocytopathy may sometimes require long-term immunosuppression even without presence of malignancy or other coexisting autoimmune disease.

A case of natalizumab‐associated progressive multifocal leukoencephalopathy followed by immune reconstitution inflammatory syndrome with difficulty in the timing of immunotherapy

AbstractBackgroundDetails regarding the clinical course of natalizumab‐associated progressive multifocal leukoencephalopathy (NAT‐PML) have not been reported in Japanese patients. We experienced a Japanese NAT‐PML case and report it for the purpose of clarifying the challenge it posed in medical treatment.Case PresentationHerein, we describe a 58‐y‐old multiple sclerosis patient who had NAT‐PML with immune reconstitution inflammatory syndrome (IRIS). Before NAT‐PML developed, the patient's Expanded Disability Status Scale (EDSS) score was 5.5. She received natalizumab (300 mg) every 3–7 w, and her EDSS score had not changed for 9.1 y. The John Cunningham virus (JCV) index was 0.43 before NAT‐PML developed. She developed a gait disturbance when NAT‐PML emerged. Natalizumab was administered a total of 108 times. The patient was diagnosed with probable NAT‐PML on the basis of punctate lesions on T2‐weighted imaging and a JCV‐PCR result of 29 copies/ml from cerebrospinal fluid (CSF) testing. Intravenous methylprednisolone (IVMP) was initiated when the contrast‐enhanced lesion was first detected, but the NAT‐PML lesion was rather enlarged despite the temporary disappearance of the contrast‐enhanced lesion. An obvious increase in the IgG index and a slight increase in the CSF cell count were recognized at the time the immunological response was activated. Three cycles of a 3‐d course of IVMP were administered every 2 w for IRIS, and the patient's worst EDSS score was 9.5.ConclusionTreatment sequencing should be executed before the onset of NAT‐PML. Changes in CSF cell count and IgG index may be useful for treatment decision; further research is needed.

54-Year-Old Man With Acute Dyspnea on Exertion

54-Year-Old Man With Acute Dyspnea on Exertion

Efficacy of Corticosteroid Therapy for HTLV-1-Associated Myelopathy: A Randomized Controlled Trial (HAMLET-P).

Corticosteroids are most commonly used to treat HTLV-1-associated myelopathy (HAM); however, their clinical efficacy has not been tested in randomized clinical trials. This randomized controlled trial included 8 and 30 HAM patients with rapidly and slowly progressing walking disabilities, respectively. Rapid progressors were assigned (1:1) to receive or not receive a 3-day course of intravenous methylprednisolone in addition to oral prednisolone therapy. Meanwhile, slow progressors were assigned (1:1) to receive oral prednisolone or placebo. The primary outcomes were a composite of ≥1-grade improvement in the Osame Motor Disability Score or ≥30% improvement in the 10 m walking time (10 mWT) at week 2 for rapid progressors and changes from baseline in 10 mWT at week 24 for slow progressors. In the rapid progressor trial, all four patients with but only one of four without intravenous methylprednisolone achieved the primary outcome (p = 0.14). In the slow progressor trial, the median changes in 10 mWT were −13.8% (95% CI: −20.1–−7.1; p < 0.001) and −6.0% (95% CI: −12.8–1.3; p = 0.10) with prednisolone and placebo, respectively (p for between-group difference = 0.12). Whereas statistical significance was not reached for the primary endpoints, the overall data indicated the benefit of corticosteroid therapy. (Registration number: UMIN000023798, UMIN000024085)

COVID-19 and Basedow disease

Coronavirus disease-2019 (COVID-19) heavily hits the human body through various and dramatic ways, and endocrine system is not an exception. Our purpose is to update the concepts around subjects diagnosed with Basedow disease (BD) before, after or during experiencing COVID-19 infection. This is a literature review based on full-length, English papers that are available through PubMed (published between 2020 and 2021). Angiotensin converting enzyme 2 and transmembrane protease serine 2 receptors are expressed at thyroid level and this might explain why an active coronavirus infection activates the BD especially in patients with a potential predisposition. Also, the new diagnostic of BD might follow a COVID-19 infection (within weeks). Simultaneously dealing with both conditions requires a more challenging multidisciplinary management, while the diagnostic of thyroid condition after recovery from infection is more likely to follow the general pattern of evolution (as seen in non-COVID-19 cases). A previous diagnostic of autoimmune hyperthyroidism means mostly either: BD is remitted after prior medication treatment with anti-thyroid drugs and/or radioiodine therapy (normal thyroid function), the patient has iatrogenic hypothyroidism that was induced after thyroidectomy or after radioiodine therapy (requiring daily oral levothyroxine substitution) or the subject is under thiamazol (or similar drugs) with either controlled or uncontrolled thyroid function. Most of the clinical studies agree (but not all) that people with treated hypothyroidism and hyperthyroidism are not susceptible to a higher morbidity or mortality concerning coronavirus infection. One exception is concurrent medication with anti-thyroid drugs with a higher risk of agranulocytosis which is a prone condition to any kind of infection. Graves’ ophthalmopathy may be synchronous or not with an active thyroid disease. Except for mild forms, typically the condition requires glucocorticoid therapy, preferably a short course of intravenous methylprednisolone which exposes the patient to a higher risk of an infection, including COVID-19. Recently, BD was suspected to be induced or aggravated by COVID-19 vaccination which is still a matter of discussion.

Cerebral Toxoplasmosis in Systemic Lupus Erythematosus.

A 37-year-old man with underlying systemic lupus erythematosus and lupus nephritis presented with an episode of generalized tonic seizure. He complained of poor concentration and forgetfulness for 1 week. He suffered a relapse of lupus nephritis 4 months ago and received a course of intravenous methylprednisolone followed by oral prednisolone and mycophenolate mofetil. Clinically, there was no focal neurological deficit. Retroviral screening was negative. Magnetic resonance imaging (MRI) of the brain revealed eccentric and concentric signs which confirmed the diagnosis of cerebral toxoplasmosis.

Steroid-Responsive Encephalopathy Associated with Thyroiditis

steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is an autoimmune entity with a strong association with elevated antithyroid antibodies. It is a rare cause of encephalopathy and is usually a diagnosis of exclusion. Responsive to corticosteroids is required to make the diagnosis. Herein, we report a male patient presented with recurrent convulsive episodes not controlled well by anticonvulsant drugs and had drops in Glasgow coma scale (GCS). After unremarkable of extensive investigations, Hashimoto's encephalitis was suspected and antithyroid peroxidase antibodies test turned out to be positive, while thyroid function tests were normal and the diagnosis of steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) was made. He received a course of intravenous methylprednisolone for 5 days and responded very well to therapy, with an improvement of his GCS to 15/15.

Isolated opsoclonus heralding neuromyelitis optica spectrum disorder

Opsoclonus is an ocular motility disorder characterized by spontaneous, arrhythmic conjugate saccades of varying amplitude occurring in all directions of gaze without normal intersaccadic interval. Etiological spectrum of opsoclonus encompasses paraneoplastic and neoplastic conditions, infectious and para-infectious encephalitis, autoimmune, metabolic and toxic encephalopathies, drugs, motor neuron diseases, multiple sclerosis and rarely neuromyelitis optica spectrum disorder (NMOSD). Opsoclonus has never been reported as a presenting manifestation heralding NMOSD. We herein report a previously healthy 37-year-old Asian Indian woman who presented with oscillopsia and opsoclonus, followed, 12 h later, by right-sided hemiparesis, right-sided appendicular ataxia, and left-sided lower motor neuron type facial palsy and dysarthria. Brain magnetic resonance imaging revealed hyperintense lesions in brainstem and thalamus in T2-weighted and fluid attenuated inversion recovery-weighted images, quite suggestive of NMOSD. Serum and cerebrospinal fluid samples were positive for anti-aquaporin-4 antibodies, which clinched the diagnosis of seropositive NMOSD. After completion of a course of intravenous methylprednisolone 1 g/day for 5 days, her opsoclonus disappeared completely. There was significant improvement in her speech and weakness within the first week of therapy and no objective deficit after day 20 of admission. After one-and-a-half-year follow-up, the patient was maintaining well on rituximab as secondary prophylaxis without any further attack. Our case highlights that isolated opsoclonus can be the presenting feature of NMOSD.

Multiple sclerosis with intractable vomiting and atypical area postrema lesion

BackgroundArea postrema syndrome is considered as one of the most typical presentations of neuromyelitis optica spectrum disorders (NMOSDs) (Wingerchuk et al., 2015). The involvement of area postrema is rarely seen in multiple sclerosis (MS). We are here report a case of a young woman with multiple sclerosis, presented with intractable vomiting, and her MRI brain showed acute T2 hyperintense signal over the area postrema. Case presentationA 36-year-old Asian woman who is known to have schizophrenia and multiple sclerosis since 2012. She was noted to have spastic gait, and the MRI brain in 2012 showed multiple perpendicular periventricular T2 lesions suggestive of multiple sclerosis (MS). However, she defaulted her neurologist follow-up and was not on any treatment for MS. She was admitted in 2016 with intractable vomiting, and her MRI brain showed T2 hyperintense signal over area postrema with focal contrast enhancement. Her MRI cervical spine was normal. The visual evoked potential study showed bilateral prolonged P100 latencies. Oligoclonal bands were detected in her CSF analysis. Both the serum aquaporin-4 IgG (AQP4 IgGs) antibody and myelin oligodendrocyte glycoprotein (MOG-IgGs) were negative. Her intractable vomiting resolved after a short course of intravenous methylprednisolone. She was treated as MS with interferon-beta 1a. She has been in remission since 2016, and her functional status also improved from the expanded disability status scale (EDSS) of 2.0 (in 2016) to 1.0 (in 2020). ConclusionWe proposed that although area postrema lesion is typically seen in NMOSDs, it may also be seen in MS. Current MRI criteria for MS and NMOSDs are not sufficiently specific, and the diagnostic criteria should only be used in the appropriate clinical context.

Leber Hereditary Optic Neuropathy in a Mother and Daughter Associated With m.10197G>A Mutation.

We read with great interest the letter by Jorstad et al (1) describing a 16-year-old boy with Leber hereditary optic neuropathy (LHON) harboring a mitochondrial DNA 10663T>C point mutation and his favorable response to idebenone treatment. We also recently encountered a patient with LHON who had an unusual mitochondrial DNA mutation and experienced marked improvement with idebenone. A 48-year-old woman in excellent health was referred to our neuro-ophthalmology clinic with subacute vision loss in her left eye 2 months previously, followed by right eye involvement 2 weeks later. Her mother had lost vision in a similar manner at a similar age. The patient's visual acuity was 20/50 in the right eye and 20/60 in the left eye. Visual fields demonstrated a central scotoma in each eye, optical coherence tomography showed temporal thinning of the retinal nerve fiber layer bilaterally, and both optic discs were pale temporally. Testing for causes of bilateral optic neuropathy, including MRI of brain and orbits, was unrevealing. Despite a 3-day course of intravenous methylprednisolone (500 mg twice a day), the patient's acuity declined to 20/200 in the right eye and 20/800 in the left eye. Although studies were pending for mitochondrial DNA analysis, the patient was prescribed idebenone (300 mg 3 times a day) and advised to avoid smoking and alcohol. Although testing was negative for the 3 most common LHON mutations, complete mitochondrial DNA sequencing was positive for m.10197G>A mutation, with a heteroplasmy level ranging from 10% to 30%. It was also positive for another mutation (c.116T>G) of unknown clinical significance. Over the next 4 months, the patient's visual acuity improved to 20/25 in the right eye and 20/30 in the left eye. The central scotomas in each eye became less dense. The patient's mother was 70 years old with a history of subacute bilateral visual loss at the age of 58 years. When examined, her acuity was counting fingers in each eye with bilateral cecocentral scotomas and pale optic discs. At the time of her visual loss, her workup included hematologic studies and brain MRI. She, too, did not respond to initial treatment with intravenous corticosteroids. The patient's mother tested negative for the 3 common LHON mutations but declined complete mitochondrial DNA sequencing. The m.10197G>A mutation is a confirmed pathogenic mitochondrial mutation in both the homoplasmic and heteroplasmic forms (2). It changes a hydrophobic alanine residue into a hydrophilic threonine in a highly conserved part of the MT-ND3 of the respiratory chain complex. This mutation has been reported to cause Leigh syndrome and dystonia (3), dystonia and mitochondrial encephalomyelopathy with lactic acidosis and stroke-like episodes/Leigh syndrome overlap syndrome (4,5). In addition, Wang et al (6) reported 18 patients (9 male; 9 female) with LHON and dystonia in one Chinese family with the m.10197G>A mutation. Ten patients had isolated optic neuropathy without dystonia. Huang et al (7) described a 23-year-old man with isolated LHON who harbored the m.10197G>A mutation. Our patient's presentation was atypical of LHON for a number of reasons: female sex, older age, and the m.10197G>A DNA mutation. Her visual recovery may have occurred spontaneously, although other reports of patients with this mutation have not documented a favorable visual outcome. It seems that idebenone treatment was effective in our patient and in the patient described by Jorstad et al despite having different DNA mutations.

Pontine hemorrhage accompanied by neuromyelitis optica spectrum disorder

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disorder caused by antibody to aquaporin-4 (AQP4). NMOSD can infrequently present as a complication of posterior reversible encephalopathy syndrome (PRES). Moreover, few cases of NMOSD patients with brain hemorrhage have been reported. We report a rare case of PRES together with NMOSD recurrence, subsequent to pontine hemorrhage after intravenous methylprednisolone (IVMP) therapy. A 51-year-old Japanese woman, with a history of hypertension and dyslipidemia, and recurrent episodes of left visual acuity disorder related to AQP4-positive NMOSD, developed blindness in the left eye. Brain MRI showed a hyperintense lesion in pons. She was initially diagnosed with recurrence of NMOSD and 1000 mg of IVMP was administered for 3 days. After the 3rd course of IVMP, she developed left-sided sensory disturbance, and blood pressure was increased to 202/127 mmHg. Brain computed tomography (CT) showed pontine hemorrhage, and she was referred to our hospital again. We diagnosed PRES associated with NMOSD recurrence, along with development of pontine hemorrhage induced by the increase in blood pressure resulting from IVMP. The patient was treated with nicardipine to strictly control blood pressure, and tranexamic acid and glycerol for pontine hemorrhage and PRES. We also extended IVMP for 5 consecutive days in total, followed by plasmapheresis. After therapy, blindness in the left eye improved to light perception. Collectively, anti-AQP4 antibody could induce PRES together with recurrent NMOSD, and pontine hemorrhage could thus be induced by blood pressure increases resulting from IVMP.

Corticosteroids in the Management of Severe Coccidioidomycosis

BackgroundThere is limited data suggesting that recovery from severe pulmonary infection with Coccidioides may be hastened by the addition of systemic corticosteroids. MethodsWe present a case report of 2 patients with persistent and progressive coccidioidomycosis who demonstrated a dramatic response to adjunctive corticosteroid therapy. ResultsBoth patients had Coccidioides immitis cultured from respiratory samples. One was a 69-year-old man who had been treated with combination fluconazole and liposomal amphotericin for over 6 weeks, with persistent fever and pneumonia. The other was a 61-year-old man treated with fluconazole and then amphotericin for 3 weeks, with progression to acute respiratory distress syndrome and shock. Both received short courses of intravenous methylprednisolone and recovered to be discharged home. ConclusionsAs opposed to associated hypersensitivity, corticosteroid treatment in these cases was directed at modulating the ongoing destructive effects of unchecked inflammation. Rapid improvement was noted in both cases and raises the possibility that the addition of systemic corticosteroids may hasten recovery in patients with severe coccidioidomycosis.

29-Year-Old Woman With Fever and Bilateral Lower Extremity Lesions

29-Year-Old Woman With Fever and Bilateral Lower Extremity Lesions

Lethal high: acute disseminated encephalomyelitis (ADEM) triggered by toxic effect of synthetic cannabinoid black mamba

A previously well 25-year-old man presented with agitation, double incontinence and left-sided incoordination. His symptoms started after smoking a synthetic cannabinoid (black mamba) 5 days earlier. Over 48 hours, he...