Overlapping syndrome mimicking infectious meningoencephalitis in a patient with MOG and GFAP IgG

Abstract Background The presence of CNS overlapping autoimmune syndrome is not uncommon, but only one case of overlapping syndrome with coexistence of MOG-IgG and GFAP-IgG had been reported. This is the first reported case of these double antibodies positive presenting as clinical meningoencephalitis. Case presentation: A 23-year-old woman presented with transient convulsions,loss of consciousness, persistent fever, headache and vomiting. The cerebrospinal fluid (CSF) analysis revealed elevated cellularity, Magnetic resonance imaging (MRI) showed diffuse leptomeningeal enhancement. She remained fever and headache with antiviral and antibiotic treatment for two weeks, then was treated with empirical anti-tuberculosis treatment and oral prednisolone therapy. She followed up at 3 months from presentation with symptoms improved and normal CSF analysis. 3-month follow-up MRI performed asymmetric lesions in the cerebellum, corona radiata, and white matter with enhancement. Anti-tuberculosis treatment was continued and steroid was discontinued. After She stopped taking the prednisolone, interrupted headache gradually appeared. MRI at 4 months after presentation revealed partial reduced extent of lesions, but enlarged areas in left cerebellum and right parietal white matter, as well as a new lesion in the region of the right ependyma with linearly enhancement. Screening for anti-myelin oligodendrocyte glycoprotein (MOG) antibody and anti-glial fibrillary acidic protein (GFAP) antibody were positive in CSF by transfected cell-based assay. She was diagnosed with overlapping syndrome of MOG‑IgG‑associated disease and GFAP astrocytopathy and received steroid pulse therapy (methylprednisolone 1 g for 5 days) followed by a gradual tapering of oral prednisolone, as well as addition of immunosuppressant (tacrolimus, 3 mg per day). 6 months after the patient’s initial presentation, no symptom was found, MRI showed the lesions had obviously diminished and no enhancement was found. Conclusions To our knowledge, this is the first reported case of overlapping syndrome with coexistence of MOG-IgG and GFAP-IgG presenting as clinical meningoencephalitis. The early screening of autoantibodies against CNS antigens was of great importance for the patient suspected of intracranial infection to make the definite diagnosis.

Overlapping syndrome mimicking infectious meningoencephalitis in a patient with MOG and GFAP IgG

BMC Neurology ◽

10.1186/s12883-021-02381-8 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Suqiong Ji ◽

Chenchen Liu ◽

Zhuajin Bi ◽

Huajie Gao ◽

Jian Sun ◽

...

Keyword(s):

White Matter ◽

Oral Prednisolone ◽

Pulse Therapy ◽

Tuberculosis Treatment ◽

Case Presentation ◽

Steroid Pulse ◽

Csf Analysis ◽

Abstract Background Central nervous system overlapping autoimmune syndromes are uncommon, especially with the coexistence of MOG-IgG and GFAP-IgG. Case presentation A 23-year-old woman presented with transient convulsions, a loss of consciousness, persistent fever, headache, and vomiting. Cerebrospinal fluid (CSF) analysis revealed elevated cellularity, and magnetic resonance imaging (MRI) showed diffuse leptomeningeal enhancement. She had fever and headache with antiviral and antibiotic treatment for 2 weeks, and she had empirical anti-tuberculosis treatment and oral prednisolone therapy. She was followed for 3 months after presentation with improved symptoms and normal CSF analysis. A 3-month follow-up MRI showed asymmetric lesions in the cerebellum, corona radiata, and white matter with enhancement. The anti-tuberculosis treatment was continued, and steroid therapy was discontinued. After she stopped taking prednisolone, an interrupted headache gradually appeared. MRI at 4 months after presentation revealed a partial reduction in lesions but enlarged areas in the left cerebellum and right parietal white matter and a new lesion in the region of the right ependyma with linear enhancement. Her CSF was positive for anti-myelin oligodendrocyte glycoprotein (MOG) and anti-glial fibrillary acidic protein (GFAP) antibodies using a transfected cell-based assay. She was diagnosed with overlapping syndrome of MOG‑IgG‑associated disease and GFAP astrocytopathy. She received steroid pulse therapy (methylprednisolone, 1 g for 5 days), followed by a gradual tapering of oral prednisolone and the addition of an immunosuppressant (tacrolimus, 3 mg per day). Six months after the initial presentation, she had no symptoms. An MRI showed that the lesions had diminished, and no enhancement was found. Conclusions We report a case that was positive for double antibodies, which was initially misdiagnosed as infectious meningoencephalitis. This case broadens the clinical and phenotypic presentation of the overlapping syndrome spectrum.

White matter volume changes in people who develop psychosis

The British Journal of Psychiatry ◽

10.1192/bjp.bp.107.043463 ◽

2008 ◽

Vol 193 (3) ◽

pp. 210-215 ◽

Cited By ~ 71

Author(s):

Mark Walterfang ◽

Philip K. McGuire ◽

Alison R. Yung ◽

Lisa J. Phillips ◽

Dennis Velakoulis ◽

...

Keyword(s):

White Matter ◽

Grey Matter ◽

White Matter Volume ◽

Volume Changes ◽

Matter Volume ◽

Original Group ◽

Longitudinal Comparison ◽

BackgroundGrey matter changes have been described in individuals who are pre- and peri-psychotic, but it is unclear if these changes are accompanied by changes in white matter structures.AimsTo determine whether changes in white matter occur prior to and with the transition to psychosis in individuals who are pre-psychotic who had previously demonstrated grey matter reductions in frontotemporal regions.MethodWe used magnetic resonance imaging (MRI) to examine regional white matter volume in 75 people with prodromal symptoms. A subset of the original group (n=21) were rescanned at 12–18 months to determine white matter volume changes. Participants were retrospectively categorised according to whether they had or had not developed psychosis at follow-up.ResultsComparison of the baseline MRI data from these two subgroups revealed that individuals who later developed psychosis had larger volumes of white matter in the frontal lobe, particularly in the left hemisphere. Longitudinal comparison of data in individuals who developed psychosis revealed a reduction in white matter volume in the region of the left fronto-occipital fasciculus. Participants who had not developed psychosis showed no reductions in white matter volume but increases in a region subjacent to the right inferior parietal lobule.DiscussionThe reduction in volume of white matter near the left fronto-occipital fasciculus may reflect a change in this tract in association with the onset of frank psychosis.

Hip pain and its correlation with cam morphology in young skiers—a minimum of 5 years follow-up

Journal of Orthopaedic Surgery and Research ◽

10.1186/s13018-020-01952-8 ◽

2020 ◽

Vol 15 (1) ◽

Author(s):

Josefin Abrahamson ◽

Pall Jónasson ◽

Mikael Sansone ◽

Anna Swärd Aminoff ◽

Carl Todd ◽

...

Keyword(s):

Morphological Changes ◽

Hip Pain ◽

Activity Level ◽

Conflicting Evidence ◽

The Right ◽

Α Angle ◽

Cam Morphology ◽

Elite Skiers

Abstract Background There is conflicting evidence regarding the association between cam morphological changes and hip pain, and it remains unclear who with cam morphology will develop hip pain and who will not. This study aimed to investigate the correlation between cam morphology, hip pain, and activity level at a 5-year follow-up in young Alpine and Mogul skiers. Method All students (n = 76) at Åre Ski National Sports High School were invited and accepted participation in this prospective study at baseline. Magnetic resonance imaging (MRI) of both hips was conducted to evaluate the presence of cam morphology (α-angle ≥ 55°) and its size alongside the reporting of hip pain, type, and frequency of training by the Back and hip questionnaire, at baseline. After 5 years, the skiers were invited to complete a shortened version of the same questionnaire. Results A total of 60 skiers (80%) completed the follow-up questionnaire, of which 53 had concomitant MRI data. Cam morphology was present in 25 skiers (47.2%, 39 hips). Hip pain at baseline and at follow-up was reported in 17 (28.3%) and 22 (36.7%) skiers, respectively. No correlations were found between the activity level, the frequency, and the size of cam morphology and hip pain, except for the right hip α-angle at 1 o’clock and hip pain in skiers with cam morphology at baseline (rs = 0.49; P = 0.03) and at follow-up (rs = 0.47; P = 0.04). A total of 73.3% skiers had retired, of which 48% reported this was due to injuries. Conclusion Hip pain was not shown to be correlated, or had a low correlation, with activity level and the presence and size of cam morphology in young skiers on a 5-year follow-up. Based on these results, cam morphology or activity level did not affect hip pain to develop during 5 years of follow-up in young skiers. Furthermore, this study highlights that almost 75% of young elite skiers had retired from their elite career with almost 50% reporting that this was due to injuries sustained from skiing.

Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation

Neurology International ◽

10.4081/ni.2012.e5 ◽

2012 ◽

Vol 4 (1) ◽

pp. 5 ◽

Cited By ~ 10

Author(s):

Go Makimoto ◽

Yasuhiro Manabe ◽

Chizuru Yamakawa ◽

Daiki Fujii ◽

Yasuko Ikeda-Sakai ◽

...

Keyword(s):

High Intensity ◽

Skin Lesions ◽

Pulse Therapy ◽

Initial Manifestation ◽

Diffusion Weighted Images ◽

Fluid Analysis ◽

Fluid Attenuated Inversion Recovery ◽

Left Caudate ◽

We report 2 cases that were considered to be neuro-Sweet disease. They initially manifested with meningoencephalitis and no skin lesions, and rapidly improved with corticosteroid therapy. In both cases, patients complained of meningitic symptoms such as fever and headache, and HLA-B54 and -Cw1 turned out to be positive over the clinical course. Cerebrospinal fluid analysis showed increased levels of lymphocytes and protein. In case #1, fluid-attenuated inversion recovery (FLAIR), magnetic resonance imaging (MRI) and diffusion-weighted images (DWI) showed high-intensity signals in the right dorsal medulla oblongata, bilateral dorsal midbrain, and left thalamus. In case #2, FLAIR and DWI showed high-intensity signals in the bilateral cerebellar cortex and left caudate nucleus. Symptoms and MRI images were markedly improved in both cases after corticosteroid pulse therapy. According to published diagnostic criteria, these 2 cases were considered possible neuro-Sweet disease. These cases suggest that the combination of meningoencephalitis and HLA specificity is important to consider the possibility of neuro-Sweet disease, even without skin lesions.

A case of chronic progressive Lyme encephalitis as a manifestation of late Lyme neuroborreliosis

Infectious Disease Reports ◽

10.4081/idr.2014.5496 ◽

2014 ◽

Vol 6 (4) ◽

Cited By ~ 2

Author(s):

Vivek Verma ◽

Matthew Roman ◽

Disha Shah ◽

Marina Zaretskaya ◽

Mohamed H. Yassin

Keyword(s):

White Matter ◽

Short Term Memory ◽

Treatment Options ◽

Chronic Fatigue ◽

Short Term ◽

Mri Findings ◽

White Matter Changes ◽

Neurological Improvement ◽

Magnetic Resonance Imaging Mri

A 54-year-old female living in Europe presented with gait ataxia, dizziness, and bilateral hearing loss. Magnetic resonance imaging (MRI) revealed non-specific white matter changes. The patient’s condition gradually deteriorated over two years without diagnosis. The patient continued to decline cognitively and neurologically with worsening ataxia and upper motor neuron signs. Repeat MRI showed worsening white matter changes. Lumbar puncture, not previously done, showed positive Lyme testing. Treatment with intravenous ceftriaxone resulted in marked neurological improvement. Four years after symptom, the patient has short-term memory deficits and chronic fatigue, but is otherwise neurologically, cognitively, and functionally intact. Follow up MRI findings remain largely unchanged. Because cases of intraparenchymal or encephalopathic neuroborreliosis in America are lacking, so are treatment options. We present a rare case and discuss our experience with antibiotic treatment. This case lends evidence to define optimal treatment of this disease, imperative for hastening neurological recovery.

Intractable Trigeminal Neuralgia Secondary to Osteoma of the Clivus: A Case Report and Literature Review

Journal of Neurosciences in Rural Practice ◽

10.1055/s-0041-1742118 ◽

2022 ◽

Author(s):

Ebtesam Abdulla ◽

Krishna Das ◽

Joseph Ravindra ◽

Tejal Shah ◽

Sara George

Keyword(s):

Trigeminal Neuralgia ◽

Pressure Effect ◽

Benign Tumors ◽

Radiographic Images ◽

Patient Reported ◽

The Right ◽

Slow Growing ◽

Serial Observation

AbstractSkull base osteomas (SBOs) are benign tumors that are frequently detected on radiographic images by coincidence. They are known for being slow-growing tumors and rarely symptomatic. The therapeutic approach for SBOs can differ substantially. Depending on the symptoms, size, and location of the tumor, this can range from serial observation to vigorous surgical extirpation. Clival osteoma is extremely rare. We report a case of clival osteoma, causing intractable trigeminal neuralgia due to the pressure effect on the trigeminal nerve at Meckel's cave. We also provide a review of pertinent literature. A 37-year-old woman presented with intractable trigeminal neuralgia. Cranial magnetic resonance imaging (MRI) demonstrated a large, lobulated, extra-axial lesion involving the right cerebellopontine angle and epicentering the clivus. Pathologically, the specimen was proven to be osteoma. The patient reported complete symptom resolution over a 4-year follow-up period. To the best of the authors' knowledge, this is the first clinical case of intractable trigeminal neuralgia due to clival osteoma.

Case Report: Anti-NMDAR Encephalitis With Anti-MOG CNS Demyelination After Recurrent CNS Demyelination

Frontiers in Neurology ◽

10.3389/fneur.2021.639265 ◽

2021 ◽

Vol 12 ◽

Author(s):

Bing-Yan Ren ◽

Yi Guo ◽

Jing Han ◽

Qian Wang ◽

Zai-Wang Li

Keyword(s):

Case Report ◽

Brain Mri ◽

Clinical Manifestations ◽

Epileptic Seizures ◽

Autoimmune Disorder ◽

Pulse Therapy ◽

Nmdar Encephalitis ◽

Cns Demyelination ◽

Introduction: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis, a serious neurological autoimmune disorder caused by autoantibodies with diverse clinical manifestations, may simultaneously onset with antimyelin oligodendrocyte glycoprotein (MOG) demyelination after recurrent central nervous system (CNS) demyelination.Case Report: We present a case of anti-NMDAR encephalitis combining with anti-MOG CNS demyelination following recurrent CNS demyelination. A 38-year-old man admitted to hospital developed epileptic seizures following recurrent episodes of cross-sensory disturbance and dizziness. Magnetic resonance imaging (MRI) showed a demyelinating lesion in the right brainstem initially. Despite a good response to methylprednisolone pulse therapy at the beginning, the patient still had relapses and progression after corticosteroid reduction or withdrawal. Then brain MRI discovered new serpentine lesions involving extensive cerebral cortex on his second relapse. Repeat autoantibodies test indicated cerebrospinal fluid (CSF) NMDAR antibodies coexisted with MOG-Abs simultaneously, suggesting the diagnosis of anti-NMDAR encephalitis with anti-MOG CNS demyelination.Results: After a definite diagnosis, the patient was treated with mycophenolate mofetil (MMF) and corticosteroid. He was discharged after his symptoms ameliorated. No neurological sequels remained, and there were no effects on his activities of daily living after 6 months of immunoregulatory therapy of MMF and corticosteroid.Conclusion: For individuals with recurrent CNS demyelination, especially combining with cortical encephalitis, repeated detection of autoantibodies against AE, and demyelination in CSF/serum can be helpful to enable a definite early diagnosis. For patients who suffer from anti-NMDAR encephalitis combining with anti-MOG CNS demyelination, second-line immunotherapy is recommended when first-line treatment such as steroids, intravenous immunoglobulin G (IVIG) and plasma exchange has been proven ineffective to prevent the relapse of disease.

A Case Study on Retinoblastoma Associated Orbital Cellulitis

Journal of Advances in Medicine and Medical Research ◽

10.9734/jammr/2021/v33i130799 ◽

2021 ◽

pp. 91-97

Author(s):

Sagili Chandrasekhara Reddy ◽

Bina Sharine Menon

Keyword(s):

Anterior Segment ◽

Oral Prednisolone ◽

Detailed Examination ◽

Orbital Cellulitis ◽

Eye Drops ◽

Upper Eyelid ◽

Bilateral Retinoblastoma ◽

Pediatric Oncologist ◽

A 20-months-old male child was brought to the Eye clinic with swelling of right upper eyelid, discharge sticking the eyelids in the right eye and redness in both eyes of three days duration. On detailed examination of anterior segment and fundus, the diagnosis of bilateral retinoblastoma with conjunctivitis was made. The conjunctivitis was cured with ciprofloxacin eye drops and eye ointment. CT scan of orbits and brain confirmed the diagnosis of retinoblastoma with calcification in both eyes. Optic nerve on both sides was normal and there was no metastasis in the brain. Since it was a bilateral case of retinoblastoma, chemoreduction followed by enucleation in the right eye, and salvaging the left eye with chemotherapy in order to save the vision was planned. Intravenous triple drug chemotherapy with carboplatin, etoposide and vincristine (six cycles) was started by pediatric oncologist in pediatric ward. After two weeks of completing the first cycle of treatment, enucleation of right eye was done. Postoperative period was uneventful. The chemotherapy was continued. The child developed marked swelling of left upper eyelid few days before the sixth cycle of chemotherapy. Examination of left eye showed signs of aseptic orbital cellulitis which was treated with oral prednisolone and topical eye drops of combination of gentamycin and dexamethasone. The inflammatory signs subsided completely in ten days time. The sixth cycle of chemotherapy was completed. On the follow up visit two weeks after discharge, the left eye ball was normal. In the first follow up, the child could pick up the toys thrown in front of him. Unfortunately the child defaulted follow up. Retinoblastoma should be excluded in all young children with orbital cellulitis because misdiagnosis is life threatening.

First Clinical Experience with Anti-myelin Oligodendrocyte Glycoprotein Antibody-Positive Optic Neuritis

Klinische Monatsblätter für Augenheilkunde ◽

10.1055/a-1068-2506 ◽

2020 ◽

Vol 237 (04) ◽

pp. 458-463 ◽

Cited By ~ 1

Author(s):

Jan Heckmann ◽

Margarita Todorova ◽

Stefanie Müller ◽

Philip Julian Broser ◽

Veit Sturm

Keyword(s):

Visual Acuity ◽

Optic Neuritis ◽

Pulse Therapy ◽

Myelin Oligodendrocyte Glycoprotein ◽

Individual Treatment ◽

Igg Antibodies ◽

Intravenous Methylprednisolone ◽

Finger Counting ◽

Abstract Background Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been consistently found in a range of demyelinating disorders. In this context, MOG-IgG-associated optic neuritis (ON) has been suggested as a new subset of optic neuropathy. However, clinical manifestations and distinctive characteristics have only rarely been described. Patients and Methods A retrospective case series of three patients with MOG-IgG-associated ON. Clinical morphological features using imaging techniques are presented. Results Three patients (8-year-old boy, 28-year-old female, 48-year-old male) were included. An 8-year-old boy suffered from a bilateral ON with severe visual loss. The best-corrected visual acuity (BCVA) was 0.05 in the right eye and finger counting in the left eye. The patient had a previous episode of acute disseminated encephalomyelitis (ADEM) with a right abducens nerve palsy. Visual acuity recovered after repeated cycles of intravenous methylprednisolone pulse therapy and 10 cycles of plasma exchange. During the last follow-up, BCVA was 0.9 in the right eye and 0.8 in the left eye. A 28-year-old female presented with a bilateral ON. Her BCVA was 0.5 in the right eye and 0.8 in the left eye. She fully recovered with pulse methylprednisolone therapy (1000 mg/d) with tapering after the second cycle and had a BCVA of 1.0 during the last follow-up visit. A 48-year-old male suffered from a relapsing bilateral ON. At first presentation, BCVA was 0.1 in the right eye and finger counting in the left eye. BCVA fully recovered after each pulse therapy with intravenous methylprednisolone (two cycles). Since the first relapse, the patient has been receiving long-term immunosuppression with rituximab. Despite rituximab and low-dose oral prednisone, the patient had another relapse with a left ON. After a third cycle with intravenous methylprednisolone, he partially recovered. BCVA at last follow-up was 1.0 in the right and 0.8 in the left eye. Conclusions MOG-IgG antibodies have been identified in different acquired demyelinating syndromes. The patients reported had an ADEM followed by bilateral ON, an isolated bilateral ON, and a relapsing bilateral ON. Individual treatment strategies led to substantial visual recovery in all patients. We recommend inclusion of MOG-IgG antibodies in the diagnostic workup at least after the first recurrence of ON since they can serve as a diagnostic and potential prognostic tool and might lead to specific therapeutic recommendations.

Huge peripapillary staphyloma with craniofacial clefts: A case report

European Journal of Ophthalmology ◽

10.1177/1120672120946292 ◽

2020 ◽

pp. 112067212094629

Author(s):

Fei Yu ◽

Yao Fu

Keyword(s):

Case Report ◽

Deep Excavation ◽

Resonance Imaging ◽

Large Defect ◽

The Right ◽

First Time ◽

Left Eyelid ◽

Peripapillary Staphyloma

Purpose: We reported the occurrence of a congenital unilateral huge peripapillary staphyloma in association with craniofacial clefts for the first time. Case report: A 1-year-old boy presented with a large defect on his left eyelid, a wide oblique columella nasi and an atypical wedge-shaped extension of the unilateral anterior hairline. Magnetic resonance imaging (MRI) examinations revealed there were cracks on his nasal septum, palate, and superior alveolar midline. Moreover, we surprisingly uncovered a gourd-shaped eyeball with the compressed optic nerve on the right side, while the right eye seemed normal from appearance. Under anaesthesia, fundus examination of the right eye showed a 15 mm-deep excavation surrounding the optic disc with defective choroid and dysplastic optic papilla. We reconstructed the left eyelid of the patient to protect his cornea and would make other solutions according to the results of follow-up. Conclusion: Peripapillary staphyloma and craniofacial clefts are two dissimilar rare congenital anomalies. In this patient, we firstly observed the co-existence of the two defects, which may provide the experience to the diagnosis and treatment of peripapillary staphyloma and craniofacial clefts. This case also gives us the pathogenic inspiration for further studies of peripapillary staphyloma and craniofacial clefts.