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

2021 ◽  
Vol 12 ◽  
Author(s):  
Bing-Yan Ren ◽  
Yi Guo ◽  
Jing Han ◽  
Qian Wang ◽  
Zai-Wang Li

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.

2020 ◽  
Vol 8 (C) ◽  
pp. 132-139
Author(s):  
Nevena Manevska ◽  
Neron Popovski ◽  
Tanja Makazlieva ◽  
Hristina Popovska ◽  
Aleksandra Pesevska-Todorcevska ◽  
...  

BACKGROUND: Infectious sacroiliitis (ISI) is an inflammation of one or both of the sacroiliac (SI) joints, relatively rare disorder, affecting between 1% and 2% of all patients with septic arthritis. The variety of symptom presentation makes the diagnosis quite challenging. Combination of laboratory hematological tests, together with diagnostic imaging tools, such as magnetic resonance imaging (MRI), computed tomography (CT), and bone scan (BS), as well as microbiological tests contribute the final diagnosis, which may take up to several months. CASE REPORT: We present a case of a 33-year-old male patient with a history of lower back pain with propagation of the pain in the right leg, accompanied by febrility and hematuria. Laboratory tests showed high values of C-reactive protein, high degradation products and hyperkalemia, leading to a diagnose of acute renal failure stage 3. MRI of the lower spine and pelvis revealed hetero- signal change more to the right where the spinal canal was expanded, accumulating contrast and involved the caudate and the right radix. Тhe displayed sequences were accompanied by an altered morphology of the spinal musculature, with intense accumulation of contrast in parts of the muscle. Paravertebral abscess was detected in the intercaudal and iliac muscles, along with inflammatory edema of the right SI with a suspicion of a sacroiliitis. One week after, a three phase BS showed positive accumulation in the right SI joint in all three phases. The SI index for the right SI joint was 2.09, while for the left SI joint was 1.125. The patient underwent surgical intervention for drainage of the paravertebral abscess. CONCLUSION: The condition of ISI may be sometimes very difficult to be recognized in many patients. Considering the diversity of the clinical manifestations, it is of great importance to select the right imaging modality. The nuclear medicine technique triple phase bone and the hybrid imaging SPECT/CT have been suggested to improve the sensitivity and specificity of the bone scan, providing better characterization of equivocal lesions, especially in the acute form for disease localization.


2016 ◽  
Vol 33 (S1) ◽  
pp. S524-S524
Author(s):  
P. Azevedo ◽  
F. Monteiro ◽  
A.P. Correia ◽  
A. Norton ◽  
A.M. Moreira

IntroductionAnti N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is an autoimmune disorder with a presentation that includes acute behavioral changes, psychosis, cognitive impairment and autonomic instability. In some cases, there are isolated psychiatric symptoms without neurological involvement.AimsTo raise awareness of the disorder among psychiatrists, considering it a differential diagnosis in a first psychotic episode since a prompt diagnosis and treatment can dramatically affect the outcome.ObjectivesTo summarize the latest literature about this field and to present a case report.MethodsA brief review of the latest literature was performed on PubMed using the keywords “anti N-methyl-D-aspartate receptor encephalitis”, “anti-NMDA encephalitis”, “psychiatric symptoms”.ResultsA 20-year-old male was admitted to our inpatient unit with bizarre delusions of grandious and religious content, somatic hallucinations, sleep cycle inversion and strange behaviour. These symptoms had been present for 1 week and remitted after 10 days of treatment with risperidone. On follow-up, he developed anhedonia, apathy and blunt affect. Brain MRI showed multiple hyperintense changes in T2 and T2-FLAIR, highly suggestive of demyelinating lesions. The cerebrospinal fluid showed mild lymphocytic pleocytosis, mildly increased proteins, oligoclonal bands and anti-NMDAR antibodies of intrathecal production. He was treated with corticoids and the antipsychotic was discontinued. No neurologic symptoms were ever present.ConclusionThis is an atypical case of anti-NMDAR encephalitis because of its isolated psychiatric presentation. Most patients develop neurological symptoms 2 to 3 weeks after onset of psychiatric symptoms. Monosymptomatic syndromes arise in less than 5% of patients.Disclosure of interestThe authors have not supplied their declaration of competing interest.


2020 ◽  
Vol 12 (3) ◽  
pp. 433-439
Author(s):  
Riwaj Bhagat ◽  
Siddharth Narayanan ◽  
Marwa Elnazeir ◽  
Thong Diep Pham ◽  
Robert Paul Friedland ◽  
...  

Gasperini syndrome (GS), a rare brainstem syndrome, is featured by ipsilateral cranial nerves (CN) V–VIII dysfunction with contralateral hemibody hypoesthesia. While there have been 18 reported cases, the GS definition remains ambiguous. We report a new case and reviewed the clinical features of this syndrome from all published reports to propose a new definition. A 57-year-old man with acute brainstem stroke had right CN V–VIII and XII palsies, left body hypoesthesia and ataxia. Brain MRI showed an acute stroke in the right caudal pons and bilateral cerebellum. After a systematic review, we classified the clinical manifestations into core and associate features based on the frequencies of occurring neurological deficits. We propose that a definitive GS requires the presence of ipsilateral CN VI and VII palsies, plus one or more of the other three core features (ipsilateral CN V, VIII palsies and contralateral hemibody hemihypalgesia). Additionally, GS, similar to Wallenberg’s syndrome, represents a spectrum that can have other associated neurological features. The revised definition presented in this study may enlighten physicians with the immediate recognition of the syndrome and help improve clinical localization of the lesions and its management.


2021 ◽  
Vol 11 (9) ◽  
Author(s):  
Nilesh Vishwakarma ◽  
Shaival Chauhan ◽  
Shrey S Binyala ◽  
Sanjeev K Singh

Introduction:Primary subacute pyogenic osteomyelitis, or Brodie’s abscess was initially documented by Sir Benjamin Brodie in 1832. We present a case report with a 6-months follow-up period, demonstrating the successful diagnosis and surgical treatment of a focal lesion of the proximal metaphysis of the right humerus in a 21-years-old female. The pathology of hematologic osteomyelitis and its role in the development of a subacute abscess along with a review of literature and an in detail description of the pathogenesis of Brodie’s abscess is discussed and submitted. Case Report:A 21- years -old healthy female with a history of fall sustaining injury to the right shoulder one 1 year back followed by which she presented to the outpatient clinic with a swelling over her right shoulder. The patient was managed conservatively with analgesics and was relieved of pain over a course of one 1 week of medications, the patient now presents with pain and swelling in the right shoulder joint on and off since the episode of fall one 1 year back, which had increased over a period of past one 1 week. A week before the most recent presentation she started experiencing some discomfort and pain in her right shoulder. No recent trauma was reported. A mild swelling appeared over the proximal part of the humerus. There were no constitutional symptoms of fever or any illness reported. On examination, there was noted a painful restriction of ROM at the right shoulder joint with no rotator cuff injury. Laboratory investigations were suggestive of raised inflammatory markers. Radiograph of the right shoulder taken in the true antero-posterior view with the shoulder in the neutral rotation was suggestive of an oval lucency with surrounding sclerosis in the proximal metaphyseal region of the humerus. Magnetic resonance imaging MRI of the right shoulder joint showed features consistent with Brodie’s abscess in the proximal metaphyseal region of the humerus. Surgical debridement of the abscess w


Author(s):  
Renjie Wang ◽  
Yankun Shao ◽  
Lei Xu

Introduction: The medulla oblongata is the lowest segment of the brain stem, located adjacent to the spinal cord, with a complex anatomical structure. Thus, a small injury to the medulla oblongata can show complex clinical manifestations. Case Presentation: A patient experienced dysesthesia, which manifested as numbness in her right lower limb and decreased temperature sense, and dizziness 20 days before admission. The numbness worsened 1 week before admission, reaching the right thoracic (T) 12 dermatomes. Her thermoception below the T12 dermatomes decreased, and the degree of dizziness increased, accompanied by nausea and vomiting. Magnetic resonance imaging (MRI) of the neck, chest, and abdomen performed at a local hospital showed no abnormalities. MRI of the brain was performed after admission. One week after admission, she experienced a severe headache in the upper left periorbital area. The numbness extended to T4, and thermoception decreased below T4. Diagnosis: Lateral medullary infarction. Interventions: Anti-platelet aggregation and mitochondrial nutritional therapies were performed along with treatments for improving circulation and establishing collateral circulation. Outcomes: The intensity of limb numbness decreased, and the symptoms of headache and dizziness resolved. Conclusion: Lesions leading to segmental sensory disorders can occur in the medulla oblongata. Ipsilateral headaches with contralateral segmental paresthesia can be a specific sign of lateral medullary infarction.


Author(s):  
Kiran Natarajan ◽  
Koka Madhav ◽  
A. V. Saraswathi ◽  
Mohan Kameswaran

<p>Bilateral temporal bone fractures are rare; accounting for 9% to 20% of cases of temporal bone fractures. Clinical manifestations include hearing loss, facial paralysis, CSF otorhinorrhea and dizziness. This is a case report of a patient who presented with bilateral temporal bone fractures. This is a report of a 23-yr-old male who sustained bilateral temporal bone fractures and presented 18 days later with complaints of watery discharge from left ear and nose, bilateral profound hearing loss and facial weakness on the right side. Pure tone audiometry revealed bilateral profound sensori-neural hearing loss. CT temporal bones &amp; MRI scans of brain were done to assess the extent of injuries. The patient underwent left CSF otorrhea repair, as the CSF leak was active and not responding to conservative management. One week later, the patient underwent right facial nerve decompression. The patient could not afford a cochlear implant (CI) in the right ear at the same sitting, however, implantation was advised as soon as possible because of the risk of cochlear ossification. The transcochlear approach was used to seal the CSF leak from the oval and round windows on the left side. The facial nerve was decompressed on the right side. The House-Brackmann grade improved from Grade V to grade III at last follow-up. Patients with bilateral temporal bone fractures require prompt assessment and management to decrease the risk of complications such as meningitis, permanent facial paralysis or hearing loss. </p>


2021 ◽  
Author(s):  
Jialin Pan ◽  
Begench Ovlyakulov ◽  
Lili zhou

Abstract BackgroundAnti-N-methyl-D-aspartate receptor (NMDAR) encephalitis can coexist with neuromyelitis optica spectrum disorder (NMOSD). Patients with overlapping anti-NMDAR encephalitis with positive NMDAR antibodies and aquaporin 4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD are rare but should not be ignored.Case presentationThis report describes a unique case of anti-NMDAR encephalitis coexisting with NMOSD is presented. A 27-year-old male presented with blurred vision, cognitive impairment, psychosis, dysphagia, gait instability and urinary incontinence. Brain magnetic resonance imaging (MRI) showed abnormal signals in the right cerebellar hemisphere, temporal lobe, and corpus callosum. NMDAR antibodies were positive in the CSF. AQP4-IgG antibodies were positive in the serum. The patient's condition was stable following intravenous gamma globulin, corticosteroids, immunosuppressants and symptomatic treatments. ConclusionsThis case provides further evidence for the occurrence of anti-NMDAR encephalitis overlapping NMOSD with AQP4-IgG-seropositive in a Chinese patient. However, the mechanisms underlying the occurrence of double positive antibodies remain elusive.


2007 ◽  
Vol 65 (2b) ◽  
pp. 524-527 ◽  
Author(s):  
Marco Antonio Orsini Neves ◽  
Marcos R.G. de Freitas ◽  
Mariana Pimentel de Mello ◽  
Carlos Henrique Dumard ◽  
Gabriel R. de Freitas ◽  
...  

Monomelic amyotrophy (MA) is a rare condition in which neurogenic amyotrophy is restricted to an upper or lower limb. Usually sporadic, it usually has an insidious onset with a mean evolution of 2 to 4 years following first clinical manifestations, which is, in turned, followed by stabilization. We report a case of 20-years-old man who presented slowly progressive amyotrophy associated with proximal paresis of the right upper limb, which was followed by clinical stabilization 4 years later. Eletroneuromyography revealed denervation along with myofasciculations in various muscle groups of the right upper limb. We call atention to this rare location of MA, as well as describe some theories concerning its pathophysiology .


2018 ◽  
Vol 46 (3) ◽  
pp. 1277-1281 ◽  
Author(s):  
Chaoyang Jing ◽  
Lichao Sun ◽  
Zhuo Wang ◽  
Chaojia Chu ◽  
Weihong Lin

Background Reversible splenial lesion syndrome is a distinct entity radiologically characterized by a reversible lesion in the splenium of the corpus callosum. According to previous reports, this condition may be associated with antiepileptic drug use or withdrawal. We herein report a case of reversible splenial lesion syndrome associated with oxcarbazepine withdrawal. Case Report A 39-year-old man presented with an 8-year history of epileptic seizures. During the previous 3 years, he had taken oxcarbazepine irregularly. One week prior to admission, he withdrew the oxcarbazepine on his own, and the epilepsy became aggravated. Magnetic resonance imaging (MRI) revealed an isolated lesion in the splenium of the corpus callosum with slight hypointensity on T1-weighted imaging and slight hyperintensity on T2-weighted imaging. Regular oxcarbazepine was prescribed. Over a 5-month follow-up period, repeat MRI showed that the abnormal signals in the splenium of the corpus callosum had completely disappeared. Conclusion Reversible splenial lesion syndrome is a rare clinicoradiological disorder that can resolve spontaneously with a favorable outcome. Clinicians should be aware of this condition and that oxcarbazepine withdrawal is a possible etiological factor.


BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Yun Jiang ◽  
Jianpeng Ma ◽  
Tao Gong ◽  
Hongjun Hao ◽  
Haibo Chen

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe and most common autoimmune encephalitis in patients under 40 years old. Anti-NMDAR encephalitis has various clinical and neuroimaging findings. Here we report a special case of an anti-NMDAR encephalitis who had diffuse lesions in bilateral hemispheres with mild mass effects in left basal ganglia area. Case presentations A 28-year-old female anti-NMDAR encephalitis patient mainly presented with headache and fever. Brain magnetic resonance image (MRI) showed slightly contrasted diffuse lesions, involving the left temporal and frontal lobes, left basal ganglia area and splenium of corpus callosum, as well as the right frontal lobe, with mild edema surrounded in the left basal ganglia area. Cerebrospinal fluid (CSF) revealed a moderate pleocytosis with normal protein and glucose levels. Anti-NMDAR antibodies were identified in CSF. Transvaginal ovarian ultrasound did not reveal an ovarian teratoma. The patient was treated with immunoglobulin and steroid, and had a good recovery. Conclusions Anti-NMDAR encephalitis has no special clinical manifestations and brain MRI is highly variable, which could be unremarkable or abnormal involving white and grey matters. The extensive lesions in frontal and temporal lobes, and basal ganglia area, with mild mass effects, have not been described previously. Recognition of various changes in brain MRI will enable the early detection of anti-NMDAR antibody and then effective treatments.


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