scholarly journals Recurrent post-partum rhombencephalitis associated with anti-centromere antibody: a case report

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Andy Jin ◽  
Jean Mamelona ◽  
Byrne Harper ◽  
Alier Marrero

Abstract Background Rhombencephalitis (RE) is a serious condition of the brain with multiple etiologies. We report a unique case of recurrent, postpartum RE that is associated with positive anti-centromere antibody (ACA). A discussion of the case, current literature on autoimmune RE and related autoantibodies are reviewed. Case presentation A healthy 33-year-old Caucasian patient (gravida 2, para 2) had two episodes of progressive focal neurological deficits during postpartum periods. Signs and symptoms included right-sided dysmetria, adiadochokinesia, weakness, ataxia, and photophobia. MRI revealed rhombencephalitis involving the mesencephalon of the brainstem. Extensive and comprehensive investigations using blood and cerebrospinal fluid (CSF) were consistently positive only for ACA. The first episode was successfully treated with empiric antimicrobial agents and steroid. Given the negative infectious work up with the prior episode and the nearly identical clinical presentations, the second episode was treated with corticosteroid only. This led to complete resolution of her symptoms and reversal of the brain magnetic resonance imaging (MRI) lesions. Conclusion To the author's knowledge, this is the first report of a primary autoimmune RE during postpartum period that is associated with ACA. Immunologic causes should be considered early with any encephalitis. Given the risk of recurrence, relapse, and neurologic deterioration, regular monitoring is recommended, especially for female patients of child-bearing age. Consistent with the current literature on autoimmune RE, steroid seems to be an effective treatment for ACA-associated RE.

2019 ◽  
Author(s):  
Andy Jin(Former Corresponding Author) ◽  
Jean Mamelona ◽  
Byrne Harper ◽  
Alier Marrero(New Corresponding Author)

Abstract Background Rhombencephalitis (RE) is a serious condition of the brain with multiple etiologies. We report a unique case of recurrent, postpartum RE that is associated with positive anti-centromere antibody (ACA). A discussion of the case, current literature on autoimmune RE and related autoantibodies are reviewed. Case Presentation A healthy 33-year-old Caucasian patient (gravida 2, para 2) had two episodes of progressive focal neurological deficits during postpartum periods. Signs and symptoms included right-sided dysmetria, adiadochokinesia, weakness, ataxia, and photophobia. MRI revealed rhombencephalitis involving the mesencephalon. Extensive and comprehensive investigations using blood and cerebrospinal fluid (CSF) were consistently positive only for ACA. The first episode was successfully treated with empiric antimicrobial agents and steroid. Given the negative infectious work up with the prior episode and the nearly identical clinical presentations, the second episode was treated with corticosteroid only. This led to complete resolution of her symptoms and reversal of the brain magnetic resonance imaging (MRI) lesions. Conclusion To the authors’ knowledge, this is the first report of a primary autoimmune RE during postpartum period that is associated with ACA. Immunologic causes should be considered early with any encephalitis. Given the risk of recurrence, relapse, and neurologic deterioration, regular monitoring is recommended, especially for female patients of child-bearing age. Consistent with the current literature on autoimmune RE, steroid seems to be an effective treatment for ACA-associated RE.


2018 ◽  
Author(s):  
Andy Jin ◽  
Jean Mamelona ◽  
Byrne Harper ◽  
Alier Marrero

Abstract Background Rhombencephalitis (RE) is a serious condition of the brain with multiple etiologies. We report a unique case of recurrent, postpartum RE that is associated with positive anti-centromere antibody (ACA). A discussion of the case, current literature on autoimmune RE and related autoantibodies are reviewed. Case Presentation A healthy 33-year-old Caucasian patient (gravida 2, para 2) had two episodes of progressive focal neurological deficits during postpartum periods. Signs and symptoms included right-sided dysmetria, adiadochokinesia, weakness, ataxia, and photophobia. MRI revealed rhombencephalitis involving the mesencephalon. Extensive and comprehensive investigations using blood and cerebrospinal fluid (CSF) were consistently positive only for ACA. The first episode was successfully treated with empiric antimicrobial agents and steroid. Given the negative infectious work up with the prior episode and the nearly identical clinical presentations, the second episode was treated with corticosteroid only. This led to complete resolution of her symptoms and reversal of the brain magnetic resonance imaging (MRI) lesions. Conclusion To the authors’ knowledge, this is the first report of a primary autoimmune RE during postpartum period that is associated with ACA. Immunologic causes should be considered early with any encephalitis. Given the risk of recurrence, relapse, and neurologic deterioration, regular monitoring is recommended, especially for female patients of child-bearing age. Consistent with the current literature on autoimmune RE, steroid seems to be an effective treatment for ACA-associated RE. Keywords: Autoimmune disease, rhombencephalitis, anti-centromere antibody, connective tissue disease, case report


2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi198-vi198
Author(s):  
Ruchi Raval ◽  
Aadi Pandya ◽  
Jaspreet Behl ◽  
Sumul Raval

Abstract PURPOSE As more information is gathered about brain metastases, it still remains that the current prognosis of brain metastases is very poor. Due to this, it is imperative that physicians are aware of the most important components regarding brain metastases. This literature review will encompass the most current literature in order to highlight the most crucial information. METHODS All mentioned studies and literature reviews cited in the paper were obtained through various sites, and were published between 1996 and 2017. The main components that were required from the papers reviewed included where in the body the brain metastases originated from, where in the brain they tended to spread to, what the signs and symptoms typical of patients with brain metastases are, and what the options are in terms of treatment. RESULTS Using the results from a variety of studies performed within the past three decades, it is apparent that brain metastases most commonly originate from, in order of increasing frequency, lung cancer, breast cancer, melanoma, and colorectal cancer. In addition, it is reaffirmed that the magnetic resonance imaging (MRI) is the best diagnostic tool to be used when dealing with brain metastases. The most frequent signs and symptoms of a brain metastases include cognitive changes, headaches, weakness, and seizures. Finally, supportive treatment includes use of corticosteroids, antiepileptic drugs (AEDs), and anticoagulation therapy. Definitive treatment for brain metastases varies based on size, location, and prevalence in the brain, but the most effective options include chemotherapy, radiation therapy, and surgery. CONCLUSIONS The study’s results confirm the need for more research to be done regarding brain metastases, and better options to increase the survival of patients.


Author(s):  
Neelu Desai ◽  
Rahul Badheka ◽  
Nitin Shah ◽  
Vrajesh Udani

AbstractReversible cerebral vasoconstriction syndrome (RCVS) has been well described in adults, but pediatric cases are yet under recognized. We describe two children with RCVS and review similar already published pediatric cases. The first patient was a 10-year-old girl who presented with severe headaches and seizures 3 days after blood transfusion. Brain magnetic resonance imaging (MRI) showed changes compatible with posterior reversible encephalopathy syndrome and subarachnoid hemorrhage. Magnetic resonance angiogram showed diffuse vasoconstriction of multiple cerebral arteries. The second patient was a 9-year-old boy who presented with severe thunderclap headaches. Brain MRI showed isolated intraventricular hemorrhage. Computed tomography/MR angiogram and digital subtraction angiogram were normal. A week later, he developed focal neurological deficits. Repeated MR angiogram showed diffuse vasospasm of multiple intracranial arteries. Both children recovered completely. A clinico-radiological review of previously reported childhood RCVS is provided.


Author(s):  
Sreelakshmi S. ◽  
Anoop V. S.

Neurological disorders are diseases of the central and peripheral nervous system and most commonly affect middle- or old-age people. Accurate classification and early-stage prediction of such disorders are very crucial for prompt diagnosis and treatment. This chapter discusses a new framework that uses image processing techniques for detecting neurological disorders so that clinicians prevent irreversible changes that may occur in the brain. The newly proposed framework ensures reliable and accurate machine learning techniques using visual saliency algorithms to process brain magnetic resonance imaging (MRI). The authors also provide ample hints and dimensions for the researchers interested in using visual saliency features for disease prediction and detection.


2017 ◽  
Vol 16 (04) ◽  
pp. 239-242
Author(s):  
Gunnar Buyse ◽  
Lieven Lagae ◽  
Philippe Demaerel ◽  
Frank Kesteloot ◽  
Ingele Casteels ◽  
...  

AbstractWyburn–Mason syndrome is a rare condition that is characterized by ipsilateral arteriovenous malformations affecting the eye, brain, and facial skin. A conjunctival vascular dilation can be a rare ocular presenting sign. We report a 6-year-old boy who attended the hospital because of the sudden appearance of a conjunctival vascular lesion in his right eye. Inspection of his facial skin showed a subtle discoloration along the right trigeminal nerve and a vascular structure of the conjunctiva. Fundoscopy showed dilated and tortuous retinal vessels. Brain magnetic resonance imaging (MRI) revealed a large arteriovenous malformation involving the thalamus and perimesencephalic area. Ophthalmologic and neuroradiologic findings were consistent with the diagnosis of Wyburn–Mason syndrome. The sudden emergence of a vascular malformation in the conjunctiva should alert the clinician to perform an ophthalmoscopy, and in our patient, this finding was the clue to diagnosis of Wyburn–Mason syndrome. Because of the association between retinal and intracranial arteriovenous malformations, an MRI of the brain is strongly recommended in all patients with ocular arteriovenous malformations.


Author(s):  
Ghazaleh Jamalipour Soufi ◽  
Siavash Iravan

Pelizaeus-Merzbacher Disease (PMD), as a rare genetically x-linked leukodystrophy, is a disorder of proteolipid protein expression in myelin formation. This disorder is clinically presented by neurodevelopmental delay and abnormal pendular eye movements. The responsible gene for this disorder is the proteolipid protein gene (PLP1). Our case was a oneyear-old boy referred to the radiology department for evaluating the Central Nervous System (CNS) development by brain Magnetic Resonance Imaging (MRI). Clinically, he demonstrated neuro-developmental delay symptoms. The brain MRI results indicated a diffuse lack of normal white matter myelination. This case report should be considered about the possibilityof PMD in the brain MRI of patients who present a diffuse arrest of normal white matter myelination.


2021 ◽  
Author(s):  
Pedro Schmidt dos Reis Matos Figueiredo ◽  
Thiago Oliveira Chaves

Context: Dyke-Davidoff-Masson (DDM) syndrome is a rare neurological condition, first described in 1933. Characteristics include cerebral hemiatrophy, contralateral hemiparesis, seizures, and cognitive dysfunction, combined into different degrees and patterns. Brain magnetic resonance imaging (MRI) is used to perform diagnosis throughout its specific findings. Case Report: A eighteen-year-old female presented to our service with a history of cognitive dysfunction and seizures since early childhood, which persistence even with adequate use of antiepileptic drugs. During Investigation were found signs and symptoms compatible with DDM syndrome, and evidence of pseudoseizures captured in a video electroencephalography monitoring. Conclusion: DDM syndrome is a rare condition that must be part of differential diagnosis in patients with seizures and cerebral hemiatrophy. Management is based on adequate control of seizures and other comorbidities.


2019 ◽  
Vol 9 (3) ◽  
pp. 569 ◽  
Author(s):  
Hyunho Hwang ◽  
Hafiz Zia Ur Rehman ◽  
Sungon Lee

Skull stripping in brain magnetic resonance imaging (MRI) is an essential step to analyze images of the brain. Although manual segmentation has the highest accuracy, it is a time-consuming task. Therefore, various automatic segmentation algorithms of the brain in MRI have been devised and proposed previously. However, there is still no method that solves the entire brain extraction problem satisfactorily for diverse datasets in a generic and robust way. To address these shortcomings of existing methods, we propose the use of a 3D-UNet for skull stripping in brain MRI. The 3D-UNet was recently proposed and has been widely used for volumetric segmentation in medical images due to its outstanding performance. It is an extended version of the previously proposed 2D-UNet, which is based on a deep learning network, specifically, the convolutional neural network. We evaluated 3D-UNet skull-stripping using a publicly available brain MRI dataset and compared the results with three existing methods (BSE, ROBEX, and Kleesiek’s method; BSE and ROBEX are two conventional methods, and Kleesiek’s method is based on deep learning). The 3D-UNet outperforms two typical methods and shows comparable results with the specific deep learning-based algorithm, exhibiting a mean Dice coefficient of 0.9903, a sensitivity of 0.9853, and a specificity of 0.9953.


2019 ◽  
Vol 6 (Supplement_2) ◽  
pp. S515-S515
Author(s):  
Ambreen Allana ◽  
Mohammed Samannodi ◽  
Michael Hansen ◽  
Rodrigo Hasbun

Abstract Background To describe the use and the impact on clinical outcomes of adjunctive steroids in adults with encephalitis. Methods We conducted a retrospective observational study of 230 adults (age >17 years) who met the international consortium definition for encephalitis. An adverse clinical outcome was defined as a Glasgow outcome score of 1–4. The study took place at three tertiary care hospitals in Houston TX, between August 2008 and September 2017. Results A total of 230 adults with encephalitis were enrolled, out of which 121 (52.6%) received steroids. Adjunctive steroids were given more frequently to those with focal neurological deficits (P = 0.013), had a positive cerebrospinal fluid (CSF) HSV PCR (P = 0.013), required mechanical ventilation (MV) (P = 0.011), required intensive care unit (ICU) admission (P < 0.001), had white matter abnormalities (P = 0.014) or had cerebral edema on the brain magnetic resonance imaging (MRI) (P = 0.003). An adverse outcome was seen in 139 (60.7%) of patients. Predictors for adverse outcomes included a Glasgow coma score (GCS) < 8, fever, seizures, ICU admission, and presence of edema on brain MRI. The use of adjunctive steroids did not impact clinical outcomes (P = 0.521). Independent prognostic factors on logistic regression analysis were edema on brain MRI (7.780 [1.717–35.263] P = 0.008), GCS < 8 (6.339 [1.992–20.168] P = 0.002), and fever (2.601 [1.342–5.038] P = 0.005). Conclusion Adults with encephalitis continues to be associated with significant adverse clinical outcomes in the majority of patients. Adjunctive steroids are used in the sicker patients and it is not associated with improved clinical outcomes. Disclosures All authors: No reported disclosures.


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