scholarly journals Clinical manifestations of neurosarcoidosis

2013 ◽  
Vol 66 (suppl. 1) ◽  
pp. 54-59
Author(s):  
Mihailo Stjepanovic ◽  
Dragana Jovanovic ◽  
Aleksandra Dudvarski-Ilic ◽  
Violeta Mihailovic-Vucinic ◽  
Vesna Skodric-Trifunovic ◽  
...  

Introduction. Sarcoidosis affects the central nervous system more frequently than it was previously believed. Since the diagnosis of neurosarcoidosis is often delayed, it may result in serious complications. Being non-specific when present, the symptoms may be subtle and resemble those of other neurological diseases. While the cranial nerves are most frequently affected, neurosarcoidosis can involve other nervous system tissues including the meninges, brain parenchyma (especially the hypothalamic region), spinal cord, peripheral nerve and muscle. Discussion and Review of Literature. During the past decade, a significant progress was made in understanding the epidemiology and pathophysiology of neurosarcoidosis, as well as the possibility to diagnose and treat this disease. Studies have shown that the optimal diagnostic imaging modality for neurosarcoidosis is magnetic resonance imaging with gadolinium because it enhances visualization of granulomatous infiltration in neural tissue. Subclinical neurosarcoidosis may not be uncommon in patients with sarcoidosis. It is now evident that neurosarcoidosis does not invariably present as a catastrophic event. Adverse effects associated with high-dose systemic corticosteroids, the standard therapy, have discouraged practitioners from initiating treatment in the absence of significant symptomatic neurological disease. However, other immunosuppressive agents as well newer biologic agents have emerged as an effective, well-tolerated therapeutic alternative to corticosteroids, which are often effective in corticosteroid- recalcitrant cases. Conclusion. Neurosarcoidosis, as a localized granulomatous disease, is possible and not so rare. Early recognition of neurological involvement in patients with undiagnosed or diagnosed sarcoidosis is crucial to prevent complications, which can sometimes be life-threatening.

2020 ◽  
Author(s):  
Juan I. Guerrero ◽  
Luis A. Barragán ◽  
Juan D. Martínez ◽  
Juan P. Montoya ◽  
Alejandra Peña ◽  
...  

Abstract Background: SARS-CoV-2 can affect the human brain and other neurological structures. An increasing number of publications report neurological manifestations in patients with COVID-19. However, no studies have comprehensively reviewed the clinical and paraclinical characteristics of the central and peripheral nervous system's involvement in these patients. This study aimed to describe the features of the central and peripheral nervous system involvement by COVID-19 in terms of pathophysiology, clinical manifestations, neuropathology, neuroimaging, electrophysiology, and cerebrospinal fluid findings.Methods: We conducted a comprehensive systematic review of all the original studies reporting patients with neurological involvement by COVID-19, from December 2019 to June 2020, without language restriction. We excluded studies with animal subjects, studies not related to the nervous system, and opinion articles. Data analysis combined descriptive measures, frequency measures, central tendency measures, and dispersion measures for all studies reporting neurological conditions and abnormal ancillary tests in patients with confirmed COVID-19.Results: A total of 143 observational and descriptive studies reported central and peripheral nervous system involvement by COVID-19 in 10723 patients. Fifty-one studies described pathophysiologic mechanisms of neurological involvement by COVID-19, 119 focused on clinical manifestations, 4 described neuropathology findings, 62 described neuroimaging findings, 28 electrophysiology findings, and 60 studies reported cerebrospinal fluid results. The reviewed studies reflect a significant prevalence of the nervous system's involvement in patients with COVID-19, ranging from 22.5% to 36.4% among different studies, without mortality rates explicitly associated with neurological involvement by SARS-COV-2. We thoroughly describe the clinical and paraclinical characteristics of neurological involvement in these patients. Conclusions: Our evidence synthesis led to a categorical analysis of the central and peripheral involvement by COVID-19 and provided a comprehensive explanation of the reported pathophysiological mechanisms by which SARS-CoV-2 infection may cause neurological impairment. International collaborative efforts and exhaustive neurological registries will enhance the translational knowledge of COVID-19's CNS and PNS involvement and generate strategies for therapeutic decision-making.Registration: This review is registered in PROSPERO (CRD42020193140), July 24, 2020.


Author(s):  
Bonnie L. Bermas

Systemic lupus erythematosus (SLE) is a multisystem disease that preferentially affects women of childbearing age. This disorder is both more common and more severe in individuals of African and Asian ancestry. The etiology of SLE is not well understood, although genetics and environmental stimuli clearly are involved. Whether this disease is caused by a T-cell, B-cell, or other immunologic malfunction is debated, but all would agree that clearly autoantibodies such as antinuclear antibodies and anti–double-stranded DNA contribute to the pathophysiology of this disorder. This multisystem disease can affect the skin, joints, lungs, heart, kidneys, and central nervous system. Most of the morbidity and mortality is from renal and central nervous system (CNS) involvement, although accelerated atherosclerosis has recently been appreciated as a major contributor to disease burden. The treatment of SLE has improved over the past decade with less reliance on high-dose corticosteroids and more emphasis on immunosuppressive agents. It is our hope that future research into the pathophysiology of this disorder and the development of more specific therapy, such as biologics, will improve the outcome of this disease.


Author(s):  
Chunquan Cai ◽  
Changhong Shen ◽  
Weidong Yang ◽  
Qingjiang Zhang ◽  
Xiaoli Hu

Background:Neurenteric cysts are rare congenital epithelium-lined cysts of the central nervous system. They are found predominantly in the spinal cord, with lower incidence in the intracranial compartment, and may be associated with various other congenital spinal anomalies. Seven patients with symptomatic intraspinal neurenteric cysts are presented.Materials and Methods:Seven patients with intraspinal neurenteric cysts aged from nine months to ten years treated at this hospital from May 2000 to July 2006 were reviewed. The clinical manifestations, imaging and surgical findings of patients were analyzed retrospectively. All patients underwent operation. One patient's cervical neurenteric cyst was resected using the lateral cervical approach, and the other six resections were performed with posterior approach.Results:All seven patients presented with neurological involvement. One patient had an intramedullary cyst, while the other six cysts were situated ventrally. Three patients' cysts occurred in the cervical region, two in the cervicothoracic region, one in the thoracic region and one in the lumbar region. One patient had bony anomalies, and one had a lumbar posterior occult spinal dysraphism. Five patients' symptoms improved rapidly after surgery.Conclusions:Intraspinal neurenteric cysts in children are rare and most occur ventral to the spinal cord. Magnetic resonance imaging (MRI) is the most effective imaging modality. Earlier diagnosis and surgical resection of spinal neurenteric cysts improves prognosis.


Author(s):  
Alvin Oliver Payus ◽  
Constance Liew Sat Lin ◽  
Malehah Mohd Noh ◽  
Mohammad Saffree Jeffree ◽  
Raymond Azman Ali

The novel coronavirus disease 2019 (COVID-19) is caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which is believed to have emerged from an animal source and has been spreading rapidly among humans. Recent evidence shows that SARS-CoV-2 exhibits neurotropic properties and causes neurological diseases. Here we review the literature on neurological involvement in SARS-CoV-2 infections and the possible mechanisms of invasion of the nervous system by this virus, to provide a summary and critical analysis of the early reporting of neurological involvement in COVID-19. An exhaustive search of scientific articles on neurological involvement in COVID-19 was performed in the Web of Science, Scopus, Medline/PubMed, and several other databases. Nineteen relevant articles that had been published or were in preprint were carefully selected according to the inclusion and exclusion criteria. Based on our research, we found that patients with COVID-19 can present with neurological symptoms that can be broadly divided into central nervous system involvement, such as headache, dizziness, altered mental state, and disorientation, and peripheral nervous system involvement, such as anosmia and hypogeusia. Most of these patients are in the older age group and exhibit comorbidities, especially hypertension, and severe infection. In extreme presentations of COVID-19, some patients exhibit seizures, stroke, flaccid paraparesis, corticospinal weakness, and even coma. Moreover, the neurological manifestations can occur independently of the respiratory system. In conclusion, SARS-CoV-2 infection can cause multiple neurological syndromes in a more complex presentation. Therefore, this review elucidated the involvement of the nervous system in SARS-CoV-2 infection and will hopefully help improve the management of COVID-19.


2017 ◽  
Vol 41 (S1) ◽  
pp. S489-S490
Author(s):  
U. Cikrikcili ◽  
B. Saydam ◽  
M. Aktan

Primary central nervous system lymphoma (PCNSL) is a high-grade malignant B-cell non-Hodgkin neoplasm that is an infrequent variant of all intracranial neoplasms (1%) and all lymphomas (< 1%)PCNSL is documented mainly in immunocompromised patient groups, although it may also be diagnosed in immunocompetent patients. It affects mainly the eyes, supratentorial areas, or the spinal cord. The lesions are typically localized in frontal lobes, corpus callosum and basal ganglia. Additionally, lesions might rarely be detected at infratentorial areas and in medulla spinalis. Even though a wide spectrum of treatment options are available, such as chemotherapy, radiotherapy, or surgery; response rates are low and prognosis is poor in spite of appropriate treatment.The case we reported here is 57-year-old male presented with symptoms of aggresivity, impulsivity, depressive mood and personality changes. Histopathological diagnosis was CD5 positive diffuse large B cell lymphoma, which is very rare in high-grade lymphomas. There were no neurological signs related to CNS tumor and the clinical manifestations responded very well to chemotherapy consisting of high dose methotrexate, vincristine and procarbazine. The significance of such neuropsychiatric symptoms in the course of treatment for PCNSL has been previously documented as well. These behavioral and emotional symptoms might manifest themselves based on where the neoplasm is localized. Therefore, psychiatrists should be more aware of the uncommon manifestation of the disorder as reported in this case. Consultation for differential diagnosis might also be necessary in such cases.Disclosure of interestThe authors have not supplied their declaration of competing interest.


2021 ◽  
Vol 11 (3) ◽  
pp. 410-429
Author(s):  
Mandeep Singh Rahi ◽  
Jay Parekh ◽  
Prachi Pednekar ◽  
Gaurav Parmar ◽  
Soniya Abraham ◽  
...  

Radiotherapy plays an important role in the treatment of localized primary malignancies involving the chest wall or intrathoracic malignancies. Secondary effects of radiotherapy on the lung result in radiation-induced lung disease. The phases of lung injury from radiation range from acute pneumonitis to chronic pulmonary fibrosis. Radiation pneumonitis is a clinical diagnosis based on the history of radiation, imaging findings, and the presence of classic symptoms after exclusion of infection, pulmonary embolism, heart failure, drug-induced pneumonitis, and progression of the primary tumor. Computed tomography (CT) is the preferred imaging modality as it provides a better picture of parenchymal changes. Lung biopsy is rarely required for the diagnosis. Treatment is necessary only for symptomatic patients. Mild symptoms can be treated with inhaled steroids while subacute to moderate symptoms with impaired lung function require oral corticosteroids. Patients who do not tolerate or are refractory to steroids can be considered for treatment with immunosuppressive agents such as azathioprine and cyclosporine. Improvements in radiation technique, as well as early diagnosis and appropriate treatment with high-dose steroids, will lead to lower rates of pneumonitis and an overall good prognosis.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Juan I. Guerrero ◽  
Luis A. Barragán ◽  
Juan D. Martínez ◽  
Juan P. Montoya ◽  
Alejandra Peña ◽  
...  

Abstract Background SARS-CoV-2 can affect the human brain and other neurological structures. An increasing number of publications report neurological manifestations in patients with COVID-19. However, no studies have comprehensively reviewed the clinical and paraclinical characteristics of the central and peripheral nervous system’s involvement in these patients. This study aimed to describe the features of the central and peripheral nervous system involvement by COVID-19 in terms of pathophysiology, clinical manifestations, neuropathology, neuroimaging, electrophysiology, and cerebrospinal fluid findings. Methods We conducted a comprehensive systematic review of all the original studies reporting patients with neurological involvement by COVID-19, from December 2019 to June 2020, without language restriction. We excluded studies with animal subjects, studies not related to the nervous system, and opinion articles. Data analysis combined descriptive measures, frequency measures, central tendency measures, and dispersion measures for all studies reporting neurological conditions and abnormal ancillary tests in patients with confirmed COVID-19. Results A total of 143 observational and descriptive studies reported central and peripheral nervous system involvement by COVID-19 in 10,723 patients. Fifty-one studies described pathophysiologic mechanisms of neurological involvement by COVID-19, 119 focused on clinical manifestations, 4 described neuropathology findings, 62 described neuroimaging findings, 28 electrophysiology findings, and 60 studies reported cerebrospinal fluid results. The reviewed studies reflect a significant prevalence of the nervous system’s involvement in patients with COVID-19, ranging from 22.5 to 36.4% among different studies, without mortality rates explicitly associated with neurological involvement by SARS-CoV-2. We thoroughly describe the clinical and paraclinical characteristics of neurological involvement in these patients. Conclusions Our evidence synthesis led to a categorical analysis of the central and peripheral neurological involvement by COVID-19 and provided a comprehensive explanation of the reported pathophysiological mechanisms by which SARS-CoV-2 infection may cause neurological impairment. International collaborative efforts and exhaustive neurological registries will enhance the translational knowledge of COVID-19’s central and peripheral neurological involvement and generate therapeutic decision-making strategies. Registration This review was registered in PROSPERO 2020 CRD42020193140 Available from: https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42020193140


2019 ◽  
Vol 11 (3) ◽  
pp. 104-109
Author(s):  
M. S. Pushkaryov ◽  
L. M. Tibekina ◽  
L. P. Churilov

The review gives data on the etiology, pathogenesis, and clinical manifestations of sarcoidosis, which are associated particularly with damage to the central and peripheral nervous system. Sarcoidosis is currently regarded as a genetically determined, polyetiologic, and multisystem, disease of mainly autoimmune origin, which is accompanied by a productive inflammatory response with the formation of space-occupying noncaseous granulomas. The detection rates for the classical forms of sarcoidosis of the lungs, lymph nodes, and eyes are increasing, while the intravital diagnosis of neurosarcoidosis is still difficult. The latter mimics many other neurological diseases, frequently in persistent disabling disorders and death. Studies focus on searching for imaging, biological and/or immune markers that can reliably diagnose this disease. The problem of small fiber neuropathy that is considered to be the most common and early manifestation of neurosarcoidosis is being actively studied. In 2018, Neurosarcoidosis Consortium Consensus Group (NCCG), USA, proposed the diagnostic criteria for neurosarcoidosis, which are presented in this article.


Lupus ◽  
2009 ◽  
Vol 18 (10) ◽  
pp. 884-888 ◽  
Author(s):  
G Zandman-Goddard ◽  
M Blank ◽  
Y Shoenfeld

This article is an update on the clinical and research data available on systemic lupus erythematosus (SLE) and intravenous immunoglobulin (IVIg) therapy that includes some studies performed under the umbrella of the European Working Party on SLE. Various mechanisms of IVIg may play a role, some synergistically, in the modulation of SLE. Recently it has been suggested that IVIg also suppresses the expansion of autoreactive B lymphocytes through signalling of the FcgRIIB, idiotype-mediated inhibition of B cell receptors and neutralisation of cytokines such as the B cell survival factors (B cell activation factor (BAFF and APRIL). In case reports and in open trials, high-dose IVIg (2 g/kg over a 5-day period) has consistently been shown to be a beneficial and safe adjunct therapeutic agent for over 20 manifestations in patients with SLE. It can be given as a first choice of therapy in some cases, for example, in neurological involvement and in those patients who refuse certain immunosuppressive agents such as cyclophosphamide, or in patients who have concomitant infections. Furthermore, IVIg may have a steroid-sparing effect although this characteristic needs further investigation. Specific IVIg (an anti-idiotype to anti-DNA, phosphorylcholine and antiphospholipids) has been shown to be effective in experimental murine models. Hence, extractable IVIg that is directed to the specific pathogenic immunoglobulins will enable the more specific therapy for patients with lupus.


2007 ◽  
Vol 49 (5) ◽  
pp. 279-287 ◽  
Author(s):  
Josef Finsterer ◽  
Herbert Auer

Infection of humans with embryonated eggs of Toxocara canis (larva migrans) remains asymptomatic, or results in covert or common toxocarosis, visceral larva migrans syndrome, or ophthalmologic and neurologic impairment. Though neurological manifestations of Toxocara canis larvae are rare, toxocarosis remains an important differential diagnosis of various neurological disorders. Manifestations of the central nervous system are dementia, meningo-encephalitis, myelitis, cerebral vasculitis, epilepsy, or optic neuritis. Manifestations of the peripheral nervous system comprise radiculitis, affection of cranial nerves, or musculo-skeletal involvement. If toxocarosis is neglected, ignored, or refused as a differential of these abnormalities, it may be easily overlooked for years. Early recognition and treatment of the infection is, however, of paramount importance since it reduces morbidity and mortality and the risk of secondary superinfection. Like the visceral manifestations, neurological manifestations of toxocarosis are treated by benzimidazole components, most frequently albendazole, corticosteroids, or diethylcarbamazine. If detected and treated early, the prognosis of neurological manifestations of toxocarosis is favourable.


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