Nervous system

Author(s):  
Andrew Graham ◽  
Clare Galton

Rheumatological conditions may be complicated by a variety of both central and peripheral nervous system disorder. Common complications such as entrapment neuropathies are familiar to rheumatologists but accurate diagnosis of less common neurological disorders may be challenging; careful clinical reasoning is essential, supplemented where necessary by imaging, neurophysiology, and other special investigations including cerebrospinal fluid examination. Complications vary according to the nature of background condition. In rheumatoid arthritis, neurological involvement is typically related to the mechanical consequences of advancing disease; the commonest complications are carpal tunnel syndrome and cervical myelopathy due to atlantoaxial subluxation. By contrast, neurological involvement in systemic lupus erythematosus (SLE) tends to occur earlier in the disease course, with a much wider range of manifestations. The management of stroke or seizures in SLE is not necessarily any different from that in the general population, unless complicated by the antiphospholipid syndrome. However, less common neurological syndromes may demand more specific investigation and treatment. For example, longitudinally extensive transverse myelitis and recurrent optic neuritis (neuromyelitis optica, or Devic's disease) is frequently associated with antibodies to aquaporin-4, and is highly likely to relapse unless treated vigorously with humoral immunosuppression. Nervous system involvement in vasculitis is common. Finally, not all neurological disorder in rheumatological disease is necessarily due to the underlying condition; neurological complications of disease-modifying therapy are increasingly recognized, in particular central and peripheral nervous system demyelination associated with TNF-α‎ inhibitors.

Author(s):  
Andrew Graham ◽  
Clare Galton

Rheumatological conditions may be complicated by a variety of both central and peripheral nervous system disorders. Common complications such as entrapment neuropathies are familiar to rheumatologists but accurate diagnosis of less common neurological disorders may be challenging; careful clinical reasoning is essential, supplemented where necessary by imaging, neurophysiology, and other special investigations including cerebrospinal fluid examination. Complications vary according to the nature of the background condition. In rheumatoid arthritis, neurological involvement is typically related to the mechanical consequences of advancing disease; most commonly, entrapment neuropathies such as carpal tunnel syndrome and cervical myelopathy due to atlantoaxial subluxation. By contrast, neurological involvement in systemic lupus erythematosus (SLE) tends to occur earlier in the disease course, with a much wider range of manifestations. The management of stroke or seizures in SLE is not necessarily any different from that in the general population, unless complicated by the antiphospholipid syndrome. However, less common neurological syndromes may demand more specific investigation and treatment. For example, longitudinally extensive transverse myelitis and recurrent optic neuritis (neuromyelitis optica, or Devic’s disease) is frequently associated with antibodies to aquaporin-4, and is highly likely to relapse unless treated vigorously with humoral immunosuppression. Nervous system involvement in vasculitis is common. Finally, not all neurological disorder in rheumatological disease is necessarily due to the underlying condition; neurological complications of disease-modifying therapy are increasingly recognized, in particular central and peripheral nervous system demyelination associated with TNF-α‎‎ inhibitors.


2011 ◽  
Vol 30 (1) ◽  
pp. 1-4 ◽  
Author(s):  
Inimioara Cojocaru ◽  
Manole Cojocaru ◽  
Isabela Silosi ◽  
Camelia Vrabie

Central Nervous System Manifestations in Rheumatic DiseasesPatients with multi-system rheumatic conditions may have a disease affecting the central nervous system (CNS). Central nervous system manifestations vary according to the location of the lesion and range from focal findings (e.g., stroke-like presentations), although serious neurological complications in rheumatic disease appear to be rare. The most prominent features of neurological involvement in rheumatic diseases include cerebral ischaemia and psychiatric symptoms. Little information is available on the prevalence of neurological disease in patients with a rheumatological diagnosis. Involvement of the CNS may be a striking early or presenting feature with a wide variety of manifestations. There is more clarity about the CNS syndromes attributable to systemic lupus erythematosus and new insights into the central mechanisms involved in the manifestations of Sjögren's syndrome and rheumatoid arthritis. Severe CNS involvement is associated with poor prognosis, and high mortality rate. We review the spectrum of neurological diseases in patients with a rheumatological diagnosis.


2010 ◽  
pp. 5158-5165
Author(s):  
Neil Scolding

Primary neuroimmune disorders such as multiple sclerosis or the Guillain–Barré syndrome are well recognized (and described elsewhere in this section), but there are numerous diverse systemic inflammatory, infective, or immunological disorders that can affect the nervous system. Autoimmune rheumatic disorders—(1) Systemic lupus erythematosus—neurological manifestations include headache, acute or subacute encephalopathy, fits, myelitis, strokes and movement disorders (including chorea and other extrapyramidal disorders), ataxia and brainstem abnormalities, cranial and peripheral neuropathies, and psychiatric and cognitive disturbances. Risk of stroke is particularly associated with the lupus anticoagulant and the primary antiphospholipid syndrome. (2) Other conditions—(a) rheumatoid arthritis: mononeuritis, cervical cord compression; (b) Sjögren’s syndrome: sensory neuropathy, myositis, various central nervous system complications; (c) Reiter’s disease: polyneuritis, radiculitis, various central nervous system manifestations....


Author(s):  
Sathiji Nageshwaran ◽  
Heather C Wilson ◽  
Anthony Dickenson ◽  
David Ledingham

Primary neuroinflammatory disorders of the central nervous system (multiple sclerosis, neuromyelitis optica (NMO), transverse myelitis, optic neuritis, acute disseminated encephalomyelitis (ADEM), primary angiitis of the central nervous system, autoimmune limbic encephalitis, and Susac’s syndrome) and multisystem diseases with inflammatory involvement of the central nervous system (sarcoidosis, systemic lupus erythematosus (SLE), giant cell arteritis, Behçet’s disease, Sjögren’s, and other vasculitides) are discussed in depth, covering the aetiology, clinical features and evidence-based treatment.


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):  
Christopher Woldstad ◽  
Michael Boska ◽  
Howard E. Gendelman

This chapter serves to highlight both the research advances made in understanding the effects of HIV on the nervous system and what lies ahead. Particular focus is given to both the effects HIV can play on the nervous system at the molecular and cellular levels and the comorbid conditions that affect neural function. Attention is also given to specific biomarkers to be used for increasing the effectiveness and availability of therapies. The pathogenesis of HIV-associated neurocognitive disorders (HAND) is comparable to that of several other neurodegenerative disorders, and their mechanistic similarities are also discussed in detail. With the introduction of antiretroviral therapy the life expectancy of persons with HIV has increased, with a concomitant decrease in the incidence of severe dementia. There has been a remarkable improvement in cognitive function with almost a complete reversal of associated symptoms of disease. Past and present disease manifestations and the implications for treatment are outlined in the chapter.


Author(s):  
Aaron E. Miller ◽  
Teresa M. DeAngelis

Rheumatoid arthritis (RA) is a systemic inflammatory disease that is characterized principally by a polyarthritis, but can result in several neurologic complications involving both the central and peripheral nervous system. In addition, several immunotherapies used to treat RA have been associated with neurological complications. In this chapter, we review the characteristic neurological sequelae of RA as well as the possible neurological consequences of its therapeutic regimens.


2021 ◽  
Vol 9 ◽  
Author(s):  
Chengbing Tan ◽  
Yan Jiang ◽  
Min Zhong ◽  
Yue Hu ◽  
Siqi Hong ◽  
...  

Background: Contactin-associated protein-like 2 (CASPR2) neurological autoimmunity has been associated with various clinical syndromes involving central and peripheral nervous system. CASPR2 antibody-associated autoimmune encephalitis is mostly reported in adults. Analysis of the clinical presentation and prognostic data of CASPR2 antibody-associated autoimmune encephalitis in children remains important.Methods: A single-center retrospective review of children diagnosed with CASPR2 antibody-associated autoimmune encephalitis from June 1st, 2018 to October 31st, 2020.Results: Six patients were identified. The median age was 12 years (range 1.8–14), with an overall male predominance of 83% (5/6). Commonest clinical features were psychiatric symptoms (6/6), movement disorders (4/6), altered consciousness (3/6), sleep disorders (3/6), and headache (3/6). Four patients (4/6) received first-line therapy alone (steroids combined with intravenous immunoglobulins), and two patients (2/6) received second-line therapy (rituximab, mycophenolate mofetil, or cyclophosphamide). All patients showed no peripheral nervous system involvement. One patient had comorbidities with systemic lupus erythematosus. No evidence of neoplastic disease was found in the whole cohort. All patients had favorable outcomes (modified Rankin Score 0–2) with recurrence rate at 0%, respectively.Conclusion: CASPR2 antibody-associated autoimmune encephalitis is rare in children. Our findings suggest that this type of encephalitis seems to occur more frequently in older children. Patients respond well to immunotherapy and usually demonstrate a favorable clinical outcome. Associated tumors are extremely rare.


Author(s):  
Adam Fisch

Chapter 6 discusses how to draw the peripheral nervous system, specifically the autonomic nervous system, including autonomic fiber arrangements, the parasympathetic nervous system, the sympathetic nervous system, the urinary system, and the cardiac reflex.


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