Paraneoplastic Disorders of the Central Nervous System: Update on Diagnostic Criteria and Treatment

Bataller, Luís; Dalmau, Josep

doi:10.1055/s-2004-861540

Cited by 61 publications

(21 citation statements)

References 66 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Therefore, it is important considering that the detection of an onconeural antibody indicates the presence of an underlying tumour. Successful treatment of the tumour is the main factor associated with stabilisation or improvement of PNS [12,13], such as we report in the present case. However, this response is not always associated with a change in the levels of the onconeural antibodies, as previously reported [14].…”

supporting

confidence: 65%

Isolated paraneoplastic optic neuropathy associated with small-cell lung cancer and anti-CV2 antibodies

et al. 2007

View full text Add to dashboard Cite

supporting

confidence: 65%

Isolated paraneoplastic optic neuropathy associated with small-cell lung cancer and anti-CV2 antibodies

et al. 2007

View full text Add to dashboard Cite

“…For this reason, orchidectomy may be necessary in face of clinical deterioration, de novo testicular enlargement, previous cryptorchidism or ultrasound evidence of testicular microcalcification, in the absence of other tumours 8 . MRI may show contrast enhancement 13 . CV2 -the presence of chorea 14 is highly suggestive.…”

Section: Antibodies To Intracellular Antigensmentioning

confidence: 99%

What should you know about limbic encephalitis?

Machado¹,

Pinto²,

Irani

2012

Arq. Neuro-Psiquiatr.

View full text Add to dashboard Cite

Limbic encephalitis was firstly described by Brierley in 1960 1 and was characterized as an inflammatory disorder involving the hippocampi, amygdala, frontobasal and insular regions, with a spectrum of symptoms, most commonly characterized by a subacute progressive impairment of short-term memory, psychiatric features and seizures. While in some cases it appears to exclusively involve limbic regions, it has become clear that several clinical features implicate involvement of areas other than the limbic system. For this reason, the authors prefer the term autoimmune encephalitis (AIE).Once regarded as a rare and paraneoplastic disorder with a poor prognosis, most forms of AIE are now recognised as being non-paraneoplastic and a substantial proportion of them may have a good response to immunotherapy, particularly if it is promptly initiated.Due to a broad differential diagnosis, the recognition of AIE is frequently difficult and delayed. The aim of this article was to facilitate recognition of the clinical features and to simplify the diagnostic approach, with the ultimate objective of rapid immunotherapy administration. CLINICAL VIGNETTEA 52-year-old, right handed, man presented to the Accident and Emergency (A&E) department after two generalized tonic-clonic seizures. He was previously healthy and was the third of four healthy siblings with no problems during development or birth. He was successful at school and worked as a lawyer. There was no previous history of epilepsy, infections of the central nervous system (CNS), trauma, substance abuse or smoking. He was a moderate consumer of alcohol and was taking no medications. There was no relevant family history. ABSTRACTAutoimmune encephalitis is an inflammatory disorder characterized by a subacute impairment of short-term memory, psychiatric features and seizures. It is often associated with a variety of other neurological symptoms, and its differential diagnosis is wide, leading to challenges in its recognition. It used to be regarded as a rare disease, usually paraneoplastic and with poor prognosis. However, with the recent recognition of membrane-surface directed antibodies, it is now known that in a substantial proportion of cases there is no association with any malignancy and there is a good prognosis if treated. Hence, early recognition and prompt initiation of immunotherapies are of great importance.Key words: limbic autoimmune encephalitis, encephalopathy, intracellular antigens, membrane surface antigens, VGKC-complex, LGI1, CASPR2, N-methylaspartate, paraneoplastic syndrome, immunotherapy. RESUMOA encefalite autoimune é uma doença inflamatória caracterizada por envolvimento subagudo da memória de curto prazo, presença de sintomas psicóticos e crises epilépticas. Dada a diversidade de sintomas na apresentação, o diagnóstico diferencial é um verdadeiro desafio. Anteriormente, era considerada uma doença rara, de etiologia paraneoplásica e com mau prognóstico. No entanto, com a recente descoberta dos anticorpos dirigidos à superfície da membrana, é atua...

show abstract

“…Although there have not been in vivo studies revealing neuronal cell death due to infiltration with onconeural antibodies against putative IAg, in vitro studies have shown somewhat contradictory results, with earlier studies demonstrating accessed cultured neurons without damage [43] and later ones demonstrating induction of cell death after antibody uptake [44]. A major concern in managing these disorders has not only been prompt treatment of the tumor, but commencement of effective immunotherapy targeting mainly cytotoxic T-cells [45]. Vasculitis has not been a recognized mechanism of injury in intraneuronal antibodies, either in life or at postmortem examination.…”

mentioning

confidence: 99%

Autoimmune Encephalitides

Younger¹

2017

WJNS

View full text Add to dashboard Cite

Autoimmune encephalitis is a potentially severe disorder of the brain of diverse causes with a complex differential diagnosis. Recent advances in the past decade have led to the elucidation of new syndromes and biological markers transforming the approach to diagnosis and management of autoimmune encephalitis. Limbic encephalitis, the commonest form of autoimmune encephalitis, combines common presentations of cognitive, psychiatric, and epileptic disorders and has until recently been considered paraneoplastic or postinfectious in origin. The autoimmune encephalitides are clinically and histopathologically associated with serum and intrathecal antibodies to intracellular and surface neuronal antigens, and constituents of the limbic system neuropil. This has led to a reconsideration of a number of neuropsychiatric and neurocognitive disorders as having shared mechanisms of origin. This chapter reviews their historical background, clinical presentation, laboratory evaluation, histopathology, diagnosis and management. KeywordsAutoimmune, Encephalitis, Hashimoto, Encephalopathy Historical PerspectiveCorsellis and colleagues [1] coined the term limbic encephalitis (LE) in 1968, noting a relation to bronchial cancer in three patients in the sixth to eighth decade of life, and showing close clinicopathologic similarity to cases described by Brierley and colleagues [2] six years earlier. All three cases had subacute temporal lobe seizures, neuropsychiatric, and memory disturbances for two years before death. Postmortem examination showed inflammatory lesions in limbic grey matter sections of the brain, especially in medial temporal lobe structures of the uncus and amygdala nuclei, and hippocampal, cingulum and dentate gyri. Case 2 had an undifferentiated non-metastatic lung carcinoma removed six months after onset of neurological symptoms, while two others had a clinically unsus- The formulation of the relation of GAD antibodies to SPS has been especially instructive in understanding how far the science of autoimmune neurologic disorder has advanced. In 1988, Solimena and colleagues [28] investigated the existence of non-paraneoplastic CNS autoimmunity in a patient with SPS, epilepsy and type-1 diabetes (T1D), and increased titers of oligoclonal CSF IgG. Both the serum and CSF produced identical intense staining of all gray-matter regions. GAD65 was thence an important autoantigen in T1D, being highly expressed in the cytoplasm of pancreatic β cells. GAD-derived peptides were presentable by main histocompatibility complex (MHC) class I molecules and recognized by CD8+ cells on the surface of β cells [29]. Activation of CD8+ GAD-specific T-cells further differentiated into memory cells suggesting a pathogenic role in T1D [30]. However, only patients with very high titers of GAD were associated with LE [31], and they typically presented with recent-onset temporal lobe epilepsy (TLE) and intrathecal secretion, defining a form of non-paraneoplastic LE. Other patients within the SPS spectrum harbored antibodies agains...

show abstract

Paraneoplastic Disorders of the Central Nervous System: Update on Diagnostic Criteria and Treatment

Cited by 61 publications

References 66 publications

Isolated paraneoplastic optic neuropathy associated with small-cell lung cancer and anti-CV2 antibodies

Isolated paraneoplastic optic neuropathy associated with small-cell lung cancer and anti-CV2 antibodies

What should you know about limbic encephalitis?

Autoimmune Encephalitides

Contact Info

Product

Resources

About