Update on Paraneoplastic Neurologic Disorders

Rosenfeld, Myrna R.; Dalmau, Josep

doi:10.1634/theoncologist.2010-0001

Cited by 28 publications

(22 citation statements)

References 122 publications

(140 reference statements)

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“…6 Prognosis is generally favorable, as 80% of patients respond to immunotherapy with improvement in memory and executive functions. 7,8 Cancer is rarely reported with LGI1 antibodies, and a series of 55 patients with confirmed LGI1 antibodies revealed no cancer after a median follow-up of 3 years 9 ; therefore, the utility of cancer screening in these patients is questionable. Though evidence is limited as to the optimal treatment regimen, most patients respond well to initial treatment with corticosteroids, plasma exchange, or IVIg, with maintenance options including corticosteroids or steroid-sparing agents such as mycophenolate, rituximab, or cyclophosphamide.…”

Section: Discussionmentioning

confidence: 99%

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Clinical Reasoning: A 72-year-old man with rapid cognitive decline and unilateral muscle jerks

Duncan¹,

Cholfin²,

Restrepo³

2014

Neurology

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A 72-year-old man presented with cognitive decline and unilateral muscle jerks. Three months prior to presentation, the patient suddenly developed violent muscle jerks involving the right side of his body and face that impaired his gait and balance. Approximately 1 week later, he acutely developed confusion and memory loss. Over the following weeks, he experienced fluctuating symptoms of confusion, memory impairment, insomnia, and paranoid delusions. His muscle jerks and unstable gait were intermittent with return to baseline in between attacks, but they increased in frequency and occurred many times throughout the day. He was found to be mildly hyponatremic and was eventually admitted to a psychiatric ward for treatment of acute psychosis.The patient's medical history was significant for hypertension, well-controlled diabetes, and a myocardial infarction 22 years previously. He was a retired mechanical engineer and was physically active prior to the onset of symptoms.On neurologic examination, the patient was alert and oriented to person only. He registered 3 items but was unable to recall them at 5 minutes and was unable to complete serial 7s. He had no language deficits and could follow 3-step commands without difficulty. His cranial nerve, motor, and sensory examination results were normal. He had a wide-based gait with prominent right lateral pulsion and retropulsion, without any observed muscle jerks during gait examination. Occasional myoclonus involving the right side of his face and right upper extremity were observed, which were associated with loss of awareness and dystonic posturing of the right arm.The patient was admitted to the general neurology ward and an MRI of the brain was performed (figure).

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Section: Discussionmentioning

confidence: 99%

“…Though evidence is limited as to the optimal treatment regimen, most patients respond well to initial treatment with corticosteroids, plasma exchange, or IVIg, with maintenance options including corticosteroids or steroid-sparing agents such as mycophenolate, rituximab, or cyclophosphamide. 6,7,10 …”

Section: Discussionmentioning

confidence: 99%

Clinical Reasoning: A 72-year-old man with rapid cognitive decline and unilateral muscle jerks

Duncan¹,

Cholfin²,

Restrepo³

2014

Neurology

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“…Наличие анти-Yo и анти-Tr АТ практически всегда сочетается с подострой дегенера-цией мозжечка вследствие избирательного поражения клеток Пуркинье. Онкологический процесс в этих случа-ях обычно локализуется в яичниках, молочных железах [16]. Другими хорошо изученными АТ являются антитела к Mа2-антигенам, появление которых характерно при лимбическом и стволовом энцефалитах, церебеллите [1].…”

Section: аэ развивающиеся в связи с онкологическим процессомunclassified

Autoimmune encephalitis

Davydovskaya¹,

Boyko²,

Beliaeva³

et al. 2015

Z. nevrol. psikhiatr. im. S.S. Korsakova

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ОБЗОРЫАутоиммунные энцефалиты (АЭ) -неврологические заболевания, характеризующиеся поражением преиму-щественно серого вещества, в патогенезе которых основ-ную роль играют аутоантитела (АТ) к внутри-и/или вне-клеточным структурам нервной системы, выступающие в качестве антигенов [1,2]. Среди этих заболеваний выде-ляют паранеопластические неврологические синдромы, при которых поражение нервной системы связано с теку-щим онкологическим процессом [1,3], и недавно выде-ленные в отдельную группу идиопатические АЭ, при ко-торых поражение нервной системы может происходить в отсутствие онкологии [2]. Патогенез АЭПатогенез АЭ в значительной степени связан с лока-лизацией структур нервной клетки, которые выступают в В обзоре рассмотрены вопросы патогенеза, клинических проявлений, диагностики и лечения аутоиммунных энцефалитов. Показано, что развитие аутоиммунных энцефалитов может быть связано с онкологическим процессом или иметь идиопатический характер. В основе патогенеза аутоиммунных энцефалитов лежит выработка антител, которые непосредственно или опосредованно (через Т-клеточный механизм) повреждают вне-и/или внутриклеточные структуры нервных клеток. Выявление в цереброспинальной жидкости (ЦСЖ) при аутоиммунных энцефалитах антител к внутриклеточным структурам нейронов в абсолютном большинстве случаев (>95%) отражает сопутствующий онкологический процесс, а выявление антител к мембранам или синапсам нейронов может быть не связано с онкологическим процессом. Диагностический алгоритм при аутоиммунных энцефалитах наряду с проведением комплексного обследования, включающего нейровизуализацию, ЭЭГ, исследование ЦСЖ и определение антител, должен включать поиск онкологического очага. Терапевтический алгоритм при аутоиммунных энцефалитах предусматривает комбинированное назначение иммуносупрессивной терапии, плазмафереза, иммуноглобулинов, цитостатиков, а также лечение онкологического процесса. Ключевые слова: аутоиммунные энцефалиты, паранеопластические синдромы, онкология, антитела.The authors consider the issues related to pathogenesis, clinical features, diagnosis and treatment of autoimmune encephalitis. It has been demonstrated that the development of autoimmune encephalitis can be associated with the oncologic process or be of idiopathic character. The pathogenesis of autoimmune encephalitis is caused by the production of antibodies that directly or indirectly (via T-cell mechanism) damage exo-and/or endocellular structures of the nerve cells. The presence of antobodies to endocellular structures of neurons in the cerebrospinal fluid of patients with autoimmune encephalitis in the vast majority of cases (>95%) indicates the concomitant oncologic process, the presence of antibodies to membranes or neuronal synapses can be not associated with the oncologic process. Along with complex examination, including neuroimaging, EEG, cerebrospinal fluid and antibodies, the diagnostic algorithm in autoimmune encephalitis should include the search for the nidus of cancer. The treatment algorithm in autoimmune encephalitis included the co...

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“…Widespread muscle pains and painful muscle spasms are observed (Rosenfeld and Dalmau, 2010). Continuous motor activity is observed in EMG.…”

Section: Stiff-person Syndromementioning

confidence: 99%

“…Patients developed it as paraneoplastic are usually women with anti-amphiphysin positive breast cancer (Folli et al, 1993;Petzold et al, 2004). Treatment primarily targets the underlying disease, however corticosteroids, IVIG, diazepam and GABAergic agents may also be used (Rosenfeld and Dalmau, 2010).…”

Section: Stiff-person Syndromementioning

confidence: 99%

Paraneoplastic neurologic disorders

Şen¹,

Terzı²

2013

jecm

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IntroductionParaneoplastic neurologic syndromes (PNS) are manifestations of cancers which are located outside the central nervous system. They appear before the diagnosis of cancer in most of patients. Identification of these disorders is very important as it leads to diagnosis and treatment of cancer subsequently. PNS are divided into two groups as classical and non-classical. Classical PNS are associated with cancer more than non-classical PNS. Non-classical PNS are rather observed in cases without cancer. PNS can also be divided into two groups as per the involvement of the central and peripheral nervous system (Table 1) (Bradley, 2008;Tüzün, 2010). EpidemiologyPNS are rare disorders in cancer patients. Frequency in all cancer patients is 0.01% (Darnell and Posner, 2003). Women/ men ratio is approximately 3:1. It is usually observed beyond mid-life. They show a closer association with some kinds of cancer. They have a more frequent association especially with hematologic diseases such as small cell lung cancer (SCLC), thymoma, and monoclonal gammopathy. Other can-cer types associated with PNS include breast, gynecological cancers such as ovarian and Hodgkin's, non-Hodgkin's lymphoma, testicular cancer and neuroblastoma (Toothaker and Rubin, 2009). Furthermore, development of PNS is possible in almost all neoplasms (Bataller and Dalmau, 2003). PathogenesisA widely accepted opinion is that these disorders occur due to stimulation of immune response by some proteins which are formed by a tumor, against both the tumor and the nervous system (Tüzün, 2010). Cytotoxic T-cell responses have a very important role in pathogenesis of PNS (Bradley, 2008). T-cells are responsible for both of the antibody-mediated processes (Albert et al., 1998). Lymphocytic pleocytosis and oligoclonal band positivity may be observed in BOS analysis (Armstrong et al., 2005).PNS are formed by antibodies developed against the intracellular antigen or neuronal surface antigen. In limbic encephalitis, there are antibodies for both regions. Anti Hu, anti Yo, anti Ri, anti Ma2, Cv2/CRMP5 are antibodies associated with intracellular antigen, whereas the VGKC complex

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Update on Paraneoplastic Neurologic Disorders

Cited by 28 publications

References 122 publications

Clinical Reasoning: A 72-year-old man with rapid cognitive decline and unilateral muscle jerks

Clinical Reasoning: A 72-year-old man with rapid cognitive decline and unilateral muscle jerks

Autoimmune encephalitis

Paraneoplastic neurologic disorders

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