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Paraneoplastic syndromes are indirect manifestations of cancer due to functional peptides/hormones produced by a tumour, or due to cross reactivity between tumour and host antigens. Here the case of a 58-year-old woman presenting with ataxia, paraesthesia and subacute and progressive loss of vision is reported. The patient exhibited strong serum positivity for anti-Hu and anti-CV2 antibodies, and a chest CT scan showed a hypodense nodule in proximity of the right upper lobe bronchus and an enlarged ipsilateral paratracheal lymph node that was not visible on a lung x-ray. Histopathological examination of a biopsy specimen from this lymph node showed that small cell carcinoma of the lung was present. The patient's deficits were subsequently diagnosed as three coexisting paraneoplastic neurological syndromes (PNSs): subacute cerebellar ataxia, sensory neuropathy and retinopathy, respectively. Although rare, PNSs can be the first manifestations of cancer, and their rapid recognition facilitates an early treatment.

We herein report the case of a 58-year-old man with advanced esophageal carcinoma who developed posterior reversible encephalopathy syndrome (PRES). He initially presented with a severe consciousness disturbance. A subsequent examination revealed hypercalcemia and an elevated serum parathyroid hormone-related peptide (PTHrP) level. Magnetic resonance imaging performed on admission and 24 days later showed reversible widespread white matter abnormalities, which confirmed a diagnosis of PRES. The patient's clinical and radiological manifestations improved upon normalization of the serum calcium level. To the best of our knowledge, this is the first report describing hypercalcemia-induced PRES occurring in association with elevated PTHrP.

Langerhans cell histiocytosis (LCH) is a very rare disease in which granulation tissue forms in various organs and the central nervous system (CNS) due to monoclonal proliferation of Langerhans cells. Some patients develop ataxia, tremor, or neurodegenerative abnormalities (such as personality changes and mental deterioration) several years after the onset as the late effects of LCH. We report a case of a 4-year-old boy with LCH, showing speech disorder, truncal ataxia and a wide-based gait with abnormal findings of central nervous system in CT and MRI image. The results of auditory brain stem response revealed a conduction block in the auditory conduction pathway, suggesting an axonopathy of the brain stem. Disequilibrium may be due to brainstem dysfunction associated with paraneoplastic syndrome because an anti-GluRε2 antibody was seen. Paraneoplastic syndrome is a neuropathy induced through an autoimmune mechanism caused by an antibody directed against the nervous system. Neuro-otological examination is helpful for the assessment of CNS neurodegeneration associated with LCH., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Purpose of Review: This review describes relevant advances in paraneoplastic neurological syndromes (PNS) with emphasis on particular syndromes and the impact of antibodies against surface antigens in their management., Recent Findings: PNS may present with symptoms that do not raise the suspicion of a paraneoplastic origin. The best example is anti-N-methyl-D-aspartate receptor encephalitis that in adult women frequently associates with ovarian teratoma. An electroencephalogram pattern described as 'extreme delta brush' was recently identified in 30% of patients with this disorder. Isolated myelopathy may have a paraneoplastic origin associated with amphiphysin or CV2 (CRMP5) antibodies. Jaw dystonia and laryngospasm can be the predominant symptom of the brainstem encephalitis associated with Ri antibodies. γ-Aminobutyric acid (GABA)(B) receptor antibodies are the most common antibodies found in patients with limbic encephalitis and small cell lung cancer, and contactin-associated protein 2 antibodies in patients with Morvan's syndrome and thymoma. Lastly, a recent study identified delta/notch-like epidermal growth factor-related receptor (DNER) as the target antigen of Tr antibodies, a marker of cerebellar ataxia and Hodgkin's lymphoma., Summary: The number of antibodies relevant to PNS is now expanded to those against surface antigens. These antibodies do not confirm the paraneoplastic origin of the syndrome but predict a better response to immunotherapy.

The objective of this paper is to review the literature published in 2011 in the field of intensive care and emergency related to oncology. Are discussed because of new original publications: prognosis, resuscitation techniques, oncologic emergencies, serious toxicities of cytotoxic chemotherapy and targeted therapies, complicated aplastic anemia, toxicity of bisphosphonates, respiratory complications, pulmonary embolism and neurological complications.

Anti-SRP (signal recognition particle) positive necrotizing myopathy is commonly not associated with neoplasms. We demonstrate two histologically confirmed cases with unusual manifestations of anti-SRP positive necrotizing myopathy. A 65-year-old man presented with rapidly progressing weakness and mild difficulties in swallowing and speaking. Screening for underlying disorders revealed a moderately differentiated renal adenocarcinoma. The muscular symptoms partially improved after tumor nephrectomy and prednisone treatment. However, the patient developed pulmonary metastases and died of the sequelae of pneumonia 11 months after the diagnosis of renal cancer. The second patient developed rapidly complete external ophthalmoplegia, severe bulbar dysarthrophonia and dysphagia, bilateral facial palsy, loss of patellar and ankle jerk reflexes, and severe symmetrical tetraparesis of both proximal and distal muscles. CSF showed mildly increased protein levels, neurography axonal impairment of motor nerves. Screening revealed no evidence for infections, ganglioside antibodies, and carcinoma. MRI was normal. The disease course suggested an overlap syndrome of Miller-Fisher-syndrome, axonal Guillain-Barré-syndrome and Bickerstaff brainstem encephalitis. In conclusion SRP antibodies might be found in necrotizing myopathies associated with autoimmune mediated overlap syndromes and neoplasms. The pathomechanism is not clear. Any otherwise unexplained evidence of necrotizing myopathy should prompt the screening for SRP antibodies., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Cancer polyarthritis is a very rare condition. Here, we present a case of cancer polyarthritis, who also had mononeuritis simplex as a manifestation of paraneoplastic neuralgic syndrome. A 71-year-old man, who initially presented symmetrical polyarthritis and unilateral posterior interosseous nerve palsy, was subsequently diagnosed to have gastric cancer. Total gastrectomy was performed, and his polyarthritis and the palsy simultaneously disappeared within 2 weeks after the resection. His gastric cancer was found to be metastasized to his liver 16 months after the total gastrectomy; however, the polyarthritis and the palsy did not recurrent throughout his course. The polyarthritis in this case was diagnosed as a cancer polyarthritis from its features. On the other hand, the isolated posterior interosseous nerve palsy in this case met the diagnostic criteria for paraneoplastic neurological syndrome. This case was also unique in that the manifestations of paraneoplastic syndromes did not recur even after the metastasis of the primary cancer, suggesting that some specific clones in the cancer were responsive to the manifestations of paraneoplastic syndromes. Our case suggested that relapse of the manifestations of paraneoplastic syndromes may not always herald the recurrence of primary or metastatic tumour, and other tumour markers and signs should be periodically followed to search for the recurrence of the tumours.

Paraneoplastic neurological syndromes are defined as the remote effects of cancer on the nervous system. Here we report a 68-year-old man who initially presented with worsening paresthesia in the lower extremities. Although the culprit lesion remained to be identified, he coincidentally had diagnosis of prostate cancer by an annual prostate-specific antigen examination. Leukocytosis and elevated granulocyte colony-stimulating factor in serum were also detected. Neurological symptoms and leukocytosis improved after initiation of androgen-deprivation therapy followed by external beam radiotherapy. A total of 9 months after treatment, the patient showed no evidence of cancer recurrence or neurological signs. Paraneoplastic neurological syndromes are rare in prostate cancer and therefore have received little attention. We should be aware that when paraneoplastic neurological syndromes occur, they usually occur as the first sign of or during progression of prostate cancer. Furthermore, we should take into account the existence of malignancy when the cause of neurological symptoms cannot be specified., (© 2012 The Japanese Urological Association.)

Introduction: Cerebellar ataxia and stiff person-syndrome are the main neurological syndromes associated with antibodies to glutamic acid decarboxylase (GAD)., Case Report: A 59-year-old patient, with history of polymyalgia rheumatica and active smoking, was admitted for subacute cerebellar ataxia and memory dysfunction explained by limbic encephalitis on brain MRI. He also presented with orthostatic hypotension and erectile dysfunction revealing autonomic dysfunction. CSF was inflammatory and antibodies to GAD were positive. Onconeuronal antibodies including GABA(B) receptor antibodies were negative. Patient's condition quickly improved after intravenous immunoglobulins. A few months later, a small cell lung carcinoma was diagnosed and precociously treated., Conclusion: This case report underlines the importance of appropriate studies to confirm a primitive neoplasia, when confronted with limbic encephalitis and cerebellar ataxia, even if anti-GAD antibodies rarely define paraneoplastic syndromes., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

A 63-year-old woman had anti-Hu-associated paraneoplastic encephalomyelitis (anti-Hu syndrome) caused by esophageal small cell carcinoma (SCC). The patient developed bilateral limbic encephalitis, followed by myelitis, brain stem encephalitis, and autonomic failure. Extensive examination demonstrated SCC of the abdominal lymph nodes that was retrospectively diagnosed as metastasis of esophageal SCC on autopsy. The neuropathological findings were characterized by widespread neuronal loss and gliosis in the central nervous system, as well as patchy loss of myelin and axons in the spinal nerve roots with perivascular lymphocytic infiltration. This is the first detailed clinical and neuropathological report of anti-Hu syndrome caused by esophageal SCC.

We report a case of elderly-onset neuromyelitis optica (NMO) positive for the anti-aquaporin-4 (AQP-4) antibody; symptoms developed after the diagnosis of prostate adenocarcinoma and relapsed after a 23-valent pneumococcal polysaccharide vaccination. We suggest that activation of CD4-positive T cells and secretion of interferon-gamma induced by adenocarcinoma and complement activation induced by vaccination are responsible for the onset and relapse of NMO, even if a patient is positive for the anti-AQP-4 antibody. This case supports the previous experimental finding that the anti-AQP-4 antibody does not cause NMO-like lesions when injected alone, but does so after the induction of T cell-mediated experimental autoimmune encephalomyelitis or when co-injected with human complement.

We report two cases of the rare neurological paraneoplastic syndrome, limbic encephalitis, as the initial presentation of small cell lung cancer. The first case responded to treatment of the underlying malignancy, while the second required more acute treatment in the intensive care setting. In this case, initial treatment was with immunosuppression to achieve a degree of stability before the underlying malignancy could be treated. Both cases had significant improvement in neurological function. These cases highlight the importance of directed investigation to try and identify an underlying malignancy in patients in whom a diagnosis of limbic encephalitis is made, and the difficulty in managing such patients., (© 2011 Blackwell Publishing Asia Pty Ltd.)

Introduction: A paraneoplastic syndrome characterized by neuropsychiatric symptoms, involuntary movements and seizures has been recently associated with antibodies targeting NMDA (N-methyl-D-aspartate) receptor in patients with an ovarian teratoma. Severe neurological impairment is frequent and treatment in the intensive care unit is often required because of ventilatory failure and life-threatening autonomic instability. Tumor removal is curative in many cases and neurological improvement is demonstrated shortly after surgery., Case Report: Here we report on a patient with paraneoplastic encephalitis manifested by unconsciousness and coreo-athetosic movements related to NMDA receptor antibodies associated with an immature ovarian teratoma grade III. She made a complete recovery after oophorectomy, intravenous immunoglobulin and corticosteroids., Conclusions: Treatment of paraneoplastic syndromes is based on specific therapy for underlying tumor associated to immunomodulators. As in this case, anti-NMDA encephalitis may significantly improve after tumor removal and intra-venous immunoglobuline.

Background: Patients with higher levels of fasting serum glucose have higher death rates from pancreatic cancer compared to patients with lower levels of fasting serum glucose. However, the reasons have not been studied. The goal of the current study was to examine the neural alterations in pancreatic cancer patients with hyperglycemia and to identify the relationship between the neural alterations and perineural invasion., Methodology/principal Findings: The clinical and pathological features of 61 formalin-fixed pancreatic cancer specimens and 10 normal pancreases as controls were analyzed. Furthermore, the expression of Protein Gene Product 9.5 (PGP9.5), Myelin P0 protein (MPP), NGF, TrkA, and p75 were examined by immunohistochemistry. The median number of nerves, the median area of neural tissue, and the median nerve diameter per 10 mm(2) were larger in the hyperglycemia group than those in the euglycemia group (p = 0.007, p = 0.009, and p = 0.004, respectively). The integrated optical density (IOD) of MPP staining was lower in the hyperglycemia group than those in the euglycemia group (p = 0.019), while the expression levels of NGF and p75 were higher in the hyperglycemia group than those in the euglycemia group (p = 0.002, and p = 0.026, respectively). The nerve bundle invasion of pancreatic cancer was more frequent in the hyperglycemia group than in the euglycemia group (p = 0.000)., Conclusions/significance: Nerve damage and regeneration occur simultaneously in the tumor microenvironment of pancreatic cancer patients with hyperglycemia; the simultaneous occurrence may aggravate the process of perineural invasion. The abnormal expression of NGF and p75 may also be involved in this process and subsequently lead to a lower rate of curative surgery.

Extraskeletal myxoid chondrosarcoma is a rare tumor with less than 100 cases reported in the literature. The prevalence of anti-Hu positive myxoid chondrosarcoma-associated paraneoplastic subacute cerebellar degeneration is exceedingly rare. We present a report of a patient with confirmed myxoid chondrosarcoma-associated paraneoplastic subacute cerebellar degeneration, who exhibited marked improvement within 1 week of receiving chemotherapy, intravenous immunoglobulin (IVIG), and hydrocortisone treatment.

Encephalomyelitis occurs in paraneoplastic syndrome and acute disseminated encephalomyelitis through different autoimmune mechanisms. No postvaccinal encephalomyelitis other than acute disseminated encephalomyelitis has been reported in patients with malignancy. A 68-year-old woman was admitted because of a headache followed by a gait disturbance and psychomotor retardation 2 days after she had received an influenza vaccination followed by abulia, limb rigidity and hyperreflexia of both legs, and meningeal irritation. Cerebrospinal fluid studies showed increased intracranial pressure, elevated immunoglobulins G and A, and pleocytosis. Contrasted brain magnetic resonance imaging revealed ventriculomegaly and multiple symmetric leptomeningeal enhancement, without demyelinating changes or cortical ribbon signs. Somatosensory evoked potentials and nerve conduction velocity studies suggested myelitis. Encephalomyelitis was diagnosed on the basis of clinical and laboratory examinations. The etiological survey identified a lung adenocarcinoma. Both the encephalomyelitis and the lung adenocarcinoma simultaneously progressed after the vaccination and then, after targeted therapy for lung cancer, simultaneously subsided. In conclusion, postinfluenza-vaccination paraneoplastic encephalomyelitis may occur in patients with lung adenocarcinoma.

Paraneoplastic movement disorders are part of the spectrum of paraneoplastic syndromes caused by the production of onconeural antibodies such as anti-Hu by underlying tumours. These attack specific neurons depending on receptor aetiology. We report the case of a 53-year-old man who presented 8 years previously with symptoms of upper limb weakness, light headedness, dizziness and falls. His condition followed a progressive course. Two years after onset he had right-sided weakness, diplopia and generalised dystonia. Initial investigations identified a positive anti-Hu antibody, but an extensive search for a primary tumour was negative. A malignant fibrous histiocytoma in his right gluteal fold was subsequently identified. At this stage he was bed bound with severe ataxia, dystonia and spasticity. Following surgical excision and treatment with high dose steroids and pulse immunoglobulin, further progression was arrested and minor improvements occurred. He can now ambulate with bilateral assistance but remains severely disabled.

Paraneoplastic syndromes are cancer-associated endocrinological, haematological, dermatological or neurological disorders, which are directly related neither to the physical effects of the tumour mass, nor to invasion by the primary tumour, nor to metastasis of the tumour; nor are they associated either with the side-effects of anticancer treatment or with any of the complications of cancer. These syndromes are brought about by the ectopic production of biological mediators by the malignant tumour cells, or by immunological responses to the malignancy. Certain cancers are typically associated with specific paraneoplastic disorders. Though uncommonly, oral carcinomata have reportedly been associated with paraneoplastic pemphigus, humoral hypercalcaemia of malignancy, syndrome of inappropriate antidiuretic hormone, and paraneoplastic leukocytosis syndrome.

Background: Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis has been reported to be associated with ovarian teratomas. In many patients, surgical excision has resulted in improvement., Case: A previously healthy 14-year-old girl presented with confusion which later evolved into a comatose state. Imaging revealed an adnexal mass which was surgically removed and confirmed to be a mature teratoma. Her cerebral spinal fluid was positive for antibodies to NR1/NR2 heteromers of NMDA receptors., Comments: Cystic teratomas are one of the most common benign ovarian lesions in children and young women. While an association of ovarian teratomas and paraneoplastic encephalitis is published in the neurologic literature, this association needs to be clarified for the practicing gynecologist who may be asked to consult on these patients., (Copyright © 2010 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

The article provides an overview on the diagnosis and pathogenesis of paraneoplastic neurological disorders (PNDs), and subsequently the current therapeutic strategies in these patients. PNDs are nervous system dysfunctions in cancer patients, which are not due to a local effect of the tumor or its metastases. Most of these clinically defined syndromes in adults are associated with lung cancer, especially small-cell lung cancer, lymphoma and gynecological tumors. In a part of the PND, an overlapping of different clinical syndromes can be observed. Highly specific autoantibodies directed against onconeuronal antigens led to the current hypothesis of an autoimmune pathophysiology. Whereas the most central nervous PNDs are more T-cell-mediated, limbic encephalitis can be caused by pathogenic receptor autoantibodies. The PND of the neuromuscular junction and paraneoplastic autonomic neuropathy are mainly associated with receptor or ion channel autoantibodies. The childhood opsoclonus-myoclonus syndrome and the PNDs associated with receptor/ion channel autoantibodies often respond to immunosuppressive therapies, plasmapheresis and intravenous immunoglobulins. By contrast, most CNS PNDs associated with defined antineuronal antibodies directed against intracellular antigens only stabilize after tumor treatment.

Introduction: Paraneoplastic neurological syndromes (PNS) are uncommon and imperfectly understood and, therefore, are frequently underdiagnosed., Methods: We review the current literature on the clinical presentation, diagnosis, pathophysiology and treatment of PNS., Results: PNS are a heterogeneous group of neurologic disorders caused by an immune response to an underlying malignancy. A 3-step diagnostic process is necessary to establish the diagnosis. We review the role of onconeural antibodies in the diagnosis and pathogenesis of PNS and describe recent advances in treatment, focusing on paraneoplastic encephalomyelitis, limbic encephalitis, paraneoplastic cerebellar degeneration, opsoclonus-myoclonus syndrome, subacute sensory neuronopathy and Lambert-Eaton myasthenic syndrome, Conclusion: PNS often antedate the diagnosis of cancer, offering an opportunity for detecting cancer at an earlier and curable stage. Tests for paraneoplastic antibodies are often negative and do not rule out the diagnosis of a paraneoplastic syndrome. Certain clinical presentations should suggest a paraneoplastic syndrome, even in the absence of paraneoplastic antibodies, and prompt a thorough search for occult malignancy.

Paraneoplastic neurological syndromes (PNSs) are a heterogeneous group of neurological disorders caused by immune-mediated mechanisms. The incidence of PNS is much less than 1% for solid tumors, except for small-cell lung cancer and thymoma. We report a rare case of gastric cancer that presented with primary clinical findings of PNS. The patient was a 63-year-old woman who was admitted for worsening neuropathy. Laboratory and neurological tests excluded a nutritional deficit, diabetes mellitus, and connective tissue disease as causes of her neuropathy. Computed tomography (CT) of the abdomen, positron emission tomography (PET)-CT, and endoscopy of the stomach revealed gastric cancer with lymph node swelling. Distal gastrectomy was performed and pathological and immunohistochemical examinations indicated endocrine cell carcinoma. The gastrectomy stopped the exacerbation of her symptoms and recurrence was not observed, but the neurological disorders were irreversible. This case suggests that early diagnosis of the primary tumor is required to improve the outcome in patients with PNS.

Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is a recently described paraneoplastic syndrome with prominent neuropsychiatric symptoms. We report a case of NMDA receptor encephalitis in a 15-year-old female related to the development of NMDA receptor autoantibodies triggered by an ovarian teratoma. Removal of the mature teratoma proved curative with eventual resolution of the paraneoplastic disease process and associated psychiatric symptoms. Increasingly, reports of anti-NMDA receptor encephalitis associated with ovarian teratomas in pediatric patients, as well as a novel assay to measure these antibodies, suggest an etiology for this disease process that may be amenable to prompt surgical excision. The clinical presentation, diagnosis, and surgical management of the disease, as well as a review of the literature, are included., (Copyright 2010 Elsevier Inc. All rights reserved.)

The remote effects of malignant tumors in most cases of paraneoplastic neurological syndromes(PNS)are mediated by autoimmune processes against antigens shared by the tumor cells and the nervous tissue(onconeural antigens). Onconeural (or paraneoplastic)antibodies are broadly categorized into two groups according to the location of the corresponding onconeural antigens, inside or on the surface of neurons. Antibodies established as clinically relevant diagnostic markers for PNS are designated as well-characterized onconeural antibodies (or classical antibodies)that target intracellular antigens(Hu, Yo, Ri, CV2/CRMP5,Ma2, and amphiphysin). They also serve as useful markers in detecting primary tumors. Recent identification of new antibodies as markers of subtypes of limbic encephalitis has also expanded the concept of autoimmune limbic encephalitis. These autoantibodies are directed to neuronal cell-surface antigens including neurotransmitter receptors(NMDA, AMPA, and GABAB receptors)and ion channels(VGKC). They are less frequently associated with cancer, so that they cannot be used as specific markers for PNS. Autoimmune limbic encephalitis with anti-neuronal cell surface antobodies and paraneoplastic limbic encephalitis with classical antibodies overlap in some clinical features but are pathophysiologically distinct. Classical antibodies are not simple tumor markers. They seem to be closely related to the disease mechanisms because specific intrathecal synthesis has been shown in PNS patients. However, attempts to produce an animal model of PNS by passive transfer of these antibodies have been unsuccessful, and there is no direct evidence demonstrating the pathogenic role of classical antibodies. Instead, some circumstantial evidence, including pathological studies showing extensive infiltrates of T cells in the CNS of the patients, supports the hypothesis that cytotoxic-T cell mechanisms cause irreversible neuronal damage. On the other hand, humoral immune response is probably the principal mechanism in autoimmune encephalitis associated with antibodies against neuronal cell-surface antigens. Those antibodies are supposed to mediate neural dysfunction which may be reversed by immunosuppression therapy, while the exact mechanism remains to be elucidated. Further accumulation of the cases and longer observation would be necessary to delineate the clinical spectrum of each type of newly-identified autoimmune limbic encephalitis. Early diagnosis and optimal oncological treatment is a prerequisite for better prognosis of PNS patients. Detection of the primary tumor at very early stages including carcinoma in situ is a challenging issue. Optimization of immunosuppression/ immunomodulation therapy for each patient according to the underlying pathophysiological processes is another important clinical issue.

Paraneoplastic neurological syndromes (PNSs) are the remote effects of cancer on the nervous system. The peripheral nervous system is an important targets of PNS. The neuropathies associated with PNS are reviewed in this article. Among the various paraneoplastic neuropathies, the main classical syndorome of PNS is subacute sensory neuronopathy that involves the cell bodies of sensory neurons in the dorsal root ganglia. Its clinical symptoms include sensory ataxia. Onconeuronal antibodies such as anti-Hu and anti-CV2/CRMP5 antibodies are frequently associated with this syndrome. In contrast to this classical form of PNS, non-classical syndromes are considered as heterogeneous neuropathies. The clinical features of non-classical syndromes are variable and no evident association between a clinical phenotype and onconeuronal antibodies has been established. Early detection and treatment of cancer is an essential for management of PNS. Neuropathies with paraproteinemia are also important clinical entities of PNS. IgM M-protein is most likely to cause neuropathy. Patients with IgM paraproteinemic neuropathy is usually characterized by predominan distal and sensory impairment, prolonged distal motor latencies in nerve conduction studies, and the presence of anti-MAG and SGPG antibodies.

The coincidence of stroke and cancer is frequently encountered. From recent epidemiological data, the stroke risk in cancer patients seems to be equally distributed as compared to the non-cancer population. However, there are several clinical conditions in cancer patients which increase the risk for stroke: Trousseau's syndrome, non-bacterial thrombotic endocarditis and disseminated intravascular coagulation. Also some tumour-specific conditions such as coagulopathies, changes of viscosity and cellular mechanisms such as leukocytosis or thrombocytopathies must be considered. In several types of tumour treatment, such as various anticancer drugs, an increased occurrence of stroke has been reported. Presently there is no indication that stroke and cancer are related to the immune-mediated "classic" paraneoplastic syndromes. However, there are several cancer-specific types and causes of stroke which need to be considered in each patient, as they can be of significance in the treatment.

We describe a 12-year-old boy male who presented with an expressive dysphasia after completion of treatment for unifocal Ewing sarcoma. CNS vasculitis was diagnosed by MRI/MRA and cerebral angiography. Extensive rheumatologic work-up failed to identify an underlying primary process. Restaging studies showed no evidence of tumor. Complete neurologic recovery was achieved on prednisone. Four months later the patient developed overt, extensive metastases, confirmed by biopsy to represent recurrent Ewing sarcoma. Despite intensive therapy the patient succumbed 6 months later. This case demonstrates the unique finding of isolated CNS vasculitis as a presenting sign of Ewing sarcoma., ((c) 2009 Wiley-Liss, Inc.)

A 21-year-old woman developed psychiatric symptoms, progressive unresponsiveness, generalized seizures, severe dyskinesia, marked fluctuation of blood pressure, and hypersalivation after a flu-like episode. Anti-glutamate receptor (GluR)ε2 and anti-N-methyl-D-aspartate receptor (NMDAR) antibodies were positive in both her serum and CSF. After she recovered five months later she underwent surgery to remove a right ovarian teratoma. Immunohistochemical examinations of her teratoma disclosed abundant expression of various GluRs including NR2B subunit of NMDAR, GluR1, and GluR2/3. These immunoreactivities of GluRs were seen not only in small areas of neural tissue identified as anti-glial fibrillary acidic protein (GFAP)-immunoreactive areas but also in other large areas of undifferentiated neuroepithelial tissue without GFAP immunoreactivity. Our findings strongly support the recent idea that neural elements in ovarian teratoma play an important role in the production of antibodies to NMDARs in anti-NMDAR encephalitis. Additionally, the study of control ovaries clearly showed NR2B-related immunoreactivity in the cytoplasm of oocytes, indicating that the normal ovary itself has expression of NMDARs. This finding might provide a clue to understand the pathogenesis of this disease in female patients without ovarian teratoma.

Patients with neurological malignancies are subject to developing a unique set of complications that require emergent evaluation and treatment. With the increasing incidence of cancer in the general population and improved survival, these emergencies will be more frequently encountered. Physicians must be able to recognize these conditions and institute appropriate therapy after a focused initial evaluation. The approach to definitive therapy is commonly multidisciplinary, involving surgeons, radiation oncologists, medical oncologists, and other medical specialists. Prompt interventions can be lifesaving and may spare patients considerable morbidity and pain. In neuro-oncology, there are some more specific complications and emergencies. The more general complications are not reviewed here.

We report the case of a 55-year-old woman with thymoma diagnosed after finding of extra limbic encephalitis. She presented neurologic symptoms as seizure and aphasia; magnetic resonance imaging (MRI) of the brain showed multiple lesions located in insular, parietal and temporal lobes (in cortical and sub-cortical area). Brain biopsies confirmed the diagnosis of encephalitis and CT-scan of the thorax showed an anterior mediastinal mass suspected for thymoma. The patient was submitted to thymectomy through a median sternotomy and we assisted to secondary reduction of cerebral lesions and total remission of symptoms.

We report on a young lady suffering from adult neuroblastoma and anti-Hu associated paraneoplastic encephalomyelitis (PEM) with a tumour free survival of nine years up to now. Treatment included tumour surgery, radiation, high dose chemotherapy, and stem cell transplantation. Serological testing demonstrated a marked decline in anti-Hu antibody titres under therapy, and subsequent disappearance of the antibody 31 months after second tumour resection.

Although most dementias are due to neurodegenerative or vascular disease, it is important to diagnose immunologically mediated dementias quickly because they can be both rapidly progressive and readily treatable. They usually affect function of limbic and cortical structures, but subcortical involvement can also occur. Because of the variety of symptoms and the rapid course, these dementias present a particular challenge to the clinician and may require evaluation and intervention in the inpatient setting. Diagnostic workup typically reveals evidence of an autoimmune process and, in some cases, cancer. In contrast to the neurodegenerative processes, many of the immunologically mediated dementias respond to immunomodulatory therapy.

Neurologic symptoms commonly occur in oncology patients, and in some cases they may be the presenting symptom of malignancy. Cancer-related neurologic syndromes are rarely pathognomonic and must be differentiated from other benign or serious conditions. This article reviews common neuro-oncologic syndromes that may lead to urgent evaluation in the emergency department, including cerebral edema, altered mental status, seizures, acute stroke, leptomeningeal metastases, and paraneoplastic neurologic syndromes.

Background: Paraneoplastic neurological syndromes constitute a heterogenous group of diseases, which often cause severe neurological symptoms. Extensive research during the last 20 years has led to identification of new antigenic targets and inclusion of additional clinical conditions into this group of syndromes., Material and Methods: The article is based on a non-systematic search of PubMed and the author's own experience with antibody analysis, treatment of patients and research within the field., Results and Interpretation: Paraneoplastic neurological syndromes affect less than 1 % of all patients with cancer. The etiology is probably autoimmune, and involves immune responses (cellular and humoral) against antigens shared by tumor cells and normal neurons. Many, but not all patients harbour onconeural antibodies (in serum and spinal fluid), which are highly useful diagnostic markers for a paraneoplastic etiology. The cornerstone of therapy is treatment of the underlying tumor, but additional immunosuppressive therapy is often administered.