Prostate In our experience, patients have improved with short-term corticosteroids. Others may be indistinguishable from more common neurological disorders (e.g. Axial T1-weighted pre- (A) and postcontrast (B) images and the coronal postcontrast image (C) from her brain MR imaging show enlargement and enhancement of the left third cranial nerve cisternal segment (B and C, arrows) and mild enhancement of the right third cranial nerve cisternal segment (C, arrowhead). Hypertrophic olivary degeneration can also be seen as a result of paraneoplastic brain stem encephalitis (Fig 5E, -F), presumably caused by disruption of the dentato-rubro-olivary pathway. Ultimately, his intracranial findings were consistent with limbic encephalitis rather than malignancy. Common examples include the secretion of adrenocorticotrophic hormone and antidiuretic hormone by the tumour.294 Antidiuretic hormone is raised in the serum of 3040% of patients with small cell carcinoma but the clinical syndrome of severe hyponatraemia, hypo-osmolality of the serum and continued urinary sodium excretion is recognised in only 510%.295 Excess antidiuretic hormone is sometimes found with non-neoplastic lung disease but here it is pituitary in origin rather than ectopic. CSF analysis showed antibodies to Kelchlike protein 11. Table 2. Treatment of neurological paraneoplastic syndromes involves treating the cancer and, in some cases, suppressing the immune response that's causing your signs and symptoms. These paraneoplastic autoantibodies are useful diagnostic markers even in syndromes in which they are not directly pathogenic. Additionally, there is patchy T2 hyperintensity in the cerebellar vermis (F, arrows). Paraneoplastic syndromes may affect diverse organ systems, most notably the endocrine, neurologic, dermatologic, rheumatologic, and hematologic systems. The most commonly associated malignancies include small cell lung cancer, breast cancer, gynecologic tumors, and hematologic malignancies. These paraneoplastic autoantibodies are useful diagnostic markers even if they are not directly pathogenic. These have demonstrated variable patterns of enhancement, with 2 patients having ANNA-1 positivity.27,29-32 We have found that enhancement can predominantly involve the dorsal (Fig 8) or ventral nerve roots (Fig 9), and the pattern of enhancement does not always correlate with symptoms. Goldman L, Schafer AI. Polymyositis/Dermatomyositis. In PEM and PSN, the autonomic dysfunction may be the first sign of the disorder. Axial CT (C) and fused FDG-PET/CT (D) images show a nonFDG-avid lumbar vertebral sclerotic lesion (C and D, arrowheads) and an adjacent mildly FDG-avid sclerotic lesion (C and D, arrows). He was diagnosed with paraneoplastic brain stem encephalitis rather than multiple system atrophy given the clinical context of the findings. FDG-PET/CT axial image (A) shows marked hypermetabolism in the bilateral mesial temporal lobes (A, arrows). Symptoms may result from adrenergic or cholinergic nerve dysfunction at the preganglionic or postganglionic level. A 64-year-old woman recently diagnosed with small cell lung cancer presented with gait difficulties and progressive bilateral lower extremity weakness lasting several months. MRI of the brain fails to show a focal lesion but may demonstrate cerebellar atrophy. The median age of onset of PND is 65years. Symptoms include mood and behavioral changes, cognitive dysfunction, memory loss, and seizure activity. Examples of paraneoplastic syndromes of the nervous system include: Cerebellar degeneration (cerebellar ataxia). She had mild improvement in her neurologic symptoms with high-dose corticosteroids but remained wheelchair dependent. A particularly devastating form of paraneoplastic syndromes ia a group of disorders classi ed as Paraneoplastic Neurological Disorders (PND).The incidence of PNS varies with the neurological syndrome and with the tumor. CSF analysis was remarkable for albuminocytologic dissociation, and the patient was initially diagnosed with Guillain-Barr syndrome. In the chronic phase, this syndrome can be mistaken for multiple system atrophy and other neurodegenerative disorders. Some examples are given below 1,2: Paraneoplastic comes from the Greek words for alongside (para), new (neo) and formation (plasis) 2. Multiple sclerosis would not generally have longitudinally extensive involvement. Careful attention to patient history, such as prior alcohol use disorder or cerebellar infection, is most important in distinguishing these entities. The endocrine system includes organs such The authors acknowledge the assistance of Desiree J. Lanzino, PT, PhD, in editing the manuscript. ADVERTISEMENT: Supporters see fewer/no ads, Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. The pathogenesis of most PND is thought to involve cytotoxic T lymphocytes, which presumably recognize autoantigens in the context of MHC Class I on both tumor cells and neurons [1]. Entities such as neurosarcoidosis or leptomeningeal carcinomatosis tend to have more nodular enhancement. A 29-year-old man with a history of mediastinal seminoma presented with progressive lower extremity weakness and frequent falls over months. The presence of perimedullary flow voids usually suggests an AVF, whereas preferential involvement of the anterior horns often suggests cord infarct. Paraneoplastic myelitis with treatment response (FI). The patients symptoms mildly improved after treatment with multiple immunosuppressive agents. Other malignancies with definite PND associations include gynecological malignancies, arising from the breast, ovary, fallopian tube and peritoneum, Hodgkin's and non-Hodgkin's lymphoma, testicular cancer and neuroblastoma. There is relative sparing of the ventral nerve roots (D, arrows), somewhat atypical for Guillain-Barr syndrome. The following diseases manifest by means of neurological dysfunction: LambertEaton myasthenic syndrome, paraneoplastic cerebellar degeneration, There are three autonomic problems that may be life-threatening: esophageal and gastrointestinal dysmotility with intestinal pseudo-obstruction, cardiac dysrhythmias, and postural hypotension. Our goal is to review the imaging findings of paraneoplastic neurologic syndromes, including less well-known entities and atypical presentations of common entities. WebFor example, a horse may be presented with haemoglobinuria and profound anaemia that could be a result of paraneoplastic changes. In those patients with a recent cancer diagnosis, the presence of new neurologic symptoms, specifically in the absence of other etiologies or cancer treatment, should raise suspicion of a paraneoplastic process. ISBN:0199772738. Neurological. The most well-recognized paraneoplastic neurologic syndrome, both clinically and on imaging, is limbic encephalitis. Axial T1-weighted pre- (A) and postcontrast (B) images from her lumbar spine MR imaging show diffuse enlargement and enhancement of the cauda equina nerve roots (B, arrowheads). Sagittal T1-weighted postcontrast (A) and sagittal FLAIR (B) images show enhancing, T2 hyperintense lesions in the midbrain and tectum (A and B, arrows), initially believed to be worrisome for metastases. This review illustrates the typical imaging findings of paraneoplastic neurologic syndromes in a pictorial essay format and briefly discusses the differential diagnosis for each syndrome when encountered on imaging (On-line Table). see full revision history and disclosures, syndrome of inappropriate antidiuretic hormone secretion (SIADH), paraneoplastic rheumatological/dermatological syndromes. This is the loss of nerve cells in the area of the brain that controls Limbic encephalitis. In many of these patients, a circulating antibody (anti-Yo) that binds to proteins shared by breast tumor cells and Purkinje cells can be identified. Paraneoplastic syndromes can affect multiple systems and have a diverse presentation. Paraneoplastic polyneuropathy mimicking Guillain-Barr syndrome. WebIntroduction Paraneoplastic neurological syndromes (PNS) describe the remote neurological immune-mediated consequences of a systemic cancer. Her symptoms have been stable on cyclophosphamide with pulsed corticosteroids. Chronic inflammatory demyelinating polyneuropathy could be considered but is less likely to show any enhancement and would have more prominent nerve enlargement. Several reports suggest that patients with PND have a favorable cancer outcome (survival and treatment response) compared to those with identical tumors without PND [58]. The patient was diagnosed with paraneoplastic cerebellar degeneration. Paraneoplastic cranial neuropathies are rare but have been previously described in several reports.16,17 Symptoms depend on the cranial nerves involved. Paraneoplastic autonomic dysfunction may occur alone but is more often associated with other paraneoplastic syndromes such as LEMS and PEM or PSN. Some paraneoplastic syndromes appear to have an autoimmune basis; thus LambertEaton syndrome is associated with the presence of anti-voltage gated calcium channel (VGCC antibodies). Saunders. Whole-body computed tomography as first-line imaging procedure to exclude cancer in patients with neurological suspicion of paraneoplastic syndromes: shall clinical practice adhere to recommendations? He was treated with chemotherapy and high-dose corticosteroids with gradual improvement in his symptoms. Proc. Despite treatment, the outcome is overall poor with most patients becoming wheelchair dependent. He was treated with chemotherapy, and his symptoms transiently improved but then worsened over the next several years. Some PNDs have unique clinical characteristics and should be easily recognized (e.g., paraneoplastic cerebellar degeneration, sensory neuronopathy, and limbic encephalitis). Imaging findings of PCD depend on the phase of the syndrome. Some of these disorders result from tissue-specific autoimmunity initiated by the immune response against cancer. We also demonstrate common diagnostic pitfalls that can be encountered when imaging these patients. In these cases, the association with cancer may be underrecognized because of the absence of clinical suspicion. Table 1. Symptoms typically develop over weeks or months, but can progress more rapidly (over a few days) in some cases. A 29-year-old man presented with tinnitus, hearing loss, diplopia, and imbalance that had progressed over at least 1 year. Others may be indistinguishable from more common neurological disorders (e.g., peripheral neuropathy, myasthenia gravis, motor neuron disease, and myelitis). The patients CSF was later found to be ANNA-1/anti-Hu antibody positive, and she was ultimately found to have small cell lung cancer. Patients can have an acute myelitis or more insidious progressive myelopathy with combinations of weakness, numbness, and bowel or bladder dysfunction. Many of these syndromes can have imaging findings that, though less well described, are important in making the correct diagnosis. Patients with cancer may develop neurological symptoms as a result of direct tumour invasion into the nervous system, or through indirect mechanisms such as a hyper-coagulable state, metabolic derangements, or nutritional disturbances. Illustration of the diverse array of paraneoplastic neurologic syndromes affecting a wide variety of anatomic structures. Endocrine. 2 There is a wide spectrum of presentations ranging from ocular dysfunction to severe paralysis. The classic features of Cushing's syndrome (Fig. Cushing syndrome is caused by ectopic secretion of corticotropin (hypercortisolism) and produces several major effects, including hyperglycemia, hyperkalemia, hypertension, and muscle weakness [176]. Cushing syndrome is associated with several types of malignant neoplasm, including small cell lung carcinoma, pancreatic carcinoma, and various neural tumors. Papulosquamous Acanthosis nigricans Acquired ichthyosis Acrokeratosis neoplastica Extramammary Paget disease Florid Web"Paraneoplastic syndrome" is an umbrella term in that there is actually a wide range of these syndromes. TABLE 13-1. A 75-year-old healthy man presented with abrupt onset of memory loss and staring spells over several days. Lung cancer (especially small-cell lung cancer ). Paraneoplastic syndromes: Some people have an autoimmune disorder that causes their bodies to attack healthy brain cells in response to a tumor. Over the next 2years, he had progressive gait ataxia and diplopia. These affect the brain and nervous system. Hypoglycemia. A 29-year-old man with a history of resected nongerm cell testicular cancer presented months after treatment with worsening memory, diminished executive function, hypogonadism, and hypothyroidism. He was diagnosed with paraneoplastic limbic encephalitis with atypical involvement of the hypothalamus and mammillary bodies. The identification of paraneoplastic antibodies in serum can establish the diagnosis of a paraneoplastic syndrome and often suggests the primary site of the cancer. Hypercalcemia refers to a condition related to elevated plasma calcium concentrations. Pathologically, there is a striking loss of Purkinje cells. Limbic encephalitis may be associated with various onconeuronal paraneoplastic antibodies, including antineuronal nuclear autoantibody type 1(ANNA-1)/anti-Hu, anti-collapsin response mediator protein-5 (CRMP5)/anti-CV2, and anti-Ma2. It is important to remember that paraneoplastic syndromes are very uncommon and may antedate the discovery of the tumor in one half of cases. The differential diagnosis for chronic PCD includes alcoholic degeneration, neurodegenerative disorders such as multiple system atrophy (cerebellar subtype), and sequelae from infectious/inflammatory cerebellitis. For PND restricted to the PNS, neuroimaging generally does not contribute much to the diagnosis. In the acute phase, increased T2 signal within the cerebellar hemispheres can be seen (Fig 4A, -B). Small cell lung carcinoma and pancreatic carcinoma can cause Trousseau syndrome by elaborating platelet-aggregating factors and procoagulants from the cancer or its necrotic products. Symptoms are mediated by cytokines, hormones or immune cross-reactivity. Other differential considerations include status epilepticus, neurosyphilis, and low- or high-grade glioma.4 When limbic encephalitis has hypothalamic involvement, inflammatory conditions such as sarcoidosis or lymphocytic hypophysitis should be considered. Paraneoplastic syndromes are defined as disorders of organ or tissue function caused by cancer that are not a direct result of tumor invasion. This article has been cited by the following articles in journals that are participating in Crossref Cited-by Linking. Enter multiple addresses on separate lines or separate them with commas. All PND are rare, but several classical syndromes are distinctive and common enough to warrant specific attention. Indicates open access to non-subscribers at www.ajnr.org. Abnormal FDG uptake can also be present in the involved area of the spinal cord with this syndrome (Fig 7E), which could potentially be mistaken for metastatic tumor involvement.23 Differential considerations for this entity include neuromyelitis optica spectrum disorder, neurosarcoidosis, spinal dural AVF, radiation myelitis, and cord infarct. As a result, misdiagnosis or delay in diagnosis of PND is common. Certain features, independent of established diagnostic criteria, should raise concern for PND: (1) subacute and progressive course, (2) multifocal pattern of neurological deficits, and (3) constitutional symptoms or cancer risk factors.27 Neuroimaging is important in the evaluation of PND of the CNS, mainly to evaluate for other etiologies, such as metastatic disease, vascular disorders, or inflammation. Pancreatic cancer. Therefore, timely diagnosis is likely to significantly impact patient outcomes. A 54-year-old woman recently diagnosed with small cell lung cancer presented with diplopia that slowly progressed over a period of days. WebMore than 100 years ago, it was recognized that certain cancers cause various symptoms not attributable to direct tumor invasion or compression. Classic limbic encephalitis (A and B). For example, deteriorating balance and sensorineural hearing loss have been observed with paraneoplastic involvement of the eighth cranial nerves.17 Symptoms may be more extensive when there is involvement of multiple cranial nerves, as seen in 1 report of paraneoplastic neuropathy of the third, sixth, and seventh cranial nerves in a patient with gallbladder carcinoma.18 Anti-Hu/ANNA-1 and the newly described antibodies to Kelchlike protein-11 associated with seminoma are especially associated with paraneoplastic cranial neuropathy. Causes Risk factors Diagnosis Treatment Coping and support Preparing for an appointment Overview Paraneoplastic syndromes of the nervous system are a group of uncommon disorders that develop in some people who have cancer. Paraneoplastic serum antibody panel revealed anti-G-AchR (ganglionic acetylcholine receptor) antibodies, and CSF analysis revealed increased protein concentration and high IgG levels. Other Types. The constituents secreted by the tumors consists mainly of hormones and cytokines that are capable of mimicking normal hormones which may affect other proteins flowing throughout the body. Symptoms are brought about by cytokines, Some of these disorders result from tissue-specific autoimmunity initiated by the immune response against cancer. Initial axial T2-weighted image of the brain (C) and subsequent axial T2-weighted image from an examination several months later (D) show interval development of mild left cerebellar atrophy (D, arrows). Darnell RB, Posner JB. When confined to the spinal cord, imaging findings include longitudinally extensive T2 hyperintensity and enhancement (Fig 7). In patients with breast cancer, the most common of these rare disorders is subacute cerebellar degeneration. These affect the glands and hormones of your body. Endocrine. The lung tumour most commonly associated with such hypercalcaemia is squamous cell carcinoma.299 Gynaecomastia and testicular atrophy are occasionally encountered, again with squamous cell carcinoma. Although the neurological symptoms vary between disorders, most PNDs share some common clinical features. WebThe most common cancers associated with paraneoplastic syndromes include Lung cancer (most common) Kidney cancer Liver cancer Leukemia Lymphoma Breast cancer Although follow-up of patients with paraneoplastic myelitis is not well documented in the literature, clinical improvement and improvement in imaging findings can occur (Fig 7FI). Paraneoplastic syndromes are complications of cancer that cannot be attributed to direct effects of the neoplasm or its metastases. But overall, men tend to have more problems with balance and coordination as they get older. {"url":"/signup-modal-props.json?lang=us"}, Knipe H, Bell D, Lorenzzoni P, et al. Her examination showed left eye ptosis and gaze abnormalities consistent with oculomotor palsy. Pelosof LC, Gerber DE. Last, it should be noted that PNSs are relatively rare disorders compared with various conditions in the differential diagnosis. Possible imaging findings include enhancement and/or enlargement of the affected cranial nerves (Fig 6). These criteria may now serve as a guide for determining the appropriateness of malignancy screening (Tables 2 and 3). Serum and CSF were positive for Kelchlike protein 11 autoantibodies. Typical treatment includes management of the underlying malignancy and immunosuppression. These conditions can cause malaise, pain and mental distress as well as irreparable damage to organs remote from the location of tumour. Pathologically, patients have progressive loss of Purkinje cells in the cerebellum.10 CSF analysis can show pleocytosis and elevated oligoclonal bands. He had multiple neurologic symptoms, including impaired thermoregulation, hypersexuality, and depression. FDG-PET axial source image of the brain (E) and processed surface rendering of the brain compared with age-matched control participants (F) show marked hypometabolism within the left cerebellar hemisphere (E and F, arrows). Follow-up MR imaging shows improved mild residual hypothalamic T2 signal (E, arrows) and decreased enhancement of the mammillary bodies (F, arrows). Axial double inversion recovery MR imaging image (B) shows corresponding increased T2 signal in both mesial temporal lobes (B, arrows). Trousseau syndrome is characterized by hypercoagulopathy and produces the major effect of venous thrombosis of the deep veins in the cancer patient [177]. Limbic encephalitis mimicking a tumor. In addition to the usual paraneoplastic work-up, MR imaging of the entire spine is particularly helpful in these patients because of the longitudinally extensive nature of spinal cord involvement. Breast cancer is a leading cause of cancer death in the United States, with an increasing incidence in recent years. Antibodies against neuronal antigens may be produced as well. Chronic paraneoplastic cerebellar ataxia (CF). PCD is associated with a variety of paraneoplastic antibodies, including anti-Yo (Purkinje cell autoantibody type-1 [PCA-1]), anti-Ri (ANNA-2), and anti-Purkinje cell autoantibody-Tr/DNER (delta/notchlike epidermal growth factor-related receptor). WebThe most dramatic examples of cancer-related autoimmunity are the paraneoplastic neurological disorders (PND). Check for errors and try again. William B. Coleman PhD, in Molecular Pathology (Second Edition), 2018. However, numerous additional clinically described syndromes affect the brain, spinal cord, and peripheral nerves. Bryan Corrin MD FRCPath, Andrew G. Nicholson DM FRCPath, in Pathology of the Lungs (Third Edition), 2011. Table 3. Cancer-associated hypercalcaemia is usually due to bone metastases but occasionally occurs without metastatic disease, when it is generally mediated by parathyroid-related protein which increases both bone resorption and renal tubular reabsorption of calcium: serum parathyroid hormone levels are characteristically low or unmeasurable in these cases. Lymphoma. Subacute paraneoplastic cerebellar ataxia (A and B). ScienceDirect is a registered trademark of Elsevier B.V. ScienceDirect is a registered trademark of Elsevier B.V. Office Practice of Neurology (Second Edition), Handbook of Neuro-Oncology Neuroimaging (Third Edition), Handbook of Neuro-Oncology Neuroimaging (Second Edition). These findings can also involve other parts of the limbic system. Additionally, we have highlighted the fact that these entities can have variable imaging findings resulting in many potential diagnostic pitfalls. This is an example of a paraneoplastic autoimmune encephalitis with NMDA-R and GFAP antibodies in context of ICI treatment in a patient Diagnosis requires a thorough neurologic work-up, and ophthalmologic consultation is helpful if ophthalmoplegia is identified. Guillain-Barr syndrome classically, but not always, has ventral predominant nerve enhancement. Although the syndromes associated with these antibodies all characteristically involve the limbic system, some can have extralimbic involvement. Inappropriate or ectopic hormone secretion is responsible for another important group of paraneoplastic syndromes. In these cases, the association with cancer may be under-recognized because of the absence of clinical suspicion. 2010;85 (9): 838-54. Paraneoplastic Corresponding axial T2-weighted (D) and FDG-PET/CT (E) images show increased T2 signal (D, arrows) and marked abnormal FDG uptake (E, arrows) in the spinal cord. Importantly, many of these syndromes respond to treatment of underlying malignancy or immunotherapy. These syndromes are often associated with serum or CSF positivity of onconeuronal or neuronal cell surface antibodies. Reference article, Radiopaedia.org (Accessed on 12 Jun 2023) https://doi.org/10.53347/rID-32421. A 45-year-old man presented with progressive aural fullness and hearing loss, initially thought to be caused by otitis media. This question is for testing whether or not you are a human visitor and to prevent automated spam submissions. Serum and CSF were positive for CRMP5 autoantibodies. 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