This case report summarises a very rare diagnosis of paraneoplastic cerebellar degeneration with a positive onconeuronal antibody associated with anaplastic nonhodgkins lymphoma. Clinical insights into paraneoplastic cerebellar degeneration. Paraneoplastic cerebellar degeneration pcd is a collection of neurological disorders resulting from tumorinduced autoimmunity against cerebellar antigens. Paraneoplastic syndromes are thought to result from an abnormal immune response to an underlying and often undetected malignant tumor.
Pcd is a rare condition that occurs in less than 1% of cancer patients. In paraneoplastic cerebellar degeneration, the average age of onset is 50, with males affected more often than females. Are there different types of paraneoplastic neurological disorders. Patients typically develop subacute cerebellar ataxia, dysarthria, diplopia, and vertigo, which often predate the cancer. The cancer may be new or recurrent, is usually limited in metastatic volume, and is often occult by standard imaging procedures. The intensity of the hypermetabolism had decreased markedly on followup fdg. Paraneoplastic syndromes an overview sciencedirect topics. A provisional diagnosis of paraneoplastic cerebellar degeneration, secondary to. The neurologic symptoms frequently precede or coincide with the diagnosis of cancer. Paraneoplastic cerebellar degeneration pcd is an immune.
We present the case of a 60year old female patient who developed paraneoplastic cerebellar degeneration upon locoregional recurrence of a poorly differentiated spindle cell carcinoma of the nasal cavity and paranasal. Antiyo mediated paraneoplastic cerebellar degeneration. Neocerebellar degeneration paraneoplastic profile with. Paraneoplastic cerebellar degeneration request pdf. A 51 year old man with paraneoplastic cerebellar degeneration from gastric adenocarcinoma showed cerebellar hypermetabolism and increased perfusion on brain fdgpet scan and spect during the acute stage of his illness. Paraneoplastic cerebellar degeneration pcd is a rare paraneoplastic syndrome, occurring in pdf. Immunemediated cerebellar ataxias include gluten ataxia, paraneoplastic cerebellar degeneration, gad antibody associated cerebellar ataxia, and hashimotos encephalopathy. Renal cell carcinoma has rarely been described as an underlying malignancy in these cases.
Paraneoplastic cerebellar degeneration associated with breast cancer. Paraneoplastic cerebellar degeneration pcd is a rare disorder, presenting with severe cerebellar dysfunction. Alcoholic or nutritional cerebellar degeneration affects alcoholics and people with thiamine deficiency. Paraneoplastic cerebellar ataxia and the paraneoplastic. These demonstrated a large left adnexal mass and a raised ca125 level. Guidelines for treatment of immunemediated cerebellar ataxias.
Nov 25, 2016 paraneoplastic neurological syndromes pns are an extensive group of neurologic disorders that occur in patients with cancer and can affect any part of the nervous system. The syndrome is often associated with small cell carcinoma of the lung, breast cancer, gynecologic cancers, and hodgkin disease. Bipolar disorder associated with paraneoplastic cerebellar. Dec 09, 2019 paraneoplastic cerebellar degeneration is caused by the secondary effects of cancer and is believed to be immune mediated. To extend the clinicalimmunologic analysis to 28 patients with antitr antibodies. Paraneoplastic cerebellar degeneration in a patient with breast cancer associated with carbonic anhydraserelated protein viii autoantibodies angeliki prevezianou, john s. Fiftytwo of them proved to have malignancies, almost exclusively breast or gynecologic cancers and usually confined to the involved. Paraneoplastic neurological disorders can involve any part of the nervous system and may affect multiple areas simultaneously table. Paraneoplastic cerebellar degeneration radiology reference.
Antitr antibodies were detected by immunohistochemistry. The tumors tend to progress quickly and often fatal. Paraneoplastic neurological disorders result from an autoimmune response against neural selfantigens that are ectopically expressed in neoplastic cells. Paraneoplastic cerebellar degeneration american journal of. Paraneoplastic cerebellar degeneration secondary to. Diagnosis can often be aided by detection of antibodies to various cerebellar proteins. We present a case of a 52yearold female presenting with diplopia, gait instability, dysarthria, dysphagia, nystagmus, and, most notably, new onset paroxysmal episodes of uncontrollable crying concerning for pseudobulbar affect pba.
Neuroimaging is usually unremarkable in paraneoplastic cerebellar degeneration pcd, at least in the early stages of the disease. Paraneoplastic cerebellar degeneration pcd is the most devastating and easily recognized paraneoplastic syndrome a remote neurological complication of cancer that cannot be attributed to direct effects of the neoplasm, its. The following diseases manifest by means of neurological dysfunction. Pdf paraneoplastic cerebellar degeneration associated with. Journal of neuroimmunology vol 344, in progress 15 july.
Paraneoplastic cerebellar degeneration an overview. In addition, 20% of huab negative paraneoplastic cerebellar degeneration patients without clinically identified lamberteaton myasthenic syndrome had pqtype voltagegated calcium channel antibodies, while only 2% of smallcell lung cancer patients without paraneoplastic symptoms had these antibodies. High titers in the patients serum and cerebrospinal fluid csf of autoantibodies directed against both neurons and tumor have been detected in some forms of this syndrome. Paraneoplastic cerebellar degeneration and antiyo antibodies in a man with prostatic adenocarcinoma. P cerebellar degeneration pcd presents with acute or subacute onset of ataxia, dysarthria, and intention tremor. Paraneoplastic cerebellar degeneration most often precedes a. Paraneoplastic cerebellar degeneration is usually associated with gynecological and breast cancer, lung cancer, and hodgkins lymphoma. Autoimmune response of patients with paraneoplastic cerebellar degeneration to a purkinje cell cytoplasmic protein antigen. Jul 09, 2019 cerebellar degeneration is defined as the loss of neurons in that particular region of the brain that controls the muscular function and balance. Typically the disorder starts with an unsteady gait which progresses rapidly to a pancerebellar syndrome rendering the patient bed bound, severely dysarthric, and tremulous within a. Pcd associated with ovarian malignancy is a rare occurrence with fewer than 100 cases reported in published work. Paraneoplastic cerebellar degeneration and dermatomyositis as first manifestations of underlying breast malignancy. For example, paraneoplastic cerebellar degeneration may improve after successful treatment of the underlying cancer.
Lawrence paraneoplastic neurological disorders pnds can be extremely complex and difficult to treat. Although cognitive function usually preserved, mild cognitive and memory deficits can occur in. Treatment options for paraneoplastic neurological disorders by carolyn z. Paraneoplastic cerebellar degeneration differential diagnoses. Paraneoplastic cerebellar degeneration associated with ovarian. Paraneoplastic cerebellar degeneration pcd is one of these neurologic diseases and patients present with acute or subacute onset of ataxia, dysarthria and intention tremor. Paraneoplastic cerebellar degeneration pcd is a heterogeneous group of disorders characterized by subacute cerebellar ataxia, specific tumour types and often associated antineuronal antibodies. Pcd is a rare, nonmetastatic complication of cancer. Paraneoplastic cerebellar degeneration pcd is a specific type of pns, associated with various autoantibodies, most commonly antiyo, which targets neurons and causes extensive loss of purkinje cells of the cerebellum.
The lymphoma preceded neurologic symptoms by 1 to 54 months in 1721 patients, but stage or activity did not correlate with severity of. Paraneoplastic cerebellar degeneration volume 20 issue s3 b. Patients whose cerebellar degeneration is a result of a tumor, for example paraneoplastic cerebellar degeneration, typically have a poor prognosis. A provisional diagnosis of paraneoplastic cerebellar degeneration, secondary to ovarian carcinosarcoma, was made. Because of malignancy, this degeneration occurs, and the condition is termed as paraneoplastic cerebellar degeneration. Antiyoassociated paraneoplastic cerebellar degeneration in a man with adenocarcinoma of unknown origin. Paraneoplastic cerebellar degeneration as initial presentation of. As the cerebellum contributes to the coordination and regulation of motor. Cerebellar degeneration is defined as the loss of neurons in that particular region of the brain that controls the muscular function and balance. The molecular layer and the cytoplasm of the purkinje cells in the cerebellum show a dotted to. We report the case of a 75 yearold woman who develop cerebellar ataxia following a respiratory viral infection. Paraneoplastic cerebellar degeneration in a patient with.
In patients older than 50 years, acute or subacute cerebellar degeneration is paraneoplastic in origin in 50% of cases. Antitr antibodies as markers of paraneoplastic cerebellar. Cerebellar hypermetabolism in paraneoplastic cerebellar. Patient with suspected paraneoplastic neurological syndrome reflex tests myasthenia gravis cerebellar degeneration, encephalomyelitislimbic encephalitis, sensory neuropathy, chorea, or optic neuritis encephalomyelitis or limbic encephalitis encephalitis with psychiatric manifestations, seizures, dyskinesias, dystonia, and autonomic instability. Paraneoplastic syndrome is a group of rare disorders that is triggered by an abnormal immune system response caused by a neoplasm. Paraneoplastic cerebellar degeneration sciencedirect. Preliminary studies suggested that antitr antibodies identify patients with paraneoplastic cerebellar degeneration pcd and hodgkin disease hd. Treatment options are not well defined and improvement is often limited.
Nine specific antineuronal antibodies are associated with pcd. Cerebellar degeneration is a condition in which cerebellar cells, otherwise known as neurons, become damaged and progressively weaken in the cerebellum. Paraneoplastic cerebellar degeneration pcd is a collection of neurological disorders resulting from tumor. Paraneoplastic cerebellar degeneration as the first. Paraneoplastic cerebellar degeneration pcd is a paraneoplastic syndrome associated with a broad variety of tumors including lung cancer, ovarian cancer, breast cancer, hodgkins lymphoma and others.
We evaluated the efficacies of immunotherapies in reported cases using a common scale of. Paraneoplastic cerebellar degeneration is a selflimited nonprogressive process in the majority of patients. Paraneoplastic cerebellar degeneration pcd is seen in a variety of tumours, most commonly breast and gynaecological cancer, lung cancer, and hodgkins disease. An initially negative ct does not preclude the diagnosis of remote effect cerebellar atrophy. Paraneoplastic cerebellar degeneration canadian journal of. Paraneoplastic cerebellar degeneration pcd is a rare neurological complication of cancer characterized by rapid development of cerebellar. There are a number of different antibodies and autoimmune reactions that are classified as pnds. There are nearly 30 different antibodies associated with this condition. Paraneoplastic cerebellar degeneration is a rare dysfunction of the cerebellum associated with malignancy. Paraneoplastic syndromes of the central nervous system repub. Antihu antibody associated paraneoplastic cerebellar. Paraneoplastic cerebellar degeneration disabled the patient in 34 months and remained as an irreversible process during the 3. A patient with proven pcd is reported in whom it could be shown that cerebellar atrophy evolved very rapidly and was present in early imaging studies. Paraneoplastic, autoantibody evaluation, serum mayo.
Even with the use of the whole spectrum of modern diagnostic tools, the underlying malignancy can be difficult to. Paraneoplastic cerebellar degeneration pcd is an uncommon disorder that can be associated with any cancer. Dec 14, 2014 for example, paraneoplastic cerebellar degeneration may improve after successful treatment of the underlying cancer. Paraneoplastic cerebellar degeneration is a rare complication of a number of cancers, particularly small cell lung cancer, gynecologic cancers and hodgkins disease. Despite the identification of an increasing number of immunemediated cerebellar ataxias, there is no proposed standardized therapy. It is hypothesized that paraneoplastic syndrome occurs when cancerfighting antibodies or white blood cells known as t cells mistakenly attack normal cells in the nervous system. Paraneoplastic syndromes are a group of rare disorders that include paraneoplastic cerebellar degeneration pcd. After a period of chemotherapy and steroid treatments, his symptoms are now stable in terms of cerebellar function.
Antibodies to cerebellar purkinje cells in patients with paraneoplastic cerebellar degeneration and ovarian carcinoma. Paraneoplastic cerebellar degeneration pcd is the most devastating and easily recognized paraneoplastic syndrome a remote neurological complication of cancer that cannot be attributed to direct effects of the neoplasm, its metastases, or treatment. Autoantibodies specific for onconeural proteins found in the plasma membrane, cytoplasm, and. Paraneoplastic cerebellar degeneration associated with cereb. Paraneoplastic cerebellar degeneration canadian journal. Paraneoplastic cerebellar degeneration with antiyo.
Paraneoplastic cerebellar degeneration is caused by the secondary effects of cancer and is believed to be immune mediated. Dec 22, 2015 paraneoplastic syndromes are most frequently associated with small cell lung carcinoma, hematologic and gynecologic malignancies while reports in head and neck cancer are rare. Paraneoplastic autoimmune neurological disorders reflect a patients humoral and cellular immune responses to cancer. Lamberteaton myasthenic syndrome, paraneoplastic cerebellar degeneration, encephalomyelitis, limbic encephalitis, brainstem encephalitis, opsoclonus myoclonus ataxia syndrome, antinmda receptor encephalitis, and polymyositis.
Pdf paraneoplastic cerebellar degeneration pcd is a rare neurological disorder characterized by a widespread loss of purkinje cells associated with. Nevertheless, it is the most common paraneoplastic syndrome affecting the brain. Paraneoplastic neurological syndromes quest diagnostics. The patient was diagnosed with an ovarian tumour, which was treated surgically. The lymphoma preceded neurologic symptoms by 1 to 54 months in 1721 patients, but stage or activity did not correlate with severity of neurologic disease. Paraneoplastic syndromes are a group of rare disorders that are triggered by an abnormal immune system response to an underlying usually undetected malignant. As no specific antineuronal antibodies have been described in the setting of renal cell carcinoma, paraneoplastic cerebellar degeneration should.
Diagnosis can often be aided by detection of antibodies to. Paraneoplastic cerebellar degeneration pcd is a rare neurological disorder characterized by a widespread loss of purkinje cells associated with a progressive pancerebellar dysfunction. Medline abstract for reference 1 of paraneoplastic. Brain imaging is usually remarkably unremarkable in pcd. Paraneoplastic cerebellar degeneration springerlink. This semiquantitative assay may be useful in monitoring. Paraneoplastic cerebellar degeneration genetic and rare.
Paraneoplastic cerebellar degeneration pcd is one of the most common pns. Paraneoplastic cerebellar degeneration pcd is a rare antiyo mediated paraneoplastic syndromes rarely that is infrequently associated with breast cancer. The most important part of treatment is to get a proper and rapid diagnosis of both the underlying cancer and the type of paraneoplastic disorder involved. It is relatively uncommon and is usually characterised by a rapid and severe cerebellar syndrome, leaving the patient dependent within a few weeks to months of onset. This form of cerebellar degeneration may precede cancer. The most common pnds include paraneoplastic cerebellar degeneration pcd, opsoclonusmyoclonus syndrome oms, eatonlambert syndrome, and paraneoplastic limbic. Subacute cerebellar ataxia in a patient with a known cancer is often due to metastatic invasion or other complications of the cancer, such as infection, coagulopathy, metabolic and nutritional deficits, or side effects of treatment. Paraneoplastic cerebellar degeneration pcd is a collection of neurological disorders resulting from tumorinduced autoimmunity against. For alcoholicnutritional cerebellar degeneration, symptoms are often relieved with discontinuation of alcohol abuse, a normal diet and dietary supplementation with thiamine and other b vitamins. Treatment options for paraneoplastic neurological disorders. Paraneoplastic cerebellar ataxia, also known as paraneoplastic cerebellar degeneration, is one of the wide array of paraneoplastic neurological syndromes in which neurological symptoms are indirectly caused by an underlying malignancy, most commonly gynecological, breast, or lung cancer or hodgkins lymphoma. May 11, 2015 paraneoplastic syndromes are a group of rare disorders that include paraneoplastic cerebellar degeneration pcd. Paraneoplastic cerebellar degeneration with bilateral. Paraneoplastic cerebellar degeneration pcd is still one of the most enigmatic of the autoimmune cns disorders.
Paraneoplastic cerebellar degeneration is a type of paraneoplastic syndrome characterized by the progressive onset of cerebellar dysfunction not explained by tumoral invasion, metastasis, or treatment side effects. Ta, antiri, antiyo, antizic4 antibodies, and antigad65 are found in patients with acute or subacute symptoms of cerebellar degeneration including gait ataxia, nystagmus andor dysarthria, and opsoclonusmyoclonus. Request pdf paraneoplastic cerebellar degeneration paraneoplastic neurological syndromes pns are an extensive group of neurologic disorders that occur in patients with cancer and can affect. Although uncommon, there have been some reports of sleep disturbances associated with cerebellar degeneration. Paraneoplastic syndromes are a rare heterogeneous group of disorders that are indicators of the underlying occult malignancy.
865 1506 1495 1219 1160 580 687 742 1369 1350 609 1391 93 606 184 680 459 1445 804 1477 643 859 954 863 1221 222 258 708 311 709 1439 404 447 174 703 608 444 660 807 743 1138 1318 290