Paraneoplastic Cerebellar Degeneration: Cerebellar degeneration associated with a remote neoplasm. Clinical manifestations include progressive limb and GAIT ATAXIA; DYSARTHRIA; and NYSTAGMUS, PATHOLOGIC. The histologic type of the associated neoplasm is usually carcinoma or lymphoma. Pathologically the cerebellar cortex and subcortical nuclei demonstrate diffuse degenerative changes. Anti-Purkinje cell antibodies (anti-Yo) are found in the serum of approximately 50% of affected individuals. (Adams et al., Principles of Neurology, 6th ed, p686)Paraneoplastic Syndromes: In patients with neoplastic diseases a wide variety of clinical pictures which are indirect and usually remote effects produced by tumor cell metabolites or other products.Cerebellar Diseases: Diseases that affect the structure or function of the cerebellum. Cardinal manifestations of cerebellar dysfunction include dysmetria, GAIT ATAXIA, and MUSCLE HYPOTONIA.Paraneoplastic Syndromes, Nervous System: Degenerative or inflammatory conditions affecting the central or peripheral nervous system that develop in association with a systemic neoplasm without direct invasion by tumor. They may be associated with circulating antibodies that react with the affected neural tissue. (Intern Med 1996 Dec;35(12):925-9)Lambert-Eaton Myasthenic Syndrome: An autoimmune disease characterized by weakness and fatigability of proximal muscles, particularly of the pelvic girdle, lower extremities, trunk, and shoulder girdle. There is relative sparing of extraocular and bulbar muscles. CARCINOMA, SMALL CELL of the lung is a frequently associated condition, although other malignancies and autoimmune diseases may be associated. Muscular weakness results from impaired impulse transmission at the NEUROMUSCULAR JUNCTION. Presynaptic calcium channel dysfunction leads to a reduced amount of acetylcholine being released in response to stimulation of the nerve. (From Adams et al., Principles of Neurology, 6th ed, pp 1471)Cerebellar Ataxia: Incoordination of voluntary movements that occur as a manifestation of CEREBELLAR DISEASES. Characteristic features include a tendency for limb movements to overshoot or undershoot a target (dysmetria), a tremor that occurs during attempted movements (intention TREMOR), impaired force and rhythm of diadochokinesis (rapidly alternating movements), and GAIT ATAXIA. (From Adams et al., Principles of Neurology, 6th ed, p90)Spinocerebellar Degenerations: A heterogenous group of degenerative syndromes marked by progressive cerebellar dysfunction either in isolation or combined with other neurologic manifestations. Sporadic and inherited subtypes occur. Inheritance patterns include autosomal dominant, autosomal recessive, and X-linked.Gastroscopes: Endoscopes used for examining the interior of the stomach.Purkinje Cells: The output neurons of the cerebellar cortex.Cerebellum: The part of brain that lies behind the BRAIN STEM in the posterior base of skull (CRANIAL FOSSA, POSTERIOR). It is also known as the "little brain" with convolutions similar to those of CEREBRAL CORTEX, inner white matter, and deep cerebellar nuclei. Its function is to coordinate voluntary movements, maintain balance, and learn motor skills.Paraneoplastic Polyneuropathy: A diffuse or multifocal peripheral neuropathy related to the remote effects of a neoplasm, most often carcinoma or lymphoma. Pathologically, there are inflammatory changes in peripheral nerves. The most common clinical presentation is a symmetric distal mixed sensorimotor polyneuropathy. (Adams et al., Principles of Neurology, 6th ed, p1334)Nerve Degeneration: Loss of functional activity and trophic degeneration of nerve axons and their terminal arborizations following the destruction of their cells of origin or interruption of their continuity with these cells. The pathology is characteristic of neurodegenerative diseases. Often the process of nerve degeneration is studied in research on neuroanatomical localization and correlation of the neurophysiology of neural pathways.Autoantibodies: Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.Nerve Tissue ProteinsAutoantigens: Endogenous tissue constituents that have the ability to interact with AUTOANTIBODIES and cause an immune response.Carcinoma, Small Cell: An anaplastic, highly malignant, and usually bronchogenic carcinoma composed of small ovoid cells with scanty neoplasm. It is characterized by a dominant, deeply basophilic nucleus, and absent or indistinct nucleoli. (From Stedman, 25th ed; Holland et al., Cancer Medicine, 3d ed, p1286-7)Limbic Encephalitis: A paraneoplastic syndrome marked by degeneration of neurons in the LIMBIC SYSTEM. Clinical features include HALLUCINATIONS, loss of EPISODIC MEMORY; ANOSMIA; AGEUSIA; TEMPORAL LOBE EPILEPSY; DEMENTIA; and affective disturbance (depression). Circulating anti-neuronal antibodies (e.g., anti-Hu; anti-Yo; anti-Ri; and anti-Ma2) and small cell lung carcinomas or testicular carcinoma are frequently associated with this syndrome.Lung Neoplasms: Tumors or cancer of the LUNG.Myoclonic Cerebellar Dyssynergia: A condition marked by progressive CEREBELLAR ATAXIA combined with MYOCLONUS usually presenting in the third decade of life or later. Additional clinical features may include generalized and focal SEIZURES, spasticity, and DYSKINESIAS. Autosomal recessive and autosomal dominant patterns of inheritance have been reported. Pathologically, the dentate nucleus and brachium conjunctivum of the CEREBELLUM are atrophic, with variable involvement of the spinal cord, cerebellar cortex, and basal ganglia. (From Joynt, Clinical Neurology, 1991, Ch37, pp60-1)Paraneoplastic Syndromes, Ocular: Ocular manifestations secondary to various NEOPLASMS in which antibodies to antigens of the primary tumor cross-react with ocular antigens. This autoimmune response often leads to visual loss and other ocular dysfunctions.Ataxia: Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharynx, larynx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or PERIPHERAL NERVE DISEASES. Motor ataxia may be associated with CEREBELLAR DISEASES; CEREBRAL CORTEX diseases; THALAMIC DISEASES; BASAL GANGLIA DISEASES; injury to the RED NUCLEUS; and other conditions.Ataxia Telangiectasia: An autosomal recessive inherited disorder characterized by choreoathetosis beginning in childhood, progressive CEREBELLAR ATAXIA; TELANGIECTASIS of CONJUNCTIVA and SKIN; DYSARTHRIA; B- and T-cell immunodeficiency, and RADIOSENSITIVITY to IONIZING RADIATION. Affected individuals are prone to recurrent sinobronchopulmonary infections, lymphoreticular neoplasms, and other malignancies. Serum ALPHA-FETOPROTEINS are usually elevated. (Menkes, Textbook of Child Neurology, 5th ed, p688) The gene for this disorder (ATM) encodes a cell cycle checkpoint protein kinase and has been mapped to chromosome 11 (11q22-q23).Retinal Degeneration: A retrogressive pathological change in the retina, focal or generalized, caused by genetic defects, inflammation, trauma, vascular disease, or aging. Degeneration affecting predominantly the macula lutea of the retina is MACULAR DEGENERATION. (Newell, Ophthalmology: Principles and Concepts, 7th ed, p304)Macular Degeneration: Degenerative changes in the RETINA usually of older adults which results in a loss of vision in the center of the visual field (the MACULA LUTEA) because of damage to the retina. It occurs in dry and wet forms.
Pulmonary Roundtable: A man with weakness and a some minor CT abnormalitiesDDx included subacute cerebellar degeneration and progressive supranuclear palsy.. Multiple CSF and serum labs were pending at ... And if it's E-L syndrome, maybe he has an underlying cancer (found in your core bx?) with paraneoplastic syndrome. ... Turns out he had sub-acute cerebellar degeneration, which is worse because (unlike LEMS) it does not get better after you treat ...
CDR1 Gene - GeneCards | CDR1 Protein | CDR1 AntibodyCerebellar Degeneration Related Protein 1, including: function, proteins, disorders, pathways, orthologs, and expression. ... Autoantibodies against CDR1 are found in patients with paraneoplastic cerebellar degeneration.. SequenceCaution:. *Sequence= ... Cloning of a brain protein identified by autoantibodies from a patient with paraneoplastic cerebellar degeneration. (PMID: ... CDR1 (Cerebellar Degeneration Related Protein 1) is a Protein Coding gene. Diseases associated with CDR1 include Cerebellar ...
Cerebellar hypermetabolism in paraneoplastic cerebellar degeneration | Journal of Neurology, Neurosurgery & PsychiatryA 51 year old man with paraneoplastic cerebellar degeneration from gastric adenocarcinoma showed cerebellar hypermetabolism and ... We suggest that the cerebellar hypermetabolism may have been due to an acute inflammatory process associated with an ...
Immune-Mediated Paraneoplasia - RedorbitParaneoplastic cerebellar degeneration Paraneoplastic cerebellar degeneration (PCD) was the first paraneoplasia to be described ... Association of anti-Yo (type 1) antibody with paraneoplastic cerebellar degeneration in the setting of transitional cell ... was established in paraneoplastic cerebellar degenerations when, in some cases, the cause of the unnatural hypersensitivity ... Subacute cortical cerebellar degeneration and its relation to carcinoma. J Neurol Neurosurg Psychiatry 1951; 14: 59-75. ...
Paraneoplastic syndromes of the nervous system - Symptoms and causes - Mayo ClinicParaneoplastic syndromes - Comprehensive overview covers symptoms, treatment of rare neurological disorders associated with ... Examples of paraneoplastic syndromes of the nervous system include:. *Cerebellar degeneration. This is the loss of nerve cells ... Paraneoplastic cerebellar degeneration. http://www.uptodate.com/home. Accessed Nov. 28, 2016. ... Neurologic paraneoplastic syndromes. In: Harrison's Principles of Internal Medicine. 19th ed. New York, N.Y.: McGraw-Hill ...
Small Cell Lung Cancer Treatment (PDQ®)-Health Professional Version - National Cancer InstituteParaneoplastic cerebellar degeneration.. *Lambert-Eaton myasthenic syndrome.. Physical examination may identify enlarged ... Infrequently, patients with SCLC may present with symptoms and signs of one of the following paraneoplastic syndromes:. * ... or paraneoplastic phenomena. The most common symptoms at presentation are worsening cough, shortness of breath, and dyspnea. ...
Biofiltration, a new method of short hemodiafiltration: preliminary report.1407576 - Paraneoplastic cerebellar degeneration. ii. clinical and immunologic findings in 21 pat.... 19379146 - Target for ...
Plus itTumor-specific killer cells in paraneoplastic cerebellar degeneration. Nat Med 1998; 4: 1321-4. ...
KAKEN - Research Projects | Studies on the structure and function of protein kinase PKN (KAKENHI-PROJECT-08458230)Publications] Takanaga, H.: 'PKN interacts with a paraneoplastic cerebellar degeneration-associated antigen,which is a ... Publications] Takanaga, H.: 'PKN interacts with a paraneoplastic cerebellar degeneration-associated antigen, which is a ... Publications] Takanaga,H.: 'PKN interacts with a paraneoplastic cerebellar degeneration-associated antigen, which is a ... detected in sera and cerebrospinal fluids of patients with paraneoplastic cerebellar degeneration syndrome, and it has been ...
Protocols and Video Articles Authored by Yolanda BlancoThe fourth patient presented with paraneoplastic cerebellar degeneration antedating a lung adenocarcinoma. The frequency of ... with paraneoplastic encephalomyelitis, a predominant gait cerebellar ataxia, and limbic encephalitis, had neuroendocrine ... Cerebellar ataxia, the second most common syndrome associated with high GAD-ab levels, shares with SPS the same demographic, ... Cerebellar Ataxia and Glutamic Acid Decarboxylase Antibodies: Immunologic Profile and Long-term Effect of Immunotherapy JAMA ...
Primecuts - This Week In The Journals | Clinical Correlations... and paraneoplastic cerebellar degeneration). Overall, PNDs were observed in 9.1% of all patients with small cell lung cancer, ... Paraneoplastic neurologic disorders (PNDs) are generally thought to be rare disorders, but they may be more common than ... Paraneoplastic neurologic disorders in small cell lung carcinoma: A prospective study. Neurology, 2015. 85(3): p. 235-9. http ... Paraneoplastic Neurologic Disorders in Small Cell Lung Cancer May Be More Common than Previously Thought ...
Topics covered | Free Medical App - Android and Apple IosParaneoplastic cerebellar degeneration. *Paraneoplastic limbic encephalitis (PLE). *Paraneoplastic neurologic syndromes. * ...
Types of Ataxia/muscular dystrophy | Pune Ataxia Support BlogParaneoplastic Cerebellar Degeneration (PCD). Neurological Paraneoplastic Syndromes are a group of rare degenerative diseases ... Disability may be greater and progress more quickly with the Cerebellar plus form of Sporadic Ataxia. The Cerebellar plus is a ... Sporadic Ataxia can be either "pure cerebellar" if only the Cerebellum is affected or Cerebellar plus. If the Ataxia is ... Ataxia Results in the Degeneration of Nerve Cells:. Eventually the affected Nerve Cells begin to function poorly and ultimately ...
Autoimmune thyroid gastrointestinal hypertension - Thyromine - Natural Herbal Thyroid Health Supplement - Apr 28, 2017Paraneoplastic Cerebellar Degeneration Clinical... (http://emedicine.medscape.com/article/1156965-clini cal) Nonparaneoplastic ...
Generation of tumor-initiating cells by exogenous delivery of OCT4 transcription factorSabater L, Bataller L, Suarez-Calvet M, Saiz A, Dalmau J, Graus F. ZIC antibodies in paraneoplastic cerebellar degeneration and ...
Paraneoplastic SyndromeThe first report of Paraneoplastic syndrome is attributed to a French physician, M Auche, who described the involvement in a ... Paraneoplastic cerebellar degeneration causes gait difficulties, dizziness, nausea and diplopia. Sensory neuropathy affects ... Paraneoplastic Syndrome. The first report of Paraneoplastic syndrome is attributed to a French physician, M Auche, who ... Paraneoplastic syndrome is defined as a rare disorder triggered by a response to neoplasm of an immune system that is altered. ...
DMOZ - Health: Conditions and Diseases: Neurological Disorders: Brain Diseases: CerebellarCardinal manifestations of cerebellar dysfunction include dysmetria, gait ataxia, and muscle hypotonia. ... NIH: Ataxias and Cerebellar/Spinocerebellar Degeneration Information sheet compiled by the National Institute of Neurological ... Cardinal manifestations of cerebellar dysfunction include dysmetria, gait ataxia, and muscle hypotonia. ...
High-Dose Intravenous Immunoglobulin to Treat Cerebellar Degeneration - Full Text View - ClinicalTrials.govKnown paraneoplastic cerebellar degeneration.. Cerebellar ataxia that is congenital, static and/or symptomatic (due to stroke, ... Adults over 18 with hereditary or sporadic cerebellar degeneration. Sporadic cerebellar degeneration may include the cerebellar ... Spinocerebellar Degenerations. Spinocerebellar Ataxias. Cerebellar Diseases. Brain Diseases. Central Nervous System Diseases. ... Patients will be selected from the motor control clinic based on a diagnosis of sporadic or hereditary cerebellar degeneration ...
Publications | Woodruff LaboratorySelective expression of Purkinje-cell antigens in tumor tissue from patients with paraneoplastic cerebellar degeneration. N ...
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Kids.Net.Au - Encyclopedia > List of rare diseases starting...Paraneoplastic cerebellar degeneration[?] * Paraomphalocele[?] * Paraparesis amyotrophy of hands and feet[?] * Paraplegia[?] * ...
CancerNeurological Function after Concurrent Treatment IVIG and Chemotherapy in a Patient with Cerebellar Degeneration Paraneoplastic ...
Overview of cerebellar ataxia in adultsAlthough cerebellar degeneration may be chronic and slowly progressive, acute cerebellar swelling due to infarction, edema, or ... Cerebellar ataxia is a common finding in patients seen in neurologic practice and has a wide variety of causes. ... Contactin-associated protein-2 antibodies in non-paraneoplastic cerebellar ataxia. J Neurol Neurosurg Psychiatry 2012; 83:437. ... Although cerebellar degeneration may be chronic and slowly progressive, acute cerebellar swelling due to infarction, edema, or ...
Surgery Definition - What is Healing By Secondary Intention? - Medical ZoneSymptom Checker - Paraneoplastic Neurologic Syndromes * Symptom Checker - Paraneoplastic Endocrine Syndrome * Symptom Checker ... Pediatric Definition - Acute Cerebellar Ataxia * Pediatric Definition - Acute Gastroenteritis * Pediatric Definition - Acute ... Symptom Checker - Macular Degeneration * Symptom Checker - Malaria * Symptom Checker - Mallory Weiss Tear ...
Rock Yo Hips: "Rock Yo Hips" is the first single of the hip hop/crunk group Crime Mob's second studio album, Hated on Mostly. The song was released on August 29, 2006 as a digital download single on iTunes.Paraneoplastic syndrome: A paraneoplastic syndrome is a syndrome (a set of signs and symptoms) that is the consequence of cancer in the body but that, unlike mass effect, is not due to the local presence of cancer cells.Paraneoplastic Syndromes, 2011, Darnell & Posner These phenomena are mediated by humoral factors (by hormones or cytokines) excreted by tumor cells or by an immune response against the tumor.Cerebellar stroke syndromeMetastatic carcinoma: Metastatic carcinoma is able to grow at sites distant from the primary site of origin; thus, dissemination to the skin may occur with any malignant neoplasm, and these infiltrates may result from direct invasion of the skin from underlying tumors, may extend by lymphatic or hematogenous spread, or may be introduced by therapeutic procedures.James, William; Berger, Timothy; Elston, Dirk (2005).Muscular Dystrophy AssociationAutosomal recessive cerebellar ataxia: Autosomal recessive cerebellar ataxia type 1 (ARCA1) is a condition characterized by progressive problems with movement. Signs and symptoms of the disorder first appear in early to mid-adulthood.Instruments used in gastroenterology: Instruments used specially in Gastroenterology are as follows:Neurochondrin: Neurochondrin proteins induces hydroxyapatite resorptive activity in bone marrow cells resistant to bafilomycin A1, an inhibitor of macrophage- and osteoclast-mediated resorption. Expression of the gene is localised to chondrocyte, osteoblast, and osteocyte in the bone and to the hippocampus and Purkinje cell layer of cerebellum in the brain.Inferior cerebellar peduncle: The upper part of the posterior district of the medulla oblongata is occupied by the inferior cerebellar peduncle (restiform body), a thick rope-like strand situated between the lower part of the fourth ventricle and the roots of the glossopharyngeal and vagus nerves.Autoantibody: An autoantibody is an antibody (a type of protein) produced by the immune system that is directed against one or more of the individual's own proteins. Many autoimmune diseases, (notably lupus erythematosus), are caused by such autoantibodies.Small-cell carcinomaLimbic encephalitisTargeted therapy of lung cancer: Targeted therapy of lung cancer refers to using agents specifically designed to selectively target molecular pathways responsible for, or that substantially drive, the malignant phenotype of lung cancer cells, and as a consequence of this (relative) selectivity, cause fewer toxic effects on normal cells.Dyssynergia: Dyssynergia is any disturbance of smooth muscular coordination, resulting in uncoordinated and abrupt movements. It is typical for dyssynergic patients to split a movement into several smaller movements.Fragile X-associated tremor/ataxia syndrome: Fragile X-associated tremor/ataxia syndrome (FXTAS) is a late onset neurodegenerative disorder associated with problems of movement, memory, and the autonomic nervous system. It is related to the disease fragile X syndrome, although FXTAS is a clinically distinct syndrome.Ataxia telangiectasia mutated: Ataxia telangiectasia mutated (ATM) is a serine/threonine protein kinase that is recruited and activated by DNA double-strand breaks. It phosphorylates several key proteins that initiate activation of the DNA damage checkpoint, leading to cell cycle arrest, DNA repair or apoptosis.Age-Related Eye Disease Study: The Age-Related Eye Disease Study (AREDS) was a clinical trial sponsored by the National Eye Institute, one of the National Institutes of Health in the United States.A Randomized, Placebo-Controlled, Clinical Trial of High-Dose Supplementation With Vitamins C and E, Beta Carotene, and Zinc for Age-Related Macular Degeneration and Vision Loss.
(1/27) Cdr2, a target antigen of naturally occuring human tumor immunity, is widely expressed in gynecological tumors.
The paraneoplastic neurological disorders provide perhaps the best known example of naturally occurring tumor immunity in humans. For example, patients with paraneoplastic cerebellar degeneration (PCD) appear to suppress the growth of occult breast or ovarian tumors that express a neuronal antigen termed cdr2. PCD patients harbor cdr2-specific CTLs in their peripheral blood, and these cells are likely mediators of the tumor suppression. Whereas cdr2 therefore appears to be the target of an effective immune response in patients with PCD, the general relevance to cancer patients has been unclear, due in part to reports indicating that cdr2 is not expressed in tumors obtained from neurologically normal patients. We have reexamined this question, and we find that cdr2 is widely expressed in such tumors, indicating that cdr2 is in fact an important tumor antigen in the general population of breast and ovarian cancer patients. (+info)
(2/27) A case of paraneoplastic syndrome accompanied by two types of cancer.
A case of paraneoplastic syndrome accompanied by two types of cancer is reported. The patient was a 62 year old man who progressively developed cerebellar ataxia, especially an abnormal gait. The anti-Hu antibody titre was high. A small tumour was detected in the middle lobe of the right lung and was surgically treated. The histology was adenocarcinoma. After lobectomy, however, the ataxia deteriorated, and plasma exchange, 250 ml/kg/day, was conducted for 6 days. After plasma exchange, the anti-Hu antibody titre decreased and the ataxia temporarily ceased to progress. A week after the last plasma exchange, a mass appeared in the anterior cervical region and rapidly increased in size. The biopsy of the neck tumour disclosed a small cell carcinoma. Five months later small cell carcinoma appeared in the left lung. This case shows the importance of searching for small cell carcinoma when anti-Hu antibodies are detected. It is assumed that plasma exchange removed not only a pathogenic factor of ataxia but also a factor which inhibited the growth of the small cell carcinoma. It is recommended that plasmapheresis should be performed with caution in paraneoplastic syndrome when the origin of a tumour is obscure. (+info)
(3/27) The immunopathogenesis of paraneoplastic neurological syndromes.
Paraneoplastic neurological syndromes are rare non-metastatic complications of cancer that have an immune-mediated aetiology. The central and peripheral nervous systems are considered to be immune-privileged sites, since the presence of the 'blood-brain/nerve barrier' means that antigens sequestered within the nervous system do not normally induce an immune response. Aberrant expression of a neuronal antigen by a tumour arising outside this barrier can lead to the breakdown of immune tolerance to the nervous system. However, in many cases the immune mechanisms that result in neurological dysfunction remain poorly defined. Furthermore, aberrant expression of neuronal antigens can be detected in many tumours that are not complicated by non-metastatic neurological syndromes. This review article examines current concepts in the immunopathogenesis of paraneoplastic neurological syndromes. (+info)
(4/27) Paraneoplastic cerebellar degeneration associated with antineuronal antibodies: analysis of 50 patients.
Paraneoplastic cerebellar degeneration (PCD) is a heterogeneous group of disorders characterized by subacute cerebellar ataxia, specific tumour types and (often) associated antineuronal antibodies. Nine specific antineuronal antibodies are associated with PCD. We examined the relative frequency of the antineuronal antibodies associated with PCD and compared the neurological symptoms and signs, associated tumours, disability and survival between groups of PCD with different antibodies. Also, we attempted to identify patient-, tumour- and treatment-related characteristics associated with functional outcome and survival. In a 12-year period, we examined >5000 samples for the presence of antineuronal antibodies. A total of 137 patients were identified with a paraneoplastic neurological syndrome and high titre (> or =400) antineuronal antibodies. Fifty (36%) of these patients had antibody-associated PCD, including 19 anti-Yo, 16 anti-Hu, seven anti-Tr, six anti-Ri and two anti-mGluR1. Because of the low number, the anti-mGluR1 patients were excluded from the statistical analysis. While 100% of patients with anti-Yo, anti-Tr and anti-mGluR1 antibodies suffered PCD, 86% of anti-Ri and only 18% of anti-Hu patients had PCD. All patients presented with subacute cerebellar ataxia progressive over weeks to months and stabilized within 6 months. The majority of patients in all antibody groups had both truncal and appendicular ataxia. The frequency of nystagmus and dysarthria was lower in anti-Ri patients (33 and 0%). Later in the course of the disease, involvement of non-cerebellar structures occurred most frequently in anti-Hu patients (94%). In 42 patients (84%), a tumour was detected. The most commonly associated tumours were gynaecological and breast cancer (anti-Yo and anti-Ri), lung cancer (anti-Hu) and Hodgkin's lymphoma (anti-Tr and anti-mGluR1). In one anti-Hu patient, a suspect lung lesion on CT scan disappeared while the PCD evolved. Seven patients improved by at least 1 point on the Rankin scale, while 16 remained stable and 27 deteriorated. All seven patients that improved received antitumour treatment for their underlying cancer, resulting in complete remission. The functional outcome was best in the anti-Ri patients, with three out of six improving neurologically and five were able to walk at the time of last follow-up or death. Only four out of 19 anti-Yo and four out of 16 anti-Hu patients remained ambulatory. Also, survival from time of diagnosis was significantly worse in the anti-Yo (median 13 months) and anti-Hu (median 7 months) patients compared with anti-Tr (median >113 months) and anti-Ri (median >69 months). Patients receiving antitumour treatment (with or without immunosuppressive therapy) lived significantly longer [hazard ratio (HR) 0.3; 95% confidence interval (CI) 0.1-0.6; P = 0.004]. Patients > or =60 years old lived somewhat shorter from time of diagnosis, although statistically not significant (HR 2.9; CI 1.0-8.5; P = 0.06). (+info)
(5/27) Acute onset paraneoplastic cerebellar degeneration in a patient with small cell lung cancer.
A patient with small cell lung cancer presented with a rare presentation of an acute onset pancerebellar dysfunction. His clinical condition markedly improved following the surgical removal of the tumor and chemo- and radiotherapy. (+info)
(6/27) Anti-Yo antibody-mediated paraneoplastic cerebellar degeneration in a man with esophageal adenocarcinoma.
We report a case of paraneoplastic cerebellar degeneration (PCD) associated with adenocarcinoma of the esophagus and anti-Yo antibodies in a male patient. The patient presented with progressive ataxia, dysarthria, and significant weight loss. Extensive work-up suggested paraneoplastic neurologic syndrome. A wide search for a cancer was undertaken and a small mass was identified in the distal esophagus on upper endoscopy. Biopsies of this lesion revealed well-differentiated invasive adenocarcinoma of the esophagus. The endoscopic ultrasound staged the tumor as T3N1M0. Despite trials of multiple therapeutic modalities, the patient's cerebellar dysfunction progressed. This is only the second report of PCD caused by esophageal adenocarcinoma and the fourth report of anti-Yo antibodies occurring in a male patient. (+info)
(7/27) Paraneoplastic cerebellar degeneration (PCD) associated with squamous cell carcinoma of the lung.
We report a case of a 71-year-old man who presented with cerebellar dysfunction. He was diagnosed as having squamous cell carcinoma of the lung (T2N3M0, Stage IIIB). No anti-onconeural antibodies were found in his serum. Cerebral spinal fluid (CSF) examination showed mild mononuclear pleocytosis alone. Magnetic resonance imaging (MRI) of the brain and spinal cord revealed no abnormalities. At autopsy, there was complete disappearance of Purkinje cells with reactive astrocytosis. These findings are compatible with paraneoplastic cerebellar degeneration (PCD). To our knowledge, no case of PCD has been reported previously in patients with squamous cell carcinoma of the lung. (+info)
(8/27) A pre-senile case of limbic encephalitis and cerebellar degeneration, with subacute onset of progressive dementia.
In case a pre-senile patient presented subacutely progressive dementia, secondary dementia, such as paraneoplastic neurological syndrome (PNS), hypothyroidism, confusion, early phase of primary degenerative dementia and prion diseases are to be considered. It is a case of pathologically confirmed, and clinico-pathologically assessed limbic encephalitis with cerebellar degeneration. The patient was a 63-year old male, with a well followed up medical history of gastric cancer 8 years earlier. Four weeks after he presented himself at our hospital his memory and disorientation progressively declined. A neurological examination revealed gaze nystagmus, with potential secondary dementia. However, no abnormal findings were detected from systemic radiological examination, or from chemical analyses. Two months later, after the onset of the disease, he presented additional symptoms, including seizure, gait disturbance, and insomnia. On admission, neurological examinations revealed gaze nystagmus and progression of dementia; however, his thought process was relatively preserved. No paroxysmal synchronized discharge was seen on electroencephalogram. Chest X-rays showed an inflammatory infiltration. In spite of anti-biotic medication, he died due to respiratory failure. The autopsy was limited to the brain. Histologically, limited lymphocytic infiltration into the hippocampus through the entorhinal cortex, with marked neuronal loss and gliosis was observed. Neuronophagia, microglial nodules, and perivascular lymphocytic infiltration were also seen. Additionally, most of the Purkinje cells in the cerebellum were lost, with Bergmann's gliosis and sparse lymphocytic infiltration. No tumor was observed in the brain. Pathological findings of the brain were compatible with paraneoplastic limbic encephalitis and cerebellar degeneration, though no neoplasm, clinically or pathologically, was detected in this patient. Consequently, it is suggested that when a senile patient presents sub-acute onset of progressive dementia, with a variety of neurological symptoms, paraneoplastic syndrome is to be taken into consideration, even if a tumor or an auto-antibody is not detected since the resection of the tumor is still the best therapeutic means. Otherwise immuno-suppressive and steroid therapies should be used. (+info)
- PCD17 is a neural antigen, which is recognized by anti-Pukinje cell autoantibodies, detected in sera and cerebrospinal fluids of patients with paraneoplastic cerebellar degeneration syndrome, and it has been suggested to be transcriptional factor by the primary structure. (nii.ac.jp)
- Signs and symptoms of paraneoplastic syndromes of the nervous system can develop relatively quickly, often over days to weeks. (mayoclinic.org)
- Signs and symptoms of paraneoplastic syndromes of the nervous system often begin even before a cancer is diagnosed. (mayoclinic.org)
- Paraneoplastic syndromes occur typically among middle-aged to older patients. (targetwoman.com)
- As Paraneoplastic syndromes may evolve over weeks to months, they usually stabilize regardless whether the patient's underlying condition improves or worsens. (targetwoman.com)
- The Cerebellar plus is a form of Sporadic Ataxia which is also known as Sporadic Olivopontocerebellar Ataxia ( sporadic OPCA) or multiple system atrophy, Cerebellar type (MSA-C). (wordpress.com)
- Sporadic cerebellar degeneration may include the cerebellar predominant variant of Multiple System Atrophy (MSA-C). Hereditary ataxia is limited to the SCAs (spinocerebellar ataxias) or those patients with clear autosomal dominant ataxia. (clinicaltrials.gov)
- Other PNDs were also seen at lower frequencies (paraneoplastic encephalomyelitis, dermatomyositis, and paraneoplastic cerebellar degeneration). (clinicalcorrelations.org)
- Paraneoplastic limbic encephalitis is characterized by depression, seizures, irritability and short term memory loss and neurologic symptoms rapidly resemble dementia . (targetwoman.com)
- Sporadic Ataxia can be either "pure cerebellar" if only the Cerebellum is affected or Cerebellar plus. (wordpress.com)
- This study will examine whether high-dose intravenous immunoglobulin (IVIG) is safe and effective for treating cerebellar ataxia-degeneration of the cerebellum, the part of the brain responsible for coordinating muscle movements and balance. (clinicaltrials.gov)
- Paraneoplastic cerebellar degeneration causes gait difficulties, dizziness, nausea and diplopia. (targetwoman.com)
- Cardinal manifestations of cerebellar dysfunction include dysmetria, gait ataxia, and muscle hypotonia. (dmoztools.net)
- Disability may be greater and progress more quickly with the Cerebellar plus form of Sporadic Ataxia. (wordpress.com)
- Patients 18 years of age and older with hereditary (genetic) or sporadic (unknown cause) cerebellar degeneration may be eligible for this 5-month study. (clinicaltrials.gov)
- There is increasing evidence that there is an antibody mediated, autoimmune component in hereditary and sporadic cerebellar degeneration. (clinicaltrials.gov)
- Patients will be selected from the motor control clinic based on a diagnosis of sporadic or hereditary cerebellar degeneration. (clinicaltrials.gov)
- Adults over 18 with hereditary or sporadic cerebellar degeneration. (clinicaltrials.gov)
- Myasthenia gravis is a common Paraneoplastic syndrome in patients with thymoma, a malignancy arising from epithelial cells of the thymus. (targetwoman.com)
- Symptoms and signs may result from the location of the primary local invasion or compression of adjacent thoracic structures, distant metastases, or paraneoplastic phenomena. (cancer.gov)
- tumor induced osteomalacia: a forgotten paraneoplastic syndrome? (nvb-nsn.gov)
- When myasthenia gravis occurs as a paraneoplastic syndrome, it is typically associated with cancer of the thymus gland (thymoma). (mayoclinic.org)
- Autoantibodies directed against the protein encoded by this intronless gene have been found in some patients with paraneoplastic cerebellar degeneration. (genecards.org)
- Autoantibodies against CDR1 are found in patients with paraneoplastic cerebellar degeneration. (genecards.org)
- The first report of Paraneoplastic syndrome is attributed to a French physician, M Auche, who described the involvement in a peripheral nervous system in cancer patients in 1890. (targetwoman.com)
- This study is a clinical, randomized, placebo-controlled trial of IVIG in adult patients with cerebellar ataxia. (clinicaltrials.gov)
- CDR1 (Cerebellar Degeneration Related Protein 1) is a Protein Coding gene. (genecards.org)
- We suggest that the cerebellar hypermetabolism may have been due to an acute inflammatory process associated with an immunological reaction. (bmj.com)
- Diseases associated with CDR1 include Cerebellar Degeneration and Paraneoplastic Cerebellar Degeneration . (genecards.org)
- When it occurs as a paraneoplastic syndrome, Lambert-Eaton myasthenic syndrome is typically associated with lung cancer. (mayoclinic.org)
- Paraneoplastic neurologic disorders (PNDs) are generally thought to be rare disorders, but they may be more common than initially thought. (clinicalcorrelations.org)
- Paraneoplastic syndrome is defined as a rare disorder triggered by a response to neoplasm of an immune system that is altered. (targetwoman.com)
- A 51 year old man with paraneoplastic cerebellar degeneration from gastric adenocarcinoma showed cerebellar hypermetabolism and increased perfusion on brain FDG-PET scan and SPECT during the acute stage of his illness. (bmj.com)