Presentation
The clinical, radiological, and histopathologic findings of the patient are presented. A 49-year-old woman presented with a history of progressive asthenia of the lower extremities persisting for 2 months. [ncbi.nlm.nih.gov]
Not all gynecologic cancers present as paraneoplastic neurologic syndrome; however, in a clinical presentation consistent with a paraneoplastic neurologic syndrome, the chances of underlying malignancy are very high. [emedicine.com]
We present here the first known case of anti-Hu seropositivity, cerebellar ataxia, and sensory neuropathy in association with cervical cancer. [jhu.pure.elsevier.com]
Entire Body System
- Inflammation
The muscle destruction (necrosis) is more prominent than the presence of inflammation. [rarediseases.org]
Fundus examination showed no signs of inflammation, no narrowing of the retinal arterioles and no diabetic retinopathy. [atlasophthalmology.net]
Polymyositis is muscle weakness and soreness resulting from muscle inflammation. When polymyositis is accompanied by skin inflammation, the condition is called dermatomyositis. Hypertrophic osteoarthropathy can occur in people with lung cancer. [merckmanuals.com]
Fatty acid oxidation, OTC/CPS deficiencies, citrullinemia Increased ICP in acute liver failure : High mortality (20-30% of ALF patients die from this) Mechanism not entirely clear, likely multifactorial Acute liver failure leads to increased ammonia and inflammation [ucsfmed.wordpress.com]
This is inflammation affecting a region of the brain known as the limbic system, which controls emotions, behaviors and certain memory functions. [mayoclinic.org]
Musculoskeletal
- Myopathy
Schl sselw rter akute lymphatische Leuk mie paraneoplastisches Syndrom Kindheit Myopathie Paraneoplastic Myopathy as a Very Rare Manifestation of Acute Lymphoblastic Leukemia Paraneoplastische Muskelschw che als sehr seltene Manifestation bei einer akuten [docslide.com.br]
Neoplasm: Waldenström's macroglobulinemia Rippling muscle syndrome Neoplasm: Thymoma Scleromyxedema Skin papules; Myopathy; Raynaud's Neoplasm: IgG or IgA λ M-protein Amyloid myopathy Metabolic/Hormonal Corticosteroid myopathy Ectopic ACTH production [neuromuscular.wustl.edu]
Neoplasms (lymphoma, leukemia, or lung cancer) may be detected in the patient either before the development of myopathy or subsequent to the development of myopathy. [yourarticlelibrary.com]
LEMS sometimes can be accompanied by xerostomia, sexual impotence, myopathy, and peripheral neuropathy. It is associated with cancer 40-70% of the time, most commonly small cell lung cancer (SCLC). [emedicine.medscape.com]
Necrotizing Autoimmune Myopathy This is a disease that associates with prominent muscle weakness at the level of the shoulders and hips. The muscle destruction (necrosis) is more prominent than the presence of inflammation. [rarediseases.org]
Urogenital
- Kidney Failure
As a result of the high calcium levels in the blood, the person develops kidney failure and confusion, which can progress to coma and even death if not recognized and treated promptly. [merckmanuals.com]
[…] or kidney failure, may result in death as well. [ citation needed ] Research directions [ edit ] Prostate cancer is the second most common urological malignancy to be associated with paraneoplastic syndromes after renal cell carcinoma. [en.wikipedia.org]
Neurologic
- Dysarthria
The first patient was a 73-year-old woman who was admitted to our hospital because of dysequilibrium and dysarthria of subacute onset. Chest CT showed swelling of the mediastinal and right hilar lymphnodes. [jstage.jst.go.jp]
To identify an autoreactivity in a 66-year-old woman who presented with combined brainstem and cerebellar syndrome including vertical gaze palsy, severe progressive ataxia, and spastic tetraparesis, an acute deterioration of vision, dysarthria, and dysphagia [ncbi.nlm.nih.gov]
Neurological symptoms may include, among others, dysarthria, truncal, limb and gait ataxia and nystagmus. [en.wikipedia.org]
After resection, his paralysis and dysarthria were resolved to the extent of being able to walk and speak fluently. Brain MP-RAGE showed no findings suggestive of metastasis or atrophy. [dear-medic.jp]
Subacute cerebellar degeneration causes progressive bilateral leg and arm ataxia, dysarthria, and sometimes vertigo and diplopia. [msdmanuals.com]
- Nystagmus
Neurological symptoms may include, among others, dysarthria, truncal, limb and gait ataxia and nystagmus. [en.wikipedia.org]
[…] resolved 2 weeks after treatment, but cognitive impairment and psychological symptoms remainedErkmen et al. [ 17 ] 201161FSeizuresLGI1-Cell surfaceIVIG, steroids, thymomectomyOver 4 weeks prior to thymomectomyWerry et al. [ 18 ] 200932MVertigo, diplopia, nystagmus [nnjournal.net]
However, SSN is usually associated with central nervous system signs, ranging from mild nystagmus to severe encephalopathy. [yourarticlelibrary.com]
Plasma exchange Ataxia with antibodies to CARP 8 10 Epidemiology: 1 patient; 77 year old female Clinical: Pure cerebellar syndrome Onset: 3 years after neoplasm Ataxia: Limb; Truncal; Gait; Dysarthria Ocular: Horizontal nystagmus Mental status: Normal [neuromuscular.wustl.edu]
- Guillain-Barré Syndrome
Therefore, motor neuron disease and peripheral neuropathic syndromes are only "possible" paraneoplastic diseases by current diagnostic guidelines. 1 The authors concluded that the low prevalence of Guillain-Barré syndrome in the EURO-Net database suggests [ahcmedia.com]
Guillain-Barré syndrome is another type of nerve dysfunction that causes a general loss of muscle strength. It is more common in people with Hodgkin lymphoma. [merckmanuals.com]
Although the frequency of cancer in patients with Guillain-Barré syndrome is not increased, the association between this neuropathy and HL is well established. 92 Patients with NHL and monoclonal gammopathy can develop typical CIDP or predominantly sensory [bloodjournal.org]
Guillain-Barré syndrome, another ascending peripheral neuropathy, is a rare finding in the general population and probably more common in patients with Hodgkin lymphoma. [msdmanuals.com]
GD1b-specific antibody induces ataxia in Guillain-Barré syndrome. Neurology. 2008;71:196–201. PubMed View Article Google Scholar Ito M, Matsuno K, Sakumoto Y, Hirata K, Yuki N. [jneuroinflammation.com]
- Paresthesia
In June 2012, she was again hospitalized because of progressive paresthesia of her lower limbs, and additionally severe constipation. An abdominal USG showed right adrenal incidentaloma. [jneuro.com]
This individual’s symptoms fluctuated until December 2004, at which time they progressed to include worsening headaches, ascending paresthesias, ataxia, and lower limb pain and hypersensitivity. [current-oncology.com]
Symptoms include fatigue, weakness, muscular pain in upper arms, difficulty walking, burning, numbness or tingling sensations in the limbs (peripheral paresthesia), dry mouth, sexual function difficulty, and drooping eyelids. [encyclopedia.com]
Symptoms and signs include fatigability, weakness, pain in proximal limb muscles, peripheral paresthesias, dry mouth, erectile dysfunction, and ptosis. Deep tendon reflexes are reduced or lost. [msdmanuals.com]
Frequency Clinical ~ 5% of patients with cancer: More common later in disease course than at onset 100% with weight loss > 15% Electrophysiological change: 30% to 40% Clinical Distribution: Distal; Legs > Arms; Symmetric Sensory: Panmodal sensory loss; Paresthesias [neuromuscular.wustl.edu]
- Tingling
[…] after thymomectomy, radiation and steroid; developed spasms and rigidity after prednisone taper, not responsive to PLEX or IVIG, but improved with steroids onlyVernino et al. [ 24 ] 200234FVertigo, tinnitus, vomiting; later, leg weakness and numbness, tingling [nnjournal.net]
Symptoms include fatigue, weakness, muscular pain in upper arms, difficulty walking, burning, numbness or tingling sensations in the limbs (peripheral paresthesia), dry mouth, sexual function difficulty, and drooping eyelids. [encyclopedia.com]
Workup
The subsequent workup and operative intervention identified a T2N0M0 SCC of the tonsil. Following resection, the patient's overall symptoms were significantly alleviated, and his gait improved. [ncbi.nlm.nih.gov]
The image below illustrates the workup of paraneoplastic cerebellar degeneration. The workup of paraneoplastic cerebellar degeneration. [emedicine.com]
Diagnostic workup showed myo-pathic changes in the EMG, but CK and electrophysiological te-sting were normal. However 3 months later ALL was diagnosed [9]. [docslide.com.br]
Polymyositis: Differential diagnoses & workup. Omaha, NE: eMedicine.com; updated November 6, 2009. Available at: http://emedicine.medscape.com/article/335925-diagnosis. Accessed March 15, 2010. Kalluri M, Sahn SA, Oddis CV, et al. [aetna.com]
Treatment
The role of systemic treatment in the progression of neurologic impairment is unclear. [ncbi.nlm.nih.gov]
Tumor removal is still the therapeutic mainstay with very early treatment being essential to prevent irreversible neuronal loss. Immunosuppressive or immunomodulatory treatments are often ineffective. [en.wikipedia.org]
Treatment • two treatment approaches • removal of the source of the antigen by treatment of the underlying tumor, and, • suppression of the immune response • Overall results disappointing in majority. 10:41 PM 83. [slideshare.net]
Greenlee, Treatment of Paraneoplastic Cerebellar Degeneration, Current Treatment Options in Neurology, 15, 2, (185), (2013). [doi.org]
Within 1 week of treatment, the patient develops a neck mass demonstrated to be a small-cell carcinoma. [link.springer.com]
Prognosis
Tumors are the main cause of death, but some types of PNS (such as dysautonomia) have a poorer prognosis than malignant neoplasms. [ncbi.nlm.nih.gov]
Prognosis of Patients with Paraneoplastic Syndromes Associated with Lung Cancer. Austin J Pulm Respir Med 2014;1(2): 1006. ISSN:2381-9022 [austinpublishinggroup.com]
Favourable prognosis in Lambert- Eaton myasthenic syndrome and smallcell lung carcinoma. Lancet 1999;353:117- 8. 10:41 PM 82. [slideshare.net]
Prognosis The prognosis for persons with paraneoplastic syndromes depends on the specific type of PS, and the progression of the underlying cancer. [encyclopedia.com]
Etiology
Therefore, for the clinician, markers are important which on the one hand prove the paraneoplastic etiology of the given neurological syndrome, and on the other hand help to diagnose the associated tumor. [degruyter.com]
Shoenfeld received the EULAR prize in 2005, in Vienna, Austria: "The infectious etiology of anti-phospholipid syndrome". [books.google.com]
Diagnostic criteria of PNSs The presence of a neurological syndrome of unclear etiology at the time of the diagnosis of a tumor does not necessarily mean that the neurological syndrome is paraneoplastic, as this could represent the coincidental occurrence [bloodjournal.org]
If the detected antibody usually does not associate with the patient’s neurologic syndrome, other etiologies for the neurologic dysfunction should be considered. [clinicalgate.com]
Only a few cases clearly demonstrate an etiologic and a pathogenetic factor. Miscellaneous syndromes Fever is thought to result from the release of endogenous pyrogens (ie, lymphokines or tissue pyrogenes). [emedicine.medscape.com]
Epidemiology
Plasma exchange Ataxia with antibodies to CARP 8 10 Epidemiology: 1 patient; 77 year old female Clinical: Pure cerebellar syndrome Onset: 3 years after neoplasm Ataxia: Limb; Truncal; Gait; Dysarthria Ocular: Horizontal nystagmus Mental status: Normal [neuromuscular.wustl.edu]
Epidemiology Frequency United States In one study, paraneoplastic cerebellar degeneration was observed in 25% of paraneoplastic neurologic syndromes, occurring in 2 of every 1000 patients with cancer. [12] Mortality/Morbidity In the study cited above, [emedicine.com]
Summary: The information about epidemiology, pathogenesis and clinical heterogeneity of paraneoplastic neurological syndrome is summarized and based on Russian and foreign literature. [ssmj.ru]
Anti-CARP VIII Clinical, paraclinical and epidemiological features So far, two patients with ACA and anti-CARP VIII have been reported. [jneuroinflammation.com]
Pathophysiology
It is written to help the clinician with understanding the pathophysiology and diagnosis of this baffling disease syndrome. [amazon.com]
The pathophysiology of PNS differs in the various entities and the underlying neoplasms also differ; in addition PNS are infrequent. [touchneurology.com]
The pathophysiological role of onconeuronal antibodies in PNS is not unequivocally established. Cellmediated immunity is also thought to play an important role. Lymphocytes could react with the same antigens as onconeuronal antibodies [ 7 ]. [jneuro.com]
Pathophysiology Paraneoplastic cerebellar degeneration is caused by the secondary effects of cancer and is believed to be immune mediated. [emedicine.com]
Prevention
Tumor removal is still the therapeutic mainstay with very early treatment being essential to prevent irreversible neuronal loss. Immunosuppressive or immunomodulatory treatments are often ineffective. [en.wikipedia.org]
It incorporates basic science, pathology, diagnosis, management, outcomes, rehabilitation, and prevention – all in one convenient resource – equipping you to overcome your toughest clinical challenges. [books.google.com]
Regardless, treatment of the underlying cancer and other interventions may prevent further damage, improve symptoms and give you a better quality of life. [mayoclinic.org]
Peripheral neuropathies from chemotherapeutics and targeted agents: diagnosis, treatment, and prevention. Neuro-Oncology 2012; 14 (4): 54 25. Gregg RV, Molepo JM, Monpetit VJ. [ssmj.ru]