Presentation
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]
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]
Eyes
- Vertical Gaze Palsy
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]
Neurologic
- Spastic Gait
We report the case of a 69-year-old man who initially presented with weakness and spastic gait. He was subsequently found to have a characteristic paraneoplastic tractopathy on thoracic magnetic resonance imaging. [ncbi.nlm.nih.gov]
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]
X-Ray
- Mediastinal Mass
An anterior mediastinal mass detected on a chest computed tomographic scan was found on resection to be a noninvasive lymphocytic thymoma. The PNNS went into remission 1 year after the thymectomy. [ncbi.nlm.nih.gov]
- Anterior Mediastinal Mass
An anterior mediastinal mass detected on a chest computed tomographic scan was found on resection to be a noninvasive lymphocytic thymoma. The PNNS went into remission 1 year after the thymectomy. [ncbi.nlm.nih.gov]
Treatment
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]
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]
Within 1 week of treatment, the patient develops a neck mass demonstrated to be a small-cell carcinoma. [link.springer.com]
The initial patient reported with Ophelia syndrome also responded to tumor treatment. 42 When limbic encephalitis is part of PEM or PSN, it usually responds poorly to treatment. [clinicalgate.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]
Paraneoplastic polyneuropathy: definition, etiology and pathogenesis, diagnosis. Siberian Health Review2010; 1:12-16 27. Grisold W, Drlicek М. Paraneoplastic neuropathy. Curr Opin Neurol 1999; 12(5): 617-625 28. Gandhi L, Johnson BE. [ssmj.ru]
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]
OTHER MEMBERS OF THE PARANEOPLASTIC NEUROLOGICAL SYNDROME EURONETWORK G Bertolin, Clinical Epidemiology laboratory, Instituto Mario Negri, Bergamo, Italy; A Carpentier, Service of Neurology, Hôpital de la Salpêtrière, Paris, France; L Lorusso, Department [jnnp.bmj.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]