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Paraneoplastic Neurologic Syndrome

PNS


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]
  • Although their presentations vary, syndromes occur when tumor antigens exhibit cross-reactivity to similar antigens expressed by these systems. The antigens in the nervous system are called "onconeural" antigens.[ncbi.nlm.nih.gov]
  • We report a case of occult breast cancer presenting as paraneoplastic sensory and motor neuropathy. The paraneoplastic antibody panel was negative and no other unknown identifiable antibody could be detected in the serum.[ncbi.nlm.nih.gov]
  • 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 findings elucidate the clinical evolution of paraneoplastic cerebellar syndrome according to the onconeural antibodies present, the heterogeneity and prognosis of dysautonomic disorders, and the clinical variability of paraneoplastic limbic encephalitis[ncbi.nlm.nih.gov]
Drooling
  • It usually affects patients younger than four years of age, and presents with sudden development of staggering and falling (ataxia) often followed by body jerks, drooling, refusal to walk or sit, opsoclonus, irritability and sleep disburbances.[rarediseases.org]
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]
Anger
  • This part of the brain is involved in memory, emotion and behavior, and controls reactions of fear and anger. It can also involve the emotions of sexual behavior.[rarediseases.org]
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]
Clumsiness
  • […] 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, left hand clumsiness[nnjournal.net]

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]
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

  • 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]
  • The lack of response of the paraneoplastic syndrome to successful treatment of HD among our three patients emphasizes the poor prognosis of longstanding paraneoplastic neurologic symptoms in HD.[ncbi.nlm.nih.gov]
  • Greenlee, Treatment of Paraneoplastic Cerebellar Degeneration, Current Treatment Options in Neurology, 15, 2, (185), (2013).[doi.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]

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]
  • The lack of response of the paraneoplastic syndrome to successful treatment of HD among our three patients emphasizes the poor prognosis of longstanding paraneoplastic neurologic symptoms in HD.[ncbi.nlm.nih.gov]
  • Favourable prognosis in Lambert- Eaton myasthenic syndrome and smallcell lung carcinoma. Lancet 1999;353:117- 8. 10:41 PM 82.[slideshare.net]
  • The prognosis is poor and patients rarely improve.[bloodjournal.org]
  • 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

  • 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]
  • 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]
  • 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]
  • . • Spontaneous neurological improvement, although reported in some syndromes, is rare and should lead to the consideration of a nonparaneoplastic etiology. – Voltz, R. D., Posner, J. B., Dalmau, J. & Graus, F.[slideshare.net]

Epidemiology

  • Summary: The information about epidemiology, pathogenesis and clinical heterogeneity of paraneoplastic neurological syndrome is summarized and based on Russian and foreign literature.[ssmj.ru]
  • 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]
  • 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]
  • Anti-CARP VIII Clinical, paraclinical and epidemiological features So far, two patients with ACA and anti-CARP VIII have been reported.[jneuroinflammation.com]
  • Anti-VGCC Clinical, paraclinical and epidemiological features Anti-VGCC autoantibodies have been reported both in patients with PCD and in patients with non-paraneoplastic cerebellar ataxia.[jneuroinflammation.com]
Sex distribution
Age distribution

Pathophysiology

  • It is written to help the clinician with understanding the pathophysiology and diagnosis of this baffling disease syndrome.[amazon.com]
  • Pathophysiology Paraneoplastic cerebellar degeneration is caused by the secondary effects of cancer and is believed to be immune mediated.[emedicine.com]
  • However, pathophysiological outcomes usually arise from when a tumor arises. Paraneoplastic syndrome often occurs alongside associated cancers as a result of activated immune systems.[en.wikipedia.org]
  • A case of finger clubbing associated with nasopharyngeal carcinoma in a young girl, and review of pathophysiology. J Pak Med Assoc 2009; 59(4): 253-4. [ PubMed ] 29. Garganese MC, et al.[medical.nikkeibp.co.jp]
  • In addition, we provide a comprehensive summary of the clinical, paraclinical and epidemiological features associated with these antibodies, briefly review the available knowledge regarding their pathophysiological relevance and discuss their oncological[jneuroinflammation.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]
  • 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]
  • • Within a few months it reaches its peak and then stabilizes – – – – – – – 10:41 PM most cannot walk without support cannot sit unsupported handwriting is impossible eating independently difficult speech very difficult to understand oscillopsia may prevent[slideshare.net]
  • Overexpression of MRGX in T98G glioblastoma cells led to morphological changes and cell death, which could be prevented by CDR2 [ 27 ].[jneuroinflammation.com]

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