Integrated treatment for autonomic paraneoplastic syndrome improves performance status in a patient with small lung cell carcinoma: a case report

Abstract

BackgroundParaneoplastic neurological syndromes (PNS) are rare disorders associated with cancer and are believed to be immune mediated. Patients with autonomic PNS suffer from variable combinations of parasympathetic and sympathetic failure. Autonomic PNS are usually associated with other PNS, such as encephalomyelitis and sensory neuropathy; however, autonomic symptoms may rarely manifest as PNS symptoms. Autonomic symptoms, therefore, may be overlooked in patients with cancer.Case presentationWe described a 65-year-old Japanese man who was diagnosed with autonomic PNS due to small-cell lung carcinoma (SCLC) with Eastern Cooperative Oncology Group (ECOG) performance status 3, who suffered from orthostatic hypotension, and urinary retention needing a urethral balloon. Laboratory studies showed decreased levels of noradrenaline, and were positive for anti-ganglionic acetylcholine receptor antibody, type 1 antineuronal nuclear antibody, and sry-like high mobility group box 1 antibody. Nerve conduction evaluations and 123I-metaiodobenzylguanidine myocardial scintigraphy showed no abnormalities. Abdominal contrast-enhanced computed tomography revealed marked colonic distention. The patient’s autonomic symptoms resolved following integrated treatment (symptomatic treatment, immunotherapy, and additional chemotherapy) enabling the patient to walk, remove the urethral balloon, and endure further chemotherapy. ECOG performance status remained at 1, 10 months after admission.ConclusionsIntegrated treatment for autonomic PNS may improve autonomic symptoms and ECOG performance status of patients with cancer.

Anti-SOX1 Antibodies in Paraneoplastic Neurological Syndrome

“… 64 Additionally, 28% of 85 SCLC patients compared with 18% of 210 NSCLC patients had more than 1 ab. 15 64 In the current review, 245 (47.1%) of the 520 patients showed the coexistence of multiple onconeuronal and cell-surface autoabs at any time point: 213 (41.0%) patients had 2 autoabs, 6 7 11 15 16 20 21 42 46 47 48 49 52 53 54 55 59 60 64 65 66 27 (5.1%) patients had 3 autoabs, 11 15 16 51 53 56 57 58 64 4 (0.8%) patients had 4 autoabs, 15 16 21 64 and 1 (0.2%) patient had 5 autoabs. 61 In the 285 patients with additional autoabs coexisting with anti-SOX1 abs, anti-VGCC P/Q ( n =157, 55.1%) 6 7 11 16 42 48 49 57 64 and anti-Hu ( n =73, 25.6%) 7 11 15 16 21 46 51 55 58 61 64 abs were the most common ( Table 3 ).…”

“…ple onconeuronal and cell-surface autoabs at any time point: 213 (41.0%) patients had 2 autoabs, 6,7,11,15,16,20,21,42,[46][47][48][49][52][53][54][55]59,60,[64][65][66] 27 (5.1%) patients had 3 autoabs, 11,15,16,51,53,[56][57][58]64 4 (0.8%) patients had 4 autoabs, 15,16,21,64 and 1 (0.2%) patient had 5 autoabs. 61 In the 285 patients with additional autoabs coexisting with anti-SOX1 abs, anti-VGCCP/Q (n=157, 55.1%) 6,7,11,16,42,48,49,57,64 and anti-Hu (n=73, 25.6%) 7,11,15,16,21,…”

“…The follow-up time ranged from 15 days to 29 months. 17,20,41,46,47,[58][59][60]66 The neurological symptoms did not improve in 10 patients. 25,42,44,46,47,51,61,65 Among the other patients with or without treatment, 14 died during a follow-up of up to 30.75 months, 40,43,45,[52][53][54][55][56][57] all of whom were SCLC patients.…”

“…The presence of anti-SOX1 abs is a potential predictor of underlying SCLC, and SCLC represented the predominant cancer (85.2%, 414 of the 486 cancer patients) ( Table 3 ). 6 7 11 12 15 16 21 40 41 42 43 44 45 46 47 48 49 51 52 53 54 55 56 57 58 59 60 61 The underlying pathogenic mechanism may be due to the immunoreactivity of SCLC patients against epitopes of the conserved high-mobility group box; 14 apart from preventing neural differentiation in progenitor cells and being mainly expressed in the developing nervous system and down-regulated in adults, SOX1 proteins also affect the airway epithelial differentiation and are highly immunogenic. 14 18 …”

“…The proportions of cases with normal and abnormal MRI findings for anti-SOX1 abs were very similar, with 46.7% of the patients ( n =14) showing normal findings regardless of the follow-up time. 17 21 40 42 43 44 51 52 56 58 59 Abnormal cerebellar changes were observed in only 3 of the 30 patients with anti-SOX1 abs: 21 25 2 patients with PCD had cerebellar atrophy 21 and 1 patient with PCD had diffuse hyperintense lesions in T2-weighted imaging of the cerebellum and brainstem. 25 …”