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Background and Aims: SOX1 antibodies are generally associated with small cell lung cancer and anti-Hu antibody overlap is common. This case demonstrates isolated anti-SOX1 antibodies with an uncommon tumor type, and relapse of a paraneoplastic syndrome with recurrence of tumor., Methods: We describe a case of a 65-year-old male with a paraneoplastic peripheral neuropathy and anti-SOX1 antibody positivity in the context of a prior male breast Grade 2 ductal carcinoma, in remission at the time of the initial neurological presentation., Results: Treatment response to intravenous immunoglobulin (IVIg) was demonstrated. After period of clinical stability on IVIg in the context of remission of breast carcinoma, the patient experienced a relapse of his neuropathy. This was associated with tumor recurrence and again responded to tumor excision, radiotherapy and IVIg., Interpretation: Male breast carcinoma has not previously been associated with anti-SOX1 antibody positive paraneoplastic neuropathy., (Copyright © 2021 Elsevier B.V. All rights reserved.)

The case of a 17-year-old man with Hodgkin's lymphoma who presented with paraneoplastic sensory neuropathy is presented. The patient visited our hospital because of acute progression of dysesthesiae in the bilateral face and extremities. He also developed an ataxic gait due to decreased deep sensation. Post-contrast T 1 -weighted MRI showed enhancement of both trigeminal nerves and the cauda equina. Cerebrospinal fluid examination was unremarkable. Intravenous immunoglobulin therapy and subsequent steroid pulse therapy did not improve his symptoms. Laboratory data showed an elevated serum soluble interleukin-2 receptor level. His chest X-ray and CT showed enlarged lymph nodes in the mediastinum, and the histopathologic examination of a lymph node biopsy specimen showed classical Hodgkin's lymphoma. He was treated with chemotherapy. His symptoms of neuropathy improved promptly while the lymphoma was being successfully treated, and he was able to walk with a cane. The present case was characterized by paraneoplastic sensory neuropathy as the initial clinical feature in association with Hodgkin's lymphoma. It is necessary to consider a paraneoplastic neurological syndrome even in a young patient with acute/subacute sensory neuropathy. Paraneoplastic sensory neuropathy associated with Hodgkin's lymphoma could be expected to improve with oncotherapy, and examination of the malignancy and early treatment are important.

Breast cancer in male is rare which accounts about 1% of all malignant breast neoplasm cases. Since paraneoplastic syndrome is unusual with male breast cancer, very few reported cases are found. A72- year-old gentleman presented with proximal myopathy in all four limbs was referred to Dr. Sirajul Islam Medical College and Hospital in April 2017. He had generalized wasting with reduced tone and reflexes. Planter responses were normal with intact sensory. There were typical Heliotrope rash bilaterally. In background, he had history of radical mastectomy due to stage IIA ductal carcinoma of left breast 7 years back. Three years later, he was found to have multiple metastases in lung and liver, however, deliberately discontinued chemotherapy after first dose. Currently he is on Tamoxifen. Two months back, he was diagnosed to have brain metastasis. Also his serum sodium level was low with low urine osmolality. Considering his background, we diagnosed him dermatomyositis with peripheal neuropathy & SIADH as paraneoplastic presentation of breast malignancy. Despite of normal CPK and NCV, we treated him with steroid as dermatomyositis can present with normal CPK. His myopathy improved after 2 weeks of steroid treatment. Fluid restriction increased his serum sodium level. The aim of reporting this case is to aware physicians about the aggressive nature of male breast cancer, its orthodox paraneoplastic presentation and to differentiate neuropathy from myopathy so that early treatment can improve the outcome.

Purpose of Review: This review describes relevant advances in paraneoplastic neuropathies with emphasis on particular syndromes and the impact of new therapies., Recent Findings: Sensory neuronopathy may present with symptoms that do not raise the suspicion of a paraneoplastic origin. A recent study on sensory neuronopathies of different causes identified paraneoplastic cases in a group of older (>60 years) male patients with subacute onset early pain, and frequent involvement of the arms. Paraneoplastic sensorimotor polyneuropathies may be confused with chronic inflammatory demyelinating polyneuropathy (CIDP) and in lymphomas with direct infiltration of nerves (neurolymphomatosis). Recent neurophysiological studies indicate that the polyneuropathy of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M component, and skin changes) can be differentiated from CIDP by the presence of diffuse demyelination and more severe axonal loss. Neuropathy in Waldenström macroglobulinemia is heterogeneous. Up to 38% have demyelinating features and the rest show axonal degeneration due to different causes (dysimmune, amyloidosis, or tumoral infiltration). Isolated case reports suggest that the combination of cyclophosphamide and rituximab may be effective in paraneoplastic neuronopathies. Lenalidomide and dexamethasone are effective to control the neuropathy of POEMS patients who are not suitable for or progress after autologous stem cell transplantation., Summary: Clinical and neurophysiological studies are helpful to correctly identify particular paraneoplastic neuropathies.

Paraneoplastic neurologic syndromes (PNS) are uncommon, affecting fewer than 1 in 10,000 patients with cancer. PNS, while rare, can cause significant morbidity and impose enormous socio-economic costs, besides severely affecting quality of life. PNS can involve any part of the nervous system and can present as limbic encephalitis, subacute cerebellar ataxias, opsoclonus-myoclonus, retinopathies, chronic intestinal pseudo-obstruction (CIPO), sensory neuronopathy, Lambert-Eaton myasthenic syndrome, stiff-person syndrome, and encephalomyelitis. The standard of care for CIPO includes the use of promotility and anti-secretory agents and the resection of the non-functioning gut segment; all of which can cause significant compromise in the quality of life. There is significant evidence that paraneoplastic neurologic syndromes are associated with antibodies directed against certain nerve antigens. We successfully treated a patient with CIPO in the setting of small cell lung cancer with a combination of rituximab and cyclophosphamide. The patient, who had failed to respond to prokinetic agents, anti-secretory therapy, and multiple resections, responded to the immunomodulatory therapy, with minimal residuals with PEG tube feeding and sustained ostomy output. The use of rituximab and cyclophosphamide should therefore be considered in patients with CIPO, especially if it can avoid complicated surgical procedures.

Paraneoplastic syndromes arise infrequently in prostate cancer and paraneoplastic subacute sensory neuronopathy has not previously been reported in association with prostate cancer. When paraneoplastic syndromes occur, it is usually in the setting of small-cell carcinoma of the prostate or advanced, hormone-resistant disease. Here the authors report a 64- year-old man who developed a progressive, severe, sensory neuronopathy in the setting of a recently diagnosed stage T4 hormone-responsive prostate adenocarcinoma. Anti-Hu antibodies were positive and screening for a concurrent neoplasm at another site was negative. Sensory neuronopathy progressed, despite hormone responsiveness of his prostate adenocarcinoma, and resulted in a severe level of disability. His symptoms did not respond to intravenous corticosteroid therapy but there was a partial response to intravenous immunoglobulin.

Paraneoplastic myeloneuropathy has rarely been reported with breast cancer. We report the case of a 59-year-old woman who presented with a peripheral neuropathy and cranial involvement and later developed a myelopathy. The neuropathy was found to be electrophysiologically and histologically demyelinating in nature. Magnetic resonance imaging studies failed to identify any structural brain or spinal cord abnormalities. The patient was diagnosed with breast carcinoma 4 months after initial presentation and underwent resective surgery, radiotherapy, and hormonotherapy. Paraneoplastic antibodies (anti-Hu, anti-Yo, anti-Ri, anti-CV2, anti-Ma, and anti-amphiphysin) were all negative. Her condition did not progress further after cancer treatment. Partial neurologic improvement occurred with oral steroid therapy, with subsequent deterioration on treatment withdrawal.

Paraneoplastic syndromes have only been reported in malignant pleural mesothelioma (MPM) in a few cases. In this case, we describe a 57-year-old man with MPM who developed sensory-motor polyneuropathy 18 days after diagnosis. Thorough endocrinological, neurological, and paraclinical examinations gave no explanation of the symptoms, and paraneoplasia was therefore suspected. The patient was treated with immunoglobulin and prednisolone, and this resulted in subjective, objective, and paraclinical improvement of the symptoms. We therefore suggest that polyneuropathy is a possible paraneoplastic syndrome in MPM.

Paraneoplastic myasthenia gravis (MG) is accompanied by a neoplasm, usually thymoma. In patients with thymoma and a specific genetic make-up, the paraneoplastic immune response develops further in thymic remnant or peripheral lymphatic tissue. Paraneoplastic MG and late-onset MG (age >or= 50 years) share a similar immunological profile with high titin and ryanodine receptor (RyR) antibody prevalence. This profile is the most important predictor of clinical outcome in paraneoplastic MG. The presence of a thymoma per se does not cause more severe MG. MG severity is linked to the patient's immunological profile. Paraneoplastic MG causes a distinctive non-limb symptom profile at MG onset, characterized by bulbar, ocular, neck, and respiratory symptoms. When the diagnosis of paraneoplastic MG is established, the neoplasm should be removed surgically. Pre-thymectomy plasmapheresis or iv-IgG should be considered in these patients to minimize post-thymectomy MG exacerbation risk. Paraneoplastic MG usually continues after thymectomy. The pharmacological treatment of paraneoplastic MG does not differ from non-paraneoplastic MG, except for tacrolimus that should be considered in difficult cases. Tacrolimus is an immunosuppressant acting specifically in RyR antibody positive patients through enhancing RyR-related sarcoplasmic calcium release that in theory might be blocked by RyR antibodies, causing symptomatic relief in paraneoplastic MG.

Paraneoplastic neurological syndromes are a rare complication of breast cancer. Nevertheless, they may be clinically relevant leading to neurological impairment. Clinicians should be aware that these neurological disorders could even precede the diagnosis of breast cancer. Here we present the case of a female patient with advanced breast cancer who developed paraneoplastic sensorimotor neuropathy. Treatment with capecitabine lead to clinical amelioration. A review of the literature on the paraneoplastic neurological syndromes in breast cancer is also included.

Combining clinical and immunological information, a neurological syndrome can now be diagnosed as a "definite" or "possible" paraneoplastic syndrome according to the newly suggested diagnostic criteria of the PNS Euronetwork. Differentiated diagnosis of a paraneoplastic syndrome is essential for differential therapy in patients. According to the response to IVIG therapy, paraneoplastic disorders may be subgrouped in group A, a clinical response is the rule (prototype Lambert-Eaton myasthenic syndrome), and in group B, IVIG may be helpful in single patients and is indicated in specific clinical settings (prototype anti-Hu associated neurological syndromes). The mode of action of IVIG may range from direct anti-idiotype effect to indirect effects on the cellular part of the pathogenesis of paraneoplastic syndromes. Due to the therapeutic relevance, it is therefore important to diagnose a PND as early as possible, and start immunotherapy including IVIG immediately.

We administered chemotherapy in three cases of small-cell lung cancer (SCLC) with renal failure under different situations. Hemodialysis (HD) was used in 2 out of the 3 cases. Case 1 was complicated by acute renal failure from extensive bilateral tumor invasion. After chemotherapy (CBDCA + ETP) under HD, renal metastases regressed and renal function improved, although the final response was PD. In case 2, HD had been introduced for diabetic nephropathy. After 2 cycles of chemotherapy (CBDCA + ETP) under HD, the patient attained a PR. Case 3 is an example of paraneoplastic nephrotic syndrome with renal failure. Chemotherapy including CBDCA or CDDP was performed and the QOL of the patient improved. Pro-GRP and serum creatinine changed in parallel during the clinical course of 6 admissions. In conclusion, individualized therapy is necessary to increase survival time of SCLC patients with renal failure. Although chemotherapy is useful, further study is needed for the selection of suitable chemotherapeutic regimens, optimal dosage of each drug and the timing of HD.

A 56 year old woman had a 19 month history of a severe subacute progressive cerebellar degeneration, peripheral sensory neuropathy, and urinary incontinence. She was confined to a wheelchair, needed assistance with eating, and her speech was almost unintelligible. No underlying cancer was found despite repeated investigations, and no autoantibodies were demonstrated. She received a 3-month course of intensive immunosuppressant therapy with intravenous immunoglobulin 400 mg/kg per day for 5 days every month, oral cyclophosphamide 50 mg twice or three times a day to maintain the total lymphocyte count between 500 and 750/mm(3), and prednisone 60 mg per day. She experienced dramatic subjective and objective improvement. The dysarthria and the upper extremity dysmetria disappeared, and she regained the ability to write and cook. The lower extremity ataxia improved and she became able to walk with a cane. Urinary incontinence disappeared. A trial of intensive immunosuppressant treatment is worth considering in a patient with a clinical syndrome resembling paraneoplastic disorders, even if an underlying neoplasm and autoantibodies are not demonstrated.