Paraneoplastic sensorimotor neuropathy and ventral cauda equina nerve root enhancement as initial presentation of small cell lung carcinoma: a case study.

BACKGROUND: Paraneoplastic neurologic syndromes (PNS) are rare, however, are important to recognize as oftentimes they precede the detection of an occult malignancy. Our case highlights a rare circumstance of paraneoplastic radiculoneuropathy and the importance of recognizing PNS in antibody negative disease, as is the case in up to 16% of sensory neuronopathies, and the process of excluding other etiologies. CASE PRESENTATION: We discuss a 51-year-old man who presented with asymmetric subacute sensorimotor deficits in the lower limbs. Initial clinical examination showed weakness throughout the right lower limb and normal strength on the left with objective numbness in a mixed dermatomal and stocking-glove distribution. Electrophysiology was consistent with axonal sensorimotor neuropathy. Cerebrospinal fluid showed pleocytosis and elevated protein. Intravenous immunoglobulin treatment was given with some improvement in pain symptoms but no measurable motor improvement. Following clinical and electrophysiologic deterioration the patient was transferred to a tertiary centre. Magnetic resonance imaging of the spine showed smooth enhancement of the ventral caudal nerve roots. Chest computed tomography revealed left lower vascular scarring. Further positron emission tomography scan imaging identified fluorodeoxyglucose avid right lung lymphadenopathy. Bronchoscopy-guided biopsy revealed small cell lung carcinoma. Onconeural and antiganglioside antibodies were negative. The patient was then transferred to a medical oncology ward where he underwent chemoradiotherapy and subsequently experienced improvement in his motor function, supporting that his neurological condition was indeed secondary to a paraneoplastic process. CONCLUSIONS: Onconeural negative paraneoplastic radiculoneuropathy can precede diagnosis of small cell lung carcinoma. If considered early and adequately investigated, it can allow earlier diagnosis and treatment of underlying malignancy, improving overall and neurological prognosis.

Dorsal cauda equina nerve root enhancement on magnetic resonance imaging due to ANNA-1-associated paraneoplastic polyneuropathy.

A 69-year-old female presented with subacute onset ascending weakness and paraesthesias. She was initially diagnosed with Guillain-Barré syndrome (GBS) based on her clinical presentation and cerebrospinal fluid (CSF) analysis showing albuminocytological dissociation. However, she was later found to have anti-neuronal nuclear antibody 1 (ANNA-1/anti-Hu)-positive CSF and was subsequently diagnosed with small-cell lung cancer. Her neurological symptoms were ultimately attributed to ANNA-1/anti-Hu-associated paraneoplastic polyneuropathy. During the course of her evaluation, she had magnetic resonance imaging findings of dorsal predominant cauda equina nerve root enhancement, which has not been previously described. The only previously reported case of cauda equina enhancement due to ANNA-1-associated polyneuropathy described ventral predominant findings. The distinction between ventral and dorsal enhancement is important, since it suggests that different patterns of nerve root involvement may be associated with this paraneoplastic syndrome. Therefore, ANNA-1-associated paraneoplastic inflammatory polyneuropathy can be considered in the differential diagnosis of cauda equina nerve root enhancement with ventral and/or dorsal predominance. This can potentially be helpful in differentiating ANNA-1 polyneuropathy from GBS, which classically has ventral predominant enhancement.

Retrospective study of paraneoplastic neurological syndromes in a Chinese Han population from Shandong, East China.

OBJECTIVE: To analyze the clinical features, diagnostic strategies and therapeutic methods associated with paraneoplastic neurological syndromes. METHODS: A retrospective study of paraneoplastic neurological syndromes was performed at a single center in Shandong, East China. The medical records and follow-up data of 28 patients were intensively reviewed between February 2011 and December 2014. RESULTS: Twenty-four (85.7%) patients experienced subacute or chronic onset of disease, and the most common symptoms reported were mild myasthenia and paresthesias. Twenty-five (89.3%) patients presented nervous system lesions prior to occult tumors, and the median time frame between paraneoplastic neurological syndromes onset and the diagnosis of a tumor was 15 weeks. Sensorimotor neuropathy, Lambert-Eaton myasthenic syndrome and limbic encephalitis were the three most common neurological syndromes reported. Elevated serum tumor markers were observed in 44.0% of patients, while 40.7% of patients were positive for onconeural antibodies. Tumors were detected in 21 (75.0%) patients after repeated whole-body screening, and lung carcinomas were the most common primary tumor detected. Seventeen patients received anti-tumor or immunological therapy, and clinical symptoms were relieved in 13 (76.5%) of these patients. CONCLUSIONS: In the majority of paraneoplastic neurological syndromes patients, the onset of disease is subacute or chronic with mild clinical symptoms. Nervous system lesions usually occur prior to occult tumors with complicated and various clinical manifestations. Neither tumor markers nor onconeural antibodies exhibit a high rate of occurrence, while repeated whole-body screening is helpful in identifying occult tumors. Early diagnosis and treatment are crucial to these patients.

Paraneoplastic neuromyelitis optica spectrum disorder: A case report and review of the literature.

Neuromyelitis optica spectrum disorders (NMOSD) are demyelinating, autoimmune diseases affecting the central nervous system. Typically, recurrent optic neuritis and longitudinal extensive transverse myelitis dominates the clinical picture. In most cases NMOSD are associated with autoantibodies targeting the water channel aquaporin-4 (AQP-4). NMOSD usually present in young adults. Clinical findings suggestive of NMOSD in elderly patients should raise the suspicion of a paraneoplastic etiology. To our knowledge, we report the first case of a 66 year-old female patient with paraneoplastic NMOSD that is associated with squamous cell lung carcinoma. Anti-AQP-4 was present in both the serum and cerebrospinal fluid of the patient. However, immunhistological staining of the malignant tissue did not show presence of AQP-4 on the surface of tumour cells.

Multiple enlarged nerves on neurosonography: an unusual paraneoplastic case.

Multiple nerve enlargements at non-entrapment sites are usually caused by hereditary or acquired immune-mediated neuropathies.We describe a case of multifocal hypertrophic mononeuropathies detected by nerve sonography with a clinical picture of progressive mononeuritis multiplex caused by a paraneoplastic syndrome associated with anti-Hu antibodies. This case illustrates an unusual but important paraneoplastic differential diagnosis of progressive multifocal hypertrophic neuropathies. It emphasizes the role of nerve ultrasound in the diagnostic work-up of peripheral nervous system disorders.

[Paraneoplastic chorea and behavioral disorders in a patient with anti-CV2/CRMP5 antibodies and two different tumors]. / Chorée paranéoplasique et troubles du comportement chez un patient présentant des anticorps anti-CV2/CRMP5 et deux cancers différents.

INTRODUCTION: Paraneoplastic movement disorders are rare. Reported cases frequently describe association with anti-CV2/CRMP5 antibodies. CASE REPORT: We report a case of an 80-year-old man who developed sensorial neuronopathy, following by movement disorders mimicking chorea and obsessive-compulsive and behavioral disorders. These manifestations were first considered to be associated with a prostatic adenocarcinoma but PET and surgical biopsy revealed a mediastinal small cell lung carcinoma classically associated with anti-CV2/CRMP5 antibodies. CONCLUSION: This case demonstrates that in a context of paraneoplastic neurological syndrome, search for a classically associated cancer is necessary in order to institute adapted treatment early, even if another tumor is obvious.

Paraneoplastic mononeuritis multiplex in non-small-cell lung carcinoma.

A 60-year-old man developed two selective peripheral mononeuropathies of the peroneal and later the radial nerve, shortly after a diagnosis of large-cell lung carcinoma. Nerve conduction studies and electromyography confirmed isolated lesions in both nerves, and in the case of the peroneal nerve lesion, focal conduction block was localised to the level of the fibula neck. Subsequent magnetic resonance imaging of the lower limb excluded focal compression or malignant infiltration along the course of the peroneal nerve, and there was no signal change within the nerve, prompting a diagnosis of paraneoplastic mononeuritis multiplex. Anti-neuronal antibodies and serological markers of systemic vasculitis were negative. Neither the patient's large-cell lung carcinoma nor mononeuritis multiplex responded to chemotherapy, and he died within 6 months of the initial diagnosis.

Antibody-positive paraneoplastic neurologic syndromes: value of CT and PET for tumor diagnosis.

OBJECTIVE: To assess use of whole-body 18F fluoro-2-deoxy-glucose (FDG)-PET and CT for diagnosing tumor in patients with antibody-positive paraneoplastic neurologic syndromes (PNS). METHODS: In order to directly compare CT and FDG-PET imaging in patients with various antineuronal antibodies, the authors performed in parallel CT scanning and FDG-PET in a series of 13 consecutive patients (9 women, 4 men, aged 59 +/- 14 years) with positive antineuronal antibodies (anti-Hu: 8, anti-Yo: 4, anti-Tr: 1) in whom the authors were searching for a tumor or tumor recurrence. RESULTS: A new tumor or tumor recurrence was found in 10/13 patients (5 small cell lung cancer, 2 ovarian cancer, and 1 each neuroblastoma, Hodgkin's lymphoma, and lymph node metastasis of adenocarcinoma). All tumors except one with good clinical evidence for small cell lung cancer were confirmed histologically. For detection of tumor or tumor recurrence, CT was positive in 3/10 patients (sensitivity of 30%), and FDG-PET in 9/10 (sensitivity of 90%, difference between methods p < 0.01), but the combination of both methods showed a sensitivity of 100%. CONCLUSIONS: FDG-PET imaging is useful in tumor screening of patients with antineuronal antibodies, but should be complemented by CT scanning to increase sensitivity and accuracy of tumor diagnosis.