Background: Non-motor symptoms are well established phenotypic components of adult-onset idiopathic, isolated, focal cervical dystonia (AOIFCD). However, improved understanding of their clinical heterogeneity is needed to better target therapeutic intervention. Here, we examine non-motor phenotypic features to identify possible AOIFCD subgroups.This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
Ethnic minority patients reach early levels of fixed disability more rapidly than Caucasian patients, but this effect diminishes at later stages of the disease. This has implications for clinical management of these patients.
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.
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