Paraneoplastic and Idiopathic Ganglionopathy: Importance of Differential Diagnosis Guilherme Perassa Gasque * , Felipe da Rocha Schmidt, Diogo Matheus Terrana de Carvalho, Salim Balassiano, José Marcelo Ferreira Bezerra and Marcia Rodrigues Jardim Department of Neurology, Pedro Ernesto University Hospital, State University of Rio de Janeiro (UERJ), Rio de Janeiro, RJ, Brazil *Corresponding author: Guilherme Perassa Gasque, Department of Neurology, Pedro Ernesto University Hospital, State University of Rio de Janeiro (UERJ), Rio de Janeiro, RJ, Brazil, Tel: +552123340639; E-mail: guigasque_4@hotmail.com Received date: Nov 10, 2015; Accepted date: Dec 11, 2015; Published date: Dec 18, 2015 Copyright: ©2015 Gasque GP, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Abstract Sensory ganglionopathies (SG) may occur in association with different diseases and are characterized by the degeneration of the primary sensory neurons located in the dorsal root ganglia. The most important associated conditions are paraneoplastic SG, HIV infection, Sjogren's syndrome, and cisplatin or pyridoxine toxicity. Even when clinical criteria help differentiate SG from other neuropathies, an etiological diagnosis remains elusive. Thus, there is a need to identify biological markers than can distinguish immune-mediated SG from other etiologies. Objectives: The present study of two cases of SG compares their neurological outcomes with their treatment responses in accordance with the etiology of each one. Methods: evaluate the clinical presentation and examination results to be able to define the etiology and institute early treatment. Results: To differentiate paraneoplastic and idiopathic causes, more precise imaging techniques and paraneoplastic antibody tests are required. These diagnostic tools might be helpful in early diagnosis and treatment, thus affording the best chance of stabilizing the neurological symptoms. Conclusions: The identification of paraneoplastic antibodies before the onset of cancer can reduce the percentage of cases misdiagnosed as idiopathic, leading to early treatment and perhaps a more favorable prognosis. Keywords: Multiple sclerosis; Cognitive impairment; Tunisia Introduction Sensory ganglionopathies (SG), characterized by the degeneration of the primary sensory neurons located in the dorsal root ganglia, may occur in association with diferent diseases [1]. Te most important associated conditions are paraneoplastic SG, HIV infection, Sjogren's syndrome, and cisplatin or pyridoxine toxicity [2]. Half of all cases are still labeled idiopathic. Te sensory defcits are usually multifocal, involving the proximal and distal parts of the limbs; and all sensory modalities - pain, temperature, sense position, and vibration - may be compromised during the course of the disease [1,3]. Gait ataxia and widespread arrefexia ofen occur [1,3,4]. Some patients have pseudoathetotic hand movements. Injury to small and medium-sized neurons can generate pain, a burning sensation, and allodynia [1,3,4] while the motor system examination is usually unremarkable. Nystagmus is not frequent, but autonomic dysfunction may be found. Tere have been reports of tonic pupils, orthostatic hypotension, gastrointestinal symptoms, and erectile dysfunction. Nerve conduction studies are the most useful tests in evaluating SG [3,5]. Te distinguishing characteristics between SG and other sensory neuropathies are important to properly focus the etiological investigation and the adoption of appropriate treatment [2]. However, the diagnosis of SG is ofen challenging for the practicing neurologist. Furthermore, there is evidence that, in some cases, the dorsal root ganglia and peripheral nerves can be afected simultaneously such as in Sjögren's syndrome [6]. Tus, reliable diagnostic criteria for SG are urgently needed as a backdrop in clinical practice [6]. Even when clinical criteria aid in diferentiating SG from other neuropathies, an etiological diagnosis remains elusive. Tus, there is a need for biological markers than can distinguish immune-mediated SG from other etiologies [7]. Other tests, such as screening for occult tumors and searching for paraneoplastic antibodies are essential for the establishment of an etiological diagnosis since paraneoplastic SG may precede the tumor within fve years of diagnosis [8]. Te present study examines two cases of SG by comparing and highlighting the importance of the clinical presentation and laboratory results mainly from the perspective of the presence of paraneoplastic antibodies in the management of patients. Case Report Case 1 A 49y, single, mixed-race female and small business owner and resident of Rio de Janeiro was admitted to the Pedro Ernesto University Hospital in Rio de Janeiro, Brazil, due to hand paresthesias and cooking difculties that, within a month, evolved into involuntary movements of the hands and numbness in the forearms and feet. He smoked a pack a day for 20 years and drank alcohol regularly. She was then taking 25 mg/day amitriptyline. Te physical examination showed the patient to be in generally good health and pale 1+/4+. Te neurological examination showed sensory ataxic gait and a Romberg sign, along with widespread arefexia, dysmetria, and dysdiadocokinesia in the upper limbs (UL) that worsened with eye closure, bilateral fexor plantar responses, tactile hypoesthesia, painful asymmetric and multifocal, apalesthesia, and loss of proprioception distal limbs and pseudoathetosis movements in the hands. He also Gasque GP, et al., J Neurol Neurophysiol 2015, 6:6 DOI: 10.4172/2155-9562.1000339 Case Report Open Access J Neurol Neurophysiol ISSN:2155-9562 JNN, an open access journal Volume 6 • Issue 6 • 339 Journal of Neurology & Neurophysiology Jo u r n a l o f N e u ro l o g y & N e u r o p h y s i o l o g y ISSN: 2155-9562