Rheumatoid arthritis and amyotrophic lateral sclerosis association: A case report and literature review

Abstract

WCN 2013 No: 2243 Topic: 6 — MS & Demyelinating Diseases Uncommon ocular and visual signs of relapsing remitting multiple sclerosis E. Kammoun, M. Zouari, E. Farhat, F. Hentati. Neurology, National Institute Mongi Ben Hamida of Neurology of Tunis, Tunis, Tunisia Background: Ocular manifestations in multiple sclerosis (MS) are classic, including both visual and motor ocular systems. Optic neuritis remains the most frequent manifestation. Objective: To report two MS patients revealed by isolated homonymous lateral hemianopsia (HLH) and sixth cranial nerve palsy (SCNP). Case 1: A 29 year-old woman, who presented a sudden right visual field defect. Neurological examination revealed an isolated right HLH. CT scan demonstrated a left occipital hypodensity. The diagnosis of ischemic stroke was made. Brain MRI showed a large T2-weighted lesion in the left juxtacortical occipital region, associated to multiple T2-weighted lesions in the periventricular regions. The visual evoked potential was normal. Oligoclonal bands were present in the cerebrospinal fluid (CSF). MS was diagnosed and the patient was treated with intravenous methylprednisolone with clinical improvement. Case 2: A 30 year-old woman, who presented sudden horizontal diplopia. The neurological examination showed isolated left SCNP. The eyeground was normal. MRI showed a T2-weighted lesion of the sixth cranial nerve, associated to multiple T2-weighted lesions in the brainstem and spinal cord. Oligoclonal bands were present in the CSF. The diagnosis of MS was made with a complete spontaneously recovery within two weeks. Discussion: HLH is infrequent in MS, occurring in less than 1% of patients, due to the large required lesions in post-chiasmal visual pathway. Isolated sixth nerve palsy is a rare presenting sign of MS, with an incidence of 1.6%. Conclusion: The diagnosis of MS should be considered in young adults with HLH or ocular motor cranial nerve involvement. doi:10.1016/j.jns.2013.07.1394 Abstract — WCN 2013 No: 2253 Topic: 6 — MS & Demyelinating Diseases Rheumatoid arthritis and amyotrophic lateral sclerosis association: A case report and literature review WCN 2013 No: 2253 Topic: 6 — MS & Demyelinating Diseases Rheumatoid arthritis and amyotrophic lateral sclerosis association: A case report and literature review I. Bekri, M. Zouari, E. Farhat, F. Hentati. Neurology, National Institute Mongi Ben Hamida of Neurology of Tunis, Tunis, Tunisia Introduction: Neurological complications are reported with rheumatoid arthritis (RA), involving especially the central nervous system. Degenerative motor neuron disease is unusual. Objective: The objective is to describe a rare association between RA and amyotrophic lateral sclerosis (ALS) and to discuss the relation between these two diseases. Case report: A 20-year-old woman presented rapidly progressive walking disorder and limb weakness without dysphagia and dysphonia. The physical examination showed signs of bilateral degeneration of upper and lower motoneurons involving the four limbs, without sensory disturbance and cranial nerve involvement. We noted also a deforming polyarthropathy of the hands. Electromyography found signs of impairment of the anterior horn of the spinal cord. Brain and spine MRI was normal. Serological test for rheumatoid factor was positive. The diagnosis of ALS associated with seropositive erosive RA was made. Discussion: ALS is a chronic disease of unknown cause. To date, only seven cases similar to our patient have been reported, most of them having long-standing but quiescent joint disease at the time of ALS onset. Our patient has an atypical bilateral presentation, without signs of medulla's involvement. There are other cases of ALS in combinationwith autoimmune diseases such as systemic lupus erythematosus, scleroderma,Wegener granulomatosis andGoujerot Sjogren's Syndrome. There is no evidence in favor of a common pathophysiological mechanism, and thus the possibility of a fortuitous association must be raised. Conclusion: The simultaneous presence of RA and ALS represents a difficult diagnostic challenge. There is no argument in favor of a shared physiopathological mechanism. doi:10.1016/j.jns.2013.07.1395 Abstract — WCN 2013 No: 2181 Topic: 6 — MS & Demyelinating Diseases A diagnostic challenge: Tumefactive demyelinating lesions, our clinical experience in Baskent University WCN 2013 No: 2181 Topic: 6 — MS & Demyelinating Diseases A diagnostic challenge: Tumefactive demyelinating lesions, our clinical experience in Baskent University S. Kibaroglu, U.S. Benli, E.D. Ciftci, M.K. Toprak, O.Y. Cakmak, U. Can. Neurology, Baskent University, Faculty of Medicine, Ankara, Turkey Background: Tumefactive demyelinating lesions (TDLs) are a rare variant ofmultiple sclerosis (MS), characterized by the presence of large lesions in white matter (N2 cm) with mass like features and edema. Differential diagnosis of TDLs from other intracranial space occupying lesions is essential to avoid unnecessary interventions and treatments. Methods: Demographic, clinical, radiological and laboratory data of 6 patients with TDLs were retrospectively collected. Results:All patientswere femalewith amean age of 42.8 (33–57 years) and mean age at disease onset was 38.8 (31–47 years). The mean duration of disease was 4 years (range 1–10 years). All patients had solitary TDLs, five had tumefactive lesions at onset and one patient developed tumefactive lesion during the course ofMS. One patientwith RRMS developed two tumefactive relapses during follow-up. Clinical presentations varied but headache, visual changes, and sensory and motor symptoms were the most common. Brain MRI showed tumefactive lesions with mild to moderate edema and contrast enhancement. MR spectroscopy was performed in all cases and revealed findings suggesting demyelination. CSF analysis was available for three patients. Oligoclonal IgG band was positive in one case. Biopsy was performed in one case, revealing nondiagnostic pathology; final diagnosis was TDLs. All patients responded to corticosteroids. Three patients were treated with interferon and one patient was treated with azathioprine. Conclusion: Six patients with TDLs who had different clinical and radiological characteristics were presented with review of the literature with a special emphasis on spectroscopic investigation. doi:10.1016/j.jns.2013.07.1396 Abstract — WCN 2013 No: 2171 Topic: 6 — MS & Demyelinating Diseases Paraneoplastic neuromyelitis optica associated with stomach carcinoid tumor WCN 2013 No: 2171 Topic: 6 — MS & Demyelinating Diseases Paraneoplastic neuromyelitis optica associated with stomach carcinoid tumor T.M. Al-Harbi,M.F. Bin Falah. Neurology, King Fahad Specialist Hospital, Dammam, Saudi Arabia; Neurology, Dr.Sulaiman Al-Habib Hospital,