Picture of the Month Myasthenic tongue Vasiliki Zouvelou a,⇑ , Michael Rentzos a , Panagiotis Toulas b , Ioannis Evdokimidis a a Department of Neurology, Eginition Hospital, University of Athens, Athens, Greece b Diagnostic Center “Euromedica-Encephalos”, Athens, Greece Received 18 March 2011; received in revised form 7 July 2011; accepted 11 October 2011 Keywords: Myasthenia gravis; Tongue atrophy; Anti-AchR positive; MRI In September 2010, a 55 year-old male patient pre- sented with fixed severe facial weakness, nasal speech, dysarthria and marked tongue atrophy with central fur- rowing, but good muscle bulk of the lateral tongue mar- gins (Fig. 1A). There was no ocular, axial, limb or respiratory involvement. The bulbar deficits were fixed for many years and the present referral was not due to clinical deterioration. In 1969, at the age of 14 he received the diagnosis of juvenile generalized myasthenia gravis with ocular, bulbar and limb muscle involvement. He was treated with pyridostigmine (180 mg qd) and prednisolone (20 mg qd) for 11 years with clinical improvement, concerning the ocular and limb muscle manifestations. For the last 30 years he remained clini- cally stable and free of medication. Thymectomy was never performed. During the whole disease course there was no episode of myasthenic crisis or requirement for short-term immunotherapy (plasma exchange or intrave- nous immunoglobulin). At the present evaluation, repet- itive nerve stimulation of nasalis, quadratus labii inferioris and trapezius muscles and single-fiber electro- myography of orbicularis oris, were positive for myas- thenia. Serum acetylcholine receptor antibodies were positive with a titer of 312 nM (normal range < 0.2 nM). Serum anti-titin and anti-MuSK antibodies were nega- tive. CT of the mediastinum was normal. MRI of the tongue demonstrated deep midline furrow and almost total fatty replacement of the tongue with predominant involvement of the intrinsic muscles (Fig. 1B–D). Myasthenia gravis is an antibody-mediated, neuromus- cular transmission disorder, where the targets are post- synaptic proteins, primarily the skeletal muscle acetylcholine receptor and the muscle-specific kinase (MuSK). The disease is characterized by weakness and fatigability of voluntary muscles. Muscle atrophy is an unusual finding in patients with myasthenia gravis, occurring usually in longstanding cases as disuse atrophy due to neuromuscular transmission failure [1]. Exception- ally, in MuSK-myasthenia muscle atrophy may be an early finding [2]. Localized muscle atrophy may occur in about 5–15% of the myasthenic patients and the tongue atrophy is the most frequent localized muscle atrophy in myasthe- nia. Visible wasting of the tongue was described by Buz- zard in 1905 and again by Wilson in 1955, who drew attention to the “triple furrowing” of the myasthenic tongue. Oosterhuis [1] reported 12 out of 418 myas- thenic patients with tongue atrophy and De Assis [3] reported 10 cases of tongue atrophy with persistent dys- phonia in a group of 752 patients with generalized myasthenia. We present the images of severe tongue atrophy of an anti-AChR positive myasthenic patient, 41 years after the disease onset. Although it is difficult to commit to the cause of the atrophy, the most probable is the long duration of myasthenia. References [1] Oosterhuis H, Bethlem J. Neurogenic muscle involvement in myas- thenia gravis. J Neurol Neurosurg Psychiatry 1973;36:244–54. 0960-8966/$ - see front matter Ó 2011 Elsevier B.V. All rights reserved. doi:10.1016/j.nmd.2011.10.006 ⇑ Corresponding author. Address: Department of Neurology, Univer sity of Athens, 74 Vas. Sophias Ave., Athens 11528, Greece. Tel.: +30 2107289404; fax: +30 2107216474. E-mail address: vzouvelu@med.uoa.gr (V. Zouvelou). www.elsevier.com/locate/nmd Available online at www.sciencedirect.com Neuromuscular Disorders 22 (2012) 289–290