%0 Journal Article
%T Anti-Glycine Receptor Antibody Mediated Progressive Encephalomyelitis with Rigidity and Myoclonus Associated with Breast Cancer
%A Sofie N. De Blauwe
%A Patrick Santens
%A Ludo J. Vanopdenbosch
%J Case Reports in Neurological Medicine
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/589154
%X We describe a 66-year-old woman who presented with a dramatic course of PERM. Anti-glycine receptor antibodies were found. She stabilized after plasma-exchange and partly recovered. Eighteen months later, a diagnosis of smouldering breast cancer with bone marrow metastasis was made. There are indications that this tumor was already present at first presentation. An overview of PERM and anti-glycine receptor antibodies is given. 1. Introduction PERM is a severe, life-threatening condition, characterized by rigidity, painful muscle spasms, sensory and brainstem symptoms, autonomic features, breathing problems, and prominent spontaneous and stimulus-evoked myoclonus. It is part of the spectrum of stiff person syndrome and has been associated with anti-glutamic acid decarboxylase (GAD) antibodies [1]. More recently, a few cases of PERM with antibodies directed against the antiglycine receptor were reported [2¨C11]. 2. Case Report A 66-year-old, previously healthy retired primary school teacher, presented in October 2009 with inability to look to the left and gait instability. She also complained about dysesthesia in the left cheek with prominent painful electric tingling upon touch of the left cheek, left nostril, and left ear. This started three weeks earlier with nightly itching in the left cheek. For this reason, she had a decayed tooth removed, without resolution of the symptoms. She was not taking any medication, and her medical history was otherwise unremarkable. Upon clinical examination, she had a horizontal gaze palsy to the left, a subtle asymmetry of expression in the left face (both eye and nasolabial fold), and had an unstable gait. There was no appendicular or truncal ataxia at that time and pyramidal signs were absent. Reflexes were normal. The next day, her symptoms had progressed to a one and a half syndrome and prominent appendicular ataxia. A high resolution brain MRI was strictly normal. Lumbar puncture showed 58£¿lymphocytes/mm3, 8£¿red blood cells/mm3, normal glucose of 63£¿mg/dL (normal 40¨C70£¿mg/dL, glycemia 91£¿mg/dL), normal lactate of 1.8£¿mmol/L (normal 1.1¨C2.4£¿mmol/L), and mildly elevated protein of 69£¿mg/dL (normal 20¨C40£¿mg/dL). Oligoclonal bands were negative, but there was an elevated albumine-index of 11.7 (normal < 8). IgG index was normal. Because of a hypothesis of rhombencephalitis, she was started on IV methylprednisone 1£¿g daily for three days and ceftriaxone 4£¿g/day, ampicillin 12£¿g/day, and aciclovir 1000£¿mg/day for 10 days. Within 3 to 4 days, she made a full recovery. After stopping the IV antibiotics, she was discharged.
%U http://www.hindawi.com/journals/crinm/2013/589154/