Transient Neurological Symptoms Preceding Cerebellar Ataxia with Glutamic Acid Decarboxylase Antibodies
- PDF / 353,802 Bytes
- 7 Pages / 595.276 x 790.866 pts Page_size
- 95 Downloads / 191 Views
ORIGINAL ARTICLE
Transient Neurological Symptoms Preceding Cerebellar Ataxia with Glutamic Acid Decarboxylase Antibodies Sergio Muñiz-Castrillo 1,2,3 & Alberto Vogrig 1,2 & Bastien Joubert 1,2 & Anne-Laurie Pinto 1,2 & David Gonçalves 4 & Hugo Chaumont 5 & Véronique Rogemond 1,2 & Géraldine Picard 1,2 & Nicole Fabien 4 & Jérôme Honnorat 1,2,6
# Springer Science+Business Media, LLC, part of Springer Nature 2020 Abstract
A prompt diagnosis and treatment of patients with autoimmune cerebellar ataxia (CA) with antibodies against glutamic acid decarboxylase (GAD-Abs) may lead to a better prognosis. Herein, we report prodromal transient neurological symptoms that should raise clinical suspicion of CA with GAD-Abs. We initially identified a 70-year-old man who presented a first acute episode of vertigo, diplopia, and ataxia lasting 2 weeks. Two months later, he experienced a similar episode along with new-onset gaze-evoked nystagmus. After 4 months, downbeat nystagmus, left limb dysmetria, and gait ataxia progressively appeared, and an autoimmune CA was diagnosed based on the positivity of GAD-Abs in serum and cerebrospinal fluid (CSF). We searched retrospectively for similar presentations in a cohort of 31 patients diagnosed with CA and GAD-Abs. We found 11 (35.4%) patients (all women, median age 62 years; 8/11 [72.7%] with autoimmune comorbidities) with transient neurological symptoms antedating CA onset by a median of 3 months, including vertigo in 9 (81.8%; described as paroxysmal in 8) and fluctuating diplopia in 3 (27.3%) patients. The identification of transient neurological symptoms of unknown etiology, such as paroxysmal vertigo and fluctuating diplopia, should lead to GAD-Abs testing in serum and CSF, especially in patients with autoimmune comorbidities. Keywords Cerebellar ataxia . Glutamic acid decarboxylase . Autoantibodies . Vertigo . Diplopia
Introduction
* Jérôme Honnorat [email protected] 1
French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France
2
Institut NeuroMyoGène, INSERM U1217/CNRS UMR 5310, Université de Lyon, Université Claude Bernard Lyon 1, Lyon, France
3
Neurology Department, Hospital Universitario Infanta Cristina, Madrid, Spain
4
Immunology Department, Hospices Civils de Lyon, Centre Hospitalier Lyon Sud, Lyon, France
5
Neurology Department, Centre Hospitalier Universitaire de la Guadeloupe, Point-à-Pitre, France
6
Service de Neuro-Oncologie, Hôpital Neurologique Pierre Wertheimer, 59 Boulevard Pinel, 69677 Bron Cedex, France
Cerebellar ataxia (CA) is one of the main immunemediated neurological syndromes associated with antibodies against glutamic acid decarboxylase (GAD-Abs), along with stiff-person syndrome (SPS) and limbic encephalitis (LE) [1–3]. Patients with CA are usually middle-aged women with chronic gait ataxia that generally responds poorly to immunotherapy [1, 2, 4]. A better prognosis has been reported in those with subacute onset and prompt treatment, high
Data Loading...
