title: Contribution of plexus MRI in the diagnosis of atypical chronic inflammatory demyelinating polyneuropathies.
abstract: Nerve enlargement has early been recognized in CIDP and plexus MRI hypertrophy has been reported in typical CIDP cases. Our aim is to determine plexus MRI value in the diagnosis of CIDP with an initial atypical presentation, which, up to now, has not been demonstrated. Retrospective study of 33 consecutive patients suspected of CIDP. Plexus MRI was performed on the most affected territory (brachial or lumbar). Were assessed: plexus trophicity, T2-STIR signal intensity and gadolinium enhancement. Final CIDP diagnosis was made after comprehensive workup. A histo-radiological correlation was performed. Final CIDP diagnosis was made in 25 (76%) including 21 with initial atypical clinical presentation. Eleven CIDP patients (52%) with initial atypical clinical presentation had abnormal plexus MRI including 9 suggestive of CIDP (43%) and none of the patients with an alternative diagnosis. Hypertrophy of the proximal plexus and/or extraforaminal roots was found in 8 cases and Gadolinium enhancement in 2 cases. Abnormalities were more frequent on brachial (86%) than lumbosacral MRIs (29%) and asymmetrical (72%) and most often associated with histological signs of demyelination. The nerve biopsy was suggestive of CIDP in 9/13 patients with normal MRI. Plexus MRI seems useful in the diagnostic strategy of patients with suspicion of CIDP with atypical presentation. Nerve biopsy remains important when other investigations are inconclusive.
authors.affiliation: French National Reference Centre for Familial Amyloid Polyneuropathies and Other Rare Diseases, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France; Department of Neurology, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France. Electronic address: pierre.lozeron@aphp.fr.
authors.forename: Pierre
authors.initials: P
authors.lastname: Lozeron
authors.affiliation: French National Reference Centre for Familial Amyloid Polyneuropathies and Other Rare Diseases, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France; Department of Neuroradiology, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France.
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Lacour
authors.affiliation: French National Reference Centre for Familial Amyloid Polyneuropathies and Other Rare Diseases, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France; Department of Neuroradiology, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Vandendries
authors.affiliation: French National Reference Centre for Familial Amyloid Polyneuropathies and Other Rare Diseases, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France; Department of Neurology, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Théaudin
authors.affiliation: French National Reference Centre for Familial Amyloid Polyneuropathies and Other Rare Diseases, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France; Department of Neurology, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Cauquil
authors.affiliation: Department of Neurology, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France; Univ Paris 11, INSERM U788, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France.
authors.forename: Christian
authors.initials: C
authors.lastname: Denier
authors.affiliation: French National Reference Centre for Familial Amyloid Polyneuropathies and Other Rare Diseases, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France; Department of Neuropathology, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Lacroix
authors.affiliation: French National Reference Centre for Familial Amyloid Polyneuropathies and Other Rare Diseases, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France; Department of Neurology, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France; Univ Paris 11, INSERM U788, Bicêtre Hospital, APHP, Le Kremlin Bicêtre, France.
authors.forename: David
authors.initials: D
authors.lastname: Adams
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001917:Brachial Plexus / Q000473:pathology*; D005260:Female; D006801:Humans; D008160:Lumbosacral Plexus / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000473:pathology*; D012189:Retrospective Studies
keywords: Atypical; CIDP; Diagnosis; MRI; Plexus