In the borderland of multifocal motor neuropathy and chronic inflammatory demyelinating polyradiculopathy
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In the borderland of multifocal motor neuropathy and chronic inflammatory demyelinating polyradiculopathy Raffi Topakian 1
&
Petra Müller 1 & Ioana-Cristina Ciovica-Oel 2 & Johannes Trenkler 3
Received: 18 May 2020 / Accepted: 6 October 2020 # Fondazione Società Italiana di Neurologia 2020
Abstract Chronic inflammatory demyelinating polyradiculopathy (CIDP) and multifocal motor neuropathy (MMN) are seen as distinct entities with marked differences in pathophysiology and clinical, laboratory, and imaging features. We report a patient with an immune-mediated neuropathy in the borderland of CIDP and MMN, whose magnetic resonance imaging and cerebrospinal fluid (CSF) features strongly resembled CIDP, while the clinical course and treatment response suggested the diagnosis of MMN without conduction blocks. There is strong evidence that MMN is not a variant of CIDP and that these conditions can be separated pathologically. Our case report widens the spectrum of MMN presentations, indicating the existence of a clinical overlap syndrome of MMN and CIDP, and emphasizing the need for more precise criteria regarding CSF and nerve root imaging abnormalities in the differentiation of chronic immune-mediated neuropathies. Keywords Cerebrospinal fluid protein . Chronic inflammatory demyelinating polyradiculopathy . Differential diagnosis . Magnetic resonance imaging . Multifocal motor neuropathy . Nerve conduction studies . Overlap
Introduction Multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyradiculopathy (CIDP) are seen as distinct entities with marked differences in pathophysiology and clinical, laboratory, and imaging features [1, 2]. However, the diagnosis of chronic immune-mediated neuropathies can be challenging, as can be derived from the fact that there exist at least 15 sets of criteria for the diagnosis of CIDP, the most common immunemediated neuropathy. Despite important progress over the last 10 years with improved definitions of “typical CIDP” and “atypical CIDP,” misdiagnosis and overdiagnosis of CIDP remain highly prevalent [3]. We report a patient with an immunemediated neuropathy in the borderland of CIDP and MMN, whose magnetic resonance imaging (MRI) and cerebrospinal
* Raffi Topakian [email protected] 1
Department of Neurology, Academic Teaching Hospital Wels-Grieskirchen, Grieskirchner Str. 42, 4600 Wels, Austria
2
Department of Neurology, Kepler University Hospital, Linz, Austria
3
Institute of Neuroradiology, Kepler University Hospital, Linz, Austria
fluid (CSF) features strongly resembled CIDP, while the clinical course and treatment response suggested the diagnosis of MMN without conduction blocks on nerve conduction studies (NCS).
Case presentation A 37-year-old woman presented with a 3-year-history of walking difficulties with insidious onset and recent worsening. Her first symptom had been mild weakness of left-sided plantar flexion. She reported recurrent self-limited diffuse arthralgias, subjective weakness in her hands, bilatera
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