TY - JOUR
T1 - Combined central and peripheral demyelination
T2 - Clinical features, diagnostic findings, and treatment
AU - Cortese, Andrea
AU - Franciotta, Diego
AU - Alfonsi, Enrico
AU - Visigalli, N.
AU - Zardini, Elisabetta
AU - Diamanti, Luca
AU - Prunetti, P.
AU - Osera, Cecilia
AU - Gastaldi, Matteo
AU - Berzero, Giulia
AU - Pichiecchio, Anna
AU - Piccolo, G.
AU - Lozza, Alessandro
AU - Piscosquito, Giuseppe
AU - Salsano, Ettore
AU - Ceroni, Mauro
AU - Moglia, Arrigo
AU - Bono, Giorgio
AU - Pareyson, Davide
AU - Marchioni, Enrico
PY - 2016/4/15
Y1 - 2016/4/15
N2 - Combined central and peripheral demyelination (CCPD) is rare, and current knowledge is based on case reports and small case series. The aim of our study was to describe the clinical features, diagnostic results, treatment and outcomes in a large cohort of patients with CCPD. Thirty-one patients entered this retrospective, observational, two-center study. In 20 patients (65%) CCPD presented, after an infection, as myeloradiculoneuropathy, encephalopathy, cranial neuropathy, length-dependent peripheral neuropathy, or pseudo-Guillain-Barré syndrome. Demyelinating features of peripheral nerve damage fulfilling European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria for CIDP were found in 23 patients (74%), and spatial dissemination of demyelinating lesions on brain MRI fulfilling the 2010 McDonald criteria for multiple sclerosis (MS) in 11 (46%). Two thirds of the patients had a relapsing or progressive disease course, usually related to the appearance of new spinal cord lesions or worsening of the peripheral neuropathy, and showed unsatisfactory responses to high-dose corticosteroids and intravenous immunoglobulins. The clinical presentation of CCPD was severe in 22 patients (71%), who were left significantly disabled. Our data suggest that CCPD has heterogeneous features and shows frequent post-infectious onset, primary peripheral nervous system or central nervous system involvement, a monophasic or chronic disease course, inadequate response to treatments, and a generally poor outcome. We therefore conclude that the current diagnostic criteria for MS and CIDP may not fully encompass the spectrum of possible manifestations of CCPD, whose pathogenesis remains largely unknown.
AB - Combined central and peripheral demyelination (CCPD) is rare, and current knowledge is based on case reports and small case series. The aim of our study was to describe the clinical features, diagnostic results, treatment and outcomes in a large cohort of patients with CCPD. Thirty-one patients entered this retrospective, observational, two-center study. In 20 patients (65%) CCPD presented, after an infection, as myeloradiculoneuropathy, encephalopathy, cranial neuropathy, length-dependent peripheral neuropathy, or pseudo-Guillain-Barré syndrome. Demyelinating features of peripheral nerve damage fulfilling European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria for CIDP were found in 23 patients (74%), and spatial dissemination of demyelinating lesions on brain MRI fulfilling the 2010 McDonald criteria for multiple sclerosis (MS) in 11 (46%). Two thirds of the patients had a relapsing or progressive disease course, usually related to the appearance of new spinal cord lesions or worsening of the peripheral neuropathy, and showed unsatisfactory responses to high-dose corticosteroids and intravenous immunoglobulins. The clinical presentation of CCPD was severe in 22 patients (71%), who were left significantly disabled. Our data suggest that CCPD has heterogeneous features and shows frequent post-infectious onset, primary peripheral nervous system or central nervous system involvement, a monophasic or chronic disease course, inadequate response to treatments, and a generally poor outcome. We therefore conclude that the current diagnostic criteria for MS and CIDP may not fully encompass the spectrum of possible manifestations of CCPD, whose pathogenesis remains largely unknown.
KW - Acute disseminated encephalomyelitis
KW - Chronic inflammatory demyelinating polyneuropathy
KW - Combined central and peripheral demyelination
KW - Guillain-Barré syndrome
KW - Multiple sclerosis
UR - http://www.scopus.com/inward/record.url?scp=84959311461&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84959311461&partnerID=8YFLogxK
U2 - 10.1016/j.jns.2016.02.022
DO - 10.1016/j.jns.2016.02.022
M3 - Article
VL - 363
SP - 182
EP - 187
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
SN - 0022-510X
ER -