Journal of Neurological Sciences (Turkish) Ahead Of Print
Combined Central And Peripheral Nervous System Demyelination: A Case Report
İlay Hilal Kılıç1, Can Emre Erdoğan1, İsa Aydın1, Figen Gökçay1, Ayşe Nur Yüceyar1
1Ege University Faculty of Medicine, Neurology, İzmir,
DOI : 10.24165/jns.10192.17 Combined central and peripheral nervous system demyelination (CCPD) is a fairly new concept and our knowledge is restricted to several case series and case reports. We aimed to contribute to the literature with our experiences in the management of a patient representing both chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multiple sclerosis (MS) features. A 32-year-male patient presented with neuropathic compaints for one year and recent onset of optic neuritis with central demyelinating lesions. Clinical, electrophysiological and radiological response was achieved with corticosteroid treatment and relapsed after cessation. His second simultaneous CCPD exacerbation did not respond on intravenous immunoglobulins (IVIG) but responded well on intravenous high dose corticosteroid. Previous studies reported inadequate responses to high-dose corticosteroids and IVIG in the majority of patients with CCPD. It?s estimated to be a unique disease beyond MS and CIDP with heterogenous features. We suggest that awareness of this steroid-dependent condition may improve our knowledge in the pathophysiology and management strategies. Keywords : Chronic inflammatory demyelinating polyneuropathy; Multiple sclerosis; Combined central and peripheral demyelination; Optic Neuritis