Combined central and peripheral demyelination (CCPD)- a case report
Conference Paper/Poster - Oct 23, 2019
Aims and Background:
Acute and chronic demyelinating diseases (AIDP and CIDP) of the peripheral nervous system as well as acute and chronic demyelinating diseases of the central nervous system (ADEM and MS) are well described diseases with typical entity, diagnostic criteria and therapeutic recommendations. The presentation of both – a combined peripheral and central demyelination – was first described in 1992 and is characterized by a heterogeneous clinical presentation, variable disease course and challenges in therapeutic decision.
A 46 year old man was presented with a syncope and paraesthesia of both hands and feet since one week. On admission the patient had spastic distal paresis of the left leg (M4) and asymmetric glove-and-stocking type sensory disturbance and painful paraesthesia on both hands and feet. He also showed a sensory ataxic gait. Magnetic resonance imaging showed multiple high intensity cerebral lesions and two lesions in the spinal cord without contrast enhancement, not completely fulfilling the criteria for multiple sclerosis (Mc Donald Criteria 2017). CSF showed mild pleocytosis and elevated protein, no oligoclonal bands. Nerve conduction studies showed severe sensory and motor demyelinating polyneuropathy, fulfilling the criteria for AIDP. nerve ultrasound showed typical signs of acquired demyelinating polyneuropathy with multiple focal swelling of different nerves. Serological tests for neurotropic infections, rheumatological disorders, antiganglioside-antibodies, neurofascin antibodies, aquaporin4-antibodies, MAG- and MOG antibodies were negative. The diagnosis of combined central and peripheral demyelination was made and treatment with intravenous immunoglobulin (IvIGs) days was initiated. Five months later the symptoms did not improve clinically and nerve conductions were unchanged, so that the diagnostic criteria for CIPD were fulfilled. The magnetic resonance imaging was stable at 6 months follow-up.
Combined peripheral and central demyelination is rare but is supposed to be a new entity in the spectrum of demyelination diseases. Specific new antibodies like neurofascin or anti-MOG are described to be present in this disease. However, some cases like the one described her are sero-negative.