Combined central and peripheral demyelination: a case report

Abstract

Overlapping central nervous system (CNS) and peripheral nervous system (PNS) demyelination is a rare clinical entity, more frequently seen in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and multiple sclerosis (MS). This case report showcases a patient with atypical CIDP and CNS demyelination lesions. Demographic data, disease course, treatment responsiveness, neurological examination, laboratory tests, nerve conduction studies (NCS), and brain and spinal cord MRI were registered. The case highlights the difficulty of diagnosis establishment and treatment selection, given the atypical course of the disease and limited answers to the indicated therapies. The data from our report suggest that specific and widely available immunological targets are necessary for diagnosing combined central and peripheral demyelination cases appropriately. The association of different immunotherapeutic agents may be necessary to induce and maintain disease remission.

Keywords: AIDP – acute inflammatory demyelinating polyneuropathy; CCPD – combined central and peripheral demyelination; CIDP – chronic inflammatory demyelinating polyneuropathy; CNS – central nervous system; CSF – cerebrospinal fluid; IVIg – intravenous immunoglobulin; LE – lower extremity; MRC – Medical Research Council; MRI – magnetic resonance imaging; MS – multiple sclerosis; NCS – nerve conduction studies; PLEX – plasma exchange; PNS – peripheral nervous; chronic inflammatory demyelinating polyneuropathy; combined central and peripheral demyelination; immunotherapy; multiple sclerosis.

Figure 1

Axial fluid-attenuated inversion recovery (FLAIR) MRI sections revealing hyperintense contrast-enhancing lesions in the corpus callosum and periventricular regions (A and B).

Figure 2

Sagittal T2-weighted MRI sequence of the cervical spinal cord revealing nonhomogeneous cervical spinal cord with a focal area of centro-posterior myelopathy (C1-C2: 3×4×15 mm), non-enhancing (white arrows).

Figure 3

Axial fluid-attenuated inversion recovery (FLAIR) MRI section revealing an acute hyperintense contrast-enhancing lesion in the right periventricular region.