Abstract
Combined Central and Peripheral Demyelinating Spectrum Disorder: A Case Report and Systematic Review
Author(s): Lourdes de Fatima Ibanez Valdes and Humberto Foyaca Sibat*Background: Combined Central and Peripheral Demyelination (CCPD) disorders are composed of uncommon clinical pathologies characterized by central and peripheral nervous system inflammatory processes of the Myelin Sheath (MS). Clinical features of CCPD are atypical for MS, with a lack of oligoclonal immunoglobulin G bands causing CCPD target nodes and paranodes of a node of Ranvier in the CNS/PNS and an associated anti-neurofascin antibody. The previous report about the speed recovery after anti-CD20 monoclonal antibody-Rituximab therapy has not been published.
Methods: We searched the medical literature for articles on CCPD and other autoimmune-mediated peripheral neuropathies between January 01, 2012, and January 01, 2022, following our inclusion/exclusion criteria. The relevant information was extracted from each publication using Microsoft Excel in a structured coding scheme. Our investigation used aggregate data where possible, following the PRISMA guidelines, and all studies were screened for bias using an internationally accepted scoring system.
Results: A total of 88 cases have been reported within this period, and 59% were females. Only 21 cases presented clinical manifestations of the Central Nervous System (CNS) and Peripheral Nervous System (PNS) simultaneously at the onset, with an overall mean age of 33.3 ± 12.4 (SD) years. The sensory disturbance was the most common sign reported (92%), followed by motor signs (89.4%) and abnormal gait (81%). Raised albumin level in the CSF was seen quite often. (79.9%).
Case presentation: A 32-year-old female patient complaining of two months history of numbness and cramps of the left foot and five days of the same paresthesias of the right lower limb. The paresthesia then involving both lower limbs and ascending. After ten days, there was an onset of weakness in both lower limbs. Two months later, the patient needed aid for walking. She had initially sought traditional medicine unsuccessfully. However, one month later noticed distal weakness in the upper limbs and symmetrical sensory disturbances in four limbs. NVC and CT/MRI of the brain and spinal cord confirmed the diagnosis of CCPD.