The Clinical Features of Combined Central and Peripheral Demyelination (CCPD)

The investigators conduct this study to clarify the clinical features and to evaluate the prevalence of anti-nodal/paranodal antibodies of patients with combined central and peripheral demyelination (CCPD) .

Study Overview

• Status: Unknown
• Conditions: Combined Central and Peripheral Demyelination Disease

Detailed Description

The investigators will review the clinical manifestation, laboratory test results, electrophysiological examination and neuroimaging findings of patients with CCPD. And we will detect antibodies to aquaporin 4(AQP4), myelin oligodendrocyte glycoprotein (MOG), neurofascin-155 (Nfasc155), neurofascin-186 (Nfasc186), and myelin-associated glycoprotein (MAG) in patients with CCPD.

• Study Type: Observational
• Enrollment (Anticipated): 30

Contacts and Locations

• Study Contact: Name: Junwei Hao, MD,PhD; Phone Number: 010-83199088; Email: haojunwei@vip.163.com
• Study Contact Backup: Name: Xiaodan Hou, MD; Phone Number: +8618935415649; Email: hxddoc@126.com

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Patients who have combined central and peripheral demyelination

Description

Inclusion Criteria:

1. T2 high-signal intensity lesions in the brain or spinal cord on MRI, or visual-evoked potentials(VEPs) abnormalities.
2. conduction delay, conduction block, temporal dispersion or F-wave abnormalities, suggesting peripheral demyelinating neuropathy regarding nerve conduction studies (NCS).

Exclusion Criteria:

1. infectious diseases(e.g., human T lymphocyte trophic virus type1-associated myelopathy, syphilis, neuroborreliosis, HIV infection or progressive multifocal leukoencephalopathy)
2. pre-existing inflammatory diseases (e.g., sarcoidosis, Behçet's disease, Sjögren's syndrome, vasculitis or other collagen diseases)
3. mitochondrial disease
4. metabolic/toxic diseases (e.g., vitamin deficiency, amyloidosis, chronic alcoholism, diabetes mellitus or subacute myelo-opticoneuropathy due to clioquinol intoxication
5. cervical spondylotic myelopathy
6. syringomyelia
7. spinocerebellar degeneration
8. multiple myeloma, other tumors
9. inherited diseases (e.g., leucodystrophies)
10. cerebrovascular disease
11. non-specific lesions on T2-weighted MRI (e.g., leucoaraiosis).

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Retrospective

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort

Patients with CCPD; Inclusion criteria： Criteria for central nervous system involvement: T2 high-signal intensity lesions in the brain, or spinal cord on MRI, or visual-evoked potentials(VEPs) abnormalities.; Criteria for peripheral nervous system involvement: conduction delay, conduction block, temporal dispersion or F-wave abnormalities, suggesting peripheral demyelinating neuropathy regarding nerve conduction studies (NCS). In the present study, it was compulsory for at least two nerves between the median, ulnar, tibial and peroneal nerves to have abnormal findings indicating demyelination. Exclusion criterion: Secondary demyelinating diseases or changes.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Laboratory findings of nodal/paranodal antibodies in blood of patients with CCPD	These antibody including anti-neurofascin 155(NF155) et.al.	Day 1 post-gathering information of patients in hospital medical record system

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Demographic characteristics	The demographic characteristics of patients with CCPD such as age,sex,and et.al	Day 1 post-gathering information of patients in hospital medical record system
Neurological symptoms and signs	The neurological symptoms and signs of patients with CCPD	Day 1 post-gathering information of patients in hospital medical record system
Laboratory findings of blood and cerebrospinal fluid	Laboratory findings of blood and cerebrospinal fluid of patients with CCPD such as C reactive protein et.al in blood and protein et.al in cerebrospinal fluid	Day 1 post-gathering information of patients in hospital medical record system
Neuroimaging and VEPs findings	Neuroimaging and VEPs findings of patients with CCPD	Day 1 post-gathering information of patients in hospital medical record system
Nerve conduction study findings	Nerve conduction study findings of patients with CCPD	Day 1 post-gathering information of patients in hospital medical record system

Collaborators and Investigators

• Sponsor: Xuanwu Hospital, Beijing
• Investigators: Principal Investigator: Junwei Hao, Xuanwu Hospital, Beijing

Publications and helpful links

General Publications

• Cortese A, Franciotta D, Alfonsi E, Visigalli N, Zardini E, Diamanti L, Prunetti P, Osera C, Gastaldi M, Berzero G, Pichiecchio A, Piccolo G, Lozza A, Piscosquito G, Salsano E, Ceroni M, Moglia A, Bono G, Pareyson D, Marchioni E. Combined central and peripheral demyelination: Clinical features, diagnostic findings, and treatment. J Neurol Sci. 2016 Apr 15;363:182-7. doi: 10.1016/j.jns.2016.02.022. Epub 2016 Feb 10.
• Ogata H, Matsuse D, Yamasaki R, Kawamura N, Matsushita T, Yonekawa T, Hirotani M, Murai H, Kira J. A nationwide survey of combined central and peripheral demyelination in Japan. J Neurol Neurosurg Psychiatry. 2016 Jan;87(1):29-36. doi: 10.1136/jnnp-2014-309831. Epub 2015 Feb 11.
• Wang YQ, Chen H, Zhuang WP, Li HL. The clinical features of combined central and peripheral demyelination in Chinese patients. J Neuroimmunol. 2018 Apr 15;317:32-36. doi: 10.1016/j.jneuroim.2018.02.006. Epub 2018 Feb 9.
• Nave KA, Werner HB. Myelination of the nervous system: mechanisms and functions. Annu Rev Cell Dev Biol. 2014;30:503-33. doi: 10.1146/annurev-cellbio-100913-013101.
• Stathopoulos P, Alexopoulos H, Dalakas MC. Autoimmune antigenic targets at the node of Ranvier in demyelinating disorders. Nat Rev Neurol. 2015 Mar;11(3):143-56. doi: 10.1038/nrneurol.2014.260. Epub 2015 Jan 27.

Study record dates

Study Major Dates

• Study Start (Anticipated): December 1, 2020
• Primary Completion (Anticipated): December 1, 2020
• Study Completion (Anticipated): January 1, 2021

Study Registration Dates

• First Submitted: December 5, 2020
• First Submitted That Met QC Criteria: December 6, 2020
• First Posted (Actual): December 11, 2020

Study Record Updates

• Last Update Posted (Actual): December 16, 2020
• Last Update Submitted That Met QC Criteria: December 11, 2020
• Last Verified: December 1, 2020

More Information

Terms related to this study

• Keywords: antibody; clinical features; CCPD
• Additional Relevant MeSH Terms: Nervous System Diseases; Demyelinating Diseases
• Other Study ID Numbers: haojunwei1

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No