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title: "CIDP"
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docid: "12e4033c-edc8-46ff-8081-3acc433cda78"
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authors:
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- key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607"
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value: "Surjith Vattoth, MD, FRCR"
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breadcrumbs:
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-
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name: "Brain"
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slug: "brain"
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treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a"
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name: "Diagnosis"
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treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8"
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name: "Pathology-Based Diagnoses"
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slug: "pathology-based-diagnoses"
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treeNodeId: "d9d3a8ed-f21b-4831-8c77-591a3500ef77"
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-
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name: "Infectious, Inflammatory, and Demyelinating Disease"
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slug: "infectious-inflammatory-and-demyel-"
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treeNodeId: "7210f860-fe5f-4a2d-81cc-4fe06c769607"
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-
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name: "Inflammatory and Demyelinating Disease"
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slug: "inflammatory-and-demyelinating-dis-"
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treeNodeId: "62ab4dc3-dbf6-45a9-8532-f0e962aa62dc"
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-
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name: "CIDP"
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slug: "cidp"
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treeNodeId: null
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category: "Brain"
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documentVersionId: "96729e13-6c4b-4fd3-be3e-4e1a940566fd"
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imageCount: 12
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isComparable: true
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isInCompareCart: false
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lastUpdated: "06/08/20"
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pageDescription: "CIDP"
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pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Infectious, Inflammatory, and Demyelinating Disease, Inflammatory and Demyelinating Disease, CIDP"
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pageTitle: "CIDP | STATdx"
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enhancedTitle: "CIDP"
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type: "DX"
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breadcrumbs:
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- "Brain"
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- "Diagnosis"
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- "Pathology-Based Diagnoses"
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- "Infectious, Inflammatory, and Demyelinating Disease"
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- "Inflammatory and Demyelinating Disease"
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- "CIDP"
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---
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# KEY FACTS
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- ## Terminology
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- Clinically heterogeneous, grossly symmetric, sensory & motor neuropathy evolving as monophasic, relapsing, or progressive disorder
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- Develops over > 8 weeks
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- ## Imaging
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- Sagittal FLAIR may reveal hyperintense brain lesions similar to multiple sclerosis
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- Enlargement & abnormal T2 hyperintensity of nerve roots, plexi, or peripheral nerves
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- ↑ nerve root diameter, cross-sectional area (CSA), & volume
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- Spinal nerve roots & peripheral nerves (extraforaminal > intradural)
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- Lumbar > cervical, brachial plexus, thoracic/intercostal > cranial nerve
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- Fair degree of CSA correlation between high-resonance nerve ultrasound (HRUS) & MR neurography (MRN)
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- ## Top Differential Diagnoses
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- Guillain-Barré (AIDP)
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- Inherited demyelinating neuropathy (Charcot-Marie-Tooth)
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- Neurofibromatosis type 1, schwannomatosis
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- ## Pathology
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- Autoimmune disease of cellular & humoral immunity
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- Hallmarks of CIDP: Enlarged nerves with onion bulb formations, demyelination
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- ## Clinical Issues
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- Usually **clinical**diagnosis based on progressive weakness/sensory loss & response to steroids
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- Typical: Symmetric proximal & distal weakness, sensory loss
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- Abnormal EMG/NCV: Key electrophysiologic features → nerve conduction block, slowed conduction velocities suggestive of demyelination
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- Diagnosis relies primarily on clinical, electrophysiologic examination supplemented by nerve biopsy
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# TERMINOLOGY
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- ## Abbreviations
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- Chronic inflammatory demyelinating polyneuropathy (CIDP)
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- ## Synonyms
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- Chronic inflammatory demyelinating polyradiculoneuropathy
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- ## Definitions
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- Chronic acquired, immune-mediated demyelinating neuropathy characterized by relapsing or progressive muscle weakness ± sensory loss
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- Enlargement & abnormal T2 hyperintensity of nerve roots, plexi, or peripheral nerves
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- Spinal nerve roots & peripheral nerves (extraforaminal > intradural)
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- Lumbar > cervical, brachial plexus, thoracic/intercostal > cranial nerves (CNs)
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- ### Size
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- Nerve size varies; small → very large
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- Mean diameter of spinal nerve roots in CIDP: Cervical 6-6.8 mm; lumbosacral 7.3-10.4 mm
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- 5-mm best cut-off value of C6, C7, C8 nerve root diameters to distinguish CIDP patients from controls
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- CIDP nerves larger volumes, which positively correlate with disease duration
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- Recent MR neurography (MRN) of L3-S1 nerve roots of lumbosacral plexus using 3D multiple echo recalled gradient-echo (3D MERGE) sequence showed
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- ↑ mean cross-sectional area (CSA): 28.04 ± 8.55 mm² in CIDP (14.91 ± 2.36 square mm² in normal); optimal cut-off value 19.20 mm²
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- ### Morphology
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- Focal or diffuse fusiform enlargement of cauda equina, nerve roots/plexi, & peripheral nerves
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- ## CT Findings
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- ### NECT
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- Isodense nerve enlargement
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- ### CECT
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- Mild to moderate nerve enhancement
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- ## MR Findings
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- ### T2WI
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- Enlargement, abnormal hyperintensity of intradural & extradural spinal nerves/branches
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- ### FLAIR
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- Sagittal FLAIR may reveal hyperintense brain lesions similar to multiple sclerosis (MS)
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- ### DWI
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- Diffusion-weighted MRN
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- DTI: ↓ nerve fractional anisotropy (FA) (mean 0.42 ± 0.08) in CIDP compared to healthy controls (0.52 ± 0.04)
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- ↓ FA due to ↑ radial diffusivity (RD); axial diffusivity (AD) not significant
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- FA & RD correlate strongly with electrophysiological markers of demyelination
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- ### T1WI C+
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- Mild to moderate nerve enhancement
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- ## Ultrasonographic Findings
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- ### Grayscale ultrasound
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- Hypoechoic, hypertrophic nerves
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- Fair degree of CSA correlation in high resonance nerve US (HRUS) & MRN of cervical plexus, & peripheral nerves in CIDP
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- CSA in HRUS correlate well with markers of nerve integrity, such as ↓ FA in DTI & with ↑ T2 signal
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- HRUS-CSA of interscalene brachial plexus correlated significantly with MRN-CSA & T2 signal of L5 & S1 lumbar plexus roots
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- ## Imaging Recommendations
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- ### Best imaging tool
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- MRN, T2WI, enhanced coronal & axial T1WI sequences with fat suppression best delineate nerve lesions
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- Brain MR to detect subclinical CNS demyelination
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# DIFFERENTIAL DIAGNOSIS
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- ## Conditions Recently Proposed to be Included Under CIDP Syndrome
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- Antimyelin associated glycoprotein (MAG) neuropathy
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- Chronic neuropathies associated with IgG4 antibodies against paranodal/nodal proteins; chronic immune sensory polyradiculopathy (CISP); multifocal motor neuropathy
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- [Guillain-Barré (Acute Inflammatory Demyelinating Polyneuropathy)](/document/guillain-barr-spectrum-disorders/c1f52a65-920e-4e28-8a75-07dfa208f290)
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- Pial, nerve root enhancement similar to CIDP
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- Differs from CIDP in onset duration, clinical course
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- Acute onset of ascending paralysis with relative sensory preservation
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- [Hereditary Motor and Sensory Neuropathy](/document/hypertrophic-neuropathy/e246f4d1-0262-4ca7-b8e1-6f2a4bd67c06)
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- Also called Charcot-Marie-Tooth (CMT) disease
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- CMT1, CMT 3 (Dejerine-Sottas disease) CMT4, CMTX1
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- Genetic testing, clinical phenotype distinguish from CIDP
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- [Neurofibromatosis Type 1](/document/neurofibromatosis-type-1-spine/89236653-e750-4fa7-b2b1-0a3c4ed31a87)
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- Diffuse nerve root enlargement, enhancement
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- Genetic testing & distinctive clinical stigmata to distinguish
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- ## Lateral Meningocele
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- CSF density/signal intensity (not solid) ± foraminal enlargement, dural ectasia
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- Usually coexisting NF1 or connective tissue disorder (Marfan syndrome)
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- ## Schwannomatosis
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- Multiple schwannomas of peripheral nerves & CNs [nonvestibular schwannomas (nVS)]
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- However, unilateral VS described with germline mutations of Schwannomatosis in SMARCB1 & LZTR1
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- ## Other Clinical Differential Diagnosis
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- Diabetic neuropathy, amyloid neuropathy due to TTR mutations, vasculitic neuropathy, POEMS syndrome
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- Exact pathogenesis of CIDP unclear; involves both cellular & humoral immune factors
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- Polyneuropathies co-occurring with MS: Underdiagnosed; extra disability burden; includes CIDP
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- 1/3 of MS-CIDP cases with serum testing show IgG4 autoantibodies to neurofascin-155
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- ## Gross Pathologic & Surgical Features
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- Extensive fusiform nerve enlargement ± gross onion bulb formations
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- ## Microscopic Features
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- Large nerve, onion bulb formations, demyelination
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- Macrophage, T-cell infiltration → perivascular inflammatory infiltrates, nerve demyelination & remyelination
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- Onion bulb formation: Excessive Schwann cell process proliferation → repetitive demyelination/remyelination
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Mixed sensorimotor neuropathy; typical form: Symmetric proximal & distal weakness, sensory loss
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- Rarer atypical form (Lewis-Sumner syndrome)
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- Predominantly uni- or multifocal as well as distal
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- CNs are occasionally affected, with particular tropism for CNVII, but ophthalmoplegia or bulbar weakness can be present
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- ### Other signs/symptoms
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- Chronic progressive: Progressively deteriorate until treatment is given
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- ## Demographics
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- ### Sex
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- M = F
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- ## Natural History & Prognosis
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- Average disease duration: 7.5 years
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- ## Treatment
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- European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy; immunomodulation or immunosuppression therapy
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# DIAGNOSTIC CHECKLIST
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- ## Consider
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- Consider CIDP in differential of nerve root/peripheral nerve enlargement
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- ## Image Interpretation Pearls
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- MR findings imperfectly correlate with clinical disease activity/severity, laboratory findings
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0f953548-b230-4137-9147-51d6ed147c6c
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## Images
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### Selected Images
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*Sagittal T1 C+ MR of the cervical spine shows marked hypertrophy and enhancement of all exiting cervical nerve roots <img src='/img/arrows/CS.png'/>. 5 mm is considered an adequate cut-off value of cervical spinal nerve root diameter, discriminating CIDP from controls. Mean diameter of spinal nerve roots in CIDP: Cervical 6-6.8 mm; lumbosacral 7.3-10.4 mm.*
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*Sagittal T1 C+ MR of the cervical spine shows marked hypertrophy and enhancement of all exiting cervical nerve roots <img src='/img/arrows/CS.png'/>. 5 mm is considered an adequate cut-off value of cervical spinal nerve root diameter, discriminating CIDP from controls. Mean diameter of spinal nerve roots in CIDP: Cervical 6-6.8 mm; lumbosacral 7.3-10.4 mm.*
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*Sagittal T2WI MR reveals enlargement and T2 hyperintensity of exiting extradural lumbosacral nerves <img src='/img/arrows/CS.png'/>. High signal of CSF should be excluded while measuring nerve root size/area in T2 MR.*
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*Axial T1WI C+ MR depicts enlargement and abnormal enhancement of exiting extradural lumbosacral nerves <img src='/img/arrows/CS.png'/>. Blood-nerve barrier breakdown can cause contrast enhancement. Axon loss associated with demyelination is the most important factor of disability and resistance to treatment. Root hypertrophy also may cause stenosis symptoms.*
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*Sagittal FLAIR MR demonstrates periventricular ovoid hyperintensities <img src='/img/arrows/CO.png'/> in a typical case of marked fusiform CIDP nerve enlargement with brain demyelination.*
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### Additional Images
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*Axial T1WI C+ MR shows thickening and enhancement of ventral and dorsal cauda equina nerve roots <img src='/img/arrows/WS.png'/>.*
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*Sagittal T2WI MR demonstrates diffuse thickening of the intradural cauda equina nerve roots.*
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*Sagittal FLAIR MR of the brain in a CIDP patient shows a typical paraventricular demyelinating lesion <img src='/img/arrows/WS.png'/> similar to those seen in multiple sclerosis patients.*
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*Sagittal T2WI MR depicts enlarged lumbar nerve roots extending into extraforaminal ventral primary rami <img src='/img/arrows/WS.png'/>.*
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*Axial T2WI MR shows diffuse thickening and hyperintensity of thoracic nerve roots and paraspinal intercostal nerves.*
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*Axial T2WI MR reveals bilateral symmetric enlargement, hyperintensity of cervical nerve roots and brachial plexus <img src='/img/arrows/WS.png'/>.*
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*Sagittal T1WI C+ MR demonstrates diffuse pial thickening and enhancement extending into the cauda equina nerve roots. Clinical course distinguished from Guillain-Barré (AIDP).*
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*Axial T2WI MR shows marked enlargement of the lumbar/sacral nerve roots <img src='/img/arrows/BS.png'/> and lumbosacral trunk <img src='/img/arrows/WS.png'/>.*
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