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Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)


KEY FACTS

Terminology

  • Clinically heterogeneous, grossly symmetric, sensory and motor neuropathy evolving as monophasic, relapsing, or progressive disorder

    • Develops over > 8 weeks

Imaging

  • Enlargement and abnormal T2 hyperintensity of nerve roots, plexi, or peripheral nerves

  • Spinal nerve roots and peripheral nerves (extraforaminal > intradural)

  • Lumbar > cervical, brachial plexus, thoracic/intercostal > cranial nerve

Top Differential Diagnoses

  • Guillain-Barré (acute inflammatory demyelinating polyneuropathy)

  • Inherited demyelinating neuropathy

  • Neurofibromatosis type 1

Pathology

  • Autoimmune disease involving cellular and humoral immunity

  • Hallmarks of chronic inflammatory demyelinating polyneuropathy (CIDP): Enlarged nerves with “onion bulb” formations, demyelination

Clinical Issues

  • Diagnosis relies primarily on clinical, electrophysiologic examination supplemented by nerve biopsy

  • Typical presentation

    • Progressive weakness/sensory loss in distal and proximal segments of 4 limbs with areflexia

    • Evolves over > 8 weeks

    • ↑ cerebrospinal fluid protein, heterogeneous slowing of nerve conduction

    • Responds to corticosteroids and/or IV immunoglobulin therapy

  • Abnormal EMG/NCV: Key electrophysiologic features → nerve conduction block, slowed conduction velocities suggestive of demyelination

Axial T1WI C+ FS MR demonstrates symmetric enlargement, mild enhancement of lumbar nerve roots
.

Axial T2WI MR in same patient shows mild enlargement & iso- to hyperintensity of bilateral lumbar nerves
.

Sagittal T2WI in the same case reveals enlargement, abnormal T2 hyperintensity of the exiting extradural lumbosacral nerves
.

Axial T2WI in the same case shows enlargement, abnormal T2 hyperintensity of the exiting extradural lumbosacral nerves
. Imaging and associated progressive motor and sensory deficits with increased CSF protein content, electrophysiological features of demyelination, are characteristic of CIDP.

TERMINOLOGY

Abbreviations

  • Chronic inflammatory demyelinating polyneuropathy (CIDP)

Synonyms

  • Chronic inflammatory demyelinating polyradiculoneuropathy

Definitions

  • Chronic acquired, immune-mediated demyelinating neuropathy characterized by relapsing or progressive muscle weakness ± sensory loss

IMAGING

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