|· Chronic Inflammatory Demyelinating Polyradiculoneuropathy-acquired PN of presumed autoimmune basis; chronically progressive or relapsing/remitting forms.·
- Sx develop sub-acutely or insidiously over weeks to months.
- Contrasts with AIDP that develops acutely over few days or weeks.·
- Responds to immune modulating treatment. Responds to steroid, IVIg. Plasma exchange, other immunosuppressive meds may be also used.·
- Early signs/sx: paresthesia; painful sx such as burning/searing jabbing pain less common. Tremors, limb/gait ataxia, loss of balance may reflect impaired proprioception. Significant disability may occur.
- · Link with pre-ceding viral or bacterial infection less apparent than AIDP.
- · Macrophage induced segmental demyelination usually accompanied by nerve edema and infiltrates of mononuclear inflammatory cells.
- · Symmetric neuropathy with proximal nerves (even CNS) involved.
- · Paranodal, internodal segmental demyelination. ?macrophage induced
- · Perivascular lymphocytes frequently seen in epineurium
- · Some axonal degeneration.
- · Edema, epineural and endoneural inflammation.
- · Onion bulb formation, scarring.
- · No strong evidence for characteristic antibody.
- · Anatomic-motor greater than sensory; large greater than small fiber; proximal (root/plexus/proximal nerves) more than distal nerves.
· Diagnostic criteria
- · Symmetric proximal/distal weakness.
- · Sensation especially proprioception impaired; numbness/tingling/parasthesia.
- · Generalized motor/sensory neuropathy; multifocal inflammatory demyelination.
- · Cranial nerves may be affected.
- · Disease progression for longer than 8 weeks or relapsing course mandatory for dx.
· Lab findings
- · Protein content in CSF elevated; lymphocyte count less than 10/mm.
- · Inflammatory demyelination with nerve biopsy; maybe some axonal loss.
- · Trial of therapy may aid dx.
- · Sensory SNAP responses may be absent.
- · Strong emphasis on focal conduction block/increased temporal dispersion in several motor nerve segments.
- · Little/no positive sharp wave, fibrillation activity.
Multifocal Motor and Sensory Demyelinating Neuropathy
CIDP varient-affects upper limbs in asymmetric fashion.
· Different designations
- · Multifocal motor and sensory demyelinating mononeuropathy (MDSMM)
- · Upper limb-predominate multifocal chronic inflammatory neuropathy.
- · Multifocal acquired demyelinating motor and sensory neuropathy (MADSAM)