CIDP

CIDP
·       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.

·       Pathology

  • ·       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.

·       EMG/NCS

  • ·       Slow motor/sensory NCV
  • ·       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)

 

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Ray Jurewicz
412-731-0173
E-mail: rj@NerveStudy.com