Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

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Chronic Inflammatory Demyelinating
Polyradiculoneuropathy (CIDP)

• Prevalence
• 0.5/100,000 in children, 2/100,000 in adult.

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CIDP and GBS

• --Similarity
• Both are widespread polyradiculoneuropathies,
  usually with cytoalbuminologic dissociation of
  the CSF (raised protein concentration with fewer
  or no cells)
• both exhibit nerve conduction abnormalities
  characteristic of a demyelinating neuropathy
  (reduced conduction velocity and partial
  conduction block in motor nerves),
• pathologically, both show similar multifocal
  perivenous inflammatory infiltrates

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Difference

• 1.mode of evolution and prognosis
• CIDP begins insidiously and evolves slowly,
  either in a steadily progressive or stepwise
  manner, attaining its maximum severity after
  several months or longer
• From the beginning it may be asymmetrical or
  involve the arms predominantly
• 2. CIDP may be distinct immunologically from GBS
• 3. Difference response to the administration of
  prednisone

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Clinical features

• Weakness of the limbs, particularly of the
  proximal leg muscles, or numbness, paresthesias,
  and dysesthesias of the hands and feet were the
  initial symptoms
• a mixed sensorimotor polyneuropathy with weakness
  of the shoulder, upper arm, and thigh muscles, in
  addition to motor and sensory loss in the distal
  parts of the limbs
• Cranial nerve abnormalities were distinctly
  unusual

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• There was a systemic condition such as
  paraproteinemia, lymphoma, an undifferentiated
  reactive adenopathy, or lupus in association with
  an inflammatory demyelinating polyneuropathy
• The clinical course was monophasic and slowly
  progressive in about one-third, stepwise and
  progressive in another third, and relapsing in
  the remaining third.

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Diagnosis

• The chronic symmetric sensorimotor loss coupled
  with EMG findings of demyelination largely define
  the illness.
• The typical EMG findings are of multifocal
  conduction block , prolonged distal latencies
  (distal block), nerve conduction slowing to less
  than 80 percent of normal in several nerves, loss
  of late responses, and dispersion of the compound
  muscle action potentials, further reflecting
  demyelination in motor nerves.

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Laboratory Features

• The CSF protein is elevated in 80 percent of
  patients with CIDP, typically in the range of 75
  to 200 mg/dL.
• In biopsy material (sural nerve), half of cases
  are found to have interstitial and perivascular
  infiltrates of inflammatory cells,
• onion-bulb formations are conspicuous in the
  recurrent and relapsing cases.

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Treatment

• high doses of gamma globulin (2 g/kg in divided
  infusions over 4 or 5 days).
• plasma exchanges
• Predinison, 60 to 80 mg of prednisone daily that
  is tapered over months to the lowest effective
  dose, typically 25 to 40 mg
• azathioprine for at least 3 months), 3 mg/kg in
  a single daily dose,
• cyclophosphamide or mycophenolate

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--Bowel and bladder incontinence --Numbness and
paralysis --Flaccid lower extremity
paralysis --T7 sensation level to
temperature --Vibration was perceived at knees.
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Central nervous system manifestation of
Varicela-Zoster Virus
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Neuralgia-----Posterior horn lesion
Weakness--- -Polyradiculoneuroritis
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Reference

• Neurologic complications of the reactivation of
  varicella-zoster virus NEJM 2000,342635-645
• Chronic Inflammatory Demyelinating
  Polyneuropathy NEJM 2005,3521343-1356