CIDP Patient Impoves with Treatment

This 11-year-ol girl had a 4-month history or progressive proximal leg weakness leading to falls and difficulty with stairs. There was no back pain, numbness in the legs or difficulty with badder or bowel control.

Her exam showed leg weakness, absent reflexes and normal sensation.

Her serum CK was normal.  Her EMG showed features of acquired demyelinating neuropathy, most notably absent F-waves.  Her CSF showed a mildly elevated protein level without cells (“albuminocytologic dissociation”).  She was treated with a course of intravenous immune globulin and made a remarkable recovery within 4-weeks.

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Chronic inflammatory demyelinating polyneuropathy (CIDP)

CIDP is an acquired immune-mediated inflammatory disorder of the peripheral nervous system, causing demyelination, conduction slowing and conduction block:

Affected nerves fail to respond to stimuli causing progressive muscle weakness, loss of deep tendon reflexes (areflexia), fatigue, and abnormal sensations.

Most cases show evidence of demyelinating neuropathy on electrodiagnostic studies and albuminocytologic dissociation in the cerebrospinal fluid.

Early diagnosis and treatment is important in preventing irreversible axonal loss and improving functional recovery.

However, CIDP is probably under-recognized and under-treated due to its variable presentation and the limitations of clinical, serologic, and electrophysiologic diagnostic criteria.

Consultation with a sub-specialty trained neuromuscular physician is critical.

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6 thoughts on “CIDP Patient Impoves with Treatment

  1. Hi Dr Holland ,
    Im Dr Abdelgadir Osman from Republic of Sudan. Im a registrar of Neurology. I have a patient whose diagnosis is Axonal variant GBS or demyelinating poly radiculoneuropathy .
    He had recieved IVI but showed no improvement at all. Im sure that you got experience in this area of neurology. Please if you can help let me know. Thanks in advance.

    • Thanks for your message. If your patient has truly axonal GBS (sometimes referred to Feasby’s GBS or AMSAN), then the outlook for recovery is unfortunately going to slow and incomplete no matter what you do. Combining PE and IVIG is no more efective than either treatment alone in GBS. All you can really do is continue supportive care, PT and wait it out. Click here for a link to a good review of the subject.

  2. Hi Dr Holland,
    My husband and I stopped by your office last week regarding our son. I just wanted to ask you a quick question.

    • I am not sure what you mean Maribel – if you mean symptoms that come and go on a monthly cycle, then that would be very unusual for CIDP, and would be more typical for something like a periodic ataxia or dystonia. If you post or email me more information, I might be able to give you a better answer. Thanks for you interest in our web site.

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