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.
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.
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.