Mononeuritis multiplex in Crest Syndrome

[James-J-Shea-MD]

  Dear Dr.
  My 62 yr. old wife with a thirty year history of Crest Syndrome with slow progression and responsive to small doses of prednisone{5 to 7.5 mg.} daily, received a pneumovax immunization 8-24-98 and developed bilateral numbness of her feet with R. foot drop on 9-15-98. She was referred to our Neurologist and a diagnosis of mononeuritis multiplex was made and prednisone wea increased to 60 mg. per day. There was no progression on this regime and she was referred to the Neurology Dept. at UCLA where the diagnosis was confirmed and a sural nerve biopsy showed axonal necrosis.
  She was transferred home for rehab. and after high doses iv of prednisone she had been gradually reduced to 55mg. po daily. In the early hours of the first night at the rehab center she developed and acute R. wrist drop. Previously she had only had ulnar sensory deficit in the R. hand onset apppros 9-16-98. Readmission followed and steroid were increased and given iv and a course of ivig times 5 days halted any other progression. About three weeks later during the same admission she was transferred to the rehab unit but developed urosepsis and was readmitted for Rx. with good response. Unfortunately she lost the L. wrist suddenly during the night about three weeks after this admission despite steroid Rx. At present she is at home with R. foot drop{Emg show sciatic lesion and sans improvement since onset 9-98}, R. and L. wrist drop with slight flexion of thumbs and index and middle fingers on left and no extension or flexion of either wrist. We are of course concerned about possibility of how much if any recovery we can hope for. Your experience and advice would be appreciated.
  Thank you.
  James J. Shea M.D.
  [e-mail]
===========================================================================
Dear Dr. Shea:
Mononeuritis multiplex is an uncommon but known complication of the CREST syndrome. It tends to be predominantly sensory and somewhat more indolent than in yours wife's case. The cause is believed to be a necrotizing vasculitis. Please refer to the following recent paper from Mayo Clinic on the subject.
A case-control and nerve biopsy study of CREST multiple mononeuropathy.
Neurology  Dec 1997, 49(6) p1641-5
Dyck PJ; Hunder GG; Dyck PJ
Although there are no trials or proof of efficacy, I would be inclined to treat your wife's neuropathy very aggressively, likely with a combination of high dose oral prednisone and oral cytoxan.
Recovery with severe proximal axon loss lesions (sciatic, for instance) tends to quite incomplete and slow. EMG studies may help prognosticate.
Good luck!