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HYPERTROPHY OF SPINAL ROOTS IN CHRONIC INFLAMMATORY DEMYELINATING NEUROPATHY
Author(s) -
Nardelli E.,
Agazzi P.,
Polo A.,
Boaretto M.,
Cavallaro T.,
Ferracci F.,
Moretto G.,
Rizzuto N
Publication year - 2000
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1046/j.1529-8027.2000.00513-41.x
Subject(s) - medicine , nerve root , muscle hypertrophy , cauda equina , sural nerve , mononeuropathy , myelopathy , nerve conduction velocity , polyradiculopathy , peripheral neuropathy , surgery , pathology , anatomy , cardiology , spinal cord , endocrinology , psychiatry , diabetes mellitus
We report on two patients (A, B) who developed unusual hypertrophy of cervical (A; disease duration (dd), 21 months) or lumbar (B; dd, 12 months) roots (LR) causing clinical features that were interpreted elsewhere initially as neurofibromas (A) or a Guillain‐Barrè syndrome (B). Nodular swellings along radial, ulnar, and the great auricular nerve were observed (A). Clinical signs of a rapid mixed motor and sensory distal impairment of all four limbs were occasionally associated with signs of spinal epiconus compression (B). A short course of 500 mg methylprednisolone/day for five days was administered without benefit (B). The clinical status rapidly improved with intense course of IVIg (B). The level of CSF protein was raised (B). Electrophysiological studies showed an impairment of both peroneal and tibial NCV overall through the proximal segments (prolonged F‐wave latency in A and B) and conduction blocks (A). Attenuated peripheral SNAPs (sural nerve, B) and absent spine and scalp SEPs from lower limbs (B) were found. Sural nerve biopsy (A) showed massive presence of onion bulbs throughout the section. Case A appears to fall somewhere between multiple mononeuropathy with persistent conduction blocks and CIDP. Case B had a relapsing‐remitting course and occasionally myelopathy insidiously appeared. Paraparesis due to the compression by enlarged and hypertrophic roots at the cauda equina was radiologically evident. MRI together with electrophysiological studies, and treatment response may be necessary to establish a correct diagnosis (A and B). The frequency of root hypertrophy (57% in Duggin's series, Brain 122, 1383, 1999) probably deserves further clinical studies and observations in the animal models of CIDP.