Muscle hypertrophy and a syndrome of continuous motor unit activity in prednisone-responsive Guillain-Barré polyneuropathy

Summary The clinical, electrophysiological and morphological findings (light and electron microscopy of the sural nerve and gastrocnemius muscle) are reported in an unusual case of Guillain-Barré polyneuropathy with an association of muscle hypertrophy and a syndrome of continuous motor unit activity. Fasciculation, muscle stiffness, cramps, myokymia, impaired muscle relaxation and percussion myotonia, with their electromyographic accompaniments, were abolished by peripheral nerve blocking, carbamazepine, valproic acid or prednisone therapy. Muscle hypertrophy, which was confirmed by morphometric data, diminished 2 months after the beginning of prednisone therapy. Electrophysiological and nerve biopsy findings revealed a mixed process of axonal degeneration and segmental demyelination. Muscle biopsy specimen showed a marked predominance and hypertrophy of type-I fibres and atrophy, especially of type-II fibres.