Perineuritis Successfully Treated with Early Aggressive Immunotherapy

Abstract

Perineuritis is a rare type of peripheral neuropathy defined by swelling and cellular infiltration in the perineurium. We herein report a 52-year-old man who presented with subacute onset pain from the back to the lower limbs, muscle weakness and hypoesthesia. A sural nerve biopsy revealed perineuritis, consisting of inflammatory cell infiltration and swelling of the perineurium. Oral prednisolone, plasma exchange and intravenous immunoglobulin treatment were all effective, leading to significant improvement of the symptoms.

Primary perineuritis, a rare but treatable neuropathy: Review of perineurial anatomy, clinicopathological features, and differential diagnosis

“…Electrodiagnostic testing generally showed evidence of a primarily axonal process, but two patients had slowing of motor conductions into the demyelinating range, one in a multifocal pattern. 27,32 MRI of the lumbar spine in three patients, cervical spine in two patients, and thoracic spine in one patient were unremarkable, 30,36 personal case Pelvic MRI was negative in one patient. 28 MR neurography has not been reported, but in our unreported patient, MRI of the lumbar plexus confirmed the presence of proximal involvement (enhancing obturator and femoral nerves) (Figure S1 in Data S1).…”

“…It was first described by Asbury and colleagues in 1971 and 1972 (duplicate patients) 4,24 . Ensuing reports of patients with a similar condition have used diverse diagnoses: “sensory perineuritis;” 25 “perineuritis;” 26,27 “idiopathic perineuritis;” 5 “nonvasculitic, steroid‐responsive mononeuritis multiplex;” 28 and “nonvasculitic neuritis.” 29,30 The initial cases were designated “sensory perineuritis” because of purely or nearly purely sensory symptoms, but most subsequently reported patients had both sensory and motor involvement 5,8,25–37 . The usual phenotype is an initially discrete and later overlapping MFN.…”

“…29,30 The initial cases were designated "sensory perineuritis" because of purely or nearly purely sensory symptoms, but most subsequently reported patients had both sensory and motor involvement. 5,8,[25][26][27][28][29][30][31][32][33][34][35][36][37] The usual phenotype is an initially discrete and later overlapping MFN. Patients are often assumed to have NSVN until a nerve biopsy is performed.…”

Central and Peripheral Nervous System Complications of Vasculitis Syndromes from Pathology to Bedside: Part 2—Peripheral Nervous System

“…In Wartenberg’s migrant sensory neuritis, the epineurial and perineum lymphocyte perivascular infiltrates with epi-, peri-, and endoneurial IgG deposition are the main morphological findings, all supporting an immune-mediated etiopathogenesis [ 11 , 33 , 34 ]. Thickening of the perineurium, inflammatory infiltrates, and necrosis of perineurial cells are responsible for the neuropathy.…”