Spinal and cranial hypertrophic neuropathy in multiple sclerosis

Abstract

Two patients with multiple sclerosis developed symptomatic chronic inflammatory demyelinating polyneuropathy with massive spinal or cranial nerve hypertrophy revealed by neuroimaging. Sural nerve biopsy in one showed only moderate demyelination, axonal loss, and onion-bulb formation, illustrating dichotomy between severe proximal and milder distal nerve involvement. Patients with coexistent central and peripheral demyelination usually are symptomatic from dysfunction at one site or the other, but not from both. Our patients showed minimal response to steroids, intravenous immunoglobulin, or azathioprine. These cases suggest that the mechanism of disease in symptomatic central and peripheral demyelination may differ from that of disease in only one region, and that optimal therapy in this situation must be explored further.

Visual Evoked Potentials in Differential Diagnosis of Multiple Sclerosis and Neurobehcet's Disease

“…Patients with Behcet's disease may have axonal polyneuropathy, more prominent in the lower extremities (Birol et al 2004;Yazıcı et al 2001), while MS patients may also show signs of peripheral nervous system involvement, often as demyelination (Couratier et al 2004;Quan et al 2005). Our results were similar to the literature, but the frequency of abnormal electrophysiological findings in our study was very low.…”

Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated With Multiple Sclerosis

“…Previous reports of definite MS with definite CIDP are presented in Table 5. 6,7,[9][10][11][14][15][16][17] Electrophysiological studies in our patients revealed evidence of greater involvement of nerve roots and proximal segments of peripheral and cranial nerves than the distal components, consistent with the pathology of the disease. 19,32,34 Partial conduction block or temporal dispersion of action potentials occurred in at least one of the tested nerves in all 5 of our patients, whereas only 4 of all 18 previously reported cases did (22.2%, P = 0.008).…”

“…However, these findings were accompanied by a loss of spasticity and a persistence of extensor plantar responses. 6,7,9,11,14,17 Increased CSF protein documented in our patients is more typical in polyneuropathy, including CIDP. Previous reports of definite MS with definite CIDP are presented in Table 5.…”

“…19,32,34 Partial conduction block or temporal dispersion of action potentials occurred in at least one of the tested nerves in all 5 of our patients, whereas only 4 of all 18 previously reported cases did (22.2%, P = 0.008). 6,7,[9][10][11][14][15][16][17] Spinal root dysfunction was detected at multiple levels by DTRL measurements. DTRLs were significantly prolonged in all the patients.…”

“…[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Involvement of both PNS and CNS myelin may represent a specific subpopulation of patients with common immunopathogenetic mechanisms, including but not limited to, shared myelin antigens, common immunologic factors, endothelial alterations, and altered expression of adhesion molecules. 12 Except for a few case reports, 6,7,[9][10][11][14][15][16][17] the majority of previous publications focused on the association of disorders indistinguishable from multiple sclerosis (MS) and any type of PNS involvement. No distinction between demyelinating and axonal PNS involvement was made.…”