Clinical characteristics of fibroblast growth factor receptor 3 antibody-related polyneuropathy: a retrospective study.

BACKGROUND AND PURPOSE: Autoantibodies are increasingly being used as a diagnostic biomarker of chronic inflammatory neuropathies. However, their role and associated clinical syndrome are not well defined. METHODS: This retrospective chart review evaluated the clinical presentation, diagnostic workup and therapeutic responses in fibroblast growth factor receptor 3 (FGFR3) antibody-associated neuropathy. RESULTS: A total of 27 patients [14 men, aged 29-87 (65 ± 14) years] with positive FGFR3 antibody were included. Distal lower-extremity paresthesia (66%), unsteady gait (26%) and foot drop (11%) were common presenting symptoms. Symptom onset was acute in four (15%) cases. Distal lower-extremity weakness (mild in eight and severe in three patients) was the most frequent motor finding. Decreased distal sensation to pinprick (59%) and loss of vibration sensation (37%) were observed. Titer of FGFR3 ranged between 3100 and 30 000 (normal < 3000) with a mean of 10 688 ± 7284. Apart from the occasional association of other neuropathy-related autoantibodies, comprehensive neuropathy workup was otherwise unrevealing. Six patients had other autoimmune disease and seven patients had a history of cancer. Electromyography reflected sensorimotor neuropathy with mixed axonal and demyelinating features in 11 cases. Pure sensory neuropathy was noted in three patients. Demyelination was found in five of six nerve biopsies. Intravenous immunoglobulin response was observed in 8/10 treated patients. CONCLUSIONS: The FGFR3 antibody appears not to be restricted to sensory neuropathy only. Its role in the pathogenicity of chronic inflammatory neuropathies is not yet well established and, although there may be a role for immunotherapy, larger studies are warranted.

Quantification and localization of mast cells in periapical lesions.

BACKGROUND: Periapical lesions occur in response to chronic irritation in periapical tissue, generally resulting from an infected root canal. Specific etiological agents of induction, participating cell population and growth factors associated with maintenance and resolution of periapical lesions are incompletely understood. Among the cells found in periapical lesions, mast cells have been implicated in the inflammatory mechanism. AIM: Quantifications and the possible role played by mast cells in the periapical granuloma and radicular cyst. Hence, this study is to emphasize the presence (localization) and quantification of mast cells in periapical granuloma and radicular cyst. MATERIALS AND METHODS: A total of 30 cases and out of which 15 of periapical granuloma and 15 radicular cyst, each along with the case details from the previously diagnosed cases in the department of oral pathology were selected for the study. The gender distribution showed male 8 (53.3%) and females 7 (46.7%) in periapical granuloma cases and male 10 (66.7%) and females 5 (33.3%) in radicular cyst cases. The statistical analysis used was unpaired t-test. RESULTS: Mean mast cell count in periapical granuloma subepithelial and deeper connective tissue, was 12.40 (0.99%) and 7.13 (0.83%), respectively. The mean mast cell counts in subepithelial and deeper connective tissue of radicular cyst were 17.64 (1.59%) and 12.06 (1.33%) respectively, which was statistically significant. No statistical significant difference was noted among males and females. CONCLUSION: Mast cells were more in number in radicular cyst. Based on the concept that mast cells play a critical role in the induction of inflammation, it is logical to use therapeutic agents to alter mast cell function and secretion, to thwart inflammation at its earliest phases. These findings may suggest the possible role of mast cells in the pathogenesis of periapical lesions.