RESUMEN
Hansen's disease, or leprosy, is a disease characterized by dermatological and neurological disorders. A neural form also exists, in which peripheral neuropathy occurs in the absence of skin lesions. However, cases of leprosy that involve the central nervous system and proximal nerves are rare in the literature. We describe the case of an oligosymptomatic patient diagnosed with the neural form of leprosy with involvement of peripheral nerves, dorsal root ganglion, and cervical spinal cord in an atypical presentation of the disease. Through complementary examinations and nerve biopsies, the bacillus was identified, and treatment was subsequently initiated. This case highlights the importance of investigating the suspicion of leprosy, even in cases with atypical manifestations, as early diagnosis and treatment can reduce neurological damage and deformities.
RESUMEN
Introduction: Pure Neural Leprosy (PNL) is a form of this long time known disease that affects only the peripheral nervous system. Since it is a rare form of the disease, its pathophisiology is still poorly understood. Objective: Describe the cytokines profile in patients with PNL. Methods: 30 Patients diagnosed with PNL in the Souza Araujo Outpatient Clinic and with cytokines evaluated were selected. They were evaluated by neurologists and diagnosed after a nerve biopsy. Serum levels of IL-1 ß, IL-6, IL-10, IL-17, TNF, CCL-2/MCP-1, IFN-Ï, CXCL-10/IP-10 and TGF-ß were evaluates at the moment of the diagnosis. Results: Neural thickening was a common clinical finding in this groups of patients. Small and medium sensitive fibers signs and symptoms were present in 92% of the patients and motor involvement in 53%. 43% of patients presented neuropathic pain and no one had neuritis TGF-beta, IL-17, CCl-2 and IP-10. CCL-2 levels were associated with demyelinating patters and IP-10 and IL-1o were associated with axonal patterns at NCS. Discussion: PNL patients' cytokine profile appears to be different of other clinical forms of leprosy, with the presence of cytokines described in both tuberculoid and lepromatous leprosy. High levels of CCl-2 may be related to the presence of silent neuritis as well as the presence of IL-10. PNL is unique a form of leprosy, therefore, understanding its immunological profiles essential to better understand the disease itself.