TY - CHAP M1 - Book, Section TI - Hereditary Sensory and Autonomic Neuropathy Type V (HSAN V) A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas Y1 - 2019 N1 - T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - Group of genetically inherited disorders that share the primary feature of progressive, generally severe, lower extremity spasticity. Type V is defined as medical condition. It is associated with congenital insensitivity to pain with partial anhidrosis. However, it is also possible to observe this disease in presence of anhidrosis. The signs and symptoms of HSAN5 appear early, usually at birth or during infancy. Characteristically, individuals loose the perception of deep pain, absence of pain with bone injuries, ligaments, or muscles. The clinical presentation of these patients is repeated severe injuries that go unnoticed. Repeated trauma is eventually associated with destruction of the articulations, a condition called Charcot joints. It is differentiated from HSAN4 by the absence of myelinated sensory fibers on large conducting nervous structures. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/10 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164072760 ER -