title: Beneficial use of steroids in hereditary neuropathy with liability to pressure palsy.
abstract: Management of hereditary neuropathy with liability to pressure palsy (HNPP) is primarily conservative, aimed at preventing nerve injury by avoiding trauma or other potential aggravating factors. No pharmacological treatment is known to be beneficial. We describe two adolescents, one with HNPP (male; aged 15y) and another with a clinical picture suggestive of HNPP (genetically unconfirmed; female; aged 14y), who showed considerable improvement of their symptoms after receiving corticosteroid therapy. Both individuals were symptomatic for at least 5 months before the treatment. Following corticosteroids, both individuals demonstrated rapid improvement leading to near-complete recovery of muscle power. Clinical improvement after corticosteroid therapy has been reported in some individuals with other hereditary neuropathies. Our cases demonstrate that corticosteroid therapy may also be beneficial in individuals with HNPP who have a protracted or incomplete course of recovery.
authors.affiliation: Department of Paediatric Neurology, Evelina Children's Hospital, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, UK.
authors.forename: Hock S
authors.initials: HS
authors.lastname: Heng
authors.forename: Shan S
authors.initials: SS
authors.lastname: Tang
authors.forename: Sushma
authors.initials: S
authors.lastname: Goyal
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Wraige
authors.forename: Ming J
authors.initials: MJ
authors.lastname: Lim
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D005260:Female; D007839:Functional Laterality; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009431:Neural Conduction / Q000502:physiology; D010243:Paralysis / Q000188:drug therapy* / Q000503:physiopathology; D010523:Peripheral Nervous System Diseases / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D011312:Pressure / Q000009:adverse effects*; D013116:Spinal Cord / Q000473:pathology
chemical_list: D000305:Adrenal Cortex Hormones