title: Is there involvement of the central nervous system in hereditary sensory radicular neuropathy?
abstract: There is pathological evidence that hereditary sensory radicular neuropathy (HSN type I) is a disorder related to multi-system atrophy with marked cell loss in the cerebral cortex, thalamus, brain stem and cerebellum. We report here a clinical study of a case of HSN-I including audiometric testing, autonomic functions, electromyography, transcranial magnetic stimulation and magnetic resonance imaging of the brain. There were no signs of central nervous system involvement. It is stated that HSN-I remains a disorder of dorsal root ganglia and sensory nerves, leading to painless perforating ulceration and mutilation, within the course of the disease peripheral motor nerve involvement, but without involvement of central motor pathways.
authors.affiliation: Department of Neurology, Hospital Enschede, The Netherlands.
authors.forename: G
authors.initials: G
authors.lastname: Hageman
authors.forename: B G
authors.initials: BG
authors.lastname: Hilhorst
authors.forename: A R
authors.initials: AR
authors.lastname: Rozeboom
mesh_terms: D001921:Brain / Q000503:physiopathology*; D004574:Electromagnetic Fields; D004576:Electromyography; D005260:Female; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000175:diagnosis / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D009119:Muscle Contraction / Q000502:physiology; D009460:Neurologic Examination; D011930:Reaction Time / Q000502:physiology; D013116:Spinal Cord / Q000503:physiopathology; D009435:Synaptic Transmission / Q000502:physiology