title: Decreased cortical somatosensory finger representation in X-linked recessive bulbospinal neuronopathy (Kennedy disease): a magnetoencephalographic study.
abstract: BACKGROUND
  Kennedy disease (KD) clinically presents as progressive lower motor neuron disease with minimal or no sensory impairment. However, electrophysiological studies found abnormal somatosensory-evoked potentials even in absence of clinical deficits. Little is known about possible influences of this sensory neuropathy on the central somatosensory processing.
  
  
  METHODS
  In this study, the cortical topography of index finger representation was studied in 7 patients with genetically proven KD compared to healthy control subjects by means of magnetoencephalography using an established stimulation paradigm. Data analysis was carried out with synthetic aperture magnetometry (SAM). Additionally, the latency and source amplitude of the earliest cortical somatosensory-evoked field (SEF) component were determined based on traditional single dipole source analysis.
  
  
  RESULTS
  In KD patients the latency of the SEF was prolonged (48.6 vs. 37.4 ms, P < .005). There was no significant difference in dipole source amplitude, but stimulus-related SAM activation of the contralateral sensorimotor cortex (pseudo-t-values -.107 vs. -.199, P < .05), including maximum activity (53.5%), was reduced.
  
  
  CONCLUSIONS
  These results implicate that even subclinical sensory neuropathy leads to possible functional reorganization of the sensorimotor cortex in KD patients and reinforces the view that in KD the somatosensory system is extensively involved.
authors.affiliation: Department of Neurology, University of Muenster, Muenster, Germany.
authors.forename: Sonja
authors.initials: S
authors.lastname: Suntrup
authors.forename: Inga
authors.initials: I
authors.lastname: Kristina Teismann
authors.forename: Olaf
authors.initials: O
authors.lastname: Steinstraeter
authors.forename: Erich
authors.initials: E
authors.lastname: Bernd Ringelstein
authors.forename: Christo
authors.initials: C
authors.lastname: Pantev
authors.forename: Rainer
authors.initials: R
authors.lastname: Dziewas
mesh_terms: D000328:Adult; D000368:Aged; D001921:Brain / Q000473:pathology / Q000503:physiopathology*; D055534:Bulbo-Spinal Atrophy, X-Linked / Q000473:pathology / Q000503:physiopathology*; D016022:Case-Control Studies; D005073:Evoked Potentials, Somatosensory; D005385:Fingers / Q000502:physiology*; D007839:Functional Laterality; D006801:Humans; D008279:Magnetic Resonance Imaging; D015225:Magnetoencephalography / Q000379:methods; D008297:Male; D008875:Middle Aged; D010812:Physical Stimulation; D012815:Signal Processing, Computer-Assisted; D013003:Somatosensory Cortex / Q000473:pathology / Q000503:physiopathology*; D013997:Time Factors