Decreased cortical somatosensory finger representation in X-linked recessive bulbospinal neuronopathy (Kennedy disease): a magnetoencephalographic study.

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.

Changing cortical excitability with low-frequency transcranial magnetic stimulation can induce sustained disruption of tactile perception.

Transcranial magnetic stimulation (TMS) is promising as a therapeutic tool, and TMS of the motor system has served as a model for regionally specific modulations of cortical excitability. It is unclear, however, to what extent response characteristics of the motor cortex are representative of other brain systems. We wanted to determine whether TMS could induce a sustained disruption of somatosensory processing beyond the stimulation duration, similar to observations in the motor system. We applied 1-Hz TMS at 110% of subjects' motor thresholds for a variable duration over the right and left somatosensory cortex before subjects performed a tactile frequency discrimination task with the left hand. Tactile discrimination was impaired only after TMS over the right somatosensory cortex (analysis of variance: p <.01). The duration of this impairment correlated with the duration of the preceding TMS; the effect lasted approximately 2 min after 5 min of TMS, 4 min after 10 min of TMS, and 8 min after 20 min of TMS. Two conclusions arise: 1) low-frequency TMS can interfere with tactile perception in a robust and sustained way, and 2) TMS dosing parameters effective in the motor system are also effective in the somatosensory system and may reflect a modality-independent response characteristic of the cerebral cortex.