title: Clinically silent dysfunction of dorsal columns and dorsal spinocerebellar tracts in hereditary spastic paraparesis.
abstract: Hereditary spastic paraparesis (HSP) is a neurodegenerative disorder, of which progressive spastic paraparesis is the clinical hallmark. Given the neuropathological evidence of degeneration of pyramidal tracts, dorsal columns, and dorsal spinocerebellar tracts, it is surprising that sensory symptoms are so indistinct compared to motor symptoms. We investigated the involvement of peripheral conduction and spinal proprioceptive pathways by nerve conduction studies, somatosensory evoked potentials of the median and tibial nerves, and quantitative assessment of the vibration perception thresholds of the hands and feet respectively in 32 patients suffering from HSP and healthy control groups. We did not find peripheral conduction abnormalities in HSP patients. Log-transformed vibration perception thresholds of the feet were abnormal in 13/32 HSP patients and in 0/64 controls (p < 0.00001), while tibial nerve somatosensory evoked potentials were abnormal in 20/32 patients and in 1/17 controls (p = 0.00001). The values for the upper extremities were within normal limits for nearly all subjects. In the HSP group, the neurophysiological disturbances did not correlate significantly with duration or severity of the disease, when age was controlled for, except for median nerve SSEP latency, which was affected by severity (p = 0.0072). We conclude that neurophysiological methods detected proprioceptive, subclinical abnormalities in several HSP patients, which may reflect degeneration of the dorsal columns, and/or dorsal spinocerebellar tracts. Since we found no correlation with several disease variables, the fact that not all HSP patients displayed these abnormalities may be caused by anatomical variations in proprioceptive pathways, rather than by phenotypical heterogeneity.
authors: Department of Neurology, Oudenrijn Hospital Utrecht, The Netherlands.
authors: R P
authors: RP
authors: Bruyn
authors: J G
authors: JG
authors: van Dijk
authors: P
authors: P
authors: Scheltens
authors: E H
authors: EH
authors: Boezeman
authors: B W
authors: BW
authors: Ongerboer de Visser
mesh_terms: D000200:Action Potentials; D000293:Adolescent; D000328:Adult; D000368:Aged; D005073:Evoked Potentials, Somatosensory; D005260:Female; D006181:H-Reflex; D006801:Humans; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D008875:Middle Aged; D009410:Nerve Degeneration; D009431:Neural Conduction; D011930:Reaction Time; D012684:Sensory Thresholds; D012720:Severity of Illness Index; D015419:Spastic Paraplegia, Hereditary / Q000503:physiopathology*; D013116:Spinal Cord / Q000503:physiopathology*; D013979:Tibial Nerve / Q000503:physiopathology; D013997:Time Factors; D014732:Vibration