2013
DOI: 10.1186/1750-1172-8-158
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Electrophysiological characterisation of motor and sensory tracts in patients with hereditary spastic paraplegia (HSP)

Abstract: BackgroundHereditary spastic paraplegias (HSPs) are characterised by lower limb spasticity due to degeneration of the corticospinal tract. We set out for an electrophysiological characterisation of motor and sensory tracts in patients with HSP.MethodsWe clinically and electrophysiologically examined a cohort of 128 patients with genetically confirmed or clinically probable HSP. Motor evoked potentials (MEPs) to arms and legs, somato-sensory evoked potentials of median and tibial nerves, and nerve conduction st… Show more

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Cited by 37 publications
(54 citation statements)
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“…TST is considered to be two or three times more sensitive than standard TMS procedures to detect corticospinal conduction deficits and is especially suited for the detection and quantification of small changes of central motor conduction 18 . A slightly delayed P40 latency of the SEP has often been reported for the LL, contrary to the SEP of the UL, which is normal 15 . …”
Section: Spg4 (Spastin)mentioning
confidence: 90%
See 1 more Smart Citation
“…TST is considered to be two or three times more sensitive than standard TMS procedures to detect corticospinal conduction deficits and is especially suited for the detection and quantification of small changes of central motor conduction 18 . A slightly delayed P40 latency of the SEP has often been reported for the LL, contrary to the SEP of the UL, which is normal 15 . …”
Section: Spg4 (Spastin)mentioning
confidence: 90%
“…Some mutation carriers can remain unaffected or very mildly affected all their lives. TMS of the UL is typically normal 15,18 and TMS of the LL is normal in up to 48% 14,15 . The axonal integrity of corticospinal projections to the UL in SPG4 patients has been investigated using the triple stimulation technique (TST) 18 and has not shown pathological changes.…”
Section: Spg4 (Spastin)mentioning
confidence: 99%
“…The majority of SPG4 cases have pure HSP with rather frequent neurogenic bladder disturbance (~70 %) and affection of the dorsal columns (~50 %). Motor evoked potentials are often normal even when there is clinically defi nite involvement of the corticospinal tracts [ 1 ]. Some cases with complicated SPG4 have been reported that feature cognitive defi cits, upper limb involvement, thin corpus callosum, or other complicating signs and symptoms.…”
Section: )mentioning
confidence: 99%
“…As a rule lower limbs are more severely and often earlier affected than the upper limbs, but electrophysiologically affection of the upper limbs can be demonstrated in about 1/3 of HSP patients [ 1 ]. HSP commonly also affects other long fi ber tracts of the spinal cord like the dorsal columns and central tracts controlling bladder function, resulting in a decrease of the vibration sense and urge incontinence or bladder voiding.…”
mentioning
confidence: 99%
“…However, upper limb affection is common in HSP and has been demonstrated neurophysiologically in >30% of cases, including genetically confirmed HSP cases. 4 As clinical criteria thus show a broad overlap and can be misleading, motor neuron diseases are increasingly classified according to their genetic basis or pathologically defined molecular background despite the abovementioned limitations of this approach. Concerning our series of HSP patients, we cannot exclude that some patients in the simplex group with unknown genetic cause may progress to PLS during their course of disease.…”
mentioning
confidence: 99%