Two patients suffering from the stationary form of hereditary spastic paraplegia,
father and son, are described. While in the father the disease - probably because
of congenital club-feet - led to flexion contractures of the hip- and knee-joints,
the son in contrary showed extreme genua recurvata. In spite of these contradictionary
findings, neurological examination of both patients led to the diagnosis of stationary
form of hereditary spastic paraplegia.
Both patients had normal latencies of their somatosensory evoked peroneal cortical
potentials (Peron-SEP). It is known that the progredient form of the disease leads
to alterations of the Peron-SEP. So it is the opinion of the authors that this electrodiagnostic
aspect might allow to distinguish between the "pure" and the progredient form of the
disease in less clear cases, if these findings can be confirmed in other sibships
with the "pure" form of hereditary spastic paraplegia.
Somatosensory evoked potentials - Hereditary spastic paraplegia - Knee-flexion contractures
- Genua recurvata
