ASSOCIATION OF LATE-ONSET SPASTIC PARAPARESIS AND DEMENTIA - PROBABLYAN AUTOSOMAL-DOMINANT FORM OF COMPLICATED PARAPLEGIA

The hereditary paraplegias are a heterogeneous group of genetic disord ers characterized mainly by spastic paraparesis, which may be found as an isolated ''pure form'' known as Strumpell-Lorrain syndrome, or ass ociated with a wide group of other manifestations [Harding, 1990; McKu sick, 1994], We studied two unrelated families, one with five members and the other with 11 members (over four generations), affected by a s yndrome of late onset spastic paraparesis and dementia, Both pedigrees suggest an autosomal dominant pattern of inheritance, However, this c annot be concluded definitely because male-to-male transmission was no t seen, Since this disorder has a late age of onset, we still do not k now who will become affected in the second, third, and fourth generati ons, The association of late onset spastic paraparesis and dementia, w ithout other pathological findings, has not been reported and probably represents a distinct entity. (C) 1997 Wiley-Liss, Inc.