Lower urinary tract dysfunction in familial amyloidotic polyneuropathy, Portuguese Type

Abstract

In addition to reduced sensation, underactive detrusor, opening of the vesical neck and external sphincter deficit, we found data suggesting failure of relaxation of the internal and external sphincter. The overdistention associated with an open vesical neck and external sphincter deficit justifies incontinence in those patients. The retention is due to inadequate contraction of the detrusor, probably associated with non-relaxing of the internal and external sphincter. These dysfunctions derive from deposition of amyloid substance in the detrusor, but overdistention is likely to play a role. Early therapeutic intervention in these patients is vital to avoid secondary injuries.