[Urogenital alterations in hereditary and sporadic neurodegenerative ataxias]

Sporadic and hereditary ataxias (HA) represent a group of clinically and genetically heterogeneous syndromes characterized by spinocerebellar degeneration producing a motoneuron coordination disorder. In these diseases urinary and sexual symptoms are commonly associated to the neurological alterations.

To define the prevalence of functional low urinary tract symptoms in an ataxic population and to compare them with the symptomatology control a group of in healthy subjects.

An observational, descriptive, transversal study of 491 subjects recruited from the HA regional associations of Spain was conducted. In addition, a case-control study of prevalent ataxic patients and healthy subjects matched by sex and age was also performed.

Among 195 patients included, 138 (70.8 %) had Friedreich ataxia (FA) and 57 (29.2 %) non-Friedreich ataxia (nFA). Global mean age was 32.3 years in FA and 43.7 in nFA patients (p < 0.05). Combined irritative and obstructive symptoms were present in 48.7 %, only irritative in 16.4 % and obstructive in 15 % of patients. Erectile dysfunction in 30.3 % (p < 0.01) and decreased libido in 13.4 % (p < 0.01), were the most common sexual problems.

In HA, urinary symptoms are present in 80 % of patients, with mainly irritative symptoms in 2/3 of them. A complete urodynamic evaluation in symptomatic patients is recommended in order to characterize potential neurogenic vesico-urethral dysfunction. Even though sexual dysfunction may be related to neurological causes, additional etiologic organic factors should be excluded.