Risk factors for spread of primary adult onset blepharospasm: a multicentre investigation of the Italian movement disorders study group
- Giovanni Defazioa,
- Alfredo Berardellib,
- Giovanni Abbruzzesec,
- Vincenzo Coviellod,
- Francesco Carellae,
- Maria T De Berardinisf,
- Giuseppe Galardig,
- Paolo Girlandah,
- Silvio Maurrii,
- Marco Mucchiutj,
- Alberto Albanesek,
- Mario Bascianil,
- Laura Bertolasim,
- Rocco Liguorin,
- Nicola Tambascoo,
- Lucio Santorop,
- Giorgio Assennatod,
- Paolo Livreaa
- aDepartment of Neurological and Psychiatric Sciences, University of Bari, Italy, bDepartment of Neurology, and Neuromed Institute, University of Rome La Sapienza, Italy, cInstitute of Neurology, University of Genova, Italy, dCIMEDOC, University of Bari, Italy, eIstituto Nazionale Neurologico C Besta, Milan, Italy, fInstitute of Ophtalmology, University of Naples, Italy, gInstitute of Neurology, S. Raffaele Hospital, Milan, Italy, h2nd Neurologic Clinic, University of Messina, Italy, i2nd Neurologic Clinic, University of Firenze, Italy, jInstitute of Neurology, University of Trieste, Italy, kInstitute of Neurology, Università Cattolica del Sacro Cuore, Rome, Italy, lDepartment of Neurology, Casa Sollievo della Sofferenza, S Giovanni Rotondo, Italy, mInstitute of Neurology, University of Verona, Italy , nInstitute of Neurology, University of Bologna, Italy, oInstitute of Neurology, University of Perugia, Italy, p2nd Neurologic Clinic, University of Naples, Italy
- Dr Giovanni Defazio, Department of Neurological and Psychiatric Sciences, Piazza Giulio Cesare 11, I-70124 Bari, Italy. Telephone 0039 80 5478511; fax 0039 80 5478532.
- Received 14 September 1998
- Revised 13 January 1999
- Accepted 26 March 1999
Abstract
OBJECTIVES Little is known about factors influencing the spread of blepharospasm to other body parts. An investigation was carried out to deterrmine whether demographic features (sex, age at blepharospasm onset), putative risk, or protective factors for blepharospasm (family history of dystonia or tremor, previous head or face trauma with loss of consciousness, ocular diseases, and cigarette smoking), age related diseases (diabetes, hypertension), edentulousness, and neck or trunk trauma preceding the onset of blepharospasm could distinguish patients with blepharospasm who had spread of dystonia from those who did not.
METHODS 159 outpatients presenting initially with blepharospasm were selected in 16 Italian Institutions. There were 104 patients with focal blepharospasm (mean duration of disease 5.3 (SD 1.9) years) and 55 patients in whom segmental or multifocal dystonia developed (mainly in the cranial cervical area) 1.5 (1.2) years after the onset of blepharospasm. Information was obtained from a standardised questionnaire administered by medical interviewers. A Cox regression model was used to examine the relation between the investigated variables and spread.
RESULTS Previous head or face trauma with loss of consciousness, age at the onset of blepharospasm, and female sex were independently associated with an increased risk of spread. A significant association was not found between spread of dystonia and previous ocular diseases, hypertension, diabetes, neck or trunk trauma, edentulousness, cigarette smoking, and family history of dystonia or tremor. An unsatisfactory study power negatively influenced the validity and accuracy of the negative findings relative to diabetes, neck or trunk trauma, and cigarette smoking.
CONCLUSIONS The results of this exploratory study confirm that patients presenting initially with blepharospasm are most likely to experience some spread of dystonia within a few years of the onset of blepharospasm and suggest that head or face trauma with loss of consciousness preceding the onset, age at onset, and female sex may be relevant to spread. The suggested association between edentulousness and cranial cervical dystonia may be apparent because of the confounding effect of both age at onset and head or face trauma with loss of consciousness. The lack of influence of family history of dystonia on spread is consistent with previous findings indicating that the inheritance pattern is the same for focal and segmental blepharospasm.
