Risk factors for spread of primary adult onset blepharospasm: a multicentre investigation of the Italian movement disorders study group

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 eVect 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. (J Neurol Neurosurg Psychiatry 1999;67:613-619)

Spread of primary dystonia in relation to initially affected region

“…Defazio et al [11] also did not find a significant association between spread of primary adult-onset blepharospasm and family history of dystonia.…”

“…Descendent spread lead to development of segmental dystonia in a third of our patients. Deffazio et al [11] reported further spread of dystonia in 35% of cases with blepharospasm. Green et al [4] found two times higher estimate of further dystonia spread in patients with blepharospasm (70%), also affecting respiratory muscles in 10%.…”

Menopause and Menarche in Patients with Primary Blepharospasm: An Exploratory Case-Control Study

“…This finding and the lack of correlation between age at menopause and age at BSP onset indicate that menopause probably exerts no significant influence on the age-dependent development of BSP. Likewise, it was previously shown that menopause did not affect the spread of dystonia to neighboring muscles in patients with BSP-onset cranial cervical dystonia [12]. …”

Cranial dystonia

“…Описаны редкие случаи поражения других мышц через 10 лет и более после начала заболевания [29][30][31][32].…”

“…Defazio и соавт. [29], обследовав 155 пациентов с блефароспазмом, выявили следующие предикторы даль-нейшего распространения заболевания: наличие в анамнезе черепно-мозговой травмы с нарушением сознания, возраст возникновения блефароспазма и женский пол. В другом ис-следовании значительных различий между мужчинами и женщинами в вероятности распространения дистоническо-го гиперкинеза не отмечено [28].…”