Hemifacial spasm non-motor and motor-related symptoms and their response to botulinum toxin therapy

Abstract

Hemifacial spasm (HFS) is a chronic movement disorder which presents as clonic and/or tonic facial muscle contractions frequently accompanied by many other sensory (visual or auditory disturbances, pain), motor (facial weakness, trismus, bruxism, dysarthria) and/or autonomic (lacrimation, salivation) symptoms. The aim of the study was to assess the occurrence of HFS non-motor and motor-related symptoms and their responsiveness to botulinum toxin type A (BTX-A) therapy. 56 HFS patients were included in the open-label design study. Patients were examined three times: before BTX-A injection, and 2 and 12 weeks later. The occurrence of non-motor and motor-related symptoms was assessed by a special questionnaire, and the severity of HFS was rated by the Clinical Global Impression-Severity scale (CGI-S) and depression symptoms by the Beck Depression Inventory (BDI). Over 81% of the patients before BTX-A therapy reported HFS non-motor and motor-related symptoms. Almost 50% of the patients reported more than three symptoms. The most frequent symptoms were: tearing (44.5%), eye irritation (39.3%), facial paraesthesia (26.8%) and hearing of a "clicking" sound (25.0%). 2 weeks after BTX-A injection 75% of the patients did not report any symptoms and 20% reported only one or two. 3 months later the number of symptoms had increased again, with 57% of patients reporting at least one. The number of HFS non-motor and other symptoms did not correlate with the patients' age, disease duration and the presence of neuro-vascular conflict, but were positively correlated with the CGI-S and BDI scores. This study showed that muscle contractions in HFS patients are commonly accompanied by non-motor and other motor-related symptoms and most of them are reduced following BTX-A treatment.

Misdiagnosis of hemifacial spasm is a frequent event in the primary care setting

“…The treatment options for HFS patients include: simple massage, oral medication, microvascular decompression and regular BoNT injections, which is considered the first line treatment 1,3,5 . Medication used to HFS includes carbamazepine, clonazepam, phenytoin, gababentin and baclofen with a mild clinical response verified in other series varying from 8 to 16.6% 1,3,8 .…”

“…BoNT injections reached good/excellent results in 76-100% of patients according to previous series 1,10,18 . Misdiagnosis leads to a direct impact in QoL, social distress and impairment in activities of daily living such as driving or reading 3,5,10,11 . Nearly 50% of the patients evaluated in this series were unemployed by the time they reached the tertiary center and considered HFS as a contributing factor for this status.…”

“…Although not life threatening, the paroxystic and chronic facial contractions in HFS significantly interferes in the activities of daily living and social activities with a major impact in quality of life (QoL) 5,11,12 . Psychiatric disorders such as obsessive symptoms are also associated to HFS 13 .…”

“…Generally the muscle twitch involves initially the periocular region but as it progresses, virtually all facial muscles could be affected [3][4][5] . First described by Schültze in 1875, in a 56-year-old man with a 10-year history of involuntary movements involving the left side of the face 6 , HFS has been reported in large series of cases worldwide, the age-adjusted annual incidence was 0.78/100.000 in a study in Rochester and Olmsted CountyMinnesota 7 and the overall prevalence is about 10/100.000 1,8,9 .…”

Evaluation of concentrations of botulinum toxin A for the treatment of hemifacial spasm: a randomized double-blind crossover trial

“…Since the 1990s, BTX-A has been used for the treatment of hemifacial spasm. Local intramuscular injection of BTX-A has improved efficacy, and the improvement of clinical symptoms lasts for 3-8 months with few side effects, and thus, studies on BTX-A have increased (Rudzińska et al, 2010;Butera et al, 2013).…”