Skurcz połowiczy twarzy – Epidemiologia

Skurcz połowiczy twarzy
Epidemiologia

Skurcz połowiczy twarzy (HFS) to rzadkie schorzenie neuromięśniowe o częstości występowania około 10-11 na 100 000 osób, z wyraźną przewagą kobiet (stosunek 2:1) i średnim wiekiem zachorowania 51-54 lata. Choroba częściej dotyczy lewej strony twarzy i populacji azjatyckiej. Etiologia dzieli się na pierwotną (77-79% przypadków, związana z uciskiem nerwu twarzowego przez naczynie) oraz wtórną (21-23%, wynikającą z uszkodzeń nerwu). Współistniejące nadciśnienie tętnicze występuje u około 40% pacjentów, a skurcze utrzymują się podczas snu, co może prowadzić do zaburzeń snu. Diagnostyka opiera się na ocenie klinicznej, MRI i badaniach elektrofizjologicznych, a Hemifacial Spasm Grading Scale (HSGS) jest rekomendowaną skalą oceny nasilenia objawów. Częstość błędnej diagnozy w podstawowej opiece zdrowotnej jest wysoka, co wydłuża czas do prawidłowego rozpoznania średnio do 8 lat.

Epidemiologia skurczu połowiczy twarzy

Skurcz połowiczy twarzy (ang. hemifacial spasm, HFS) jest rzadkim schorzeniem neuromięśniowym charakteryzującym się krótkimi lub długotrwałymi, mimowolnymi skurczami mięśni unerwionych przez nerw twarzowy. Analiza danych epidemiologicznych pozwala lepiej zrozumieć skalę występowania tego zaburzenia oraz jego charakterystykę demograficzną.¹²

Częstotliwość występowania

Skurcz połowiczy twarzy jest schorzeniem stosunkowo rzadkim. Według większości badań, średnia częstość występowania na świecie wynosi około 10-11 przypadków na 100 000 osób. Dane z hrabstwa Olmsted w stanie Minnesota (USA) z lat 1960-1984 wskazują, że średnia roczna zapadalność wynosiła 0,81 na 100 000 u kobiet i 0,74 na 100 000 u mężczyzn.¹²

W ujęciu globalnym rozpowszechnienie skurczu połowiczy twarzy szacuje się na 14,5 na 100 000 u kobiet i 7,4 na 100 000 u mężczyzn, co wskazuje na dwukrotnie częstsze występowanie choroby u kobiet niż u mężczyzn.¹² W Niemczech szacowana liczba chorych wynosi 8000-9000 osób. Badania z Oslo w Norwegii wykazały podobne wyniki w zakresie ogólnej częstości występowania, choć z różnicami w rozkładzie wiekowym pacjentów.¹

W Wielkiej Brytanii liczbę osób z HFS ocenia się na około 4000. W Szwajcarii, przy założeniu częstości występowania 10 na 100 000 osób, szacuje się, że na skurcz połowiczy twarzy cierpi około 800 osób.¹²

Rozkład demograficzny

Analiza danych epidemiologicznych wskazuje na wyraźne tendencje w rozkładzie demograficznym skurczu połowiczy twarzy:

Płeć: Stosunek kobiet do mężczyzn wynosi około 2:1. Dane wskazują, że około 60-70% wszystkich przypadków dotyczy kobiet.¹²
Wiek: Choroba zwykle rozpoczyna się w wieku dorosłym, najczęściej między 40. a 60. rokiem życia. Średni wiek w momencie wystąpienia objawów wynosi 51-54 lata.¹²
Rasa/pochodzenie etniczne: Badania sugerują, że HFS występuje częściej w populacji azjatyckiej w porównaniu do populacji kaukaskiej, choć przyczyny tej różnicy nie są do końca poznane.¹²
Strona ciała: Skurcz połowiczy twarzy częściej dotyczy lewej strony twarzy (stosunek 2:1 w stosunku do prawej strony), co zaobserwowano w większości opisywanych przypadków.¹²³

Wiek zachorowania i rozkład w populacji

Szczegółowa analiza rozkładu wiekowego pacjentów ze skurczem połowiczy twarzy wskazuje na interesujące tendencje. W badaniu amerykańskim największe rozpowszechnienie odnotowano w przedziale wieku 40-59 lat, podczas gdy badanie norweskie wykazało ciągły wzrost częstości występowania aż do maksimum 39,7 na 100 000 u osób powyżej 70. roku życia.¹²

Tylko 1-6% wszystkich pacjentów ze skurczem połowiczy twarzy doświadcza objawów przed 30. rokiem życia. Wystąpienie HFS u pacjentów poniżej 40. roku życia jest nietypowe i często zwiastuje podstawową chorobę neurologiczną, np. stwardnienie rozsiane.¹²³

Typy i przyczyny skurczu połowiczy twarzy

Na podstawie przyczyn występowania, skurcz połowiczy twarzy można podzielić na dwa główne typy:¹²

Pierwotny skurcz połowiczy twarzy (około 77-79% przypadków) – spowodowany uciskiem nerwu twarzowego przy wyjściu z pnia mózgu, najczęściej przez naczynie krwionośne.
Wtórny skurcz połowiczy twarzy (około 21-23% przypadków) – będący wynikiem obwodowego porażenia nerwu twarzowego lub uszkodzenia nerwu spowodowanego guzami, demielinizacją, urazem lub infekcją.

Jedno z badań obejmujące 2050 pacjentów ze skurczem połowiczy twarzy z lat 1986-2009 wykazało, że tylko dziewięć przypadków było spowodowanych zespołem kąta mostowo-móżdżkowego, co stanowi zaledwie 0,44% przypadków.¹

Związki z innymi schorzeniami

Badania wskazują na interesujące korelacje między skurczem połowiczy twarzy a innymi schorzeniami:¹²

Nadciśnienie tętnicze: U około 40% pacjentów z HFS współistnieje nadciśnienie tętnicze. Metaanalizy potwierdzają pozytywną korelację między nadciśnieniem a skurczem połowiczy twarzy. W jednym z badań 74% pacjentów z HFS miało nadciśnienie.¹²³
Zaburzenia snu: W przeciwieństwie do innych zaburzeń ruchowych, skurcze utrzymują się podczas snu, co może przyczyniać się do zaburzeń snu i bezsenności. Badania z wykorzystaniem elektromiografii twarzy (EMG) i elektrokardiogramu (EKG) w nocy wykazały, że stany czuwania lub lekkiego snu częściej towarzyszą objawom HFS.¹

Genetyka i przypadki rodzinne

Większość przypadków skurczu połowiczy twarzy występuje sporadycznie. Skurcz połowiczy twarzy rzadko ma charakter rodzinny – w literaturze naukowej opisano jedynie pojedyncze przypadki rodzinnego występowania HFS. Badania nie wykazały związku genetycznego dla HFS, co oznacza, że schorzenie to nie jest dziedziczone.¹²³

Występowanie obustronnego skurczu połowiczy twarzy

Skurcz połowiczy twarzy typowo występuje tylko po jednej stronie twarzy, co odzwierciedla termin „połowiczy” w nazwie schorzenia. Przypadki obustronnego skurczu połowiczy twarzy są rzadkie, a częstość ich występowania szacuje się na około 2,6% wszystkich przypadków.¹²

Wyzwania w diagnostyce i nadzorze

Pomimo że skurcz połowiczy twarzy jest chorobą o wyraźnie zdefiniowanych objawach klinicznych, jego diagnoza nierzadko stanowi wyzwanie dla lekarzy, zwłaszcza w podstawowej opiece zdrowotnej.¹

Problemy z diagnostyką

Badanie przeprowadzone na grupie 66 pacjentów wykazało, że żaden z nich nie otrzymał prawidłowego rozpoznania skurczu połowiczy twarzy jako pierwszej diagnozy. Wszyscy pacjenci byli najpierw badani przez lekarzy rodzinnych lub lekarzy ogólnych. Co zaskakujące, 43,9% pacjentów nie otrzymało nawet diagnozy różnicowej w podstawowej opiece zdrowotnej. Wśród tych, którzy otrzymali jakąś diagnozę, najczęstsze hipotezy obejmowały:¹²

Zaburzenia czynnościowe (lub psychogenne) – 37,8%
Porażenie nerwu twarzowego – 9%
Tiki – 3%
Inne różnorodne schorzenia

Szacuje się, że pacjenci doświadczają objawów średnio przez około 8 lat, zanim otrzymają prawidłową diagnozę, która może ukierunkować leczenie.¹²

Wykorzystanie badań diagnostycznych

Diagnoza skurczu połowiczy twarzy opiera się głównie na ocenie klinicznej, jednak w celu wykluczenia innych schorzeń, które mogą powodować podobne objawy, stosuje się różne metody diagnostyczne:¹²

Rezonans magnetyczny (MRI) – powinien być wykonany w celu wykluczenia guzów, innych nieprawidłowości strukturalnych i stwardnienia rozsianego. MRI może również zazwyczaj wykryć nieprawidłową pętlę tętnicy uciskającą nerw.
Badania elektrofizjologiczne – są wymagane do oceny innych patologii kąta mostowo-móżdżkowego.

Wśród dostępnych skal klinicznych, Hemifacial Spasm Grading Scale (HSGS) jest uważana za obiektywne, szybkie i niezawodne narzędzie do oceny HFS.¹

Wpływ nieprawidłowej diagnozy na jakość życia

Błędna diagnoza prowadzi do bezpośredniego wpływu na jakość życia, stresu społecznego i upośledzenia w codziennych czynnościach, takich jak prowadzenie pojazdu czy czytanie. Prawie 50% pacjentów ocenianych w jednym z badań było bezrobotnych w momencie dotarcia do ośrodka trzeciego poziomu referencyjnego i uważało skurcz połowiczy twarzy za czynnik przyczyniający się do tego stanu.¹

Chociaż skurcz połowiczy twarzy zwykle nie zagraża życiu, może znacząco wpływać na jakość życia pacjenta ze względu na związany z nim stres psychospołeczny. Skurcz połowiczy twarzy jest mimowolny i w wielu przypadkach prowadzi do lęku i zawstydzenia społecznego.¹

Nadzór i monitorowanie

Ze względu na wykazaną korelację między nadciśnieniem tętniczym a skurczem połowiczy twarzy, zaleca się regularne monitorowanie ciśnienia krwi podczas wizyt kontrolnych w celu wczesnego wykrycia i interwencji w przypadku rozwoju nadciśnienia.¹²

Przyszłe badania powinny skupić się na ocenie, czy kontrola nadciśnienia może pomóc w zarządzaniu HFS. Obecnie badania kliniczne dotyczące HFS i nadciśnienia mają pewne ograniczenia, ale metaanaliza przeprowadzona w tej dziedzinie podkreśla pozytywną korelację między tymi dwoma schorzeniami.¹

Różnice regionalne w leczeniu

Interesującym aspektem nadzoru epidemiologicznego nad skurczem połowiczy twarzy są różnice regionalne w dostępie do leczenia, szczególnie do dekompresji mikronaczyniowej (MVD), która jest uznawana za najbezpieczniejszą i najskuteczniejszą metodę leczenia tego schorzenia.¹

W Ameryce Północnej szacuje się, że wykorzystanie MVD w leczeniu HFS stanowi zaledwie 10% częstości występowania choroby. Obecny poziom prawdopodobnie nie jest wystarczający do odpowiedniego leczenia populacji z HFS. Skorygowane wskaźniki wykorzystania MVD w leczeniu HFS w poszczególnych prowincjach kanadyjskich wykazały dramatyczną dysproporcję geograficzną, wahającą się od 0,04 do 0,62 na 100 000 osób rocznie.¹

Postępy w monitorowaniu i leczeniu

W ostatnich latach nastąpił znaczący postęp w metodach monitorowania i leczenia skurczu połowiczy twarzy, co przyczyniło się do lepszego zrozumienia patofizjologii tego schorzenia oraz poprawy wyników leczenia.¹

Innowacje w monitorowaniu śródoperacyjnym

Od czasu gdy Møller i Jannetta po raz pierwszy przeprowadzili zapisy elektrofizjologiczne nerwów i mięśni twarzowych podczas dekompresji mikronaczyniowej (MVD) w 1984 roku, zastosowano różne metodologie śródoperacyjnego monitorowania neurofizjologicznego (ION), w tym:¹

Potencjały wywołane pnia mózgu (BAEPs)
Odpowiedź rozprzestrzeniająca się bocznie (LSR)
Odpowiedź Z-L (ZLR)
Odruch mrugania (BR)
Korowo-opuszkowe potencjały ruchowe twarzy (FCoMEPs)

Wiele grup badawczych wykorzystało monitorowanie śródoperacyjne, aby zapewnić głębszy wgląd w patologiczne mechanizmy HFS i przeanalizować wartość predykcyjną danych ION dla długoterminowych neurologicznych wyników pooperacyjnych.¹²

Skuteczność metod leczenia

Obecnie dostępnych jest kilka opcji leczenia skurczu połowiczy twarzy, z których każda ma swoje zalety i ograniczenia:¹²

Leki – najczęściej stosowane to karbamazepina, fenytoina, baklofen i klonazepam.
Toksyna botulinowa typu A – uważana za leczenie pierwszego wyboru. Ogólnie 76-100% pacjentów osiąga co najmniej 75% poprawę, przy typowym czasie trwania odpowiedzi od 3 do 4 miesięcy.
Dekompresja mikronaczyniowa (MVD) – metoda o najwyższych długoterminowych wskaźnikach wyleczenia. Niedawna metaanaliza wykazała, że ogólny wskaźnik ustąpienia skurczów po MVD wynosił 90,5% przy ostatniej kontroli po 1,25 ± 0,04 roku.

Warto zauważyć, że pomimo ograniczonych danych z wysokiej jakości badań klinicznych, toksyna botulinowa typu A jest uważana za leczenie z wyboru dla pacjentów z HFS.¹

Współpraca interdyscyplinarna

Zespół interdyscyplinarny jest niezbędny do optymalnej opieki nad pacjentami ze skurczem połowiczy twarzy. Współpraca w zakresie obrazowania MRI i radiologicznego jest ważna w identyfikacji przyczyny. Ponadto pacjenci są współprowadzeni przez specjalistów okuloplastyki, otolaryngologii i neurologii w zakresie długoterminowego leczenia i zarządzania.¹

Dokładna diagnoza, edukacja pacjenta i terminowe zarządzanie są kluczowymi elementami poprawy jakości życia pacjentów ze skurczem połowiczy twarzy.¹

Wyniki leczenia chirurgicznego

Dekompresja mikronaczyniowa pozostaje najbezpieczniejszą i najskuteczniejszą metodą leczenia skurczu połowiczy twarzy, z wskaźnikiem powodzenia 80-100% opisywanym na całym świecie. Główne obszary zainteresowania dla operującego neurochirurga to utrata słuchu czuciowo-nerwowa i niepowodzenie procedury w łagodzeniu skurczu.¹

W jednym z badań wskaźnik wyleczenia wynoszący 95% porównuje się korzystnie z danymi z istniejącej literatury, które wahają się od 90 do 98%. W metabadaniu obejmującym 22 badania i dane od łącznie 5685 pacjentów, 91,1% wszystkich pacjentów uznano za wyleczonych po medianie okresu obserwacji wynoszącej prawie 3 lata. Nawroty wystąpiły tylko u 2,4% pacjentów, którzy przeszli pierwotną udaną operację.¹²

W serii 194 pacjentów, 30 pacjentów (15,5%) miało złe wyniki (w tym 1 zgon) po pierwszej operacji, co jest porównywalne z wynikami innych badań. Obecne raporty dokumentują zmienny wskaźnik niepowodzeń po pierwszej operacji, który waha się od mniej niż 15% do wysokości 22,7%.¹

Potrzebne są dodatkowe badania, aby w pełni zrozumieć naturę HFS, odpowiedź pacjentów na różne opcje leczenia i rozwój nawrotów. Jak dotąd, dekompresja mikronaczyniowa oferuje najlepszy długoterminowy wynik leczenia HFS, z niską chorobowością i śmiertelnością.¹

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Materiały źródłowe

#1 Hemifacial Spasm (12.10.2012)
https://di.aerzteblatt.de/int/archive/article/131637
Hemifacial spasm is a neuromuscular movement disorder characterized by brief or persistent involuntary contractions of the muscles innervated by the facial nerve. Its prevalence has been estimated at 11 cases per 100 000 individuals. […] Few epidemiological data are available for the pathological entity that is hemifacial spasm. One study evaluated data from patients in Olmsted County, Minnesota, from 1960 through 1984. The mean annual incidence was 0.81 per 100 000 in women and 0.74 per 100 000 in men. The mean prevalence was 11 per 100 000 in the total population. Women developed the disorder at a rate of 14.5 per 100 000 and men at 7.4 per 100 000. The distribution by sex is thus 2:1. In Germany, the estimated prevalence is 8000 to 9000 persons. […] A study from Oslo, Norway, showed comparable results. Differences were, however, seen in the age distribution of the patients. In the US study the age peak with the highest prevalence was between 40 and 59 years, whereas the Norwegian study showed a continual increase in the prevalence to a maximum of 39.7 per 100 000 in those older than 70. The mean age at disease onset in the Norwegian study was 54. Only 1% to 6% of all patients with hemifacial spasm before age 30. In our own group of patients, too, more women than men developed the disease (women 60%, men 40%). […] Familial clustering is rare for this condition. The prevalence of bilateral hemifacial spasm has been reported to be 2.6% of cases. None of our own patients had bilateral symptoms.
#1 Hemifacial Spasm – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK526108/
HFS is a rare condition, as the estimated worldwide prevalence of HFS is 14.5 per 100,000 women and 7.4 per 100,000 men suggesting women are twice as prone to have HFS than men. […] Most cases of HFS are sporadic; however, there are some rare case reports of familial HFS in the scientific literature. […] The disease onset, especially in primary HFS, usually begins in adulthood, around the fourth through sixth decades of life. This condition commonly involves the left side more than the right, as observed in most reported cases. Researchers have reported that 40% of patients with HFS have coexisting hypertension.
#1 Hemifacial Spasm: Causes, Symptoms, and Treatment
https://patient.info/bones-joints-muscles/hemifacial-spasm-leaflet
Hemifacial spasm is a rare condition. In the UK there are thought to be about 4,000 people with hemifacial spasm. […] Symptoms usually start in middle age, the average age of onset being 45 to 52 years old.
#1 Hemifacial spasm | MedLink Neurology
https://www.medlink.com/articles/hemifacial-spasm
Data from Olmsted County, Minnesota suggest that the average age-adjusted annual incidence of hemifacial spasm for all ages is 0.78 per 100,000 (0.81 per 100,000 in women and 0.74 per 100,000 in men). The prevalence is 14.5 per 100,000 in women and 7.4 per 100,000 in men. […] Hemifacial spasm has been found to be more common in the Asian population than in other populations. […] Women account for about 60% of cases.
#1 Botulinum Toxin for the Treatment of Hemifacial Spasm: An Update on Clinical Studies
https://www.mdpi.com/2072-6651/13/12/881
Hemifacial spasm (HFS) is a hyperkinetic movement disorder characterized by short or persistent, intermittent synchronous twitching of the muscles innervated by the facial nerve, which is a chronic condition, and spontaneous recovery is rare. […] The mean prevalence of the disorder is around 10 in 100,000 (14.5 and 7.4 per 100,000 in females and males, respectively). […] Currently, HFS is classified as primary (79%) or secondary to facial nerve damage (21%). […] The average age at onset of primary HFS ranges from the fifth to sixth decades of life. […] HFS is commonly sporadic, with a few familial cases having been reported. […] Diagnosis of HFS is mainly based on clinical recognition. […] The distinction between primary or secondary HFS is fundamental to properly direct the treatment strategy.
#1 Hemifacial Spasm | Treatment & Management | Point of Care
https://www.statpearls.com/point-of-care/22719
HFS is a rare condition, as the estimated worldwide prevalence of HFS is 14.5 per 100,000 women and 7.4 per 100,000 men suggesting women are twice as prone to have HFS than men. […] As reported in some studies, those in the Asian population have a slightly higher prevalence as compared to Caucasian populations for unknown reasons. […] Most cases of HFS are sporadic; however, there are some rare case reports of familial HFS in the scientific literature. […] The disease onset, especially in primary HFS, usually begins in adulthood, around the fourth through sixth decades of life. […] Researchers have reported that 40% of patients with HFS have coexisting hypertension.
#1 Hemifacial spasm – Wikipedia
https://en.wikipedia.org/wiki/Hemifacial_spasm
The incidence of hemifacial spasm is approximately 0.8 per 100,000 persons. […] Hemifacial spasm is more prevalent among females over 40 years of age. […] The estimated prevalence for women is 14.5 per 100,000 and 7.4 per 100,000 in men. […] Prevalence for hemifacial spasm increases with age, reaching 39.7 per 100,000 for those aged 70 years and older. […] One study divided 214 hemifacial patients based on the cause of the disease: primary hemifacial spasm, where the patients had a compression in the facial nerve at the end of the brainstem; and secondary hemifacial spasm, where the patients had peripheral facial palsy or nerve lesion due to tumors, demyelination, trauma or infection. […] The study found that 77% of cases were primary and 23% secondary. […] The study also found that the two sets of patients shared a similar age at onset, male to female ratios, and similar affected side. […] In another study of 2050 patients who had presented with hemifacial spasm between 1986 and 2009, only nine cases were caused by a cerebellopontine angle syndrome, an incidence of 0.44%.
#1 Revisiting the link between hypertension and hemifacial spasm | Scientific Reports
https://www.nature.com/articles/srep21082
The prevalence of hypertension was higher in HFS patients as compared to non-HFS patients. […] This meta-analysis highlights a positive correlation between hypertension and HFS. […] Blood pressure should be closely monitored during the follow-up of HFS patients. […] The results of the meta-analysis showed that there is indeed a higher prevalence of hypertension among HFS patients as compared to non-HFS controls. […] Therefore, regular blood pressure monitoring should be performed during follow-up visits for early detection and intervention of hypertension should it develop. […] Future studies should address if controlling hypertension can help in managing HFS. […] Current case-control studies in HFS and hypertension have limitations. […] In conclusion, this meta-analysis highlights a positive correlation between hypertension and HFS. Blood pressure should be closely monitored during the follow-up of HFS patients.
#1 Hemifacial spasm is not affected by state of consciousness: a case report | European Journal of Medical Research | Full Text
https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-021-00616-5
Hemifacial spasm (HFS) is a movement disorder caused by mechanical compression of the facial nerve after it has left the brainstem and is characterized by brief or sustained twitching of the muscles innervated by that nerve. […] To our knowledge, there were no reports on how HFS manifests under disturbance of consciousness. […] Although HFS is not fatal, it often leads to social embarrassment and interference with vision from involuntary eye closure leading to functional disability. […] Unlike other movement disorders, contractions persist in sleep which may add to the morbidity of the condition by predisposing the affected individual to disturbed sleep and insomnia. […] One recent research using facial electromyogram (EMG) and electrocardiogram (ECG) overnight indicated that wake or light sleep stages were more often accompanied by HFS.
#1
https://www.scielo.br/j/anp/a/JchcnXwmH8WDHYdJYMvHjqx/
Hemifacial spasm (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 County Minnesota and the overall prevalence is about 10/100.000. […] None of the patients had HFS as their first diagnosis. All patients were seen by their family physicians or a general practitioner. Surprisingly, 29 of out 66 (43.9%) not even received a differential diagnosis at the primary care services and, for those who did, the most frequent hypothesis were: functional disorder (or psychogenic) in 25/66 (37.8%), facial palsy in 6/66 (9%), tics in 2/66 (3%) and a miscellaneous group. […] Even though HFS is not a common disease, with an estimated prevalence of 10/100000, the diagnosis of HFS is imminently based on clinical grounds.
#1 Hemifacial Spasm | Expert Surgeon | Aaron Cohen-Gadol, MD
https://www.aaroncohen-gadol.com/en/patients/hemifacial-spasm/overview
Hemifacial spasm is estimated to occur in 11 per 100,000 individuals and is more common in females. […] On average, patients suffer from this condition for approximately 8 years before finding definitive treatment. […] Hemifacial spasms are a rare disorder that’s often misdiagnosed or overlooked for nearly a decade before most patients can be diagnosed.
#1 Botulinum Toxin for the Treatment of Hemifacial Spasm: An Update on Clinical Studies
https://www.mdpi.com/2072-6651/13/12/881
Among the available clinical scales, the Hemifacial Spasm Grading Scale (HSGS) is regarded as an objective, quick and reliable tool for the assessment of HFS. […] The most efficacious therapy has been reported to be BoNT, a biological toxin derived from the Clostridium botulinum. […] Despite limited data from high-quality clinical trials, BoNT-A is considered the treatment of choice for HFS patients. […] Overall, 76% to 100% of patients have at least a 75% improvement with a typical duration of response lasting from 3 to 4 months. […] A recent meta-analysis reported that the overall spasm freedom rate after MVD was 90.5% at the last follow-up of 1.25 Â± 0.04 years. […] Presently, international guidelines recommend the use of BoNT for HFS. However, these indications have been based upon observational data.
#1
https://www.scielo.br/j/anp/a/JchcnXwmH8WDHYdJYMvHjqx/
Misdiagnosis leads to a direct impact in QoL, social distress and impairment in activities of daily living such as driving or reading. 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.
#1 A Multidisciplinary Approach in the Management of Hemifacial Spasm – The Journal of Medical Optometry (JoMO)
https://journalofmedicaloptometry.com/volume1-issue1/a-multidisciplinary-approach-in-the-management-of-hemifacial-spasm/
An interesting consideration reported in some studies is the possible association between arterial hypertension and hemifacial spasm. Several case reports have documented arterial hypertension occurring more frequently among patients with hemifacial spasm. Therefore, regular blood pressure monitoring should be advised in patients with hemifacial spasm. Good blood pressure control should be encouraged. Future studies are needed to evaluate the link between the two. […] An interprofessional team is essential for optimal care of patients with hemifacial spasm. MRI imaging and radiographic collaboration is important in identifying the cause. Additionally, patients are co-managed with oculoplastics, otolaryngology and neurology for long-term treatment and management. Although hemifacial spasm is typically not life-threatening, it can significantly affect a patients quality of life due to associated psychosocial stress. Hemifacial spasm is involuntary, and in many cases the condition leads to anxiety and social embarrassment. Accurate diagnosis, patient education, and timely management are all key elements to improve the quality of life in these patients.
#1 Microvascular decompression for hemifacial spasm: a review of twenty-one operated cases | The Egyptian Journal of Neurology, Psychiatry and Neurosurgery | Full Text
https://ejnpn.springeropen.com/articles/10.1186/s41983-020-00179-y
Hemifacial spasm has an incidence of approximately 10 per 100,000 population with a 2:1 female predominance and a mean age of onset between 45 to 55 years. […] Microvascular decompression remains the safest and the most effective method of treating this condition with a success rate of 80-100% reported around the world. […] Sensorineural hearing loss and failure of the procedure to relieve the spasm are the two main areas of concern for the operating neurosurgeon. […] Our cure rate of 95% compares favourably with those in the existing literature which ranges from 90-98%.
#1 Hemifacial spasm: a neurosurgical perspective in: Journal of Neurosurgery Volume 140 Issue 1 (2023) Journals
https://thejns.org/view/journals/j-neurosurg/140/1/article-p240.xml
Hemifacial spasm (HFS) is rare, with an estimated incidence of 0.8/100,000 persons/year, a 2:1 female preponderance, and a mean onset age of 45-55 years. […] The utilization of MVD for HFS in North America is estimated to be only 10% of the disease incidence. […] The current levels likely do not adequately treat the HFS population. […] The adjusted utilization rates of MVD for HFS in each Canadian province demonstrated a dramatic geographic disparity, ranging between 0.04 and 0.62/100,000 persons/year.
#1 :: JCN :: Journal of Clinical Neurology
https://thejcn.com/DOIx.php?id=10.3988/jcn.2022.18.4.410
Hemifacial spasm (HFS) is a rare condition that causes involuntary contraction of facial muscles, usually on one side of the face, accompanied by abnormal compression of the adjacent facial nerve near the brainstem by a vein or artery. HFS is known to develop via two representative pathologic mechanisms: 1) peripheral ephaptic transmission and 2) central hyperexcitability of the facial motor nucleus (FMN). […] Since Mller and Jannetta first performed electrophysiologic recordings of facial nerves and muscles during MVD in 1984, various intraoperative neurophysiologic monitoring (ION) methodologies have been applied during MVD, including brainstem auditory evoked potentials (BAEPs), lateral-spread response (LSR), Z-L response (ZLR), blink reflex (BR), and facial corticobulbar motor evoked potentials (FCoMEPs).
#1 < ?php wp_title( '|', true, 'right' ); ?>
https://surgicalneurologyint.com/surgicalint-articles/hemifacial-spasm-20-year-surgical-experience-lesson-learned/
The diagnosis of HFS is clinical; electrophysiology and imaging studies are required to assess other pathologies of the cerebellopontine angle. […] Therapeutic options include medication, botulinum toxin type A injections, and surgical decompression. The most commonly used medications are carbamazepine, phenytoin, baclofen, and clonazepam. […] In our series, the most commonly offending vessel was the AICA (75.8%). Other affected vessels were the PICA (6.2%), the basilar artery (5.2%), and the superior cerebellar artery (4.1%). Similar findings have been reported by Wilson et al. […] Current reports document a variable failure rate after a first surgery that ranges from lows of less than 15% to highs of 22.7%. In our series of 194 patients, 30 patients (15.5%) had bad results (including 1 death) after the first surgery, which is comparable with outcomes of other studies. […] Additional studies are needed to fully understand the nature of HFS, the response of patients to different treatment options, and the development of recurrence. So far, microvascular decompression offers the best long-term outcome for treatment of HFS, with low morbidity and mortality.
#2 Botulinum Toxin for the Treatment of Hemifacial Spasm: An Update on Clinical Studies
https://www.mdpi.com/2072-6651/13/12/881
Hemifacial spasm (HFS) is a hyperkinetic movement disorder characterized by short or persistent, intermittent synchronous twitching of the muscles innervated by the facial nerve, which is a chronic condition, and spontaneous recovery is rare. […] The mean prevalence of the disorder is around 10 in 100,000 (14.5 and 7.4 per 100,000 in females and males, respectively). […] Currently, HFS is classified as primary (79%) or secondary to facial nerve damage (21%). […] The average age at onset of primary HFS ranges from the fifth to sixth decades of life. […] HFS is commonly sporadic, with a few familial cases having been reported. […] Diagnosis of HFS is mainly based on clinical recognition. […] The distinction between primary or secondary HFS is fundamental to properly direct the treatment strategy.
#2
https://www.scielo.br/j/anp/a/JchcnXwmH8WDHYdJYMvHjqx/
Hemifacial spasm (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 County Minnesota and the overall prevalence is about 10/100.000. […] None of the patients had HFS as their first diagnosis. All patients were seen by their family physicians or a general practitioner. Surprisingly, 29 of out 66 (43.9%) not even received a differential diagnosis at the primary care services and, for those who did, the most frequent hypothesis were: functional disorder (or psychogenic) in 25/66 (37.8%), facial palsy in 6/66 (9%), tics in 2/66 (3%) and a miscellaneous group. […] Even though HFS is not a common disease, with an estimated prevalence of 10/100000, the diagnosis of HFS is imminently based on clinical grounds.
#2 Hemifacial Spasm | Treatment & Management | Point of Care
https://www.statpearls.com/point-of-care/22719
HFS is a rare condition, as the estimated worldwide prevalence of HFS is 14.5 per 100,000 women and 7.4 per 100,000 men suggesting women are twice as prone to have HFS than men. […] As reported in some studies, those in the Asian population have a slightly higher prevalence as compared to Caucasian populations for unknown reasons. […] Most cases of HFS are sporadic; however, there are some rare case reports of familial HFS in the scientific literature. […] The disease onset, especially in primary HFS, usually begins in adulthood, around the fourth through sixth decades of life. […] Researchers have reported that 40% of patients with HFS have coexisting hypertension.
#2 Hemifacial spasm | Neurosurgery Inselspital Bern
https://neurochirurgie.insel.ch/en/diseases-specialities/functional-neurosurgery-and-pain-management/hemifacial-spasm
About 10 out of 100,000 people suffer from hemifacial spasm. For Switzerland, that is about 800 sufferers. The typical age of onset is between the 45th and 65th year of life, whereby women are more frequently affected than men (in a ratio of 2:1) and left-sided hemifacial spasm is more frequent than right-sided hemifacial spasm (also in a ratio of 2:1). […] In a metastudy covering 22 studies and data from a total of 5685 patients, 91.1% of all patients were considered cured after a median observation period of almost 3 years. Recurrences occurred in only 2.4% of patients who underwent primary successful surgery.
#2 Hemifacial Spasm (12.10.2012)
https://di.aerzteblatt.de/int/archive/article/131637
Hemifacial spasm is a neuromuscular movement disorder characterized by brief or persistent involuntary contractions of the muscles innervated by the facial nerve. Its prevalence has been estimated at 11 cases per 100 000 individuals. […] Few epidemiological data are available for the pathological entity that is hemifacial spasm. One study evaluated data from patients in Olmsted County, Minnesota, from 1960 through 1984. The mean annual incidence was 0.81 per 100 000 in women and 0.74 per 100 000 in men. The mean prevalence was 11 per 100 000 in the total population. Women developed the disorder at a rate of 14.5 per 100 000 and men at 7.4 per 100 000. The distribution by sex is thus 2:1. In Germany, the estimated prevalence is 8000 to 9000 persons. […] A study from Oslo, Norway, showed comparable results. Differences were, however, seen in the age distribution of the patients. In the US study the age peak with the highest prevalence was between 40 and 59 years, whereas the Norwegian study showed a continual increase in the prevalence to a maximum of 39.7 per 100 000 in those older than 70. The mean age at disease onset in the Norwegian study was 54. Only 1% to 6% of all patients with hemifacial spasm before age 30. In our own group of patients, too, more women than men developed the disease (women 60%, men 40%). […] Familial clustering is rare for this condition. The prevalence of bilateral hemifacial spasm has been reported to be 2.6% of cases. None of our own patients had bilateral symptoms.
#2 (Primary) hemifacial spasm: epidemiological data | SpringerLink
https://link.springer.com/chapter/10.1007/978-3-7091-6829-5_5
Epidemiological and clinical analyses of hemifacial spasm (HFS) are presented and pertinent literature is reviewed. A group of 61 patients with HFS treated with botulinum toxin (BT) was studied, and a structured questionnaire took into account: age at onset (range: 1877, mean: 51.1 years) and age at time of initiation of treatment (range: 2282, mean: 58.1 years), sex distribution (showing a female predominance: 70%), side distribution (showing a predominant involvement of the left side of the face: 67%), initiation and evolution of HFS, personal and family history, symptoms associated with HFS, and response to treatment given before BT. Data on age, sex, and side distributions on these 61 patients were compared to those obtained in a group of 50 patients with HFS who underwent operation to perform vascular decompression. All of the parameters that were analyzed in patients with HFS were compared to those obtained in patients with blepharospasm (BSP). Thus, 57 patients with BSP treated with BT were age-matched with a similar number of the patients with HFS (belonging to the group of 61 patients).
#2 Hemifacial Spasm | Neurological Surgery
https://neurosurgery.weillcornell.org/condition/hemifacial-spasm
Hemifacial spasm can affect both men and women, but it is most common in middle-aged and older women; its incidence is also higher among Asian people. […] Hemifacial spasm is most commonly caused by a small blood vessel (usually an artery) compressing the facial nerve at the brainstem. […] Hemifacial spasm typically occurs on only one side of the face (hemi- meaning half), but in some cases both sides of the face are involved.
#2 Hemifacial spasm – Wikipedia
https://en.wikipedia.org/wiki/Hemifacial_spasm
The incidence of hemifacial spasm is approximately 0.8 per 100,000 persons. […] Hemifacial spasm is more prevalent among females over 40 years of age. […] The estimated prevalence for women is 14.5 per 100,000 and 7.4 per 100,000 in men. […] Prevalence for hemifacial spasm increases with age, reaching 39.7 per 100,000 for those aged 70 years and older. […] One study divided 214 hemifacial patients based on the cause of the disease: primary hemifacial spasm, where the patients had a compression in the facial nerve at the end of the brainstem; and secondary hemifacial spasm, where the patients had peripheral facial palsy or nerve lesion due to tumors, demyelination, trauma or infection. […] The study found that 77% of cases were primary and 23% secondary. […] The study also found that the two sets of patients shared a similar age at onset, male to female ratios, and similar affected side. […] In another study of 2050 patients who had presented with hemifacial spasm between 1986 and 2009, only nine cases were caused by a cerebellopontine angle syndrome, an incidence of 0.44%.
#2 Hemifacial Spasm: Background, Pathophysiology, Epidemiology
https://emedicine.medscape.com/article/1170722-overview
Hemifacial spasm is observed in both men and women but is more common in middle-aged or older women. Studies also note that the condition is more prevalent among Asian populations. However, it generally affects all races without significant disparity. […] Idiopathic hemifacial spasm typically begins in the fifth or sixth decade of life. Onset of hemifacial spasm in patients younger than 40 years is unusual and often heralds an underlying neurologic illness (eg, multiple sclerosis).
#2 Revisiting the link between hypertension and hemifacial spasm | Scientific Reports
https://www.nature.com/articles/srep21082
The prevalence of hypertension was higher in HFS patients as compared to non-HFS patients. […] This meta-analysis highlights a positive correlation between hypertension and HFS. […] Blood pressure should be closely monitored during the follow-up of HFS patients. […] The results of the meta-analysis showed that there is indeed a higher prevalence of hypertension among HFS patients as compared to non-HFS controls. […] Therefore, regular blood pressure monitoring should be performed during follow-up visits for early detection and intervention of hypertension should it develop. […] Future studies should address if controlling hypertension can help in managing HFS. […] Current case-control studies in HFS and hypertension have limitations. […] In conclusion, this meta-analysis highlights a positive correlation between hypertension and HFS. Blood pressure should be closely monitored during the follow-up of HFS patients.
#2 Prevalence of hemifacial spasm in Northern Cyprus – MDS Abstracts
https://www.mdsabstracts.org/abstract/prevalence-of-hemifacial-spasm-in-northern-cyprus/
Prevalence of hemifacial spasm in Northern Cyprus […] Objective: To estimate the prevalence and analyze the clinical profile of hemifacial spasm in Northern Cyprus. […] The association between hemifacial spasm and hypertension was frequently indicated in the literature. […] Seventeen (74%) of the patients had hypertension. […] Hypertension is a frequent seen condition among our HFS patients, replicating the association between hemifacial spasm and hypertension indicated in the literature. […] The prevalence rate of our study is similar with the previous studies published in the literature.
#2 What Is Hemifacial Spasm? | Expert Surgeon | Aaron Cohen-Gadol, MD
https://www.aaroncohen-gadol.com/en/patients/hemifacial-spasm/types/what-is-hemifacial-spasm
Hemifacial spasm is a rare condition, affecting approximately 11 in 100,000 individuals. […] Hemifacial spasm is more common in elderly women and people of Asian descent. Estimates suggest that approximately 11 in 100,000 people are affected by this disorder. […] Unfortunately, hemifacial spasm is commonly misdiagnosed, and patients tend to experience symptoms for an average of 8 years before receiving a proper diagnosis that can guide treatment.
#2 Hemifacial Spasm – Brain, Spinal Cord, and Nerve Disorders – Merck Manual Consumer Version
https://www.merckmanuals.com/home/brain-spinal-cord-and-nerve-disorders/cranial-nerve-disorders/hemifacial-spasm
Hemifacial spasm affects men and women but is more common among middle-aged and older women. […] Doctors diagnose hemifacial spasm based on symptoms but do magnetic resonance imaging to check for other disorders that can cause similar symptoms. […] Magnetic resonance imaging (MRI) should be done to rule out tumors, other structural abnormalities, and multiple sclerosis, which can cause similar symptoms. Also, MRI can usually detect the abnormal loop of artery pressing against the nerve.
#2 A Multidisciplinary Approach in the Management of Hemifacial Spasm – The Journal of Medical Optometry (JoMO)
https://journalofmedicaloptometry.com/volume1-issue1/a-multidisciplinary-approach-in-the-management-of-hemifacial-spasm/
An interesting consideration reported in some studies is the possible association between arterial hypertension and hemifacial spasm. Several case reports have documented arterial hypertension occurring more frequently among patients with hemifacial spasm. Therefore, regular blood pressure monitoring should be advised in patients with hemifacial spasm. Good blood pressure control should be encouraged. Future studies are needed to evaluate the link between the two. […] An interprofessional team is essential for optimal care of patients with hemifacial spasm. MRI imaging and radiographic collaboration is important in identifying the cause. Additionally, patients are co-managed with oculoplastics, otolaryngology and neurology for long-term treatment and management. Although hemifacial spasm is typically not life-threatening, it can significantly affect a patients quality of life due to associated psychosocial stress. Hemifacial spasm is involuntary, and in many cases the condition leads to anxiety and social embarrassment. Accurate diagnosis, patient education, and timely management are all key elements to improve the quality of life in these patients.
#2 :: JCN :: Journal of Clinical Neurology
https://thejcn.com/DOIx.php?id=10.3988/jcn.2022.18.4.410
Many research groups have used ION to provide deeper insights into the pathologic mechanisms of HFS and to analyze the predictive value of ION data for long-term postoperative neurologic outcomes. […] There have been new advances reported in this field since that time. Therefore, this article aimed to review and present advances in ION techniques and evaluate their electrophysiological/clinical significance in MVD for HFS based on studies published after 2012. […] The application of the new advances in ION methodologies used during MVD for HFS summarized in this review have not only provided clinicians with deeper insights into the pathophysiology of HFS, but they have also led to improvements in the efficacy of MVD and the prognosis of HFS after treatment.
#2 < ?php wp_title( '|', true, 'right' ); ?>
https://surgicalneurologyint.com/surgicalint-articles/hemifacial-spasm-20-year-surgical-experience-lesson-learned/
The diagnosis of HFS is clinical; electrophysiology and imaging studies are required to assess other pathologies of the cerebellopontine angle. […] Therapeutic options include medication, botulinum toxin type A injections, and surgical decompression. The most commonly used medications are carbamazepine, phenytoin, baclofen, and clonazepam. […] In our series, the most commonly offending vessel was the AICA (75.8%). Other affected vessels were the PICA (6.2%), the basilar artery (5.2%), and the superior cerebellar artery (4.1%). Similar findings have been reported by Wilson et al. […] Current reports document a variable failure rate after a first surgery that ranges from lows of less than 15% to highs of 22.7%. In our series of 194 patients, 30 patients (15.5%) had bad results (including 1 death) after the first surgery, which is comparable with outcomes of other studies. […] Additional studies are needed to fully understand the nature of HFS, the response of patients to different treatment options, and the development of recurrence. So far, microvascular decompression offers the best long-term outcome for treatment of HFS, with low morbidity and mortality.
#3 < ?php wp_title( '|', true, 'right' ); ?>
https://surgicalneurologyint.com/surgicalint-articles/hemifacial-spasm-20-year-surgical-experience-lesson-learned/
Hemifacial spasm is characterized by unilateral, paroxysmal, and involuntary contractions. It is more common in women on the left side. Its evolution is progressive, and it rarely improves without treatment. […] HFS occurs almost exclusively in adults. It is not hereditary, although some familial cases have been described. HFS occurs slightly more often in women, typically on the left side. In rare cases, it is bilateral, but not symmetrical or synchronous. […] HFS is a relatively rare entity that occurs most commonly in middle-aged women. According to Auger and Whisnant, HFS has an incidence of 0.8 cases per 100,000 persons, with a mean prevalence of 7.4 cases per 100,000 men and 14.5 per 100,000 women. […] The pathogenesis of HFS, as described by Jannetta and colleagues, is believed to be secondary to neurovascular compression of the root of cranial nerve VII at its entry zone (at the transition between the central myelin and the peripheral myelin). This theory is widely accepted and recognized, but is also highly disputed.
#3 Hemifacial Spasms – Facial Pain AssociationIcon / Teal / print@1xicon-heart@1xicon-plane
https://www.facepain.org/understanding-facial-pain/diagnosis/hemifacial-spasms/
Hemifacial spasm is a fairly rare condition, with a worldwide prevalence of 9.8-11 per 100,000. Women are affected about twice as often as men are. […] In cases of primary HFS, symptoms usually begin between 40 to 60 years, and only 1-6% of those with HFS have symptoms before the age of 30. Researchers have not found a genetic link for HFS, meaning it does not run in families.
#3 A Multidisciplinary Approach in the Management of Hemifacial Spasm – The Journal of Medical Optometry (JoMO)
https://journalofmedicaloptometry.com/volume1-issue1/a-multidisciplinary-approach-in-the-management-of-hemifacial-spasm/
An interesting consideration reported in some studies is the possible association between arterial hypertension and hemifacial spasm. Several case reports have documented arterial hypertension occurring more frequently among patients with hemifacial spasm. Therefore, regular blood pressure monitoring should be advised in patients with hemifacial spasm. Good blood pressure control should be encouraged. Future studies are needed to evaluate the link between the two. […] An interprofessional team is essential for optimal care of patients with hemifacial spasm. MRI imaging and radiographic collaboration is important in identifying the cause. Additionally, patients are co-managed with oculoplastics, otolaryngology and neurology for long-term treatment and management. Although hemifacial spasm is typically not life-threatening, it can significantly affect a patients quality of life due to associated psychosocial stress. Hemifacial spasm is involuntary, and in many cases the condition leads to anxiety and social embarrassment. Accurate diagnosis, patient education, and timely management are all key elements to improve the quality of life in these patients.