Arq Neuropsiquiat r 2002;60(2-A):288-289
HEMIMASTICATORY SPASM TREATED
WITH BOTULINUM TOXIN
Case report
Hélio A.G. Teive
1, Élcio J. Piovesan
2, Francisco M.B. Germiniani
3,
Carlos Henrique A. Camargo
3, Daniel Sá
3, Rosana H. Scola
4, Lineu C. Werneck
5ABSTRACT - We describe a f em ale pat ient w it h hem im ast icat ory spasm , a rare m ovem ent disorder due t o dysf unct ion of t he m ot or t rigem inal nerve of unknow n origin. This pat ient had an excellent response t o bot ulinum t oxin t herapy.
KEY WORDS: hemimast icat ory spasm, paroxysmal spasms, bot ulinum t oxin.
Espasmo hemimastigatório tratado com toxina botulínica: relato de caso
RESUM O - Relat amos o caso de pacient e feminina com espasmo hemimast igat ório, dist úrbio do moviment o raro decorrent e de disfunção da porção mot ora do nervo t rigeminal, de et iologia desconhecida. A pacient e t eve excelent e respost a clínica ao t rat ament o com t oxina bot ulínica.
PALAVRAS-CHAVE: espasmo hemimast igat ório, espasmos paroxíst icos, t oxina bot ulínica.
St udy performed at t he M ovement Disorders Unit , Service of Neurology, Hospit al de Clínicas, Universidade Federal do Paraná (UFPR), Curit iba PR, Brazil: 1Assist ant Professor of Neurology; 2Neurologist ; 3Resident in Neurology; 4Adjunct Professor of Int ernal M edicine; 5Full
Professor of Neurology Head of t he Service of Neurology. This paper w as present ed as a post er at t he Sixt h Int ernat ional Congress of Parkinson’s Disease and M ovement Disorders in Barcelona, Spain, 11-15th June, 2000.
Received 8 July 2001, received in final form 12 November 2001. Accept ed 21 November 2001.
Dr. Hélio A. G. Teive - Rua General Carneiro 1103/102 - 80.060–150 Curitiba – PR – Brasil. E-mail: hagteive@mps.com.br
Hem im ast icat ory spasm (HM S) represent s a rare movement disorder due t o a dysfunct ion of t he mo-t or mo-t rigeminal nerve of unknow n origin. Imo-t is frequen-t ly m isdiagnosed as hem if acial spasm , w hich is a disorder due t o dysf unct ion of t he f acial nerve1-3.
The most st riking feat ures of HM S are t he excruciat -ing pain t hat accom panies t he spasm it self and t he f act t hat init ially m ast icat ory m ovem ent s act as a t rigger f or t he spasm1-3. There are m any opt ions f or
t he t reat ment of HM S, raging from medical t o sur-gical approaches. A m ilest one in t he t reat m ent of HM S w as bot ulinum t oxin, w hich has becom e t he t reat m ent of choice due t o it s excellent result s. We report a HM S case.
CASE
A 44 years old f em ale pat ient cam e t o our service com -plaining of f acial spasm s and severe pain com prom ising t he right t em poral region and t he right side of t he f ace and last ing 1 up t o 2 m inut es. The sym pt om s w ere t rig-gered by m ast icat ory m ovem ent s at f irst , but m ore re-cent ly t hey needed no t riggering f act or and appeared
spont aneously. She also com plained of dif f icult y in t alk-ing and sw allow alk-ing w hen she had t he spasm s.
Neurological exam inat ion w as norm al except f or in-t ense conin-t racin-t ion of in-t he m assein-t er and in-t em poral m uscles on t he right w it h severe f acial pain w hen t he pat ient had t he spasm s. Rout ine laborat ory t est s (WBC, biochem ist ry, VDRL, ERS) and ceruloplasmine w ere all normal.
The pat ient also had a previous hist ory of irregular m enst rual cycles and even am enorrhea f or m ore t han o n e year . En d o cr i n o l o g i cal i n vest i g at i o n d i scl o sed increased prolact in levels (61.34; norm al: 1.39 – 24.20) and norm al t hyroid t est s: TSH 1.458 (norm al range: 0.490 – 4.670), T3 86.12 (norm al range: 45 – 137) and T4 7.64 (norm al range: 4.5 – 12). Cranial com put ed t om ography (CT) and a m agnet ic resonance im aging (M RI) w ere nor-m al. The pat ient w as t reat ed w it h several drugs such as carbam azepine, clonazepam and gabapent in w it h no im provem ent w hat soever.
Arq Neuropsiquiat r 2002;60(2-A) 289
of bot ulinum t oxin (Bot ox, Allergan) in bot h t he right masset er and right t emporal muscles w as t hen performed, leading t o a rem arkable im provem ent of sym pt om . The durat ion of effect w as t hree mont hs, w hen a new inject ion of bot ulinum t oxin had t he sam e result .
DISCUSSION
HM S is a rare m ovem ent disorder of unknow n origin1, 2 t hat af f ect s m ore w om en in t he t hird and
f ourt h decades. It ’s probably due t o a disorder of t he t rigem inal nerve, sim ilar t o t he one f ound in or-dinary hem if acial-spasm . Ent rapm ent of t he m ot or branches of t he t rigem inal nerve in t he inf rat em po-ral f ossa2, 3 leads t o paroxysmal involunt ary cont
rac-t ion of rac-t he jaw m uscles w irac-t h involunrac-t ary jaw clos-ing1. Ot her aut hors believe t hat a vascular
compres-sion near the brainstem is the cause of HM S2. To some
ext end, lack of inhibit ion of m asset er m uscle con-t raccon-t ion m ay play a role in con-t he econ-t iology of HM S.
The muscles commonly affect ed are t he masset er, t em poralis and m edial pt erigoid1. Brief, paroxysmal
spasms are separat ed in t ime by variable sympt oms-free int ervals and are not linked t o specific t asks1.The
af orem ent ioned m uscles can be hypert rophied in HM S, as opposed t o t he f acial m uscles af f ect ed in hemifacial spasm, w hich do not get hypert rophied1.
Such hypert rophy, as w ell as dislocat ion of t he jaw , can be seen by CT or M RI of t he face2. There can also
be asym m et rical jaw jerks2. M uscle biopsy is usually
normal, somet imes w it h signs of skin at rophy.
Anot her clinically distinct feature of HM S is that it may be painful, w hile hem if acial spasm t ends t o be not relat ed t o pain of any kind. HM S can also be associat ed w it h hemifacial at rophy1- 3 in some cases,
but t his associat ion is not obligat ory.
Electrophysiologic study discloses an irregular bursts of motor unit potentials, similar to the findings of ordinary hem if acial spasm and absent m asset er inhibitory reflex1,2 .This electrographic pattern suggests
that there is an ectopic excitation of the motor root of the trigeminal nerve, rather than a central origin1.
The elect rophysiologic findings are charact erist ic and help in t he dif f erent ial diagnosis w it h ot her condit ions, such as unilat eral dyst onia of t he jaw w it h jaw closure, t em porom andibular joint syn-drom e, paroxysm al event s in m ult iple sclerosis and t et any4, 5. To t hat ef f ect , EM G show s unilat eral
dis-charges and f ocal hypert rophy1.
Treatment options for HM S include the use of oral drugs, surgical t reat ment and inject ion of bot ulinum t oxin1, 3, 5. The lat t er seem s t o be t he t reat m ent
op-tion w ith the best outcome. The drugs that have been used in t he t reat m ent of HM S are phenyt oin, carm azepine, clonazepacarm , diazepacarm , dant rolene, ba-clof en, valproat e, haloperidol, am it ript yline, cyclo-benzaprine and t rihexyphenidril, all of t hem w it h poor clinical improvement , except maybe for carba-m azepine and phenyt oin1.
Bot ulinum t oxin is now available for a w ide spec-trum of conditions characterized by muscle over-con-t racover-con-t ion, including dysover-con-t onia, hem if acial spasm and spast icit y am ong ot her indicat ions6. Remarkable
re-sult s have occurred in t he t reat m ent of f ocal dyst o-nia and part icularly hemifacial spasm, an ent it y very similar t o HM S6. Auger et al. published a clinical and
elect rophysiologic observat ions on 3 cases of hem i-m ast icat ory spasi-m , and one of t hei-m responded f a-vorably t o bot ulinum t oxin inject ions1. Ebersbach et
al. report ed t w o cases of hem im ast icat ory spasm in hem if acial at rophy; inject ions of bot ulinum t oxin t ype A int o t he m ast icat ory m uscles proved t o be a successf ul t reat m ent in bot h pat ient s3. Kim et al.
report ed a case of hem im ast icat ory spasm associa-t ed w iassocia-t h localized scleroderm a and f acial hem iaassocia-t ro-phy; t he aut hors described excellent result s aft er t he use of bot ulinum t oxin t ype A inject ions7.
HM S is a highly debilit at ing m ovem ent disorder. It ’s probably due t o a dysf unct ion of t he t rigem inal nerve, sim ilar t o t he one f ound in ordinary hem if a-cial-spasm. Our patient show ed an excellent response t o bot ulinum t oxin t herapy.
REFERENCES
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2. Cruccu G, Inghilleri M, Berardelli A, et al. Pathophysiology of hemimasticatory spasm. J Neurol Neurosurg Psychiatry 1994;57:43-50.
3. Ebersbach G, Kabus C, Schelosky L, Terstegge L, Poewe W.
Hemimasticatory spasm in hemifacial atrophy: diagnostic and thera-peutic aspects in two patients. Mov Disord 1995;10:504-507. 4. Thompson PD, Obeso JA, Delgado G, Gallego J, Marsden CD. Focal
dystonia of the jaw and the differential diagnosis of unilateral jaw and masticatory spasm. J Neurol Neurosurg Psychiatry 1986;49:651-666. 5. Lagueny A, Deliac MM, Julien J, Demotes Mainard J, Ferrer X. Jaw
closing spasm—a form of focal dystonia? An electrophysiological study. J Neurol Neurosurg Psychiatry 1989;52:652-655.
6. Moore AP. General and clinical aspects of treatment with botulinum toxin. In Moore P. Handbook of botulinum toxin. Oxford, UK: Blackwell Science 1995:28-53.
