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Typical symptoms of HFS begin with an involuntary movement of the eyelid that gradually progresses to involve the oral muscles&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> TN manifests as neuropathic facial pain&#44; defined by the International Pain Society as &#8220;periodic&#44; sudden&#44; intense and transient tingling of the skin in the area innervated by one or more branches of the TgN&#46;&#8221; Typical clinical features of TN include paroxysmal pain&#44; remission&#44; precipitating factors&#44; and trigger points&#46; The annual incidence of TN is 4&#46;5&#8211;28&#46;9&#47;100&#44;000&#46; It is more common in middle-aged women&#44; with the right side being more common than the left&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">In the case of overactive cranial nerve syndrome&#44; the possibility of dolichoectasia of the vertebrobasilar artery is generally considered&#44; which is common in women and also involves the glossopharyngeal nerve &#40;GPN&#41;&#44;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> but there is the possibility&#44; not common&#44; of a mononeural compression and that due to the neurophysiological relationships&#44; for example between the V and VII pair&#44; stimuli capable of generating hyperactivity and the patient&#39;s symptoms are established&#44; the objective of this study being to present a case where this latter situation is evident&#44; through the abnormal activation of the trigemino-facial reflex&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0020" class="elsevierStylePara elsevierViewall">A 55-year-old white&#44; right-handed woman&#44; with no history of personal chronic diseases&#44; unremarkable family history&#44; reported left-sided HFS for 8 years&#44; for which she was followed up by Neurology and periodically received 21 infiltrations with botulinum toxin&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">2 years ago&#44; episodic pain of maximum severity was added to the clinical picture &#40;VAS-10&#47;10&#41; in the region corresponding to the territory of V1 and V2 on the left side&#44; which originated from stimulating trigger points&#44; with characteristics of typical TN&#46; The patient underwent several medical treatment regimens&#44; the pain being refractory to them&#46; The HFS remained between the pain crises&#44; not being related to them&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">The physical examination shows the patient conscious&#44; oriented&#44; without meningeal signs&#44; with pupils isochoric and reactive to light&#44; without ophthalmoparesis&#44; with sustained&#44; painless spasms&#44; which begins in the orbicularis oculi of the affected side with subsequent irradiation to the face and mouth&#44; including the forehead and platysma on the left side of the face and with reproduction of pain to sensory stimulation of the skin of the forehead and upper dental arch&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">MRI with protocol for neurovascular conflict showed a vessel in intimate contact with the entry zone of the left trigeminal nerve&#46; At the level of the exit zone of the left facial nerve&#44; the finding of a vessel in contact with the nerve was not clear &#40;See <a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>A and E&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">The case is discussed in our group and with informed consent from the patient it is decided to perform surgery for microvascular decompression&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">A left retrosigmoid approach was performed&#44; in a semi-sitting position&#46; First&#44; the entry zone of the TgN was accessed through a lateral supracerebellar pathway&#44; finding a clear conflict between the anterior inferior cerebellar artery and the lower sector of the sensory root of the TgN&#44; with even a notch and change in colour at that level&#46; Using a microsurgical technique&#44; a fragment of Teflon was placed between the artery and the nerve&#44; achieving adequate separation &#40;See <a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>B&#8211;D&#41;&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Then&#44; the trajectory was changed&#44; and through a lateral infracerebellar route the exit zone of the FN was accessed&#44; without identifying any type of vascular conflict&#46; For greater safety&#44; a 30-degree endoscope was used&#44; and no vascular conflict was identified with the endoscopic view &#40;See <a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>F&#8211;H&#41;&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">The patient&#39;s initial symptoms &#40;See <a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>A and B&#41;&#44; after surgery&#44; presented complete resolution&#44; both the HFS and the associated trigeminal pain&#46; Medical follow-up at one year has shown no recurrence of the initial symptoms &#40;See <a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>C and D&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0060" class="elsevierStylePara elsevierViewall">The pathophysiology of HFS and TN evidences an injury in the transition between the central myelin &#40;originated by oligodendrocytes&#41; and the peripheral myelin &#40;originated by Schwann cells&#41;&#44; creating patches of demyelination<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> and anomalous activation of transmembrane sodium channels in the axons&#46; This&#44; combined with triggering factors such as transient fluctuations in blood pressure or pulse&#44; causes these voltage-dependent channels to reach the threshold and generate action potentials that can be conducted by the damaged nerve&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">When the nerve is injured&#44; Schwann cells and macrophages phagocytose the degenerated myelin&#44; producing cytokines that further aggravate the demyelination&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> The only radical solution for this cyclical destructive process is microvascular decompression&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">In TN&#44; the vessel responsible is usually the superior cerebellar artery&#44; while in HFS it is usually the anterior inferior cerebellar artery&#46; However&#44; there are cases in which an enlarged or dolichoectatic vertebral artery can be the causative vessel of both TN and HFS&#44; as well as NGF&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> In the case we present&#44; this pathophysiological mechanism&#44; which is the most common&#44; partially explains the symptoms&#44; since clear vascular compression was evident at the level of the TN&#44; but not at the level of the FN&#46; One hypothesis is that hyperactivation of the facial nerve is produced by a sustained and abnormal stimulation of the trigemino-facial reflex&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">At the end of the 1910s&#44; the presence of HFS coexisting with facial pain due to compression of the TgN was described&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> In the last 30 years&#44; several series have been published describing the presence of HFS&#44; with or without facial pain&#44; in the context of trigeminal injury or compression&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">9&#44;10</span></a> In the last decade&#44; during skull base surgeries with intraoperative neurophysiological monitoring&#44; the presence of robust electromyographic responses of muscles innervated by the facial nerve&#44; evoked with stimulation of the TgN&#44;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a> which as a whole has been called the trigemino-facial reflex&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">The structures involved in this response constitute an oligosynaptic reflex arc&#44; formed by an afferent portion through the sensory branches of the TgN&#44; which synapses in two structures&#58; first&#44; with the main sensory nucleus of the trigeminal located in the middle portion of the pons&#44; which in turn synapses with the motor nucleus of the ipsilateral facial nerve&#46; The second synapse of this afferent portion is with the nucleus of the trigeminal spinal tract in the rostral portion of the medulla oblongata&#44; which in turn activates the nucleus of the facial nerve&#44; both ipsilateral and contralateral&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">That is why today&#44; in addition to these responses being able to be evoked and evaluated during physical examination &#40;corneal reflex&#44; corneomandibular reflex&#44; etc&#46;&#41;&#44; the evocation of multiple trigeminal reflexes has also been widely implemented during intraoperative monitoring to promote the functional preservation of these structures&#44; such as the blink reflex &#40;trigeminal afferent branch and facial efferent branch<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a> and the hypoglossal trigeminal reflex&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">14</span></a></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusions</span><p id="par0090" class="elsevierStylePara elsevierViewall">In a patient with HFS with or without pain &#40;our patient spent 6 years without pain&#41;&#44; during microvascular decompression of the FN&#44; if frank proximal compression is not evident&#44; the trigeminofacial structural relationship must be taken into account&#44; making it necessary to explore the TN&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Funding for this publication</span><p id="par0095" class="elsevierStylePara elsevierViewall">No&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conflict of interest</span><p id="par0100" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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            0 => "Hemifacial spasm"
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            3 => "Teflon sheet"
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            0 => "Espasmo hemifacial"
            1 => "Neuralgia trigeminal"
            2 => "Descompresi&#243;n microvascular"
            3 => "La&#769;mina de teflo&#769;n"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">The coincidence in a patient of Hemifacial Spasm and Trigeminal Neuralgia is not frequent&#46; A case is presented with the objective of showing this association due to the abnormal activation of the Trigemino-Facial Reflex&#46;</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">A 55-year-old woman with an 8-year history of left-sided hemifacial spasm and typical trigeminal pain in the ipsilateral V1 and V2 territory&#46;</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">The physical examination shows spasms in the left hemiface&#44; with reproduction of intense pain upon sensory stimulation of the skin on the forehead and upper dental arch&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">The MRI showed a vessel in intimate contact with the entrance area of &#8203;&#8203;the left trigeminal nerve&#46;</p><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">A left retrosigmoid approach was performed&#46; First&#44; the entrance area of &#8203;&#8203;the trigeminal nerve was accessed&#44; finding a clear vascular conflict&#44; which was isolated with Teflon&#46; Then&#44; the trajectory was changed and the exit zone of the facial nerve was accessed&#44; and no type of vascular conflict was identified&#46;</p><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">The patient presented complete resolution of the Hemifacial Spasm and the associated trigeminal pain&#46;</p><p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">The analysis of this case allows us to conclude that during microvascular decompression of the Facial Nerve&#44; if frank proximal compression is not evident&#44; the Trigeminofacial structural relationship must be taken into account&#44; making it necessary to explore the Trigeminal Nerve&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">La coincidencia en un paciente de espasmo hemifacial y de neuralgia trigeminal no es frecuente&#46; Se presenta un caso con el objetivo de mostar esta asociaci&#243;n por la activaci&#243;n anormal del reflejo trig&#233;mino-facial&#46;</p><p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Mujer de 55 a&#241;os&#44; con antecedentes de 8 a&#241;os de espasmo hemifacial de lado izquierdo y dolor trigeminal t&#237;pico en el territorio de V1 y V2 ipsilateral&#46;</p><p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">El examen f&#237;sico muestra espasmos en hemicara izquierda&#44; con reproducci&#243;n de dolor intenso al est&#237;mulo sensitivo de la piel en la frente y arcada dentaria superior&#46;</p><p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">La RM mostr&#243; un vaso en &#237;ntimo contacto con la zona de entrada del nervio trig&#233;mino izquierdo&#46;</p><p id="spar0070" class="elsevierStyleSimplePara elsevierViewall">Se realiz&#243; un abordaje retrosigmoideo izquierdo&#46; Primero se accedi&#243; a la zona de entrada del nervio trig&#233;mino encontr&#225;ndose un claro conflicto vascular&#44; el cual se aisla con tefl&#243;n&#46; Luego&#44; se cambi&#243; de trayectoria y se accedi&#243; a la zona de salida del nervio facial&#44; no identificandose ningun tipo de conflicto vascular&#46;</p><p id="spar0075" class="elsevierStyleSimplePara elsevierViewall">La paciente present&#243; resoluci&#243;n total del espasmo hemifacial y del dolor trigeminal asociado&#46;</p><p id="spar0080" class="elsevierStyleSimplePara elsevierViewall">El an&#225;lisis de este caso nos permite concluir que durante la descompresion microvascular del nervio facial si no se evidencia una franca compresi&#243;n proximal&#44; se debe tener en cuenta la relaci&#243;n estructural trigeminofacial&#44; siendo necesario la exploraci&#243;n del nervio trigeminal&#46;</p></span>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; MRI at the level of the trigeminal nerve &#40;T2 heavy and T1 sequences with gadolinium&#41;&#46; A vascular conflict is observed in the lower part of the nerve&#46; &#40;B&#8211;D&#41; microsurgical images&#46; &#40;B and C&#41; The vascular conflict can be seen with the generation of a deformation of the trigeminal nerve caused by the cerebellar artery&#46; &#40;D&#41; Teflon placed between the artery and the nerve&#46; &#40;E&#41; MRI at the level of the facial nerve &#40;T2 heavy and T1 sequences with gadolinium&#41;&#46; No clear vascular conflict is observed&#46; &#40;F&#41; Microsurgical image&#46; No vascular conflict is seen in the exit zone of the facial nerve&#46; &#40;G and H&#41; Endoscope images&#46; No vascular conflict is seen in the exit zone of the facial nerve&#46;</p>"
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Case Report
Available online 5 August 2024
Hemifacial spasm associated with trigeminal neuralgia secondary to trigeminal vascular compression
Espasmo hemifacial asociado a neuralgia trigeminal secundario a compresión vascular trigeminal
Ernesto F. Ardisanaa,
Corresponding author
ernestofas2018@gmail.com

Corresponding author.
, Juan F. Villalongaa, Mauro M. Suáreza, Alvaro Camperoa,b
a Laboratorio de Innovaciones Neuroquirúrgicas de Tucumán (LINT), Facultad de Medicina, Universidad Nacional de Tucumán, Tucumán, Argentina
b Hospital General Angel C. Padilla, Tucumán, Argentina
Received 06 April 2024. Accepted 16 July 2024
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Abstract

The coincidence in a patient of Hemifacial Spasm and Trigeminal Neuralgia is not frequent. A case is presented with the objective of showing this association due to the abnormal activation of the Trigemino-Facial Reflex.

A 55-year-old woman with an 8-year history of left-sided hemifacial spasm and typical trigeminal pain in the ipsilateral V1 and V2 territory.

The physical examination shows spasms in the left hemiface, with reproduction of intense pain upon sensory stimulation of the skin on the forehead and upper dental arch.

The MRI showed a vessel in intimate contact with the entrance area of ​​the left trigeminal nerve.

A left retrosigmoid approach was performed. First, the entrance area of ​​the trigeminal nerve was accessed, finding a clear vascular conflict, which was isolated with Teflon. Then, the trajectory was changed and the exit zone of the facial nerve was accessed, and no type of vascular conflict was identified.

The patient presented complete resolution of the Hemifacial Spasm and the associated trigeminal pain.

The analysis of this case allows us to conclude that during microvascular decompression of the Facial Nerve, if frank proximal compression is not evident, the Trigeminofacial structural relationship must be taken into account, making it necessary to explore the Trigeminal Nerve.

Keywords:
Hemifacial spasm
Trigeminal neuralgia
Microvascular decompression
Teflon sheet
Trigemino-Facial reflex
Resumen

La coincidencia en un paciente de espasmo hemifacial y de neuralgia trigeminal no es frecuente. Se presenta un caso con el objetivo de mostar esta asociación por la activación anormal del reflejo trigémino-facial.

Mujer de 55 años, con antecedentes de 8 años de espasmo hemifacial de lado izquierdo y dolor trigeminal típico en el territorio de V1 y V2 ipsilateral.

El examen físico muestra espasmos en hemicara izquierda, con reproducción de dolor intenso al estímulo sensitivo de la piel en la frente y arcada dentaria superior.

La RM mostró un vaso en íntimo contacto con la zona de entrada del nervio trigémino izquierdo.

Se realizó un abordaje retrosigmoideo izquierdo. Primero se accedió a la zona de entrada del nervio trigémino encontrándose un claro conflicto vascular, el cual se aisla con teflón. Luego, se cambió de trayectoria y se accedió a la zona de salida del nervio facial, no identificandose ningun tipo de conflicto vascular.

La paciente presentó resolución total del espasmo hemifacial y del dolor trigeminal asociado.

El análisis de este caso nos permite concluir que durante la descompresion microvascular del nervio facial si no se evidencia una franca compresión proximal, se debe tener en cuenta la relación estructural trigeminofacial, siendo necesario la exploración del nervio trigeminal.

Palabras clave:
Espasmo hemifacial
Neuralgia trigeminal
Descompresión microvascular
Lámina de teflón
Reflejo trigémino-facial

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