Anterior Opercular Syndrome Research Articles

A Rare Case of Unilateral Foix-Chavany-Marie Syndrome

Background: Bilateral anterior opercular syndrome, or Foix-Chavany-Marie Syndrome (FCMS), is a neurological condition characterized by bilateral anterior opercular lesions. Common presenting symptoms of this rare suprabublar palsy include dysarthria, or slurred speech, as well as paralysis of facial, pharyngeal, lingual, and masticatory voluntary muscles while autonomic function is preserved.1 Most cases of FCMS are bilateral, yet some rare cases have been reported with unilateral opercular lesions with preexisting contralateral white matter lesions.2 In this case report we present a rare case of unilateral FCMS in a patient who had an acute anterior left opercular infarct, as well as a residual right parietal subcortical stroke.
 Project Methods: The patient we present is a 49 year old African American female with a history of hypertension and previous stroke with residual spastic right hemiplegia who presented to the ED for evaluation of right sided facial droop, right sided weakness, and slurred speech. Patient was evaluated by acute stroke team and was treated with intravenous thrombolysis (Alteplase). On hospital day 2, the patient developed complete disarticulation, unable to produce any speech but able to communicate by appropriate gestures and writing. The patient’s symptoms included anarthria, weakness of bilateral masseters, and lateral/medial pterygoids without dysphagia. Results: The MRI brain showed left insular region/frontal opercular ischemic stroke and a small right parietal subcortical ischemic stroke, likely embolic in nature. Over the hospital stay, the patient’s motor function improved but her anarthria persisted. These radiologic findings along with the symptomology proved consistent with FCMS. She was discharged to home with an NIH stroke scale of 5 and recommended outpatient speech therapy.
 Conclusion: In this case report, we describe a patient that presents with an extremely rare case of unilateral FCMS, with a preexisting contralateral parietal infarct that could have collectively caused anarthria and masseter weakness.
 
 Works Cited 1. Milanlioglu A, Aydın MN, Gökgül A, Hamamcı M, Erkuzu MA, Tombul T. Ischemic Bilateral Opercular Syndrome. Case Reports in Medicine. 2013;2013:1-3. doi:10.1155/2013/513572.
 2. Sa F, Cordeiro IM, Mestre S, Nzwalo H. Unilateral opercular infarction presenting with FoixChavany-Marie syndrome. Case Reports. 2014;2014. doi:10.1136/bcr-2014-206439.

Dysphagia management in bilateral frontal opercular syndrome (Foix-Chavany-Marie syndrome)

An 80-year-old woman was admitted to hospital with acute anarthria and severe dysphagia, characterised by loss of voluntary control of the muscles of facial expression, mastication, bolus manipulation (tongue) and...

Bilateral Anterior Opercular Syndrome With Partial Kluver-Bucy Syndrome in a Stroke Patient: A Case Report.

Bilateral anterior opercular syndrome and partial Kluver–Bucy syndrome are associated with bilateral middle cerebral artery lesions. The combination of these two syndromes has only been reported in a child with limbic encephalitis. In this case, a 44-year-old woman with bilateral middle cerebral artery infarction, which occurred 2 years prior, could walk independently. However, she showed automatic-voluntary dissociation and anarthria with preserved writing skills. She also presented hypersexuality, hypermetamorphosis, and memory disturbances. Here, we report a case of an adult stroke patient who suffered from bilateral anterior opercular syndrome accompanied by partial Kluver–Bucy syndrome.

Anterior opercular syndrome induced by Epstein–Barr virus encephalitis

We report a 19-year-old female presenting with fever, drooling, anarthria, and voluntary facial movement disruption, characteristic of anterior opercular syndrome (AOS). Serological examination revealed Epstein–Barr virus (EBV) infection following acute encephalitis with severe ataxia. A single-photon emission computerized tomography (SPECT) examination indicated hypoperfusion in the left perisylvian region, bilateral thalamus, occipital lobe, and cerebellum. This is the first report of AOS related to EBV encephalitis. SPECT was a useful method for detecting the damaged region of the operculum. In addition, AOS is a clinically distinct entity that may help us understand the mechanisms of language circuits within the operculum.

A case report of Partial Kluver - Bucy Syndrome with Anterior Opercular syndrome in childhood: A sequel of limbic encephalitis

Kluver Bucy Syndrome is a rare neuropsychiatric disorder and a sequel of limbic encephalitis. Its presentation may vary in different ages. The patients may have all the features of Kluver Bucy Syndrome like – Hyper orality, Hyper sexuality, hyper metamorphosis, affective blunting, memory difficulties, eating disturbances etc; or may have only few of the symptoms which are commonly known as partial Kluver Bucy syndrome. Partial Kluver Bucy Syndrome is relatively more common than classic Kluver Bucy Syndrome. Anterior opercular syndrome is characterized by diminished voluntary control of facial, pharyngeal, lingual, and masticatory muscles. It usually follows bilateral lesions of the anterior opercular area surrounding the insula. In this case report, we highlight the clinical phenomenology and management issues of Kluver Bucy Syndrome with Anterior opercular syndrome in a young girl with limbic encephalitis.

Anterior Biopercular Syndrome Caused by Unilateral Infarction

The anterior biopercular syndrome is characterized by facio-pharyngo-glosso-masticatory diplegia, with automatic dissociation of movements. It generally translates bilateral opercular lesion, often of vascular etiology. There are very few cases described with unilateral lesions. We present the case of a patient with a bilateral anterior opercular syndrome caused by unilateral infarction.

Anterior Opercular Syndrome as a First Presentation of Herpes Simplex Encephalitis

We report a 5-year-old girl who presented with fever, drooling, dysphagia, and anarthria. Moreover, voluntary facial movements were disturbed, but the emotional facial movements were completely normal. This clinical phenomenon is known as the anterior opercular syndrome. There was a positive polymerase chain reaction for herpes simplex in the cerebrospinal fluid. The diagnosis herpes simplex encephalitis was supported by both magnetic resonance images (MRI) as by electroencephalogram (EEG). Herpes simplex encephalitis is a rare, but severe, cause of the anterior opercular syndrome that demands treatment as soon as possible in order to prevent high morbidity or mortality. The phenomenon of autonomic-voluntary dissociation, associated with other clinical and radiologic findings related to an underlying neurologic disorder, alerts clinicians to the anterior opercular syndrome as a critical diagnostic observation with time-dependent therapeutic consequences.

Anterior Opercular Syndrome Caused by Acute, Simultaneous, Isolated Bilateral Infarcts

Anterior Opercular Syndrome Caused by Acute, Simultaneous, Isolated Bilateral Infarcts

Bilateral anterior opercular (Foix–Chavany–Marie) syndrome

The bilateral anterior opercular (Foix–Chavany–Marie) syndrome is a rare condition most commonly encountered after bilateral middle cerebral artery stroke. Clinically, the syndrome is characterised by a loss of voluntary control of the facio-pharyngeo-glosso-masticatory muscles, while reflex movements and limb strength are preserved. We report the clinical presentation of a patient with anterior biopercular syndrome due to a bilateral middle cerebral artery stroke.

The Classical Type of Foix-Chavany-Marie Syndrome: Assessment and Treatment of Dysphagia

Foix-Chavany-Marie syndrome (FCMS) or anterior opercular syndrome is a rare neurological syndrome most commonly caused by cerebrovascular accident. The anatomical substrate of the classical (clinical type A) FCMS is constituted by any combination of bilateral cortical (opercular) or subcortical (subopercular) lesion, typically as a result of subsequent strokes. FCMS is characterized by facio-labio-pharyngo-glosso-masticatory paralysis with automatic-voluntary dissociation where involuntary movements of the affected muscles are preserved. A 79 year old woman with FCMS is presented to discuss the assessment,

Síndrome biopercular: presentación de dos casos y revisión de la literatura

The anterior opercular or biopercular syndrome is a cortical pseudobulbar palsy due to bilateral lesions of the anterior brain operculum. It is characterized by preservation of reflex function and automatic activity, without mental impairment. Two cases are reported herein and the relevant literature reviewed. The first case was a 73-year-old female with a history of a stroke occurring seven years previously, without sequelae in the interim. She presented with sudden loss of consciousness. The neurological examination showed a right facial central palsy and anarthria, with reflex acts such as smiling, blinking and yawning, not elicited by commands; she also had a right hemiparesis and walking impairment. A brain CT scan showed an old ischemic infarction in the region of the right medial cerebral artery. Because the right motor involvement did not correlate with the findings of the initial CT scan, another CT scan two days later showed an acute brain infarction in the vicinity of the left medial cerebral artery. The second case was an 8-year-old girl with mental retardation and impairment of verbal development, caused by of biopercular pachygyria. Facio-pharyngo-glosso-masticatory diplegia and volitional selective palsy of the oro-facial muscles was seen in both patients. The neuropsychological assessment showed cognitive, emotional and social interaction impairment in both cases -as part of the frontal convexity syndrome in the first case and of mental retardation in the second. The two patients had difficulty in mastication and swallowing. The prognosis for recovery of verbal capacity is poor, although generally most patients recover the ability to swallow.

Transient anterior opercular syndrome in a child with benign epilepsy with centrotemporal spikes complicated with continuous spike-waves during sleep

Transient anterior opercular syndrome in a child with benign epilepsy with centrotemporal spikes complicated with continuous spike-waves during sleep

Anterior opercular syndrome in frontotemporal lobar degeneration with ubiquitin-only immunoreactive neuronal changes

This paper presents for the first time an anterior opercular syndrome in association with a gait disorder, dropped head syndrome, dysphagia, and sialorrhea in a neuropathologically proven case of frontotemporal lobar degeneration with ubiquitin inclusions.

An autopsy case of frontotemporal dementia with severe dysarthria and motor neuron disease showing numerous basophilic inclusions

We report a clinicopathological study of a patient suffering from frontotemporal dementia (FLD) with severe dysarthria and concomitant motor neuron disease (MND). The patient was a 52-year-old woman with almost simultaneous emergence of severe dysarthria and FTD. The severe dysarthria subsequently evolved into anterior opercular syndrome. Motor neuron signs then emerged, and the patient developed akinetic mutism approximately 2 years after the onset of the disease. The patient died of pneumonia after a 7-year clinical illness. Pathologically, severe and widespread degeneration in the frontal and temporal lobes, including the anterior opercular area, limbic system, basal ganglia, spinal cord and cerebellum, and frequent ubiquitin- and tau-negative basophilic inclusions were observed. The pyramidal tracts and anterior horns of the cervical cord also showed marked degeneration. Cases showing basophilic inclusions reported so far have been divided into two groups: early onset FTD and MND with basophilic inclusions. Our case presented clinicopathological features of both FTD and MND, which suggests that cases showing basophilic inclusions may constitute a clinicopathological entity of FTD/MND.

A Case of Frontotemporal Lobar Degeneration with Progressive Dysarthria

We investigated the evolution of the neurological and neuropsychological characteristics in a right-handed woman who was 53-years-old at the onset and who showed personality changes and behavioral disorders accompanied by progressive dysarthria. She had hypernasality and a slow rate of speech with distorted consonants and vowels, which progressed as motor disturbances affecting her speech apparatus increased; finally, she became mute two years post onset. Her dysarthria due to bilateral voluntary facio-velo-linguo-pharyngeal paralysis accompanied with automatic-voluntary dissociation fit the description of anterior opercular syndrome. She showed personality changes and behavioral abnormalities from the initial stage of the disease, as is generally observed in frontotemporal degeneration (FTD), and her magnetic resonance image showed progressive atrophy in the frontotemporal lobes; thus, she was clinically diagnosed with FTLD. This patient’s symptoms suggest that FTLD, including bilateral anterior operculum degeneration, causes progressive pseudobulbar paretic dysarthria accompanied by clinical symptoms of FTD, which raises the possibility of a new clinical subtype in the FTLD spectrum.

Childhood Acute Disseminated Encephalomyelitis Presenting as Foix-Chavany-Marie Syndrome

A 10-year-old boy presented with anarthria and bilateral central faciolinguovelopharyngeomasticatory paralysis with "automatic voluntary dissociation" suggestive of Foix-Chavany-Marie syndrome or anterior opercular syndrome following a nonspecific upper respiratory infection. Brain magnetic resonance imaging revealed bilateral subcortical perisylvian demyelination in addition to multiple subcortical white-matter demyelination. The clinicoradiologic picture suggested acute disseminated encephalomyelitis. The child recovered completely following intravenous methylprednisolone and intravenous immunoglobulin therapy. This is the first report of anterior opercular syndrome in childhood acute disseminated encephalomyelitis.

Acute Bilateral Opercular Strokes Causing Loss of Emotional Facial Movements

The classic anterior opercular syndrome of Foix-Chavany-Marie presents with loss of voluntary facial, pharyngeal, lingual, and mastication movements, with preservation of emotional and automatic movements. Most commonly, sequential strokes affecting bilateral opercula cause this syndrome. The inverse clinical presentation, with selective loss of emotional facial movements, has only rarely been reported, and is less well-localized. We report a case of selective loss of emotional facial movements which resulted from bilateral acute infarcts. No etiology was discovered, and the syndrome was reversible. The available literature, and findings in this case, suggest that voluntary and automatic facial movements have distinct pathways, and damage to the insula bilaterally may lead to the selective loss of emotional facial movements. The clinical presentation of this inverse automatic/voluntary dissocation needs to be recognized as a rare syndrome with bilateral localization, so that patients at higher risk of further stroke can quickly be identified.

Síndrome opercular anterior como manifestación de estado epiléptico no convulsivo

Foix-Chavany-Marie syndrome, or bilateral anterior opercular syndrome, is characterised by facio-pharyngo-glosso-masticatory diplegia with 'automatic-voluntary dissociation', which consists in the abolition of voluntary movements while involuntary movements and reflexes are preserved. It is produced by bilateral involvement of the anterior or frontal opercular region. In adults it is related to ischemic lesions. In childhood it presents congenitally in perisylvian dysplasias and as an acquired disorder in encephalitis or can be episodic in symptomatic or idiopathic epilepsies such as benign rolandic epilepsy. A 13-year-old patient who presented, over five straight days, four episodes of facial dysplegia, anarthria, dysphagia, drooling, paralysis of the upper limbs, while involuntary facial expression was normal and the corneal, cough and gag reflexes were preserved. The first three come to an end spontaneously at 2, 4 and 20 hours, respectively; the fourth episode concluded an hour and a half after onset, following administration of intravenous phenytoin for 5 minutes. Computerised axial tomography and magnetic resonance images of the brain, as well as the interictal electroencephalograms (EEG), were normal. Administration of oxcarbazepine was started but at 8 months was stopped after a normal EEG during nocturnal sleep was obtained. After 15 months, the patient has not presented any more episodes. The paroxysmal character of the disorder together with normal interictal periods, the normality of the neuroimages, and the speedy recovery achieved after the administration of phenytoin support the notion of an epileptic origin. We believe that we are dealing with a bilateral anterior opercular syndrome due to a non-convulsive epileptic state, compatible with the presentation of benign rolandic epilepsy.

The MRI Appearances of Anterior Opercular Syndrome in a Child with Recurrent Herpes Simplex Encephalitis: Case Reports

The MRI Appearances of Anterior Opercular Syndrome in a Child with Recurrent Herpes Simplex Encephalitis: Case Reports

Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes

We describe eight patients with slowly progressive speech production deficit combining speech apraxia, dysarthria, dysprosody and orofacial apraxia, and initially no other deficit in other language and non-language neuropsychological domains. Long-term follow-up (6–10 years) in 4 cases showed an evolution to muteness, bilateral suprabulbar paresis with automatic-voluntary dissociation and frontal lobe cognitive slowing without generalised intellectual deterioration. Most disabled patients presented with an anterior opercular syndrome (Foix-Chavany-Marie syndrome), and pyramidal or extrapyramidal signs. CT and MRI findings disclosed asymmetric (left > right) progressive cortical atrophy of the frontal lobes predominating in the posterior inferior frontal region, notably the operculum. SPECT and PET revealed a decreased cerebral blood flow and metabolism, prominent in the left posterior-inferior frontal gyrus and premotor cortex, extending bilaterally in the most advanced cases. Pathological study of two cases showed non-specific neuronal loss, gliosis, and spongiosis of superficial cortical layers, mainly confined to the frontal lobes, with no significant abnormalities in the basal ganglia, thalamus, cerebellum, brain stem (except severe neuronal loss in the substantia nigra in one case), and spinal cord. We propose to call this peculiar syndrome Slowly Progressive Anarthria (SPA), based on its specific clinical presentation, and its metabolic and pathological correlates. SPA represents another clinical expression of focal cortical degeneration syndromes, that may overlap with other similar syndromes, specially primary progressive aphasia and the various frontal lobe dementias.