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 [journals.iupui.edu]

We present a biopercular syndrome associated with HIV. A 41 year old female who presents a progressive automatic- voluntary motor dissociation of face and lower cranial nerves with later involvement of limbs. [biomedsearch.com]

In the previous 24 hours, she had presented 3 episodes suggestive of focal seizures, of 5 min duration each: the first episode consisted of tonic rigidity of the upper limbs preceded by snoring during sleep; in the second episode, she presented jaw rigidity [elsevier.es]

• Epilepsy

  Acquired bilateral opercular lesions or Foix-Chavany-Marie syndrome and eating epilepsy. J Neurol Neurosurg Psychiatry. 1995;59(5):559–560. 5. Sasaguri H, Sodeyama N, Maejima Y, Kanda T, Mizusawa H. [dovepress.com]

  In children, FCMS is usually associated with delayed psychomotor milestones and epilepsy. Etiology FCMS is caused by developmental or acquired bilateral lesions of the anterior opercula. [orpha.net]

  […] and most common form associated with cerebrovascular disease, (b) a subacute form caused by central nervous system infections, (c) a developmental form probably most often related to neuronal migration disorders, (d) a reversible form in children with epilepsy [ncbi.nlm.nih.gov]

  The underlying pathogenesis of transient form is epilepsy. [jnnp.bmj.com]

  Bilateral central macrogyria: epilepsy, pseudobulbar palsy, and mental retarda- tion-a recognizable neuronal migration disorder. Ann Neurol 2. Graff-Radford NR, Bosch EP, Stears JC, Tranel D. [docslide.com.br]

• Sepsis

  The side effects of this medicine are: Weight gain, (particularly in the face and trunk), metabolic abnormalities, hypertension, severe irritability, sepsis, osteoporosis and congestive heart failure. [epainassist.com]

• Pharyngitis

  As involuntary movements were relatively preserved and no sensory loss was suspected, it was hypothesised that, once initiated or triggered, pharyngeal swallowing could be adequate. [ncbi.nlm.nih.gov]

  Bilateral trigemino-facio-linguo-pharyngeal palsy was noticed on volitional control but automatic and emotional motor movements were well preserved. [jpgmonline.com]

  There have further been case reports of maintenance of the pharyngeal stage [ 19 ]. [omicsonline.org]

  This syndrome consists of anarthria, bilateral volitional paresis of the facial, lingual, pharyngeal, and masticatory muscles bilaterally with preservation of the reflexive, emotional and autonomic innervations of the same muscles. [journals.ju.edu.jo]

• Yawning

  Automatic movements, such as laughter and yawning, were preserved (video 1 and figure). She was diagnosed with Foix-Chavany-Marie syndrome (FCMS). [n.neurology.org]

  Thus, although volitional movements are absent, emotional smiling, laughter, crying, and automatic yawning are preserved. [medlink.com]

  These patients are unable to open or close their mouth, protrude the tongue or close their eyes on command; however, they can still smile, cry, yawn or yell in an automatic manner. [kundoc.com]

  Thus, although volitional movements are absent, emotional smiling, laughter, crying, and automatic yawning are preserved… CONTINUE READING [semanticscholar.org]

  He did, however, blink in response to threat, smile when his wife came to the bedside, and swallow and yawn spontaneously. [jamanetwork.com]

• Dysphagia

  Materials and Methods: A 62-year-old man was admitted to the emergency department after 9 hours of acute dysarthria and dysphagia. MRI showed restricted diffusion in the right operculum on diffusion-weighted imaging (DWI). [ejcrim.com]

  F, Despland PA, Opercular Cheiro-Oral Syndrome, US National Library of Medicine, National Institute of Health, PubMed.gov, https://www.ncbi.nlm.nih.gov/pubmed/2039391 I Chernev, R Petrea, M Reynolds, F Wang, Assessment and Treatment of Dysphagia, The [wwjournals.com]

  Keywords: Dysarthria, dysphagia, opercular syndrome, seizures How to cite this article: Desai SD, Patel D, Bharani S, Kharod N. Opercular syndrome: A case report and review. [jpgmonline.com]

  He also had a severe dysphagia. Radiological studies revealed an acute stroke in the left anterior operculum, indicating Foix-Chavany-Marie Syndrome (FCMS) caused by a unilateral opercular lesion. [ncbi.nlm.nih.gov]

  The neurological examination revealed anarthria, central facial diplegia, dysphagia, and paralysis of tongue and jaw movements. She could, however, yawn and laugh. [kundoc.com]

• Gagging

  The twins, aged 41 years, were the product of a normal pregnancy and birth, but had subsequent delayed motor milestones, seizures, poor language development, mild mental retardation, drooling, absent gag reflexes, inability to protrude the tongue, brisk [mayoclinic.pure.elsevier.com]

  Further examination identified bilateral central facial weakness and loss of the gag reflex. Yawning was otherwise preserved resulting in a clinical diagnosis of FCMS. [content.sciendo.com]

  His gag reflex was decreased and emotional incontinence was absent. Brain magnetic res-onance (MR) imaging revealed high signal lesions in the right middle cerebral artery territory and left anterior opercu-lum. [jkna.org]

  His examination results were significant for volitional paralysis of his bilateral upper and lower face, including the orbicularis oculi, orbicularis oris, and masseter muscles, anarthria, absence of gag reflex, inability to swallow on command, and inability [jamanetwork.com]

• Trismus

  The jaw reflex is exaggerated (sometimes leading to trismus). In children, FCMS is usually associated with delayed psychomotor milestones and epilepsy. Etiology FCMS is caused by developmental or acquired bilateral lesions of the anterior opercula. [orpha.net]

  Eur J Case Rep Intern Med. 2021, 8:002462. 10.12890/2021_002462 Giannini L, Maccari A, Chiesa V, Canevini MP: Trismus, the first symptom in a challenging diagnosis of tetanus. [cureus.com]

• Thrombosis

  A 74-year-old man with metastatic pancreatic cancer and a recent deep vein thrombosis (for which he was taking enoxaparin sodium) was brought to our emergency department by his wife. [jamanetwork.com]

  […] same author(s) Eduardo Soriano-Navarro, Vanessa Cano-Nigenda, Fernanda Menéndez-Manjarrez, Esmirna Farington-Terrero, Juan José Méndez-Gallardo, Alejandro García-Irigoyen, Antonio Arauz, Bilateral Decompressive Craniectomy in Malignant Cerebral Venous Thrombosis [ejcrim.com]

  MRA of brain showed thrombosis of right internal carotid artery. [annalsofian.org]

  Polycythemia vera causing cerebral venous thrombosis, Bombay Hospital Journal vol52,2010 pg. 122 A rare case of mercurial encephalopathy .Bombay Hospital Journal .vol 53 no 4 2011pg 796 Adult onset stills disease, Bombay Hospital Journal, vol 54 2012 [drhemantgupta.com]

• Muscular Atrophy

  The tongue is almost immobile and does not show muscular atrophy and fibrillation. The jaw reflex is exaggerated (sometimes leading to trismus). In children, FCMS is usually associated with delayed psychomotor milestones and epilepsy. [orpha.net]

• Incontinence

  Intermittent incontinence and suspected hemifacial seizures. The MRI (day 6) showed cytotoxic edema bilaterally in the insula/operculum as well as in the right temporobasal, temporomesial, and right frontobasal areas. [thieme-connect.com]

  Her salivary incontinence was ameliorated by administration of an anticholinergic drug (trihexyphenidyl 2 mg twice daily). [dovepress.com]

  His gag reflex was decreased and emotional incontinence was absent. Brain magnetic res-onance (MR) imaging revealed high signal lesions in the right middle cerebral artery territory and left anterior opercu-lum. [jkna.org]

  […] form of pseudobulbar palsy is clinically distinguished from the noncortical type of pseudobulbar palsy by its acute onset, prominent voluntary-automatic dissociation of the bulbar muscles, absence of emotional lability, and absence of bowel and bladder incontinence [medlink.com]

• Stroke

  Roberto Abel Toledo-Trevino Stroke Clinic, National Institute of Neurology and Neurosurgery Manuel Velasco Suárez, Mexico City, Mexico Diana Manrique-Otero Stroke Clinic, National Institute of Neurology and Neurosurgery Manuel Velasco Suárez, Mexico City [ejcrim.com]

  Baseline disease activity, hyperlipemia, and hypertension are predictive factors for ischemic stroke and stroke severity in systemic lupus erythematosus. Stroke. 2007;38:281-5. 5. [kundoc.com]

  Jones First published May 27, 2019, DOI: https://doi.org/10.1212/WNL.0000000000007569 A 75-year-old woman with a history of multiple resolved hemiparetic strokes presented with unilateral ischemic stroke causing sudden loss of volitional control of the [n.neurology.org]

  Results: The MRI brain showed left insular region/frontal opercular ischemic stroke and a small right parietal subcortical ischemic stroke, likely embolic in nature. [journals.iupui.edu]

• Dysarthria

  He had dysarthria, nasal twang, and drooling of saliva with difficulty in chewing and swallowing. Hearing and understanding were normal. [jpgmonline.com]

  However, the dysarthria remained prominent. Multi-modal rehabilitation therapy may be helpful in the recovery of FCMS. [longdom.org]

  Typical FCMS patients present with severe dysarthria and dysphagia. They are mute or can only produce inarticulate sounds; however, they can comprehend conversations and communicate through writing or gestures. [questia.com]

  In Worster-Drought Syndrome (WDS), dysarthria is remarkable. We report an 18-year-old female, with clinical and radiological findings of intermediary form of FCMS/WDS, and showing involuntary movements, an unusual fact. [curehunter.com]

  Foix-Chavany-Marie syndrome (FCMS) is a cortico-subcortical suprabulbar or pseudobulbar palsy of the lower cranial nerves, characterized by severe dysarthria and dysphagia associated with bilateral central facio-pharyngo-glosso-masticatory paralysis, [monarchinitiative.org]

• Apraxia

  Facio-linguo-velopharyngeo-masticatory diplegia with automatic-voluntary dissociation was accompanied by motor aphasia and oral apraxia leading to a state of almost complete anarthria. [ncbi.nlm.nih.gov]

  Abstract The Foix-Chavany-Marie syndrome (FCMS) is characterized by apraxia of speech associated to bilateral central facio-linguo-velo-pharyngeal paralysis, with automatic-voluntary dissociation. [curehunter.com]

  The symptoms include linguo-bucco-facial apraxia with facial weakness, drooling, palatal and lingual speech disorders, masticatory problems, and jaw jerks. Bibliography C. Foix, J. A. Chavany, J. [whonamedit.com]

  The typical clinical manifestations of this syndrome include severe dysarthria, aphasia (expressive, receptive or both), generalized facial weakness, orofacial apraxia, and difficulty chewing and swallowing, without mental impairment. [n.neurology.org]

• Involuntary Movements

  movements, also called “automatic-voluntary dissociation”. [longdom.org]

  movements of the affected muscles are preserved. [monarchinitiative.org]

  It was described in 1926 by Charles Foix, Jean Alfred Émile Chavany and Julien Marie in the Revue Neurologique.[1] As a characteristic, there is no paralysis of upper or lower limbs and those affected can still make involuntary movement like smiling, [bionity.com]

  It was described in 1926 by Charles Foix, Jean Alfred Émile Chavany and Julien Marie in the Revue Neurologique. [1] As a characteristic, there is no paralysis of upper or lower limbs and those affected can still make involuntary movement like smiling, [wikidoc.org]

• Slurred Speech

  Here, we report the clinical and neuroradiological characteristics of a patient with FCMS due to sequential, bilateral, and symmetrical infarcts of corona radiata, presenting with slurred speech, severe hypophonia, and right central‐type facial palsy [onlinelibrary.wiley.com]

  A 9-year-old male presented with unclear and slurred speech, drooling of saliva and inability to protrude tongue and difficulty swallowing since early childhood. [jpgmonline.com]

  Unclear and slurred speech, as well as problems forming certain sounds, are signs of dysarthia whith as associated drooling and swallowing difficulties. [neuroradiologycases.com]

She was taken back to the operating room 7 days later after further workup was negative for a shunt revision to a medium pressure valve without an anti siphon device. She remained neurologically the same. [academic.oup.com]

Hypercoagulability workup: coagulation factors, antithrombin III, protein C resistance, prothrombin gene 20210, protein C and homocysteine with no alterations. Free protein S antigen at 45% (normal > 50). [kundoc.com]

In these patients, treatment with oral anticoagulants has proven effective in reducing new cerebrovascular12 events, although we must not forget aggressive treatment of vascular risk factors4. [kundoc.com]

[…] and behavioralñhealth interventions for impairments created by cancers and their treatments Represents a collaboration between some of the foremost scholars and practitioners in neuropsychology and oncology [books.google.com]

The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]

There was no further deterioration in her neurological state after these treatments. [jnnp.bmj.com]

Treatment for West's Syndrome The treatment of West's syndrome is difficult when compared to other types of epilepsy. [epainassist.com]

Prognosis The disease may have a persistent or intermittent course. Reversible forms have also been described. The prognosis for life expectancy is usually good, but usually patients do not recover voluntary swallowing and speech ability. [orpha.net]

The prognosis is favorable, but the patient should be informed of this particular hazard, and the surgeon should anticipate the surgical strategy in case the syndrome occurs intraoperatively in an awake patient. [ncbi.nlm.nih.gov]

Prognosis & Life Expectancy for West's Syndrome The prognosis is good for children having idiopathic West's syndrome when compared to prognosis of children with cryptogenic or symptomatic type of West's syndrome. [epainassist.com]

Prognosis - Foix Chavany Marie syndrome Not supplied. Treatment - Foix Chavany Marie syndrome Not supplied. Resources - Foix Chavany Marie syndrome Not supplied. [checkorphan.org]

Routine investigations were done to screen for etiological factors. [paper.researchbib.com]

In review of previous reports, the most common etiology of FCMS is stroke in the region of either operculum. [ncbi.nlm.nih.gov]

Export Citation: APA/MLA Format Download EndNote Download BibTex MeSH Terms Descriptor/Qualifier: Brain / radiography Cerebral Infarction / complications, radiography Cerebral Palsy / etiology, physiopathology* Facial Muscles / physiopathology* Female [biomedsearch.com]

The prognosis is variable, dependent on the severity of clinical disease, the type of the etiology, and the time of diagnosis. [jpgmonline.com]

The common etiologies include stroke in the regions of bilateral opercula, infections of the central nervous system such as HSV and CMV, neoplasms, trauma and epileptic disorders. [n.neurology.org]

Summary Epidemiology Less than 150 cases have been described in the literature so far. [orpha.net]

Epidemiology of Ideapathic Normal Pressure Hydrocephalus: A systematic Review of the literature. Martín Láez R, Caballero-Arzapalo H, López Menéndez LA, Arango-Lasprilla JC, Vázquez- Barquero A. [neurocirugia.humv.es]

Pathophysiology of the previous cases reported as unilateral FCMS remains controversial, but in our case, it could be delineated by the combination of the new lesion in the unilateral operculum and the old one in the contralateral pons. [ncbi.nlm.nih.gov]

In this article, the author explains the clinical presentation and pathophysiology of Foix-Chavany-Marie syndrome (bilateral anterior opercular syndrome). [semanticscholar.org]

Firstly, the Landau–Kleffner syndrome (and there may well be shared pathophysiological mechanisms) and secondly, those first described by Worster–Drought. [rcpe.ac.uk]

Overview In this article, the author explains the clinical presentation and pathophysiology of Foix-Chavany-Marie syndrome (bilateral anterior opercular syndrome). [medlink.com]

Results: The patient was discharged 5 days later, with a modified Rankin scale (mRs) score of 3, and secondary stroke prevention. He had achieved an excellent functional outcome (mRs 1) at 6-month follow-up. [ejcrim.com]

[…] minus' sign Virchow -triad Use Parentheses water AND (cup OR glass) Add an asterisk (*) at end of a word to include word stems Neuro* will search for Neurology, Neuroscientist, Neurological, and so on Use quotes to search for an exact phrase "primary prevention [read.qxmd.com]

Prevention - Foix Chavany Marie syndrome Not supplied. Diagnosis - Foix Chavany Marie syndrome The diagnosis is made with CT scan and MRI. Most of the time the lesion is seen on both side of the brain, in the Operculum. [checkorphan.org]

She was briefly anticoagulated with heparin, which was discontinued to prevent hemorrhagic conversion. Her antihypertensive medications were also held to optimize cerebral blood flow. [cureus.com]

If the cause can be established early, early treatment may prevent residual sequelae. 1. Gordon N. The opercular syndrome, or, the Foix-Chavany-Marie syndrome. J R Coll Physicians Edinb 2007;37:103-6. 2. Thapa L, Paudel R, Rana PV. [jpgmonline.com]