Title : Opercular syndrome as a sequelae of herpes simplex virus infection: A case report
Abstract:
Introduction: Opercular Syndrome also known as Foix-Chavany-Marie Syndrome (FCMS) is a rare cortical form of pseudobulbar palsy that preserves reflexive and autonomic functions but results in voluntary paralysis of the masticatory, lingual, pharyngeal, and facial muscles. The estimated prevalence of <1/1,000,000. The dissociation between autonomic and voluntary muscle actions may be explained by different neural pathways that connect the brainstem to the amygdala and hypothalamus. Although unilateral lesions or isolated pontine infarcts have been documented, bilateral sequential anterior opercular or subcortical insular infarcts are usually the cause of FCMS. Epstein-Barr virus (EBV) and Herpes Simplex virus (HSV) are the uncommon causes of FCMS . We report a 26-year-old male case of ischemic bilateral opercular syndrome caused by HSV infection.
Case report: A 26 year male patient presented with complains of headache, slurred speech, and focal seizures for 2 days. During presentation he developed multiple focal onset seizures with a decline in Glasgow Coma Scale (GCS-7, E3V1M3), necessitating intubation. Imaging(MRI Scan) showed an infarction in the bilateral insular cortex, and further clinical examinations verified characteristics that were in line with FCMS. Notably, HSV and EBV infections were detected by Polymerase Chain Reaction(PCR) in Lumbar Puncture. Amidst a complex clinical trajectory necessitating comprehensive interdisciplinary approach spanning neurology, infectious diseases, critical care, and the ENT department, the patient underwent a multifaceted treatment approach. Antiviral therapy and respiratory support were complemented by physiotherapeutic interventions and speech therapy. After a month of intubation, successful extubation was achieved. Subsequent improvements in feeding and speech function were observed, with voluntary control nearing normalization, underscoring the efficacy of the integrated therapeutic regimen.
Conclusion: This case highlights the viral infection as a causal factor for FCMS. Timely recognition and comprehensive management involving various medical specialties are essential for optimizing outcomes in patients with this rare neurological condition. Increased awareness of FCMS and its associations with viral etiologies are crucial for facilitating prompt diagnosis and effective intervention.
Audience Take Away Notes:
- Recognizing the clinical features of Opercular Syndrome, including speech abnormalities and facial muscle paralysis, is crucial for prompt diagnosis by neurologists.
- Neurologists should be vigilant about rare etiologies such as viral infections, like Herpes Simplex virus (HSV) and Epstein-Barr virus (EBV), which can cause bilateral opercular syndrome.
- A thorough diagnostic workup, including neuroimaging and cerebrospinal fluid analysis, is essential to detect viral infections in young patients presenting with acute neurological symptoms.
- Interdisciplinary collaboration between neurology, infectious diseases, critical care, and other specialties ensures comprehensive care and optimal management of patients with Opercular Syndrome.
- An integrated treatment approach, combining antiviral therapy, respiratory support, physiotherapy, and speech therapy, promotes recovery and restoration of voluntary muscle control in patients with Opercular Syndrome.
- Despite initial severity, patients with Opercular Syndrome can show significant improvement over time with appropriate interventions, emphasizing the importance of rehabilitation and comprehensive care in achieving favorable outcomes.