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Neurosarcoidosis: A Diagnostic Challenge.

Kiruba Shankar Manoharan1, Agalya Pauline Vasanthi Leoni1, Deepa Mary Samuel1

  • 1Deptartment of ENT, Skull Base, Head and Neck Surgery, Apollo Main Hospitals, Chennai, 600006 Tamilnadu India.

Indian Journal of Otolaryngology and Head and Neck Surgery : Official Publication of the Association of Otolaryngologists of India
|April 14, 2025
PubMed
Summary

Neurosarcoidosis mimicking other conditions presents a diagnostic challenge. Biopsy confirmed non-caseating granuloma, leading to diagnosis and successful steroid treatment for cranial nerve involvement.

Keywords:
Endoscopic transnasal skullbase biopsyInfratemporal fossaNeurosarcoidosis of cranial nervesNon-caseating granuloma

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Area of Science:

  • Neurology
  • Pathology

Background:

  • Neurosarcoidosis with isolated cranial nerve involvement poses diagnostic challenges, mimicking conditions like infections, vasculitis, IgG4 disease, and malignancy.
  • Early diagnosis and treatment are crucial for managing neurosarcoidosis and preventing long-term neurological deficits.

Purpose of the Study:

  • To highlight the diagnostic challenges of neurosarcoidosis presenting with isolated cranial nerve involvement.
  • To present a case report illustrating the diagnostic pathway and successful management of neurosarcoidosis.

Main Methods:

  • A case study of a 59-year-old female with symptoms of facial pain, headache, and diplopia.
  • Clinical examination revealed 5th and 6th cranial nerve involvement.
  • Diagnostic imaging included MRI and PET-CT.
  • Histopathological examination of nerve biopsy confirmed non-caseating granuloma.

Main Results:

  • MRI showed enhancing soft tissue lesions in the pterygopalatine fossa and pterygomaxillary fissure, extending along V2 and V3.
  • PET-CT revealed a hypermetabolic lesion along the left mandibular nerve.
  • Vasculitis panel was inconclusive.
  • Biopsy confirmed non-caseating granuloma, establishing the diagnosis of neurosarcoidosis.

Conclusions:

  • Isolated cranial nerve involvement in neurosarcoidosis is a diagnostic challenge requiring a high index of suspicion.
  • Histopathological confirmation of non-caseating granuloma, excluding infection and malignancy, is key to diagnosis.
  • Steroid therapy can lead to significant clinical and symptomatic improvement in neurosarcoidosis.