Cranial base manifestations of neurosarcoidosis

A review of 305 patients

Matthew L. Carlson, James R. White, Mana Espahbodi, David S. Haynes, Colin L.W. Driscoll, Allen Jr. Aksamit, Siddharama Pawate, John I. Lane, Michael J. Link

Research output: Contribution to journalReview article

25 Citations (Scopus)

Abstract

Objective: Neurosarcoidosis is a rare granulomatous disease that can result in cranial neuropathy, chronic meningitis, and intracranial granuloma formation. Meningeal involvement may cause focal nodular enhancement that can simulate common cranial base tumors. The objective of the current study is to further define the clinical features of neurosarcoidosis in a large cohort of patients, focusing on characteristics relevant to the skull base surgeon.

Study Design: Retrospective series. Setting: Two tertiary academic referral centers.

Patients: Consecutive patients diagnosed with neurosarcoidosis. Intervention(s): Review of clinical presentation, physical examination, radiologic findings, biopsy results, and laboratory testing.

Main Outcome Measures: Prevalence and distribution of cranial neuropathy, radiologic features of meningeal enhancement, and patterns of simulated tumors.

Results: A total of 305 patients met study criteria. The mean age at diagnosis was 47 years and 53% were female. The optic nerve was the most commonly involved cranial nerve, followed by the trigeminal and the facial nerve. Meningeal enhancement was present in 67% of cases with 17% demonstrating focal or multicentric nodular enhancement simulating tumor. The most common locations of inflammatory tumor development included the cavernous sinus, petrous temporal bone, and sphenoid wing; six patients had bilateral internal auditory canal lesions, several mimicking neurofibromatosis type II.

Conclusion: Establishing the diagnosis of neurosarcoidosis remains challenging. Meningeal involvement and cranial neuropathy often mimic other more common conditions. Careful review of patient history and clinical imaging can reveal important clues toward the diagnosis of neurosarcoidosis. The clinician must maintain a high index of suspicion in patients with atypical presentation to avoid misdiagnosis and facilitate early medical treatment.

Original languageEnglish (US)
Pages (from-to)156-166
Number of pages11
JournalOtology and Neurotology
Volume36
Issue number1
StatePublished - Jan 20 2015

Fingerprint

Skull Base
Cranial Nerve Diseases
Neoplasms
Petrous Bone
Neurofibromatosis 2
Cavernous Sinus
Trigeminal Nerve
Cranial Nerves
Temporal Bone
Facial Nerve
Optic Nerve
Rare Diseases
Neurosarcoidosis
Granuloma
Diagnostic Errors
Meningitis
Tertiary Care Centers
Physical Examination
Retrospective Studies
Outcome Assessment (Health Care)

Keywords

  • Cranial base
  • Cranial nerves
  • Neurosarcoidosis
  • Sarcoidosis

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Sensory Systems
  • Clinical Neurology

Cite this

Carlson, M. L., White, J. R., Espahbodi, M., Haynes, D. S., Driscoll, C. L. W., Aksamit, A. J., ... Link, M. J. (2015). Cranial base manifestations of neurosarcoidosis: A review of 305 patients. Otology and Neurotology, 36(1), 156-166.

Cranial base manifestations of neurosarcoidosis : A review of 305 patients. / Carlson, Matthew L.; White, James R.; Espahbodi, Mana; Haynes, David S.; Driscoll, Colin L.W.; Aksamit, Allen Jr.; Pawate, Siddharama; Lane, John I.; Link, Michael J.

In: Otology and Neurotology, Vol. 36, No. 1, 20.01.2015, p. 156-166.

Research output: Contribution to journalReview article

Carlson, ML, White, JR, Espahbodi, M, Haynes, DS, Driscoll, CLW, Aksamit, AJ, Pawate, S, Lane, JI & Link, MJ 2015, 'Cranial base manifestations of neurosarcoidosis: A review of 305 patients', Otology and Neurotology, vol. 36, no. 1, pp. 156-166.
Carlson ML, White JR, Espahbodi M, Haynes DS, Driscoll CLW, Aksamit AJ et al. Cranial base manifestations of neurosarcoidosis: A review of 305 patients. Otology and Neurotology. 2015 Jan 20;36(1):156-166.
Carlson, Matthew L. ; White, James R. ; Espahbodi, Mana ; Haynes, David S. ; Driscoll, Colin L.W. ; Aksamit, Allen Jr. ; Pawate, Siddharama ; Lane, John I. ; Link, Michael J. / Cranial base manifestations of neurosarcoidosis : A review of 305 patients. In: Otology and Neurotology. 2015 ; Vol. 36, No. 1. pp. 156-166.
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abstract = "Objective: Neurosarcoidosis is a rare granulomatous disease that can result in cranial neuropathy, chronic meningitis, and intracranial granuloma formation. Meningeal involvement may cause focal nodular enhancement that can simulate common cranial base tumors. The objective of the current study is to further define the clinical features of neurosarcoidosis in a large cohort of patients, focusing on characteristics relevant to the skull base surgeon.Study Design: Retrospective series. Setting: Two tertiary academic referral centers.Patients: Consecutive patients diagnosed with neurosarcoidosis. Intervention(s): Review of clinical presentation, physical examination, radiologic findings, biopsy results, and laboratory testing.Main Outcome Measures: Prevalence and distribution of cranial neuropathy, radiologic features of meningeal enhancement, and patterns of simulated tumors.Results: A total of 305 patients met study criteria. The mean age at diagnosis was 47 years and 53{\%} were female. The optic nerve was the most commonly involved cranial nerve, followed by the trigeminal and the facial nerve. Meningeal enhancement was present in 67{\%} of cases with 17{\%} demonstrating focal or multicentric nodular enhancement simulating tumor. The most common locations of inflammatory tumor development included the cavernous sinus, petrous temporal bone, and sphenoid wing; six patients had bilateral internal auditory canal lesions, several mimicking neurofibromatosis type II.Conclusion: Establishing the diagnosis of neurosarcoidosis remains challenging. Meningeal involvement and cranial neuropathy often mimic other more common conditions. Careful review of patient history and clinical imaging can reveal important clues toward the diagnosis of neurosarcoidosis. The clinician must maintain a high index of suspicion in patients with atypical presentation to avoid misdiagnosis and facilitate early medical treatment.",
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AU - Haynes, David S.

AU - Driscoll, Colin L.W.

AU - Aksamit, Allen Jr.

AU - Pawate, Siddharama

AU - Lane, John I.

AU - Link, Michael J.

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N2 - Objective: Neurosarcoidosis is a rare granulomatous disease that can result in cranial neuropathy, chronic meningitis, and intracranial granuloma formation. Meningeal involvement may cause focal nodular enhancement that can simulate common cranial base tumors. The objective of the current study is to further define the clinical features of neurosarcoidosis in a large cohort of patients, focusing on characteristics relevant to the skull base surgeon.Study Design: Retrospective series. Setting: Two tertiary academic referral centers.Patients: Consecutive patients diagnosed with neurosarcoidosis. Intervention(s): Review of clinical presentation, physical examination, radiologic findings, biopsy results, and laboratory testing.Main Outcome Measures: Prevalence and distribution of cranial neuropathy, radiologic features of meningeal enhancement, and patterns of simulated tumors.Results: A total of 305 patients met study criteria. The mean age at diagnosis was 47 years and 53% were female. The optic nerve was the most commonly involved cranial nerve, followed by the trigeminal and the facial nerve. Meningeal enhancement was present in 67% of cases with 17% demonstrating focal or multicentric nodular enhancement simulating tumor. The most common locations of inflammatory tumor development included the cavernous sinus, petrous temporal bone, and sphenoid wing; six patients had bilateral internal auditory canal lesions, several mimicking neurofibromatosis type II.Conclusion: Establishing the diagnosis of neurosarcoidosis remains challenging. Meningeal involvement and cranial neuropathy often mimic other more common conditions. Careful review of patient history and clinical imaging can reveal important clues toward the diagnosis of neurosarcoidosis. The clinician must maintain a high index of suspicion in patients with atypical presentation to avoid misdiagnosis and facilitate early medical treatment.

AB - Objective: Neurosarcoidosis is a rare granulomatous disease that can result in cranial neuropathy, chronic meningitis, and intracranial granuloma formation. Meningeal involvement may cause focal nodular enhancement that can simulate common cranial base tumors. The objective of the current study is to further define the clinical features of neurosarcoidosis in a large cohort of patients, focusing on characteristics relevant to the skull base surgeon.Study Design: Retrospective series. Setting: Two tertiary academic referral centers.Patients: Consecutive patients diagnosed with neurosarcoidosis. Intervention(s): Review of clinical presentation, physical examination, radiologic findings, biopsy results, and laboratory testing.Main Outcome Measures: Prevalence and distribution of cranial neuropathy, radiologic features of meningeal enhancement, and patterns of simulated tumors.Results: A total of 305 patients met study criteria. The mean age at diagnosis was 47 years and 53% were female. The optic nerve was the most commonly involved cranial nerve, followed by the trigeminal and the facial nerve. Meningeal enhancement was present in 67% of cases with 17% demonstrating focal or multicentric nodular enhancement simulating tumor. The most common locations of inflammatory tumor development included the cavernous sinus, petrous temporal bone, and sphenoid wing; six patients had bilateral internal auditory canal lesions, several mimicking neurofibromatosis type II.Conclusion: Establishing the diagnosis of neurosarcoidosis remains challenging. Meningeal involvement and cranial neuropathy often mimic other more common conditions. Careful review of patient history and clinical imaging can reveal important clues toward the diagnosis of neurosarcoidosis. The clinician must maintain a high index of suspicion in patients with atypical presentation to avoid misdiagnosis and facilitate early medical treatment.

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