Learning objectives
To present clinical presentation,
magnetic resonance imaging (MRI) findings,
and relevant diagnostic criteria observed in 22 patients with neurosarcoidosis (NS).
Background
Sarcoidosis is a long-term inflammatory systemic disease that manifests as non caseating granulomas.
It's a disorder of undetermined aetiology and any organ can be affected but more commonly are interested respiratory and lymphatic system.
When the nervous system is involved is termed Neurosarcoidosis.
Even though localizations to central nervous system (CNS) typically accounts for 5-15% of all cases,
neurological impairment represents first clinical evidence in more than half of them.
Autoptic studies reveal a higher prevalence of CNS involvement.
Neurosarcoidosis can occur in people with...
Findings and procedure details
From a series of more than 400 sarcoidosis Patients,
a retrospective review of clinical and neuroradiological charts identified 22 affected by CNS manifestations:
- 13 males,
9 females;
- mean age at first neurological presentation: 44 years.
At first admission,
-systemic disease was present in 20 (91%) Patients,
-isolated NS in the remaining 2 (9%).
-Ten Patients showed others coexistent immune-mediate disorders.
CNS localizations included brain,
meninges,
spinal cord,
cranial nerves and hypothalamus/hypophysis.
Findings in more than one site for 7 (32%) Patients.
MRI showed...
Conclusion
Most adopted diagnostic criteria have their advantages and disadvantages,
so that isolated neurosarcoidosis needs radiological expertise in Patients for whom CNS biopsy is not affordable.
Specific CSF biomarkers are under promising development,
such as chitotriosidase,
but at present time collaboration among neuroradiologist,
neurologist and pulmonologist is imperative in order to correctly evaluate Patients’ diagnosis,
treatment regimen and timing of follow-up.
References
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Dubaniewicz-Wybieralska M,
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J Neurol 262:258–267
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Bennett D,
Maggiorelli C,
Di Sipio P,
Margollicci M,
Bianchi N,
Rottoli P (2013) Human Chitotriosidase: a sensitive biomarker of sarcoidosis.
J Clin Immunol 33:264–270
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Lee HK,
Park JK,
Choi CG,
Suh DC (2003) Cavernous sinus syndrome: clinical features and differential diagnosis with MR imaging.
AJR Am J Roentgenol 181:583–590