Rare organ manifestations of sarcoidosis

Albaba, Isam; Feustel, Paul J.; Kenneth, M. Fish; Judson, Marc A.

doi:10.1016/j.rmed.2022.106945

Cited by 4 publications

(2 citation statements)

References 8 publications

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“…Sarcoidosis, especially isolated organ involvement types such as neurosarcoidosis (NS), is far less likely to be high on the list of differential diagnoses for patients in the non-endemic areas of the US, such as our Southwestern US region, than for those in the areas of high prevalence. While sarcoidosis most commonly involves the lungs, skin, and eyes ( 4 ), sarcoid can affect any organ system in the body ( 5 , 6 ), and is highly diverse when involving the nervous system ( 7–9 ). NS is identified in 5–10% of patients, in half of which it may be the initial clinical presentation ( 10 ).…”

Section: Introductionmentioning

confidence: 99%

“…Common or “usual” neurologic manifestations include cranial nerve palsy, peripheral neuropathy, seizures, and neuropsychological and cerebellar symptoms. Less common clinical symptoms include hydrocephalus and aseptic meningitis ( 6 ). Typical imaging findings of NS include multiple white matter lesions coupled with meningeal enhancement or “sugarcoating” near the skull base, as well as focal nodular enhancement ( 14 ).…”

Section: Introductionmentioning

confidence: 99%

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Diagnostic challenges of neurosarcoidosis in non-endemic areas

Shrestha,

Kleinschmidt-DeMasters,

Ormond

2024

Front. Neurol.

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BackgroundNeurosarcoidosis (NS) is a challenging diagnosis, particularly when cases occur in low-prevalence, non-endemic geographic regions. In the United States, the highest incidence is in the Midwest and Northeast, compared to our Southwest location. While it is well known that NS may clinically and neuroradiographically mimic meningeal carcinomatosis, autoimmune or infectious pachymeningitis, neurosyphilis, or tuberculosis, diagnosis may be particularly challenging if systemic signs of sarcoidosis are lacking or unconfirmed or if dural-based masses are present. We reviewed our Colorado experience with NS cases, focusing our study on cases where NS represented the first histological confirmation of disease.MethodsA search of departmental databases was conducted with the search term “neurosarcoidosis” to identify cases 1-2008 to 12-2019, inclusive of the given case numbers. Patients were only included if their clinical and neuroimaging features were unusual and only when a biopsy of the central nervous system (CNS) represented the first confirmed diagnosis of sarcoidosis.ResultsA total of 17 cases were identified, of which the biopsy of the CNS was used for the initial confirmation of the disease in 9 of them. The most unusual findings were two patients with dural-based masses, one of which had pure NS as the cause of meningioma-like lesions and the second of which had coexistent meningioma and intimately admixed non-necrotizing granulomas of NS.ConclusionNS with unusual features, especially in non-endemic areas, continues to yield diagnostic challenges for neurologists, neuroradiologists, and pathologists.

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