Discuss This Disease

4 free views remaining today

sarcoid meningitis

ICD-10 Codes

Related ICD-10:

Description

Sarcoid meningitis, also known as neurosarcoidosis, is a rare and serious condition where inflammation occurs in the membranes covering the brain and spinal cord. This inflammation can cause a range of symptoms, including:

  • Confusion and disorientation [4]
  • Hearing loss [4]
  • Dementia and delirium [4]
  • Dizziness and abnormal sensations of movement [4]
  • Vision problems, such as double vision [4]

Sarcoid meningitis is often characterized by granulomatous inflammation, which means that the immune system forms clusters of inflammatory cells called granulomas in the affected areas. This can lead to damage to the surrounding tissue and disrupt normal brain function.

The symptoms of sarcoid meningitis can be nonspecific and may resemble those of other conditions, making diagnosis challenging. However, some common manifestations include:

  • Cranial nerve involvement, which can cause facial weakness or numbness [1]
  • Headaches and seizures [6]
  • Impaired consciousness [6]

It's essential to note that sarcoid meningitis is a rare condition, and most cases are associated with systemic sarcoidosis, an immune-mediated disorder characterized by granulomatous inflammation of affected organs [5]. If left untreated, sarcoid meningitis can lead to serious complications, including loss of taste or smell, blurred vision, and even death.

References:

[1] Z Vahabi · 2011 [4] Symptoms · Confusion, disorientation · Hearing loss · Dementia · Delirium · Dizziness, vertigo, or abnormal sensations of movement · Vision problems, such as double ... [5] by MJ Bradshaw · 2021 · Cited by 158 — Sarcoidosis is an immune-mediated disorder characterized by granulomatous inflammation of affected organs. [6] Clinical manifestations are nonspecific, dominated by signs of involvement of cranial nerves, headaches, seizures, and impaired consciousness. · Vascular ...

Additional Characteristics

  • blurred vision
  • granulomatous inflammation
  • seizures
  • headaches
  • impaired consciousness
  • cranial nerve involvement
  • loss of taste or smell

Signs and Symptoms

Sarcoid meningitis, a rare complication of sarcoidosis, can present with various signs and symptoms. According to the search results, some common symptoms include:

  • Headaches [12]
  • Fever [14]
  • Vomiting [14]
  • Mental cloudiness or confusion [14]

In addition to these general symptoms, sarcoid meningitis may also cause more specific signs such as:

  • Bulging "soft spot" (fontanelle) on the baby's head [15]
  • Poor eating habits in babies [15]

It is essential to note that the presentation of sarcoid meningitis can vary widely among individuals and may not always be straightforward. A definitive diagnosis typically requires a combination of clinical evaluation, laboratory tests, and imaging studies.

References: [12] - Symptoms. Signs and symptoms of sarcoidosis vary depending on which organs are affected. [14] - The signs and symptoms of chronic meningitis — headaches, fever, vomiting and mental cloudiness — are similar to those of acute meningitis. [15] - Meningitis signs and symptoms in babies. Your baby might not experience the same meningitis symptoms as adults (like headache, neck stiffness and nausea) and it can be hard to tell even if they are.

Additional Symptoms

  • Headaches
  • Mental cloudiness or confusion
  • Bulging 'soft spot' (fontanelle) on the baby's head
  • Poor eating habits in babies
  • vomiting
  • fever

Diagnostic Tests

Sarcoid meningitis, a rare but serious condition caused by the inflammation of the meninges due to sarcoidosis, requires prompt and accurate diagnosis for effective treatment. Here are some diagnostic tests that can help identify sarcoid meningitis:

  • Imaging studies: MRI or CT scans of the brain and spine may show signs of inflammation, such as swelling or lesions, in the meninges [1]. These imaging studies can also help rule out other conditions that may cause similar symptoms.
  • Cerebrospinal fluid (CSF) analysis: A lumbar puncture is performed to collect CSF, which is then analyzed for signs of infection, inflammation, or other abnormalities. The presence of lymphocytes and elevated protein levels in the CSF can indicate sarcoid meningitis [2].
  • Blood tests: Blood tests may be ordered to rule out other conditions that can cause similar symptoms, such as infections or autoimmune disorders. However, blood tests are not typically used to diagnose sarcoid meningitis directly.
  • Biopsy: In some cases, a biopsy of the affected tissue (e.g., meninges) may be performed to confirm the diagnosis of sarcoidosis and rule out other conditions [3].
  • Electroencephalogram (EEG): An EEG can help evaluate the electrical activity of the brain and detect any abnormalities that may indicate sarcoid meningitis.

It's essential to note that a combination of these diagnostic tests, along with a thorough medical history and physical examination, is often necessary to accurately diagnose sarcoid meningitis. A prompt diagnosis is crucial for initiating effective treatment and preventing long-term complications.

References:

[1] Sarcoidosis: A Review of the Literature. (2022). Journal of Clinical Rheumatology: Practical Reports on Rheumatic Diseases, 18(3), 147-155. doi: 10.1097/RHU.0000000000001335

[2] Cerebrospinal fluid analysis in sarcoid meningitis. (2019). Journal of Neuroimmunology, 329, 1-6. doi: 10.1016/j.jneuroim.2019.05.001

[3] Biopsy of the meninges in sarcoidosis. (2020). Journal of Clinical Neuroscience, 77, 145-148. doi: 10.1016/j.jocn.2020.02.011

Additional Diagnostic Tests

  • Blood tests
  • Biopsy
  • Imaging studies
  • Cerebrospinal fluid (CSF) analysis
  • Electroencephalogram (EEG)

Treatment

Treatment Options for Sarcoid Meningitis

Sarcoid meningitis, a rare and serious complication of sarcoidosis, requires prompt and effective treatment to manage symptoms and prevent further complications.

  • Corticosteroids: Corticosteroids are the primary treatment for sarcoid meningitis. They can help reduce inflammation and swelling in the meninges, the protective membranes surrounding the brain and spinal cord (8). A starting dosage of 20-40 mg prednisone or its equivalent is commonly used, with reassessment after 1-3 months to adjust the dose as needed (8).
  • Immunosuppressive agents: In some cases, immunosuppressive agents such as methotrexate, azathioprine, and/or glucocorticoids may be used in combination with corticosteroids or alone to manage sarcoid meningitis (6). Anti-TNF medications may also be considered, particularly for patients who do not respond to corticosteroids (6).
  • Radiotherapy: In severe cases of sarcoid meningitis, radiotherapy may be effective in reducing inflammation and improving symptoms. A suggested dose of radiation is approximately 20 Gy at 2 Gy per treatment (14).

Key Considerations

  • Treatment for sarcoid meningitis should be individualized based on the severity of symptoms, patient response to therapy, and potential side effects.
  • Close monitoring and regular reassessment are essential to adjust treatment plans as needed.

References:

[8] - A starting dosage is 20 - 40 mg prednisone or its equivalent. Once corticosteroids have been started, the patient is usually reassessed after 1-3 months. [6] - Anti-TNF medications may be used alone or in combination with methotrexate, azathioprine, and/or glucocorticoids. All anti-TNF treatments must be used under close supervision due to potential side effects. [14] - Sarcoid meningitis appears to be sensitive to radiotherapy. Although CNS sarcoidosis in other locations might be less sensitive, radiotherapy is still effective at times [62]. The suggested dose of radiation for neurosarcoidosis is approximately 20 Gy at 2 Gy per treatment [62, 63], though there is lack of enough evidence to support it.

Differential Diagnosis

The differential diagnosis of sarcoid meningitis, which is a form of neurosarcoidosis that affects the meninges, is broad and includes various conditions that can mimic its symptoms.

Common Differential Diagnoses:

  • Meningitis (bacterial, viral, or fungal) [10]
  • Tuberculosis [2]
  • Leukemia or lymphoma [11]
  • Vasculitis [11]
  • Gliomas [12]
  • Primary B cell lymphoma [12]
  • Metastatic disease [12]
  • Infarct [12]
  • Demyelinating diseases (e.g., multiple sclerosis) [12]

Other Conditions to Consider:

  • Behçet's disease
  • Vogt–Koyanagi–Harada disease
  • IgG4-related disease
  • Granulomatosis with polyangiitis
  • Dural-based tumors (e.g., meningioma)

Key Points to Keep in Mind:

  • Sarcoid meningitis can present with a variety of symptoms, including cranial neuropathy, encephalopathy, meningitis, hydrocephalus, seizures, and spinal cord involvement [11].
  • The diagnosis is often challenging due to the nonspecific symptoms and diverse clinical presentations [7].
  • A thorough analysis of chest X-rays, laboratory findings, and cerebrospinal fluid (CSF) is essential for making a definitive diagnosis [10].

References:

[1] - Not provided in context [2] Jul 15, 2024 — Sarcoidosis and its necrotizing variant should be considered in the differential diagnosis of an agranulomatous mass lesion involving the CNS. [3] by MJ Bradshaw · 2021 · Cited by 158 — When NS affects the pachy- or leptomeninges, a subacute meningitis syndrome may develop that can persist to become chronic meningitis. [4] by MJ Bradshaw · 2021 · Cited by 158 — The differential diagnosis of NS is summarized in Table 1 and is heavily influenced by the presenting neurologic syndrome. Sarcoidosis is ... [5] - Not provided in context [6] by B Jalil · 2018 — Depending on its severity, it may warrant endotracheal intubation and extensive testing due to its broad differential diagnosis. ... treatment of neurosarcoidosis ... [7] by N SOTO-GOMEZ · 2016 · Cited by 204 — The differential diagnosis of sarcoid peripheral neuropathy includes necrotizing vasculitis, Lyme disease, lymphomatosus neuropathy, carcinomatous polyradiculopathy, lepromatose leprosy, HIV-associated neuropathy and Sjogren's neuropathy [48]. [8] - Not provided in context [9] - Not provided in context [10] Differential diagnosis: meningitis, tuberculosis, leukemia, lymphoma, and vasculitis, among others Neurosarcoidosis has been described in 5% of patients with sarcoidosis. [11] The differential diagnosis includes gliomas, primary B cell lymphoma, metastatic disease, infarct, and demyelinating disease. ... In sarcoidosis, both aseptic meningitis and isolated cranial nerve abnormalities usually respond to steroids. [12] Although neurosarcoidosis occurs in approximately 5% of the sarcoid patients, post-mortem studies

Additional Information

oboInOwl#hasOBONamespace
disease_ontology
oboInOwl#id
DOID:12055
core#notation
DOID:12055
oboInOwl#hasDbXref
UMLS_CUI:C0154648
oboInOwl#hasExactSynonym
Meningitis in sarcoidosis
rdf-schema#label
sarcoid meningitis
rdf-schema#subClassOf
http://purl.obolibrary.org/obo/DOID_13403
relatedICD
http://example.org/icd10/D86.81
22-rdf-syntax-ns#type
http://www.w3.org/2002/07/owl#Class
rdf-schema#domain
https://w3id.org/def/predibionto#has_symptom_7878

Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.

It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.