From the Guidelines
Neurosarcoidosis is a condition that presents with a variety of neurological symptoms, including headaches, vision problems, facial weakness, hearing loss, seizures, and cognitive dysfunction, which can significantly impact morbidity, mortality, and quality of life if left untreated or improperly managed. The symptoms of neurosarcoidosis can be diverse and depend on the specific part of the nervous system that is affected. Common symptoms include:
- Headaches
- Vision problems, such as blurred vision or blindness
- Facial weakness or paralysis
- Hearing loss
- Seizures
- Cognitive dysfunction
- Weakness or numbness in limbs
- Balance problems or difficulty walking
- Cranial nerve involvement leading to facial drooping, double vision, or swallowing difficulties
- Meningitis-like symptoms with neck stiffness and sensitivity to light
- Pituitary gland involvement causing hormonal imbalances resulting in excessive thirst, urination, or reproductive issues
- Spinal cord inflammation leading to pain, weakness, or sensory changes below the affected area
- Peripheral nerve involvement causing numbness, tingling, or burning sensations in extremities
These symptoms typically develop gradually over weeks to months, though acute presentations can occur, and are the result of granulomatous inflammation in nervous tissue, causing disruption of normal neurological function 1. Diagnosis often requires excluding other conditions since these symptoms overlap with many neurological disorders. According to the European Respiratory Journal, the treatment of neurosarcoidosis often involves the use of glucocorticoids as first-line therapy, with additional drugs used in combination with glucocorticoids to lower the relapse rate of neurosarcoidosis 1. The use of glucocorticoids as first-line therapy for neurosarcoidosis is supported by limited but available data, and is recommended due to the high risk of significant irreversible neurologic loss.
From the Research
Symptoms of Neurosarcoidosis
- The clinical manifestations of neurosarcoidosis are varied and include:
- Cranial neuropathy
- Aseptic meningitis
- Hydrocephalus
- Headache
- Seizure
- Neuropsychiatric symptoms
- Neuroendocrine dysfunction
- Myelopathy
- Peripheral neuropathy 2
- Neurosarcoidosis can involve either the central nervous system (CNS), the peripheral nervous system (PNS), or both 2
- Patients with cranial mononeuropathy other than optic neuropathy were more likely to be treated with steroids alone, whereas patients with other presentations were likely to require second and third level treatments 3
Diagnosis and Treatment
- The diagnostic work up of neurosarcoidosis should include an evaluation for potential extra-neural involvement and histologic confirmation of sarcoidosis 2
- Gadolinium-enhanced magnetic resonance imaging (MRI) of the brain and spinal cord is the most sensitive test for neurosarcoidosis, while the diagnostic specificity of cerebrospinal fluid (CSF) analysis is limited 2
- Corticosteroids are the mainstay of treatment for neurosarcoidosis, with oral corticosteroids used for mild to moderate cases and high-dose intravenous methylprednisolone used in severe cases or refractory cases 2
- Immunomodulating and cytotoxic agents, such as methotrexate, azathioprine, and cyclophosphamide, are often required for steroid-refractory neurosarcoidosis or for patients who develop significant corticosteroid adverse effects 2, 3, 4
- Anti-TNF-α therapies, such as infliximab, may be considered in refractory disease, but caution should be exercised as there is evidence to suggest they may unmask disease 5, 6