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Infiltrative Optic Neuropathies
Published in Vivek Lal, A Clinical Approach to Neuro-Ophthalmic Disorders, 2023
Aniruddha Agarwal, Sabia Handa, Vishali Gupta
Treatment of sarcoidosis in general and neurosarcoidosis, in particular, may be extremely difficult. Corticosteroids are the mainstay of therapy; there are no standardized treatment protocols available as yet. Although corticosteroids are very effective in controlling inflammation initially, their long-term use is often associated with significant complications.70,71 Among patients where chronic steroid usage (≥7.5 mg of prednisone or equivalent daily) is required to control the inflammation, the use of steroid-sparing agents is indicated. Immunosuppressive agents such as hydroxychloroquine, methotrexate, azathioprine, mycophenolate mofetil, or cyclosporine may be useful. Rarely, patients may require biological agents including antagonists of tumor necrosis factor (TNF) such as adalimumab, infliximab or etanercept.75,76
Inflammatory Disorders of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Neurosarcoidosis is a chronic, multisystem, granulomatous, inflammatory disease of unknown etiology in which any part of the nervous system (and indeed any organ of the body) may be affected.37,38
Systemic Illnesses (Diabetes Mellitus, Sarcoidosis, Alcoholism, and Porphyrias)
Published in Jacques Corcos, Gilles Karsenty, Thomas Kessler, David Ginsberg, Essentials of the Adult Neurogenic Bladder, 2020
The prevalence of neurosarcoidosis is registered in 5% of patients with sarcoidosis.19 Peripheral nervous system involvement ranges between 25% and 67%.20 The central nervous system can also be affected; in the brain, patients can have leptomeningeal and intraparenchymal granulomatous infiltrates, but these lesions can be found anywhere along the spinal cord.21 In a review of neurosarcoidosis literature, cranial neuropathy was the most common manifestation, with micturition abnormalities reported in 23/206 (11%) patients. Spinal cord disease and peripheral neuropathies were identified in a larger portion of patients (185); however, it is not clear how many patients were evaluated for urinary tract symptoms.
Neurosarcoidosis: an update on diagnosis and therapy
Published in Expert Review of Neurotherapeutics, 2022
Patompong Ungprasert, Persen Sukpornchairak, Brandon P Moss, Manuel L Ribeiro Neto, Daniel A Culver
The most common sites of symptomatic involvement by neurosarcoidosis are cranial nerves. Facial nerve (cranial nerve VII) is the most frequently affected, accounting for up to one-third of all patients with neurosarcoidosis [8–11,14,15]. Facial nerve palsy in neurosarcoidosis is often bilateral (seen in up to one-third) and could be either concurrent or sequential [9,12,14]. Earlier studies usually described facial nerve palsy as a consequence of parotitis caused by sarcoidosis (or Heerfordt’s syndrome) [16]. However, subsequent studies found that most patients with parotitis did not develop facial nerve palsy and most patients with facial nerve palsy did not have preceding parotitis [12,17]. Epineural inflammation and perineural inflammation of the nerve itself or external compression caused by granulomatous inflammation in brain parenchyma or leptomeninges are now the widely accepted pathogenesis for cranial neuropathies, including facial nerve palsy.13 Cranial nerve VII palsy carries one of the best prognoses compared with other types of neurosarcoidosis as complete recovery is expected for most patients [10,18].
A confusing manifestation: a case report of neurosarcoidosis presenting with confusion
Published in Journal of Community Hospital Internal Medicine Perspectives, 2018
Ateeq Mubarik, Syed Moin Hassan, Monicka Felix, Salman Muddassir, Furqan Haq
In a systematic review and meta analysis of 1088 patients of neurosarcoidosis the main presenting neurological symptoms were cranial nerve palsies (55%), headache (32%), sensory abnormalities like hypoaesthesia, paraesthesia, and neuropathic pain (29%) and gait abnormalities (28%). Visual impairment, hemiparesis and paraparesis, ataxia, vertigo, seizures, psychiatric symptoms, nystagmus and papilledema also occurred frequently [5]. Confusion without any other neurological symptoms, as was present in our patient, is a unique presenting symptom. It is present in sporadic case reports in combination with other neurological symptoms like hemiparesis and gait disturbances [14,15]. We used the Zajicek et al criteria to diagnose our patient with neurosarcoidosis [16]. This criteria divided neurosarcoidosis into confirmed neurosarcoidosis, probable neurosarcoidosis and possible neurosarcoidosis. Our patient fit the picture of probable neurosarcoidosis with clinical presentation of neurosarcoidosis with evidence of inflammation in the central nervous system, and evidence of systemic sarcoidosis and exclusion of other diseases. Marangoni et al. recently proposed some changes to the existing diagnostic criteria by adding a High Resolution computed tomography (HRCT) instead of a CXR and measuring the CD4/CD8 T cell ratio in the Cerebrospinal fluid (CSF) [17]. However, it is still unclear what the sensitivity and specificity of the diagnostic criteria of zajicek et al is and how manrangoni et al changes may affect the existing criteria.
An uncommon case of neurosarcoidosis presenting as trigeminal neuralgia
Published in Journal of Community Hospital Internal Medicine Perspectives, 2021
Lydia Slater, Andrew Slater, John Braca, Brian Le, Ryan Zimmerman
Neurosarcoidosis remains a difficult diagnosis to make based solely on presenting symptoms and initial imaging and laboratory findings. This case emphasizes the importance of obtaining a complete workup for sarcoidosis if the diagnosis is in question, including chest imaging and tissue biopsies for pathological evaluation. As described in prior case reports, neurosarcoidosis may be isolated to the CNS without extracranial manifestations [9–14], and full workup with obtaining intracranial tissue samples should still be pursued in these cases. The internist should maintain a high clinical index of suspicion for the possibility of sarcoidosis as the diagnosis in patients presenting with unexplained neurological symptoms, even in the absence of typical pulmonary manifestations.