Neurosarcoidosis Presenting as Benign Intracranial Hypertension

Redwood,; Jb, Winer; Rossor, Martin N.

doi:10.1159/000117381

Cited by 5 publications

(2 citation statements)

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“…Sarcoidosis presenting as benign intracranial hypertension is an extremely rare occurrence, with only a few reported cases till now [ 19 , 20 ]. The presence of biopsy-proven non-caseating granulomas support the diagnosis of sarcoidosis [ 21 , 22 ].…”

Section: Discussionmentioning

confidence: 99%

Benign Intracranial Hypertension: A Rare Manifestation of Neurosarcoidosis

Dubey,

Nirhale,

Rohatgi

et al. 2023

Cureus

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Sarcoidosis is an immune-mediated disease that can involve multiple systems. Sarcoidosis of the nervous system or neurosarcoidosis may present as cranial mononeuropathy, hypothalamic involvement, aseptic meningitis, granulomatous inflammation in the brain parenchyma or spinal cord, peripheral neuropathy, and, in rare cases, as myopathy and benign intracranial hypertension. The most common cranial nerve involvement is the facial nerve, which can present as unilateral or bilateral facial nerve palsy, often with recurrent episodes. Involvement of other cranial nerves such as the second and eighth cranial nerves has also been reported. Granulomatous inflammation in the spinal cord presents as myelopathy or radiculopathy. Peripheral neuropathy can manifest as mononeuropathy, mononeuritis multiplex, or generalized sensory-motor neuropathy. Carpal tunnel syndrome is more common in patients with sarcoidosis compared to the general population. Here, we describe the case of a 40-year-old female who presented with heaviness of the head and blurred vision, with a prior history of left-sided Bell's palsy. Bilateral papilledema was observed during the fundus examination. MRI of the brain revealed signs suggestive of benign intracranial hypertension. The cerebrospinal fluid (CSF) opening pressure was measured at 40 cmH2O. Biopsy of bilateral hilar lymphadenopathy indicated granulomatous inflammation consistent with sarcoidosis. The patient was started on steroids and acetazolamide, and she had a dramatic improvement in symptoms.

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Section: Discussionmentioning

confidence: 99%

Benign Intracranial Hypertension: A Rare Manifestation of Neurosarcoidosis

Dubey,

Nirhale,

Rohatgi

et al. 2023

Cureus

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“…Papilledema has been described in a sarcoid lymphocytic meningitis presenting initially as idiopathic intracranial hypertension (IIH). 30 In some cases, differentiating between IIH and sarcoid-induced intracranial hypertension can be difficult. 31 If a case of IIH is atypical, or unrelenting, other causes of papilledema should be sought.…”

Section: Optic Nervementioning

confidence: 99%

Neuro-ophthalmic Complications of Sarcoidosis

Constantino¹,

Digre²,

Zimmerman³

2000

Semin Neurol

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Sarcoidosis is a systemic disorder that is caused by granulomatous changes of uncertain etiology and commonly has multiorgan involvement. Ocular involvement may occur in up to 32% of persons afflicted by sarcoid. The neuro-ophthalmic manifestations of sarcoid, in particular, are varied and may affect any portion of the visual system, including neural structures. Diagnosis is often difficult due to the fact that the clinical presentation can mimic other disorders, such as Multiple Sclerosis, and therefore a systematic approach to testing must be used once the diagnosis has been considered. The importance of diagnosing neuro-ophthalmic sarcoid lies in the fact that it is a treatable disease. The mainstay of treatment is corticosteroids although other immunosuppressive agents may be used. The long-term prognosis of neuro-ophthalmic sarcoid has not been studied in large patient populations, but the data that is available suggests that remission may occur in up to 47%.

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