Not everything is as it seems: neurosarcoidosis presenting as leptomeningitis

Galnares-Olalde, Javier Andrés; Berebichez-Fridman, Roberto; Gómez-Garza, Gilberto; Mercado, Moisés; Moreno-Sánchez, Francisco; Alegría-Loyola, Marco Antonio

doi:10.1002/ccr3.1418

Cited by 7 publications

(12 citation statements)

References 17 publications

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“…cases of sarcoidosis and of the cranial nerves facial, and the ophthalmic nerves are the most likely to be involved [2]. There are myriads of presentations of the neurological involvement but presenting as progressive bulbar palsy is extremely rare [7].…”

Section: Discussionmentioning

confidence: 99%

“…A range of diagnostic tools is required to diagnose neurosarcoidosis, including radiological studies combined with biopsy of the accessible areas when possible. Angiotensin-converting enzyme (ACE) levels are positive in sarcoidosis, but it is not specific; moreover, ACE levels from the bronchoalveolar lavage (BAL) are more specific for the sarcoidosis compared to ACE levels in the plasma [2]. In our case, we tried to get the biopsy sample from the mediastinal lymph node but we failed to obtain enough tissue, however, we obtained biopsy from the inguinal lymph node which showed characteristic granuloma's making diagnosis obvious, we could not obtain ACE levels as the test was not available in the country.…”

Section: Discussionmentioning

confidence: 99%

“…Approximately 5%-10% of patients with sarcoidosis develop neurosarcoidosis, and only 1% of patients present solely with neurosarcoidosis [1]. It commonly presents with unilateral or bilateral seventh nerve palsy; leptomeningitis is also a common presentation [1][2]. In rare instances, neurosarcoidosis can present as dangerously progressive bulbar palsy, which is a diagnostic and clinical challenge.…”

Section: Introductionmentioning

confidence: 99%

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Isolated Bulbar Palsy: A Rare Presentation of Neurosarcoidosis

Chaudhry

Ata

Abdelghani

et al. 2020

Cureus

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Sarcoidosis, which is a non-caseating granulomatous chronic inflammatory disease, can affect virtually any organ system, including the central nervous system (CNS). Very rarely, patients may present solely with neurosarcoidosis. It commonly presents with unilateral or bilateral seventh nerve palsy. Rarely it can present as dangerous progressive bulbar palsy and is a diagnostic and clinical challenge. We present a case of sarcoidosis with a unique presentation of isolated bulbar palsy. A 38-year-old male presented to the emergency with a sudden onset of dysphonia followed by dysphagia for both solids and liquids for one week and 5 kg weight loss in two months. The rest of the CNS exam was unremarkable. On labs, he had hypercalcemia and suppressed parathyroid hormone (PTH) intact. Detailed radiological investigations, lab tests, and lymph node biopsy helped confirm the diagnosis of neurosarcoidosis. The patient did not respond to first-line steroid therapy and hence received intravenous immunoglobulin (IVIG) subsequently with adequate response and complete neurologic recovery, confirmed by a follow-up visit.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Isolated Bulbar Palsy: A Rare Presentation of Neurosarcoidosis

Chaudhry

Ata

Abdelghani

et al. 2020

Cureus

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“…Available data suggests that genetic, individual and environmental factors play a role in the etiology of neurosarcoidosis. Sarcoid tissues have shown the presence of protein antigens and mycobacterial DNA which indicates that the establishment of neurosarcoidosis may have microbial involvement [4].…”

Section: Discussionmentioning

confidence: 99%

“…Neuropsychiatric features such as dementia, depression, psychosis, poor concentration and hallucinations can be found in some cases of patients with neurosarcoidosis ( [5]. Some patients can develop granulomas on the fourth ventricle leading to hydrocephalus demanding emergency treatment [4].…”

Section: Discussionmentioning

confidence: 99%

Case Report: A Case of Central Diabetes Insipidus Secondary to Neurosarcoidosis

Johns¹,

Johns²

2020

Cureus

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We present a case of central diabetes insipidus (DI) secondary to neurosarcoidosis. The path to final diagnosis was challenging. Along with reporting the case, we review the available medical literature relating to neurosarcoidosis and central diabetes insipidus in this case report. Patient is a 56-year-old female with notable history of rheumatoid arthritis, anxiety, asthma, hypertension, spinal stenosis, and seizures of unknown etiology who presented to the emergency department for worsening headache for one week. She also endorsed decreased vision, photophobia, nausea, vomiting, gait abnormality, polyuria, and polydipsia over the past three months. Physical exam and neurological exam were unremarkable. Labs on presentation were notable for hypernatremia, increased serum osmolality and urine output of 5 L/day. Given her persistent headache and history of seizure, she underwent a CT head without contrast which showed a posterior suprasellar soft tissue fullness measuring 6 mm in the hypothalamic region. She then had additional imaging studies of the brain including MRI brain w/wo contrast and MRI pituitary w/wo contrast. MRI of the brain showed enlarged optic chiasm with increased T2 signal involving the proximal optic nerves and bilateral optic tracts, no brain parenchymal lesions to suggest multiple sclerosis. MRI of the pituitary w/wo contrast showed suprasellar mass arising from either the hypothalamus or less likely the chiasm which was concerning for high-grade glioma initially. Lumbar puncture was done that showed lymphocytic pleocytosis. Patient underwent right supraorbital craniotomy for biopsy of the suprasellar lesion. Surgical pathology showed noncaseating granulomatous inflammation most consistent with neuro-sarcoidosis. The diagnosis of neurosarcoidosis was made and patient was started on high dose steroids. Although central DI can be seen as a post-op complication, in our case, based on the clinical presentation, labs and imaging, there was concern of central DI on initial presentation. Patient was started on desmopressin 50 mg twice a day which resulted in marked improvement in urine output, serum sodium, and osmolality. Although it is rare to have nervous system involvement of sarcoidosis, symptoms of central diabetes insipidus could represent the first clinical manifestations of neurosarcoidosis. Proper treatment should be initiated in a timely fashion to avoid complications such as hydrocephalus.

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Multiple sclerosis in sarcoidosis patients: Two case reports

Etemadifar

Mehri

Sedaghat

et al. 2022

Clinical Case Reports

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Two cases of sarcoidosis referred to our clinic with neurological symptoms. They were diagnosed with multiple sclerosis using non‐invasive studies. The first patient refused treatment and died of myocardial infarction 6 months after visiting our clinic. The second received interferon‐beta and methotrexate with a favorable outcome after 3 years. Since the possible similar presentation of the two conditions could appear indistinct for certain diagnosis, accurate evaluation of symptoms and paraclinical data can provide the best approach to each condition.

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Not everything is as it seems: neurosarcoidosis presenting as leptomeningitis

Cited by 7 publications

References 17 publications

Isolated Bulbar Palsy: A Rare Presentation of Neurosarcoidosis

Isolated Bulbar Palsy: A Rare Presentation of Neurosarcoidosis

Case Report: A Case of Central Diabetes Insipidus Secondary to Neurosarcoidosis

Multiple sclerosis in sarcoidosis patients: Two case reports

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