2017
DOI: 10.1007/s10143-017-0917-x
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Systemic autoimmune diseases complicated with hydrocephalus: pathogenesis and management

Abstract: Systemic autoimmune diseases (SAIDs) represent a group of syndromes involving at least two organ systems. Classical SAIDs include connective tissue diseases, vasculitis, and granulomatous diseases, many of which involve the nervous system and result in different neurological manifestations. Hydrocephalus can be a rare but lethal complication of various SAIDs, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), sarcoidosis, and primary vasculitis. However, the pathogenesis of SAIDs complica… Show more

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Cited by 7 publications
(7 citation statements)
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“…A recent study reported that 1.8% of CNS vasculitis complicate with hydrocephalus, that is a major cause of in-hospital mortality 4. Apart from infectious meningitis, hydrocephalus has also been described in some causes of CNS vasculitis secondary to systemic autoimmune diseases like Wegener’s granulomatosis and lupus 5 6…”
Section: Discussionmentioning
confidence: 99%
“…A recent study reported that 1.8% of CNS vasculitis complicate with hydrocephalus, that is a major cause of in-hospital mortality 4. Apart from infectious meningitis, hydrocephalus has also been described in some causes of CNS vasculitis secondary to systemic autoimmune diseases like Wegener’s granulomatosis and lupus 5 6…”
Section: Discussionmentioning
confidence: 99%
“…The presenting symptoms of neurosarcoidosis are similar to several other pathologies such as fungal, bacterial or viral meningitis, other autoimmune diseases, or demyelinating diseases. These issues raise a concern for delayed diagnosis and treatment initiation and necessitate other means of differentiating between neurosarcoidosis and other pathologies, especially in individuals who have not had a prior diagnosis of sarcoidosis in other organ systems [ 13 , 14 , 15 , 16 , 17 , 18 , 19 ].…”
Section: Discussionmentioning
confidence: 99%
“…As mentioned previously, the differential diagnosis in patients with neurological signs concerning for neurosarcoidosis includes fungal, viral, and bacterial infections [ 17 , 18 ]. Six of the ten patients with confirmed neurosarcoidosis were initially treated with antibiotics or antifungals due to erroneous diagnosis of infection.…”
Section: Discussionmentioning
confidence: 99%
“…Our study shows that hydrocephalus is a rare but potentially fatal manifestation of neurosarcoidosis that requires a combination of neurosurgical intervention and immunosuppressive treatment. In the absence of a positive history of (neuro)sarcoidosis, the differential diagnosis of acquired hydrocephalus is diverse and includes among others inflammatory, (post-) infectious, (post-)hemorrhagic and (post-) traumatic disorders and intracranial tumors such as colloid cysts or malignancy [5,12,43,44]. To our knowledge, there is no literature regarding the percentage of hydrocephalus cases caused by neurosarcoidosis.…”
Section: Discussionmentioning
confidence: 99%
“…The diagnosis of neurosarcoidosis depends on a combination of clinical characteristics and results of ancillary investigations mostly used to rule out other disorders [3]. Hydrocephalus due to neurosarcoidosis was found to occur in 9% of patients with neurosarcoidosis in a meta-analysis [2,4,5]. There are no large series describing clinical characteristics of patients with hydrocephalus due to neurosarcoidosis and data regarding imaging characteristics, optimal treatment strategy and outcome are lacking.…”
Section: Introductionmentioning
confidence: 99%