Systemic autoimmune diseases complicated with hydrocephalus: pathogenesis and management

Wei, Junji; Yin, Hexiang; Wang, Li; Wang, Renzhi

doi:10.1007/s10143-017-0917-x

Cited by 7 publications

(7 citation statements)

References 41 publications

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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%

Hydrocephalus: a rare complication of primary central nervous system vasculitis

et al. 2023

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Hydrocephalus is a known complication of central nervous system (CNS) vasculitis secondary to infectious diseases. We present an unusual case of primary CNS vasculitis (PCNSV) complicated by communicating hydrocephalus. A patient in their 50s with a few months’ history of headache, psychomotor slowing and frequent falls presented with an acute left temporo-parieto-occipital infarction. Angiography revealed multiple arterial irregularities in the anterior circulation bilaterally, CSF was inflammatory and the remaining study was negative, fulfilling criteria for possible PCNSV. One month after successful treatment with corticosteroid, there was worsening of gait, urinary incontinence and neuropsychiatric symptoms. The investigation was remarkable only for active hydrocephalus. An external ventricular shunt was placed, followed by a ventriculoperitoneal shunt, and cyclophosphamide was started with subsequent recovery. Our discussion is that communicating hydrocephalus in PCNSV, due to impaired CSF flow, should be considered on subacute/chronic worsening of patients with PCNSV.

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Section: Discussionmentioning

confidence: 99%

Hydrocephalus: a rare complication of primary central nervous system vasculitis

et al. 2023

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“…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%

Relevance of Medullary Vein Sign in Neurosarcoidosis

Liberio

Kramer

Memon

et al. 2022

Neurology International

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Background: Central nervous system involvement is uncommon in patients with sarcoidosis. It remains a diagnostic challenge for clinicians, as there is a broad differential diagnosis that matches the presenting neurological signs. Often, the imaging findings also overlap with other disease entities. One understudied finding in patients with neurosarcoidosis is the presence of medullary vein engorgement on SWI imaging, termed the “medullary vein sign”, which has been postulated to be a specific sign for neurosarcoidosis. This study aims to provide an understanding of the diagnostic potential of the medullary vein sign. Methods: Thirty-two patients who presented with neurologic signs concerning for possible neurosarcoidosis were analyzed retrospectively for the presence of the medullary vein sign. Results: Out of these cases, 7 cases of definitive neurosarcoidosis cases were found based on other imaging signs, biopsy and CSF analysis; the remaining were classified into groups as possible (16), probable (5) and (4) cases of other infectious meningoencephalitis including 2 cases of autoimmune encephalitis. Seven patients among all of these cases were found to have the medullary vein sign on imaging, with five cases with confirmed and two cases from possible neurosarcoidosis. The sensitivity of the medullary vein sign in this study was 71.4%, and the specificity was 92.3%. Discussion: The benefits of improving diagnostic criteria for neurosarcoidosis include more rapid diagnosis leading to more prompt treatment, less exposure to potentially harmful antibiotics or antifungals, and less long-term neurological effects. Our results support that the medullary vein sign will potentially fill in the diagnostic gaps that have challenged the timely diagnosis of neurosarcoidosis. Conclusions: Our findings support that the medullary vein sign has a high specificity and should be included in the diagnostic criteria for neurosarcoidosis.

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“…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%

Clinical characteristics and outcome of hydrocephalus in neurosarcoidosis: a retrospective cohort study and review of the literature

2021

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Hydrocephalus is reported in approximately one-tenth of neurosarcoidosis patients. However, data on clinical characteristics and outcome are lacking. In this retrospective study, we present 11 patients with neurosarcoidosis and hydrocephalus on neuroimaging. Median age was 52 years and seven were female (64%). Presenting symptoms consisted of headache in 8 out of 11 (73%), vertigo in 5 (46%), gait abnormalities in 4 (36%), diplopia in 2 (18%) and decreased visual acuity in 1 (9%). Cranial imaging showed obstructive hydrocephalus in 10 (91%) and non-obstructive hydrocephalus in 1 (9%) out of 11, obstruction occurred at the level of the fourth ventricle in 6 out of 10 (60%). Treatment consisted of glucocorticoids in all the patients with additional methotrexate or azathioprine in 6 (55%) and infliximab in 1 (9%) patient. Neurosurgical intervention was performed in 10 out of 11 (91%) patients. Treatment led to remission, improvement or stabilization of disease in 9 out of 10 (90%) of patients. One patient died due to cerebral herniation despite neurosurgical decompression and CSF shunting. Median modified Rankin scale score at last follow-up was 2 (range 0–6). A systematic review and meta-analysis of studies on hydrocephalus due to neurosarcoidosis identified 36 patients that compared to our patients had a lower median age at onset and a higher mortality. Acute obstructive hydrocephalus due to neurosarcoidosis is a potentially fatal medical emergency requiring neurosurgical intervention and initiation of immunosuppressive therapy. If patients survive the initial phase, the outcome is generally favorable.

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Systemic autoimmune diseases complicated with hydrocephalus: pathogenesis and management

Cited by 7 publications

References 41 publications

Hydrocephalus: a rare complication of primary central nervous system vasculitis

Hydrocephalus: a rare complication of primary central nervous system vasculitis

Relevance of Medullary Vein Sign in Neurosarcoidosis

Clinical characteristics and outcome of hydrocephalus in neurosarcoidosis: a retrospective cohort study and review of the literature

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