Retrospective, dual-centre review of imaging findings in neurosarcoidosis at presentation: prevalence and imaging sub-types

Bathla, Girish; Freeman, Colbey W.; Moritani, Toshio; Song, Jae Woo; Srivastava, Sanvesh; Soni, Neetu; Derdeyn, Colin P.; Mohan, Suyash

doi:10.1016/j.crad.2020.05.008

Cited by 17 publications

(40 citation statements)

References 25 publications

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“…There were a total of 13 consecutive patients with NS who underwent VWI between July 2018 and June 2020. Because recent studies have shown a higher prevalence of cerebrovascular events in NS, 4 , 9 our institutional protocol was modified to include VWI in all patients with NS. None of the imaged patients in the current cohort, however, were imaged for suspected vasculitis or infarct.…”

Section: Methodsmentioning

confidence: 99%

“…6 - 8 This was likely an underrepresentation secondary to multiple contributory factors such as absence of more sensitive, high-resolution sequences and absence of dedicated imaging review. 4 , 5 , 9 More recently, involvement of the medullary veins on susceptibility-weighted imaging (SWI) and a relatively high proportion of cerebral infarcts (presumably secondary to underlying vasculitic/vasculopathic process) have been described and raise important questions about the extent and distribution of vascular involvement in patients with NS. 9 , 10 Herein, we describe our preliminary findings of high-resolution black-blood imaging, also referred to as vessel wall imaging (VWI) in a series of 13 patients with NS.…”

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confidence: 99%

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Vascular Involvement in Neurosarcoidosis

Bathla

Abdel-Wahed

Agarwal

et al. 2021

Neurol Neuroimmunol Neuroinflamm

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Background and ObjectivesCerebrovascular manifestations in neurosarcoidosis (NS) were previously considered rare but are being increasingly recognized. We report our preliminary experience in patients with NS who underwent high-resolution vessel wall imaging (VWI).MethodsA total of 13 consecutive patients with NS underwent VWI. Images were analyzed by 2 neuroradiologists in consensus. The assessment included segment-wise evaluation of larger- and medium-sized vessels (internal carotid artery, M1-M3 middle cerebral artery; A1-A3 anterior cerebral artery; V4 segments of vertebral arteries; basilar artery; and P1-P3 posterior cerebral artery), lenticulostriate perforator vessels, and medullary and deep cerebral veins. Cortical veins were not assessed due to flow-related artifacts. Brain biopsy findings were available in 6 cases and were also reviewed.ResultsMean patient age was 54.9 years (33–71 years) with an M:F of 8:5. Mean duration between initial diagnosis and VWI study was 18 months. Overall, 9/13 (69%) patients had vascular abnormalities. Circumferential large vessel enhancement was seen in 3/13 (23%) patients, whereas perforator vessel involvement was seen in 6/13 (46%) patients. Medullary and deep vein involvement was also seen in 6/13 patients. In addition, 7/13 (54%) patients had microhemorrhages in susceptibility-weighted imaging, and 4/13 (31%) had chronic infarcts. On biopsy, 5/6 cases showed perivascular granulomas with vessel wall involvement in all 5 cases.DiscussionOur preliminary findings suggest that involvement of intracranial vascular structures may be a common finding in patients with NS and should be routinely looked for. These findings appear concordant with previously reported autopsy literature and need to be validated on a larger scale.

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Section: Methodsmentioning

confidence: 99%

mentioning

confidence: 99%

Vascular Involvement in Neurosarcoidosis

Bathla

Abdel-Wahed

Agarwal

et al. 2021

Neurol Neuroimmunol Neuroinflamm

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“…Literature review yielded 95 articles of which 46 were assessed for eligibility based on title and abstract. Of these articles, five consisted of case series of neurosarcoidosis patients that included patients with hydrocephalus but did not report clinical characteristic, ancillary investigations, treatment and outcome of this subgroup [4,[6][7][8][9]. Seven articles were excluded based on language and one article reported on a patient that is included in our case series.…”

Section: Literature Reviewmentioning

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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“…MRI is the elective examination for the diagnosis of NS, as intense physiological uptake in the brain may limit the value of FDG/PET. However, although some MRI findings can be rather typical [ 40 , 41 ], they alone are not sufficient for diagnosis and need to be part of a context of other compatible clinical, imaging, and laboratory data, while other causes are ruled out. Cranial neuropathy is the most common manifestation of PNS involvement, sometimes causing unilateral or bilateral facial palsy.…”

Section: Neurosarcoidosismentioning

confidence: 99%

A Comprehensive Review of Sarcoidosis Diagnosis and Monitoring for the Pulmonologist

et al. 2021

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Sarcoidosis is a systemic granulomatous disease with heterogenous clinical manifestations. Here we review the diagnosis of sarcoidosis and propose a clinically feasible diagnostic work-up and monitoring protocol. As sarcoidosis is a systemic disease, a multidisciplinary approach is recommended for best outcomes. However, since the lungs are frequently involved, the pulmonologist is often the referral physician for diagnosis and management. When sarcoidosis is suspected, diagnosis needs to be confirmed and organ involvement/impairment assessed. This process is also required to establish whether the patient is likely to benefit from treatment, as many cases of sarcoidosis are selflimited and remit spontaneously. Whether or not treatment is started, effective regular followup is necessary to monitor changes in the disease, including extension, progression, remissions, flare-ups, and complications.

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Retrospective, dual-centre review of imaging findings in neurosarcoidosis at presentation: prevalence and imaging sub-types

Cited by 17 publications

References 25 publications

Vascular Involvement in Neurosarcoidosis

Vascular Involvement in Neurosarcoidosis

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

A Comprehensive Review of Sarcoidosis Diagnosis and Monitoring for the Pulmonologist

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