Neurosarcoidosis and multiple intracerebral hematomas: An unusual clinical presentation

Vukojevic, Zoran; Ilić, Tihomir V.; Dominović-Kovačević, Aleksandra; Grgić, Sanja; Mavija, Srdjan

doi:10.1016/j.jns.2017.05.030

Cited by 9 publications

(10 citation statements)

References 7 publications

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“…14,28 Although some of the apparent lobar predilection may be from a reporting bias of larger symptomatic lesions, the reasons for relative sparing of the basal ganglia in the reported studies are unclear and unlikely to be entirely coincidental. Like some of the recently reported cases 37,39,42 and the authors' personal experiences, distinct peripheral distribution of the ICH with sparing of the basal ganglia may be seen, though its significance is not clear (Fig 5). The overall heterogeneity of the lesion distribution may be secondary to pathologically demonstrated "mixed" venous and arterial involvement.…”

Section: Hemorrhagic Lesionssupporting

confidence: 49%

“…36,37 Because the common denominator in both ischemic and hemorrhagic lesions is underlying vasculitis, it is not surprising that they both have a male predilection, unlike sarcoidosis, which is more common in females. 36,38,39 Table 2 lists the cases of NS with intracerebral hemorrhage (ICH) reported in the English language literature where imaging was performed. 28,36-47 A review of these cases reveals that ICH in patients with NS is often intraparenchymal and is more likely to be supratentorial.…”

Section: Hemorrhagic Lesionsmentioning

confidence: 99%

“…36,37,43 Similar to ischemic strokes, multiple or recurrent hemorrhages may also be seen. 37,39,42 Also analogous to cases with ischemic lesions, angiographic studies (DSA/MRA/CTA) are often unyielding. As shown in Table 2, of the 11 cases in which an angiographic evaluation was performed, positive findings were only noted in 2 cases.…”

Section: Hemorrhagic Lesionsmentioning

confidence: 99%

“…36,37 An increase in intraparenchymal hemorrhages associated with steroid tapering or discontinuation has also been reported in multiple recent cases, highlighting the need for close follow-up while tapering immunosuppressive therapy. 37,39,44,45 The primary differential consideration of ICH in NS includes vasculitis, especially primary angiitis of the CNS, as discussed previously. Similar to the NS patients with ischemic lesions, the angiographic studies in patients with ICH are also often negative, though some cases may show large vessel vasculitis.…”

Section: Hemorrhagic Lesionsmentioning

confidence: 99%

See 3 more Smart Citations

Cerebrovascular Manifestations of Neurosarcoidosis: An Underrecognized Aspect of the Imaging Spectrum

Bathla¹,

Watal²,

Gupta

et al. 2017

AJNR Am J Neuroradiol

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Involvement of the central nervous system by sarcoidosis, also referred to as neurosarcoidosis, is seen clinically in about 5% of patients with systemic disease. CNS involvement most frequently affects the leptomeninges and cranial nerves, though the ventricular system, brain parenchyma, and pachymeninges may also be involved. Even though the involvement of the intracranial vascular structures is well-known on postmortem studies, there is scant literature on imaging manifestations secondary to the vessel wall involvement, being confined mostly to isolated case reports and small series. The authors present a review of various cerebrovascular manifestations of neurosarcoidosis, along with a brief synopsis of the existing literature.

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Section: Hemorrhagic Lesionssupporting

confidence: 49%

Section: Hemorrhagic Lesionsmentioning

confidence: 99%

Section: Hemorrhagic Lesionsmentioning

confidence: 99%

Section: Hemorrhagic Lesionsmentioning

confidence: 99%

See 2 more Smart Citations

Cerebrovascular Manifestations of Neurosarcoidosis: An Underrecognized Aspect of the Imaging Spectrum

Bathla¹,

Watal²,

Gupta

et al. 2017

AJNR Am J Neuroradiol

View full text Add to dashboard Cite

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“…7 Previously documented case reports of intracranial hemorrhage in NS had established improvement with our initial strategy of high dose corticosteroids. 8,9 Due to our patient’s severe presentation, we elected to pursue a more aggressive, early therapy with infliximab in addition to methotrexate and a steroid taper. We hoped to avoid further complications from disease progression in a patient with an already precarious neurological state.…”

Section: Discussionmentioning

confidence: 99%

A Case of Neurosarcoid Presenting as Multiple Intraparenchymal Hemorrhages

Mehta

Khan

Wagner

et al. 2021

The Neurohospitalist

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This report explores the case of a 49-year-old African American male with a six-month history of multifocal neurological deficits who presented to an outside hospital after a generalized seizure. Patient was transferred to our tertiary medical center after brain imaging showed multiple bilateral supratentorial intraparenchymal hemorrhages (IPH). A brain biopsy confirmed parenchymal and perivascular non-caseating granulomas with vasculitis. The patient was definitively diagnosed with neurosarcoidosis (NS) and his condition improved with high dose corticosteroids and additional immunosuppressive therapies. Intracranial hemorrhage in the setting of NS is extremely rare, with fewer than thirty documented cases; however, this is likely an underestimation of its true prevalence. This case illustrates the difficulty in diagnosis as many other etiologies of IPH must be considered. Additionally, the clinical course and manifestations of NS is often quite variable. The uniqueness of this case lies in the rapid progression from seemingly incidental microhemorrhages to multiple large IPHs over two months. While the cause of this progression is not immediately apparent, a possible cause may be inadequate initial treatment due to delayed diagnosis. Our case demonstrates the importance of early recognition and initiation of immunosuppressive therapy, potentially leading to dramatic clinical improvement, as seen in this patient.

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Recurrent Ischemic and Hemorrhagic Strokes in a Young Adult

Rocha

Singhal

2018

JAMA Neurol

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An Indian man in his early 40s was admitted for evaluation of recurrent ischemic and hemorrhagic strokes. His medical history included hypertension and gout. He first presented 7 years ago with dizziness, dysarthria, and left hemiparesis. Brain magnetic resonance imaging (MRI) showed an acute right corona radiata infarct and 2 right perisylvian ring-enhancing and nodular granulomatous lesions (Figure 1A). Results of head and neck MR angiography were normal. Two weeks after admission he developed fever and asthenia. Brain MRI showed a new right perisylvian lesion with meningeal enhancement. Follow-up MRI after 8 months showed a new right parietal nodular lesion and a right parietal hemorrhage.He remained asymptomatic without specific treatment for 7 years, but then developed diplopia; an MRI showed a new pontine infarct. Two months later, skin lesions developed below both knees; skin biopsy showed erythema nodosum. One month later he developed generalized seizures. Brain MRI showed acute parenchymal hemorrhages in the right cerebellum and left frontotemporal and right perisylvian regions, as well as chronic infarcts, multiple microbleeds, and periventricular white-matter lesions (Figure 1B).Results of routine blood tests, erythrocyte sedimentation rate, antiphospholipid and autoimmunity panels, and cerebrospinal fluid (CSF) testing were within the reference ranges, and cerebral angiography results were normal. Tests for tuberculosis suggested latent disease (Mantoux test result positive, but CSF adenosine deaminase level and mycobacterial cultures negative). Serum angiotensin-converting enzyme level was 72 U/L (normal <40 μg/L; to convert to nanokatals per liter, multiply by 16.667). Fluorescein angiogram results were normal. A whole-body computed tomography scan showed nonspecific small, calcified granulomas in the anterior mediastinum. Whole-body fluorodeoxyglucose positron emission tomography results were normal. A brain biopsy was performed.

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Neurosarcoidosis and multiple intracerebral hematomas: An unusual clinical presentation

Cited by 9 publications

References 7 publications

Cerebrovascular Manifestations of Neurosarcoidosis: An Underrecognized Aspect of the Imaging Spectrum

Cerebrovascular Manifestations of Neurosarcoidosis: An Underrecognized Aspect of the Imaging Spectrum

A Case of Neurosarcoid Presenting as Multiple Intraparenchymal Hemorrhages

Recurrent Ischemic and Hemorrhagic Strokes in a Young Adult

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