Sarcoidosis of the central nervous system

doi:10.1016/0022-510X(69)90086-0

Journal of the Neurological Sciences

Volume 9, Issue 3, November–December 1969, Pages 405-422

https://doi.org/10.1016/0022-510X(69)90086-0 Get rights and content

Abstract

Clinical and pathological findings are reported in 7 cases of sarcoidosis of the central nervous system. In 2 cases the brains only were examined. The remaining 5 fully-examined cases all showed evidence of generalized sarcoidosis. In the central nervous system typical sarcoid granulomata were present in the leptomeninges and in the parenchyma where they appeared to be largely perivascular. Involvement of blood vessels, both arteries and veins, in the granulomatous process, leading to obliteration of their lumina, was frequently observed. Acute necrotizing arteritis was found in 1 instance.

A tendency to subependymal aggregation of lesions was observed. In 2 cases this granulomatous ependymitis led to stenosis of the aqueduct and obstructive hydrocephalus.

Perivascular calcifications were found in the hypothalamus of 2 cases, in one of which the cerebellar cortex was similarly affected. Insufficient data were avaialble to correlate this finding with deranged calcium metabolism.

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The occurrence of cerebral vasculitis in individuals with neurosarcoidosis (NS) is considered to be rare. Although the number of relevant publications has increased in recent years, evidence is mostly limited to case reports. To obtain a better understanding of this rare and severe manifestation of disease, we carried out a scoping review on cerebral vasculitis in patients diagnosed with NS. The results of the review indicate that the diagnosis of cerebral vasculitis in patients with NS is made especially in patients with systemic sarcoidosis. However, recurrent strokes in patients with NS remains the main indicator of cerebral vasculitis. A tissue biopsy is considered the gold standard to confirm the diagnosis despite occasional false-negative results. Glucocorticoids and steroid-sparing agents are the most successful current treatments. Favorable outcomes were observed with strategies targeting TNFα and B cells. The goal of this review is to summarize the current literature and treatment options for cerebral vasculitis in patients with NS.
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Systemic sarcoidosis can lead to heart failure, conduction abnormalities and ventricular arrhythmias although data on concomitant valvular heart disease (VHD) is limited. We reported the prevalence and outcomes of VHD in systemic sarcoidosis. A retrospective cohort study was conducted using National Inpatient Sample between 2016 and 2020 with respective ICD-10-CM codes. 406,315 patients were hospitalized with sarcoidosis, out of which 20,570 had comorbid VHD (5.1%). Mitral disease was most common (2.5%), followed by aortic, and tricuspid disease. Tricuspid disease was associated with increased mortality in sarcoidosis (OR 1.6, 95% CI, 1.1-2.6, P = 0.04), while aortic disease was associated with higher mortality in only 31-50 years age cohort. Patients with sarcoidosis and VHD have higher hospitalization charges and lower or similar valvular intervention rates than those without sarcoidosis. VHD has a prevalence of 5% in sarcoidosis, predominantly affecting mitral and aortic valves. Underlying VHD is associated with worse outcomes in sarcoidosis.
Management of neurosarcoidosis
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Citation Excerpt :
Vascular complications in neurosarcoidosis are rare; cerebral infarction may arise with and without evidence for an adjacent leptomeningitis (Kidd et al., 2018), an occlusive arteritis resembling moya moya has been seen (Ko et al., 2009), and small cerebral and cerebellar haemorrhages are associated with more widespread leptomeningeal involvement, in which leakage from capillaries and small veins has been seen (O'Dwyer et al., 2013; Vukojevic et al., 2017). Inflammation of vessels has been cited in early pathological descriptions (Herring and Urich, 1969) and recent advances in imaging including the development of so-called black blood imaging has shown enhancement of the vessel wall (Bathla et al., 2021; Bathla et al., 2019). It seems reasonable to assume that cases of neurosarcoidosis in which infarction and/or bleeding is seen have vasculitis as a prominent part of the pathological process causing the disorder.
This is a brief and purposefully practical approach to the therapeutic and rehabilitative management of patients affected by neurological complications of systemic sarcoidosis.
The review notes the drugs used and their monitoring, and their role in the series of clinical subgroups identified to form the condition. Treatment guidelines for individual clinical subtypes of the disorder are provided, and the importance of rehabilitative measures and lifestyle changes are emphasised.
Inflammatory profiles in plasma and cerebrospinal fluid of patients with neurosarcoidosis
2022, Journal of Neuroimmunology
Cerebrospinal fluid (CSF) and plasma levels of 38 biomarkers from 20 neurosarcoidosis (NS) patients were compared to healthy controls (HC).
In CSF, 25 biomarkers were significantly elevated compared to HC: IFNγ, TNFα, TNFβ, IL-2, IL-6, IL-10, IL-12B, IL-15, IL-16, CCL2, CCL3, CCL4, CCL11, CCL13, CCL17, CCL22, CCL26, CXCL8, CXCL10, TNFR2, VEGF-A, PIGF, SAA, VCAM1, and ICAM1.
In plasma, 12 biomarkers were significantly elevated compared to HC: IFNγ, TNFα, CCL2, CCL3, CCL4, CCL17, CXCL10, VEGFR1, PIGF, SAA, VCAM1, and ICAM1.
NS patients have profoundly elevated cytokines, chemokines, vascular angiogenesis, and vascular injury biomarkers in CSF and plasma.
Neurosarcoidosis mimicry of MS: Clues from cases with CNS tissue diagnosis
2021, Journal of the Neurological Sciences
Citation Excerpt :
Autopsy and biopsy reports of acute and chronic inflammatory CNS lesions in patients with systemic sarcoid have occasionally been described in detail, and can be used to explain the considerable overlap in the MRI appearance of deep white matter lesions seen in both disorders. In the pre-MRI era, Herring et al. provided autopsy description of a case of NS involving periventricular white matter, clearly demonstrating a rounded area of granulomatous inflammation in this location [4]. Also in the pre-MRI era, periventricular predominance of neurosarcoidosis was clearly demonstrated by both histopathology and gross pathology by Matthews et al. [5].
The clinical picture of neurosarcoidosis (NS) shares many aspects with multiple sclerosis (MS). I examine whether or not clinical measures can reliably distinguish NS mimicking MS from NS coexisting with MS, and the informative role biopsy and autopsy evidence may play in understanding these two disorders. Uniquely challenging, I explore the rare patients presenting with the differential of MS or acute disseminated encephalomyelitis (ADEM) versus NS, including MS or ADEM as an associated illness in patients with systemic sarcoidosis. In most but not all NS patients, red flags against a diagnosis of MS are strong enough to rule out this more common disorder. Biopsy and autopsy findings indicate a tendency of NS granulomatous changes in the CNS to involve the same deep white matter perivascular spaces as expected to occur in MS, and hence correlate with a tendency of NS involving white matter to produce classic MRI findings of MS. The spectrum of NS includes some cases limited to a single anatomical site, including sites classically involved in demyelinating CIS (optic nerve, brainstem, and transverse myelitis). Asymptomatic “non-specific” periventricular MRI changes are described in many studies as “MS-like”. No biopsied or autopsied cases have yet proven associated classic pathological changes of MS in patients with NS.
Retrospective, dual-centre review of imaging findings in neurosarcoidosis at presentation: prevalence and imaging sub-types
2020, Clinical Radiology
Citation Excerpt :
The pathogenesis of NEWM lesions in NS remains to be fully defined. Small infarcts in NS secondary to granulomatous angiopathy have been histopathologically described previously and may be contributing to the overall lesion burden, as also noted in recent studies in non-NS population.29,30 Another postulated mechanism is extension of subependymal granulomatous process, which is again well documented pathologically.24
To assess the prevalence of various imaging manifestations in neurosarcoidosis (NS) patients at presentation and to explore if specific imaging findings may cluster in different sub-groups.
A retrospective, dual-institution, systematic imaging review was undertaken of the magnetic resonance imaging (MRI) findings in 100 consecutive NS patients who presented over a 15-year period. Clustering analysis (k-mode) was performed to evaluate co-occurrence of imaging findings.
Non-enhancing white matter (NEWM) lesions were the most common imaging abnormality (56%), followed by leptomeningeal (47%) and pachymeningeal (32%) involvement. Other common manifestations included cranial nerve involvement (30%), parenchymal granulomas (27%), hypothalamic–pituitary–adrenal axis involvement (26%), and hydrocephalus (14%). Additionally, a higher prevalence of perivascular enhancement (23%), cerebrovascular events (including ischaemic and haemorrhagic events; 17%), and ependymal involvement (20%) were noted than recognised previously. Additional k-mode analysis was performed to explore underlying disease sub-clusters. This was evaluated for clusters varying between two though five (k=2–5). For k=4, the analysis revealed that the imaging findings may possibly be divided into disease sub-sets of four groups, each with varying distribution of imaging manifestations and clinical manifestations.
Overall, NEWM lesions and meningeal involvement are the most common imaging manifestations of NS. The prevalence of perivascular enhancement, cerebrovascular events, and ependymal involvement is likely higher than reported previously. Additionally, different imaging findings in NS may cluster together and imaging subtypes in NS possibly exist.

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^∗

Present address: The Royal Cornwall Hospital, Truro, Cornwall (Great Britain).

A brief summary of the pathological findings was presented to the 4th International Conference on Sarcoidosis (Urich 1967).

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Sarcoidosis of the central nervous system

Abstract