Letter to the EditorNeurosarcoidosis and multiple intracerebral hematomas: An unusual clinical presentation
Introduction
Neurosarcoidosis (NS) represents a heterogeneous clinical entity accountable for the variety of neurological and other symptoms and signs. Pathological changes (non-caseating granulomas, vasculitic and ischaemic changes) can affect meningeas, brainstem, spinal cord and peripheral nerves [1]. Based on the Zajicek criteria diagnosis can be classified as definitive, probable and possible. In addition to a clinical findings suggestive of NS, definitive diagnosis requires a pathohistological confirmation of CNS granuloma and exclusion of other causes of neurological symptoms. Probable NS lacks a pathohistological confirmation, but in addition to typical presentation, can be supported by MRI findings, elevated CSF protein levels and/or pleocytosis, oligoclonal bands, confirmed systemic sarcoidosis and elevated serum angiotensin converting enzyme level (ACE). Possible NS requires a clinical findings suggestive of NS and exclusion of other causes of neurological symptoms [2]. Intracerebral hemorrhage in neurosarcoidosis is rare. To our knowledge, only twenty cases have been recorded so far [3]. Our patient had a single granulomatous change in the pons which showed significant radiological regression after the introduction of steroids, followed by a clinical improvement, too. Upon gradual discontinuation of steroids multiple cerebral and cerebellar hematomas were observed. Neurosarcoidosis treatment is less effective in comparison to sarcoidosis without CNS involvement and it's not supported by evidence-based guidelines or controlled randomised trials. Algorithm created by Nozaki and Judson, can be helpful especially in treatment of severe cases, comprises of methylprednisolone (500–1000 mg/day 3–5 days), followed by prednisone (40–100 mg/day) and another immunomodulatory drug, with methotrexate as a first choice [4].
Section snippets
Case report
A 45-year-old female was admitted to neurology department due to a headache and imbalance. Symptoms were slowly progressive over the past 3 months.
Four years prior, she experienced left-sided facial weakness, parotid swelling, and fatigue. Ancillary tests, at the time, revealed hilar lymphadenopathy (chest radiograph and CT scan). Biopsy of the parotid gland showed granulomatous inflammation. Based on diagnostic criteria (Zajicek 2000), diagnosis of probable neurosarcoidosis was established [2].
Discussion
Intracerebral hemorrhage in NS is extremely rare and to our knowledge, there are only twenty published cases so far. It occurs twice often in men, opposite to sarcoidosis and neurosarcoidosis without bleeding, which is more common in women. Hematomas can resemble to those seen in stroke, particularly in younger patients without typical risk factors for cerebrovascular disease. Intracerebral hemorrhage in NS was the first manifestation of the disease in 46% of all sarcoid cases [6]. Hematomas
References (7)
- et al.
Presentation and outcomes of neurosarcoidosis: a study of 54 cases
QJM
(2009) Neurosarcoidosis
Curr. Opin. Neurol.
(2000)- et al.
Spinal cord hemorrhage in a patient with neurosarcoidosis on long-term corticosteroid therapy: case report
BMC Neurol.
(2015)
Cited by (9)
Management of neurosarcoidosis
2022, Journal of NeuroimmunologyCitation Excerpt :So the length of treatment depends on the imaging and not its duration. 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).
Acute central nervous system vasculitis as a manifestation of neurosarcoidosis: A case report and literature review
2021, Radiology Case ReportsCitation Excerpt :However, some reports demonstrate the efficacy of immunosuppressive therapy over hemorrhagic lesions [16,17]. Multiple reports show that intracranial hemorrhages increase with steroid tapering and discontinuation [17–20]. In this case, microhemorrhages and microinfarct relapsed after corticosteroid reduction, but restarting and continuing corticosteroid treatment was sufficiently effective.
Neuroimaging Findings in Intracranial Sarcoid Phlebitis: A Case Report
2019, Journal of Stroke and Cerebrovascular DiseasesCitation Excerpt :The authors present a case of venous phlebitis in NS diagnosed using VWI. ICH in NS often tends to be small and present in atypical locations, a finding felt to reflect both arterial and venous involvement.4-6 Bathla et al recently reported imaging findings in 4 patients with NS phlebitis, with all patients showing evidence of ICH.
Cerebrovascular manifestations in neurosarcoidosis: how common are they and does perivascular enhancement matter?
2018, Clinical RadiologyCitation Excerpt :Another interesting observation, although of unclear significance, was occurrence of new symptomatic bleed in patient 18, within a week of reducing the dose of oral steroids once the patient was clinically stable. Few prior reports have also made similar observations.16–19 Taken together, these clusters of cases may suggest that the reduction in immunosuppressive dose and the increased friability of the inflamed veins may predispose to symptomatic ICH.
A Case of Neurosarcoid Presenting as Multiple Intraparenchymal Hemorrhages
2022, NeurohospitalistNeurosarcoidosis: Clinical manifestations, investigation and treatment
2020, Practical Neurology