Radiological manifestations of neurosarcoidosis: Report of three cases and review of literature

doi:10.1016/S0009-9260(74)80165-0

Clinical Radiology

Volume 25, Issue 3, January–October 1974, Pages 343-348

https://doi.org/10.1016/S0009-9260(74)80165-0 Get rights and content

The neuroradiological findings in three anergic patients with pituitary and endocrine disturbances, as well as typical pulmonary findings of sarcoidosis during the course of their disease, are presented. Biopsy confirmation of the intracranial lesion was obtained in one case. The pertinent literature is reviewed.

Suprasellar calcification was found in one case. Polytome pneumoencephalography demonstrated suprasellar lesions in two cases. One case had angiographic findings which could represent a type of arteritis not previously described in association with neurosarcoidosis.

The authors conclude that patients with sarcoidosis and neurological changes should undergo thorough neuroradiological evaluation to define the extent of disease. This should include, in addition to the usual plain skull radiographs, arteriography and pneumoencephalography with tomography.

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Cited by (34)

Neurologic manifestations of sarcoidosis
2014, Handbook of Clinical Neurology
Citation Excerpt :
Granulomatous masses are typically avascular (Kendall and Tatler, 1978). Rarely, angiographic abnormalities are found such as smooth narrowing of the caudal internal carotid artery (Kendall and Tatler, 1978), tapering occlusion of the anterior cerebral artery (Corse and Stern, 1990), or multiple small vessel segmental narrowings suggesting an arteritis (Lawrence et al., 1974). Patients with a diffuse encephalopathy or stroke-like episodes are the most likely to demonstrate clinically relevant angiographic findings (Oksanen, 1986).
Neurologic manifestations occur in more than 5% of sarcoidosis patients and may be the presenting feature. Neurosarcoidosis can manifest in a myriad of ways including: cranial neuropathy, aseptic meningitis, mass lesions, encephalopathy, vasculopathy, seizures, hypothalamic-pituitary disorders, hydrocephalus, myelopathy, peripheral neuropathy, and myopathy. Because its etiology is unknown, its neurological manifestations are so diverse, and its diagnosis cannot be readily confirmed by laboratory tests, neurosarcoidosis poses many clinical problems. The diagnosis of neurosarcoidosis is usually based on the identification of characteristic neurologic findings in an individual with proven systemic sarcoidosis as established by clinical, imaging, or histologic findings. Although corticosteroids are regarded as the foundation of treatment, they are not always successful and have serious side-effects. Moreover, some patients with neurosarcoidosis are refractory to conventional therapy, and approximately 5–10% die. Optimal management of patients with neurosarcoidosis benefits from an understanding of the broad clinical spectrum of neurosarcoidosis, appreciation of the ways to best confirm a diagnosis, and awareness of the full range of treatment options, including the use of alternative therapies such as immunotherapy.
Adult primary central nervous system vasculitis
2012, The Lancet
Citation Excerpt :
Additionally, intravascular lymphoma characterised by the proliferation of clonal lymphocytes within small vessels with little to no association with the organ parenchyma can affect the CNS and resemble cerebral vasculitis.93 Angiographic changes suggestive of arteritis have occasionally been reported in neurosarcoidosis; however, histological changes characteristic of vasculitis in the CNS are uncommon in this disorder.94 Secondary CNS vasculitis has also been described in patients with inflammatory bowel disease and graft-versus-host disease.95,96
Primary CNS vasculitis is an uncommon disorder of unknown cause that is restricted to brain and spinal cord. The median age of onset is 50 years. The neurological manifestations are diverse, but generally consist of headache, altered cognition, focal weakness, or stroke. Serological markers of inflammation are usually normal. Cerebrospinal fluid is abnormal in about 80–90% of patients. Diagnosis is unlikely in the presence of a normal MRI of the brain. Biopsy of CNS tissue showing vasculitis is the only definitive test; however, angiography has often been used for diagnosis even though it has only moderate sensitivity and specificity. The size of the affected vessels varies and determines outcome and response to treatment. Early recognition is important because treatment with corticosteroids with or without cytotoxic drugs can often prevent serious outcomes. The differential diagnosis includes reversible cerebral vasoconstriction syndromes and secondary cerebral vasculitis.
Neurosarcoidosis
2006, Revue Neurologique
Maladie inflammatoire, granulomateuse multisystémique, la sarcoïdose demeure toujours de cause inconnue. Le système nerveux représente une des localisations rares et graves de cette maladie bénigne. La méningite lymphocytaire, les troubles psychiques, le diabète insipide, la paralysie faciale périphérique en sont les manifestations les plus classiques. L’étude du LCR et l’IRM cérébrale et médullaire cervicale avec injection de gadolinium sont les investigations de choix. Une preuve histologique est parfois obtenue au prix de biopsies neuromusculaires, méningées, cérébrales stéréotaxiques. Les risques fonctionnels et vitaux imposent une corticothérapie précoce. En cas d’aggravation, de non-réponse ou d’une corticodépendance, d’autres immunosuppresseurs ou exceptionnellement une irradiation encéphalique seront associés. Le traitement d’entretien, le plus souvent à vie permet de maintenir le succès thérapeutique et évite les rechutes.
The origins of neurosarcoidosis, a multisystemic granulomatous disease, remain unknown. Nervous system localizations remain rare, but severe. Lymphocytic meningitis, psychiatric disorders, diabetes insipidus and cranial nerve palsy are the most frequent signs. Cerebral fluid test and cervical medullar and cerebral MRI with gadolinium have to be performed first. In some cases, histological evidence of granuloma have to be obtained with neuromuscular, meningeal or cerebral biopsies. Functional impairment and life-threatening conditions require early corticosteroid therapy. In worsening cases or in the event of no therapeutic response or poor tolerance to corticosteroids, other immunosuppressive agents should be associated. Maintenance therapy and most often life long maintenance therapy allow a continuous success while avoiding relapse.
Multiple arterial ectasias in patients with sarcoidosis and uveitis
2001, American Journal of Ophthalmology
Citation Excerpt :
Old lesions show extensive intimal fibrosis with recanalization, disruption of the elastica, and scarring of the media and adventitia. Interestingly, in sarcoidosis, arterial aneurysms and beading have been described in the brain,21,22 the aorta,23 and the heart24; in cardiac involvement, biopsy-proven noncaseating granulomas at the site of the aneurysm have been seen. Our analysis of the newly acquired arterial ectasias suggests that inflammation related to sarcoidosis may cause arterial, arteriolar, and capillary occlusion or chronic subocclusion.
PURPOSE: To describe and evaluate the cause of a clinical entity characterized by bilateral intraocular inflammation, multiple arterial ectasias including beading, macroaneurysms, comma-like ectasias and kinking, with vasculitis, staining of the optic disk and multiple peripheral round punched-out hypopigmented chorioretinal scars in elderly patients. The formation and the course of the arterial ectasias is analyzed.
METHODS: Seven patients with this syndrome were evaluated by clinical examination, fluorescein angiography, and systemic investigations.
RESULTS: Three of the seven patients had a biopsy characteristic of sarcoidosis, two others showed positive bronchoalveolar lavage, as well as other analyses and tests suggesting sarcoidosis, and two showed other findings suggestive of sarcoidosis. The patients were all over 60 years of age and had arterial hypertension. In two patients, an arterial ectasia developed at the site of previous focal inflammation. The macroaneurysms either remained unchanged, became comma-like ectasias, arterial kinks, or completely vanished.
CONCLUSION: Elderly patients with multiple arterial ectasias, uveitis, disk staining, and peripheral chorioretinitis should be thoroughly investigated for sarcoidosis. We suggest that sarcoidosis may cause some degree of arteritis, which may weaken the arterial wall, with resulting ectasia. Arterial hypertension may play a role in the formation of the ectasias by increasing the pressure on the arterial wall weakened by inflammation.
Primary (granulomatous) angiitis of the central nervous system: A clinicopathologic analysis of 15 new cases and a review of the literature
1992, Human Pathology
The clinical and pathologic features of 15 new cases of the uncommon primary or granulomatous angiitis of the central nervous system (PACNS) are described. To date, only 108 such cases have been reported in the English literature. Clinically, most PACNS patients have been young or middle-aged (mean age, 45 years; range, 3 to 96 years), with men outnumbering women slightly by a ratio of 4 to 3. The most frequent presenting complaints are headache, weakness, and confusion; less common complaints are aphasia, dysphasia, nausea or vomiting, loss of memory, and seizure disorder. There is usually no evidence of a systemic disease; the erythrocyte sedimentation rate is almost invariably normal, and there are no diagnostic laboratory tests. The cerebral angiogram usually shows multifocal, segmental stenosis or irregularity of small and mediumsized leptomeningeal and intracranial blood vessels, often with a beading or aneurysmal apprarance, and alterations in blood flow in the affected regions. Anatomically, the angiitis is focal and segmental in distribution. An isolated negative biopsy, therefore, does not rule out the disease. Histologically, PACNS may be granulomatous, necrotizing, or lymphocytic in character, and mixed morphologic types often occur. Large- and small-vessel thrombosis is common. Acute lesions frequently coexist with healing or healed lesions. Involvement of extracranial blood vessels occurs only rarely. Past or current herpes zoster infection and Hodgkin's lymphoma are the most noteworthy clinical associations of PACNS, but whether they are causally related remains uncertain.
Neurological manifestations in sarcoidosis
1988, La Revue de medecine interne

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Radiological manifestations of neurosarcoidosis: Report of three cases and review of literature

Journal of Neurologic Science

Radiologic Clinics of North America

Advances in Pediatrics

British Journal of Diseases of the Chest

Sarcoidosis of the central nervous system

Journal of Neuropathology and Experimental Neurology

Allergic granulomatosis, allergic angiitis, and periarteritis nodosa

American Journal of Pathology

Sarcoidosis with involvement of the nervous system

Brain

Principles of X-ray Diagnosis of the Skull

Extra-ventricular (communicating) hydrocephalus; some observations on the midline ventricles

American Journal of Roentgenology, Radium Therapy and Nuclear Medicine

Angiography in tuberculous meningitis

Acta Radiologica

Giant cell (cranial) arteritis

American Journal of Roentgenology, Radium Therapy and Nuclear Medicine

The relationship between periarteritis nodosa and sarcoidosis

Annals of Internal Medicine

Sarcoidosis of the nervous system

Brain

Sarcoidosis: A rare cause of spinal cord widening

Radiology