Regression of intracranial Rosai–Dorfman disease following corticosteroid therapy

2006 ◽  
Vol 104 (5) ◽  
pp. 840-844 ◽  
Author(s):  
Christopher M. McPherson ◽  
Justin Brown ◽  
Angela W. Kim ◽  
Franco Demonte

✓ Rosai–Dorfman disease (RDD) is an idiopathic histioproliferative disorder usually presenting with massive, painless lymphadenopathy. Extranodal involvement has been reported including at least 50 cases affecting the central nervous system (CNS). The treatment of CNS RDD as reported in the literature has primarily involved a surgical technique. The authors report on the case of a 53-year-old man presenting with multiple skull base lesions mimicking meningiomas. The patient suffered visual deterioration and underwent a right orbitopterional craniotomy as well as optic nerve decompression. Histopathological analysis revealed histiocytic cells and emperipolesis consistent with RDD. Following surgery, corticosteroid agents were administered, leading to marked resolution of both the remaining surgically untreated lesions and the balance of the patient's symptoms. This report represents the first case of the resolution of intracranial RDD following corticosteroid therapy. Corticosteroid agents should be considered an effective option in the treatment of CNS RDD.

2013 ◽  
Vol 5 (1) ◽  
pp. 51-63 ◽  
Author(s):  
Stuart I. Jenkins ◽  
Mark R. Pickard ◽  
Melinda Khong ◽  
Heather L. Smith ◽  
Carl L.A. Mann ◽  
...  

Neurosurgery ◽  
2005 ◽  
Vol 56 (1) ◽  
pp. E187-E190 ◽  
Author(s):  
Jun Shinoda ◽  
Satoru Murase ◽  
Katsunobu Takenaka ◽  
Noboru Sakai

Abstract OBJECTIVE AND IMPORTANCE: The first case of histologically proven hemophagocytic lymphohistiocytosis (HLH) isolated to the central nervous system (CNS) is reported. HLH affecting the CNS mimics several neurological disorders and may be misdiagnosed. The diagnostic and therapeutic problems of this disease are discussed. CLINICAL PRESENTATION: We report a case of a 5-year-old girl with a 2-month history of right hemiparesis. The initial magnetic resonance imaging scan mimicked the appearance of malignant glioma or cerebral infarction. By use of neuroimaging alone, it was extremely difficult to reach an appropriate diagnosis. INTERVENTION: Pathological examination of a surgical specimen of the lesion revealed histological characteristics typical of HLH. Because of the absence of both physical and blood chemical findings of systemic HLH, the patient was diagnosed as having HLH isolated in the CNS without systemic HLH. Radiotherapy with corticosteroid administration led to complete resolution of the CNS lesions, but the duration of remission was only 3 months. The patient died secondary to refractory progression of the CNS lesion. CONCLUSION: Radiotherapy with corticosteroid administration led to a complete resolution of the lesions, although for only a transitory remission. Although HLH is extremely rare, the existence of this disease isolated in the CNS should be documented, and further case accumulation and therapeutic investigations are needed to clarify the pathophysiological characteristics of this disease.


2015 ◽  
Vol 157 (9) ◽  
pp. 1565-1571 ◽  
Author(s):  
Yongji Tian ◽  
Junmei Wang ◽  
Mingtao Li ◽  
Song Lin ◽  
Guihuai Wang ◽  
...  

2017 ◽  
Vol 127 (3) ◽  
pp. 687-690 ◽  
Author(s):  
Jiro Akimoto ◽  
Kenta Nagai ◽  
Daisuke Ogasawara ◽  
Yujiro Tanaka ◽  
Hitoshi Izawa ◽  
...  

Sarcoidosis is a systemic granulomatous disease with unknown cause, which very rarely occurs exclusively in the central nervous system. The authors performed biopsy sampling of a mass that developed in the left tentorium cerebelli that appeared to be a malignant tumor. The mass was diagnosed as a sarcoid granuloma, which was confirmed with the onset of antibody reaction product against Propionibacterium acnes. Findings suggesting sarcoidosis to be an immune response to P. acnes infection have recently been reported, and they give insight for diagnosis and treatment of this disease. The authors report the possible first case that was confirmed with P. acnes infection in a meningeal lesion in solitary neurosarcoidosis.


Neurosurgery ◽  
2007 ◽  
Vol 61 (6) ◽  
pp. E1336-E1337 ◽  
Author(s):  
Daina Kashiwazaki ◽  
Kazutoshi Hida ◽  
Shunsuke Yano ◽  
Toshitaka Seki ◽  
Yoshinobu Iwasaki

Abstract OBJECTIVE Hemangiopericytomas, vascular tumors arising in soft tissue, are relatively rare in the central nervous system; they comprise less than 1% of all hemangiopericytomas. Central nervous system hemangiopericytomas occur primarily in the epidural space of the brain and spinal cord. There are no previous reports of subpial, extramedullary growing central nervous system hemangiopericytomas. CLINICAL PRESENTATION We document the first case of a subpial hemangiopericytoma with extramedullary growth in the thoracic spine. The patient was a 31-year-old man who developed progressively worsening left lower limb numbness that was followed by gait disturbance over the course of 4 months. INTERVENTION Magnetic resonance imaging revealed an intradural tumor at the T4–T6 level of the thoracic spine. Because the patient's symptoms progressed, he underwent resection of the tumor, which had arisen in the spinal cord subpially without attachment to the dura mater. CONCLUSION The pathological diagnosis was hemangiopericytoma. Differential diagnoses include hemangioblastoma, meningioma, schwannoma, and solitary fibrous tumor, the clinical course and prognosis of which are different from hemangiopericytoma. Our experience indicates that hemangiopericytomas can occur as intradural tumors arising from the subpial portion.


2013 ◽  
Vol 62 (2) ◽  
pp. 217-219 ◽  
Author(s):  
ROBERT WALTEREIT ◽  
ULRICH HERRLINGER ◽  
MAIK STARK ◽  
STEFAN BORGMANN

Infection of the central nervous system by streptococci is known to result in severe bacterial meningitis, however some strains have low pathogenic potential and affect the brain only in immunocompromised patients. Here we report the first case of an otherwise healthy non immunocompromised young adult woman who developed meningitis caused by Streptococcus dysgalactiae subspecies equisimilis. The patient was in the 17th week of her 3rd pregnancy. The course of the disease was quickly remittent under antibiotic treatment.


2016 ◽  
Vol 25 (2) ◽  
pp. 158-162 ◽  
Author(s):  
José Ledamir Sindeaux Neto ◽  
Michele Velasco ◽  
José Mauro Vianna da Silva ◽  
Patricia de Fátima Saco dos Santos ◽  
Osimar Sanches ◽  
...  

Abstract The genus Myxobolus, parasites that infect fishes, which cause myxobolosis, includes spore organisms belonging to the phylum Myxozoa and represents approximately 36% of all species described for the entire phylum. This study describes lymphocytic meningoencephalomyelitis associated with Myxobolus sp. infection in the brain and spinal cord (the central nervous system, CNS) of Eigenmannia sp., from the Amazon estuary region, in the Administrative District of Outeiro (DAOUT), Belém, Pará, Brazil. In May and June 2015, 40 Eigenmannia sp. specimens were captured from this region and examined. The fish were anesthetized, slaughtered and dissected for sexing (gonad evaluation) and studying parasites and cysts; after diagnosing the presence of the myxozoans using a light microscope, small fragments of the brain and spinal cord were removed for histological processing and Hematoxylin-Eosin and Ziehl-Neelsen staining. Histopathological analysis of the brain and spinal cord, based on histological sections stained with Hematoxylin-Eosin, pronounced and diffuse edema in these tissues, and congestion, degeneration, and focal necrosis of the cerebral cortex. The present study describes lymphocytic meningoencephalomyelitis associated with infection by Myxobolus sp. in the central nervous system of Eigenmannia sp.


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