Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 45-year-old woman had pyrexia, headaches, collapse and hyponatraemia. Intracerebral abscess, bacterial meningitis and subarachnoid haemorrhage were excluded. She was given intravenous antibiotics and gradually recovered. One month later she was readmitted with diplopia, headache and vomiting. Serum sodium was low (107 mmol/l) and a diagnosis of inappropriate ADH secretion was made. MRI scan showed a suprasellar tumour arising from the posterior pituitary gland. A skin rash gradually faded. Serum cortisol, prolactin, gonadotrophins and thyroid hormone levels were low. A pituitary tumour was removed trans-sphenoidally, she had external pituitary radiotherapy, and replacement hydrocortisone and thyroxine. She was well for 12 months when she developed progressive weakness and numbness of both legs. Examination suggested spinal cord compression at the level of T2 where MRI scanning showed an intradural enhancing mass. This spinal tumour was removed and her neurological symptoms disappeared. Nine months after this she developed facial pain and nasal obstruction. CT scan showed tumour growth into the sphenoid sinus and nasal cavities. A right Cauldwell-Luc operation was done and residual tumour in the nasal passages was treated by fractionated external radiotherapy and Prednisolone. Histological examination of the specimens from pituitary, spinal mass, and nasal sinuses showed Rosai-Dorfman disease, a rare entity characterized by histiocytic proliferation, emperipolesis (lymphophagocytosis) and lymphadenopathy. Aged 48 she developed cranial diabetes insipidus. Although Rosai-Dorfman syndrome is rare, it is being reported with increasing frequency, and should be borne in mind as a possible cause of a pituitary tumour.
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PMID:Rosai-Dorfman disease presenting as a pituitary tumour. 1034 67

Rosai-Dorfman disease (RDD) is an idiopathic proliferation of histiocytes that affects the lymph nodes. Central nervous system involvement in the absence of nodal disease is extremely rare. On neuroimaging studies, intracranial RDD appears as solitary or multiple well-circumscribed, dura-based lesions. The authors report on two cases of RDD with locally aggressive features including dural sinus invasion, which to their knowledge has never before been described. A 60-year-old woman presented with progressive dizziness and vertigo that had lasted for 1 week. Cranial computerized tomography and magnetic resonance imaging revealed an extraaxial homogeneous lobulated enhancing mass involving the right occipital lobe and the right cerebellar hemisphere. Invasion of the right transverse sinus was identified on a cerebral digital subtraction angiogram. A 59-year-old man with no prior medical illness experienced progressive weakness of both upper extremities and a partial complex seizure. Magnetic resonance imaging of his brain revealed a well-circumscribed enhancing mass in the left frontal lobe with extension to the right frontal lobe and invasion of the superior sagittal sinus. Both patients underwent resection of their brain masses. Pathological studies identified the disease as RDD in both patients.
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PMID:Rosai-Dorfman disease with dural sinus invasion. Report of two cases. 1579 95

Rosai-Dorfman disease is a well-established autoimmune histioproliferative disorder, and solitary central nervous system involvement is rare. A 35-year-old man presented with headache and transient blurred vision of 4 months' duration and weakness of the left extremities for 1 month. He had left temporal hemianopsia, a right nasal visual field defect of the upper medial quadrant and decreased muscle strength of the left extremities. Magnetic resonance imaging (MRI) showed a contrastenhancing tentorium-based lesion in the right trigone, intruding into the right lateral ventricle. The lesion was totally resected. Rosai-Dorfman disease was confirmed pathologically by an inflammatory infiltrate in the absence of an infectious agent, emperipolesis and positive S100 staining. From a review of the literature on Rosai-Dorfman disease in the central nervous system it is concluded that follow-up MRI should be performed in order to detect possible recurrence and that this rare entity should be considered in the differential diagnosis of intracranial lesions.
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PMID:Solitary intracranial Rosai-Dorfman disease: case report and literature review. 2211 11

We report a 48-year-old man who presents with a 20-month history of left parotid enlargement despite treatment with antibiotics and steroids. He presented with a non-painful palpable mass in his left parotid without facial weakness or otologic symptoms. Fine needle aspiration was inconclusive, and an attempted excisional biopsy was aborted due to involvement of the facial nerve and suspicion of lymphoma. Subsequently, he underwent a total parotidectomy with facial nerve preservation. The specimen yielded results that were consistent with Rosai-Dorfman disease. Of note in our patient is the lack of cervical lymphadenopathy, which classically presents with this condition. Head and neck extranodal involvement, while not unusual for Rosai-Dorfman, is rarely seen in the parotid. Previous reports of this disease found in the parotid indicate an underlying autoimmune process, which was not found in this patient.
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PMID:Rosai Dorfman Disease presenting as unilateral chronic parotitis. 2496 Jun 76

A 19-year-old woman presented with a history of severe lower backache and asymmetric proximal lower limb weakness during the past 3 months. In addition, she also suffered from lower motor neuron-type bladder and bowel symptoms. On examination, paraparesis was noted. Further, sensory examination suggested patchy asymmetric sensory loss in both lower limbs with saddle anaesthesia and areflexia. A clinical diagnosis of Conus-Cauda syndrome was made and contrast-enhanced MRI of the lumbar and sacral spine was done, which confirmed the presence of a mass lesion within the spinal canal involving the cauda equina extending up to the sacral level. She underwent partial resection of the lesion following which the neurological deficits and lower backache resolved. Histopathological evaluation and immunohistochemical analyses uncovered Rosai-Dorfman disease. There was no evidence of disease elsewhere in the body. Since the patient improved significantly following surgery and exhibited no further neurological worsening, she remains under close follow-up.
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PMID:A quintessential syndrome with a rare marvelling aetiology: Rosai-Dorfman disease presenting as Conus-Cauda syndrome. 2945 79

Rosai-Dorfman disease (RDD) is a benign histioproliferative disorder characterized by generalized lymphadenopathy, weakness, anemia, and rarely extranodal involvement. The disease affects the central nervous system (CNS) very rarely and at a relatively older age. Isolated intracranial involvement is very rare. RDD tends to present as dural masses mimicking meningioma, though very rare case reports describe isolated parenchymal involvement in the CNS. Only one case of intraventricular RDD has been described so far to the best of our knowledge. We present a very rare presentation of this disease as an isolated intraventricular presentation in the region of the atria of the lateral ventricles.
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PMID:Isolated Intraventricular Rosai-Dorfman Disease. 3045 19

Rosai-Dorfman disease (RDD) with spinal cord involvement is a rare clinical entity. We report a case of RDD with paravertebral and intraspinal epidural involvement in a 24-year-old male Bangladeshi patient who presented with progressive bilateral lower limb weakness for 20 days duration associated with spasticity and muscle spasm. MRI demonstrated an enhancing paravertebral soft tissue lesion extending from C7 through T4 with intraspinal epidural extension encasing the spinal cord with focal cord oedema. Histopathology of the paraspinal-epidural lesion reported a finding consistent with RDD. The patient was initiated on high-dose steroids. Follow up after 2 months demonstrated symptomatic improvement as the patient was able to move on the crutch and repeated MRI showed lesion regression.
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PMID:Rosai-Dorfman disease with paravertebral and epidural thoracic spine involvement: A case report and literature review. 3214 Jan 92

A 46-year-old male experienced progressive neurocognitive decline, weight loss, intermittent headaches, and weakness over 6 months. Magnetic resonance imaging of the brain revealed hydrocephalus and the spinal cord imaging showed diffuse leptomeningeal enhancement with prominent nerve root involvement. Intradural biopsy of lumbar arachnoid tissue found mixed inflammatory infiltrate consisting predominantly of histiocytes, S100 and CD68 positivity, and lymphocytophagocytosis (emperipolesis) consistent with extranodal Rosai-Dorfman disease. Rosai-Dorfman disease, a non-Langerhans cell histocytic disorder, can mimic the appearance of neurosarcoidosis and leptomeningeal carcinomatosis and should remain on the differential of a patient presenting with diffuse leptomeningeal enhancement, a common occurrence on a neurohospitalist service.
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PMID:Rosai-Dorfman Disease: A Less Common Cause of Leptomeningeal and Nerve Root Enhancement. 3298 53