Neural and visceral xanthomatosis in adults

Miller, A. A.; Ramsden, F.

doi:10.1136/jcp.18.5.622

Cited by 16 publications

(2 citation statements)

References 11 publications

(12 reference statements)

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“…3,4,17) Spinal cord involvement is rare, and usually occurs by direct extension from the surrounding bone tissue, but may also arise as metastatic spread from other tissues. 1,5,8,9,11,15,17,20,22,24,27) Isolated extramedullary or intramedullary lesion of the spinal cord is extremely rare. 10,24) We report an extremely rare case of primary intramedullary spinal LCH of the thoracic spine.…”

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confidence: 99%

Primary Intramedullary Langerhans Cell Histiocytosis of the Thoracic Spinal Cord

Yamagata

Takami

Yamamoto

et al. 2013

Neurol. Med. Chir.(Tokyo)

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A 28-year-old male presented with a rare case of primary intramedullary spinal Langerhans cell histiocytosis (LCH) manifesting as the chief complaint of a 6-month history of gait disturbance and back pain, and difficulty with sphincter control. Serial T 2 -weighted magnetic resonance imaging of the thoracic spine revealed enlargement and intramedullary hyperintensity of the spinal cord at T2 to T4. Biopsy of the lesion was performed. Histological examination of the biopsy specimens verified vascular proliferation and remarkable infiltration of histiocytes that were positive for CD1a, suggesting a diagnosis compatible with LCH. The patient was treated successfully by steroid pulse therapy. LCH is a rare disease that occurs mainly in children and may cause a broad range of manifestations, from a single osseous lesion to multiple lesions involving more than one organ or system. The present case illustrates the unexpected occurrence and important differential diagnosis of primary intramedullary spinal LCH of the thoracic spine in adult patients presenting with progressive paraparesis and back pain.

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Section: Introductionmentioning

confidence: 99%

Primary Intramedullary Langerhans Cell Histiocytosis of the Thoracic Spinal Cord

Yamagata

Takami

Yamamoto

et al. 2013

Neurol. Med. Chir.(Tokyo)

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“…Beard e col. difuso e infiltratives ou de modo localizado, tumoral, geralmente periventricular. O comprometimento das meninges raquidianas ou da medula espinhal é excepcional 23 . Apesar de em certos casos registrados na literatura 10 .…”

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Comprometimento de estruturas encefálicas não hipotalâmicas na moléstia de Hand-Schüller-Christian: relato de caso e revisão da literatura

Souza-Lima

Silva

1975

Arq. Neuro-Psiquiatr.

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Histiocitoses X são enfermidades raras do sistema retículo endotelial caracterizadas pela proliferação de células reticulares e da linhagem histiocitária. contendo estas últimas no citoplasma corpúsculos ou granulos de Langerhans visíveis ao microscópio eletrônico 12 > 25 . Três são os seus representantes: 1) moléstia de Letterer-Siwe que incide em crianças até os dois anos de idade e se caracteriza por equimoses cutâneas, hépato-esplenomegalia, trombocitopenia, com evolução aguda e fatal; 2) moléstia de Hand-Schüller-Christian que compromete grupo etário de 2 a 6 anos, caracterizando-se pela tríade diabete insí-pido, exoftalmo e granulomas ósseos, com evolução sub-aguda, sendo referidos casos de regressão espontânea dos sinais clínicos e dos achados de exames complementares 1 ; 3) granuloma eosinófilo que aparece na idade adulta, caracterizando-se pelo achado granulomatoso ao exame radiográfico, com evolução crônica.Em numerosos casos de moléstia de Hand-Schüller-Christian o diabete insípido e o exoftalmo são devidos ao comprometimento da região hipotálamo-hipofisária e da cavidade orbitaria por lesões ósseas vizinhas. Aragonés e col.2 , Beard e col. 4 , Duval e col. 12e Henschen 17 mostraram que esses elementos da tríade clássica podem ser devidos também a lesão primária das meninges cranianas, estruturas essas freqüentemente atingidas nesta moléstia, quer de modo difuso predominando na base do encéfalo, quer de modo localizado determinando a forma meningeana tumoral. Beard e col. 4 dão ênfase ao fato que o hipotálamo, devido a sua rica vascularização, não raramente é sede de processo infiltrative primário nesta moléstia. Assim, o diabete insípido que aparece na enfermidade de Hand-Schüller-Christian, além de poder ser devido à compressão de estruturas diencefálicas por processos ósseos ou meningeanos vizinhos, pode também ser devido à uma infiltração histiocitária primária da região hipotalâmica. Exceptuando o hipotálamo, o comprometimento de outras regiões do encéfalo nessa moléstia é raro e, quando isso se dá, pode ser de modo

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Plasma cell granuloma (inflammatory pseudotumor) of the spinal cord meninges.Report of a case

Eimoto

Yanaka

Kurosawa

et al. 1978

Cancer

110

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A case of plasma cell granuloma (inflammatory pseudotumor) arising in the meninges of cervical spinal cord of a 37-year-old Japanese man is presented. The diagnosis was made by analogy with the similar lesions known in the lung. Its histology was characterized by infiltration of three kinds of cells; histiocytes with slight epithelioid appearance, plasma cells with numerous Russell bodies, and lymphocytes. Electron microscopy of the formalin-fixed tissue was useful for the identification of these cells, particularly of histiocytes. Although extrapulmonary plasma cell granulomas have been reported, this is the first case, to our knowledge, of this type "tumor" in the spinal cord meninges. Differential diagnoses have been briefly discussed. xanthoma, lo fibrous xant h~m a ,~ xanthogranuloma, mast cell granuloma," and even plasma cell tumor.' While these multiple designations refer to pathogenesis and/or a predominant histologic appearance of a single entity, the benign, nonneoplastic character of the lesion has to be recognized since it can be confused with a true neoplasm both clinically and pathologically.Most of the cases have occurred in the lung and extrapulmonary lesions have been rare. Although a few cases were noted in the liver,lg stomach, '3 and peritoneal and retroperitoneal spaces,31 no previous case has been reported as plasma cell granuloma in the meninges. T h e purpose of this investigation is to describe such a From the Clinical Laboratory and Neurosurgical Division, Koshigaya City Hospital, Saitama-ken, Japan. "tumor" presenting itself as a n intradural and extramedullary mass in the cervical spinal cord and to discuss some differential diagnoses. CASE REPORTA 37-year-old ,Japanese man, a leatherette salesman, was admitted to the Koshigaya City Hospital because of weakness and sensory deficiency in the extremities. He had been well until a year prior to the admission when he developed paresthesia and progressive weakness of the left hand with subsequent involvement of the left lower extremity and right hand as well. There was no nausea, back pain or urinary disturbance, however. Upon admission the neurologic examination revealed hyperactive left tendon reflexes with positive Babinski reflex and bilateral decrease of pain, touch, and vibratory sensation below the level of C6. The extremities showed weakness, prominent in the left side. A lumbar puncture revealed a normal opening pressure but absence of Queckenstedt sign, 49 cells/mm3 (mostly lymphocytes), and elevated CSF protein (380 mg/100 ml). Myelography showed a nodular defect at C4 to C5 level with deviation of the spinal cord to the right (Fig. 1). All the other laboratory findings were negative including normal serum levels of gamma globulin and of lipids and negative serological tests for syphilis. At laminectomy the meningeal incision demonstrated an oval, extramedullary mass in the left posterior angle between the third and fourth posterior nerve roots, compressing the cervical spinal cord. However, there was no adhesion between the mas...

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Neural and visceral xanthomatosis in adults

Cited by 16 publications

References 11 publications

Primary Intramedullary Langerhans Cell Histiocytosis of the Thoracic Spinal Cord

Primary Intramedullary Langerhans Cell Histiocytosis of the Thoracic Spinal Cord

Comprometimento de estruturas encefálicas não hipotalâmicas na moléstia de Hand-Schüller-Christian: relato de caso e revisão da literatura

Plasma cell granuloma (inflammatory pseudotumor) of the spinal cord meninges.Report of a case

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