: 59 4 2000 (Cerebral demyelinating disease) 1, *, * = A bs trac t= A c as e of ce re bral de my elinating dis e as e af ter allog e ne ic bone marrow trans plantation Ji- Min Lee, M.D., Hong Ghi Lee, M.D., Jong Rak Hong, M.D., Sook- In Jung, M.D., Yeon- Lim Suh, M.D. and Dong Gyu Na, M.D. Department of Internal M edicine, Sungkyunkwan University, College of M edicine, Samsung M edical Center, Seoul, Korea We report a case of cerebral demyelinating disease developed in a 24- year- old male who underwent allogeneic bone marrow transplantation (BMT) from a HLA- matched unrelated donor for the treatment of acute myelogenous leukemia in the first complete remission. At the sixteenth month after BMT, he revealed rapidly progressive neurological signs as motor weakness of left leg, urinary incontinence, and dysarthria. T here was no evidence of chronic graft- versus- host disease or any infection. Cyclosporin was tapered off two months before the event. Magnetic resonance imaging (MRI) demonstrated widespread changes in white matter with peripheral rim enhancement in contrast media. Stereotactic brain biopsy from the lesions disclosed demyelination associated with active phagocytosis of myelin. He was treated with dexamethasone 4mg IV every 12 hours for 20 days, which resulted in the complete recovery of neurological defects and the slightly decreased extent of brain lesions on MRI. With oral prednisolone 50mg daily, which were tapered over the next 3 months, the follow- up MRI in a month interval demonstrated markedly decreased size and number of the previous lesions. He has been doing well without any neurological sequelae for the last twelve months since the last MRI.(Korean J Med 59:433-437, 2000) Key Words : Bone marrow transplantation; Leukemia, Myelocytic, Acute; Demyelinating Diseases; Steroids : 1999331 : 1999913 :, 50, (135-710) E- mail : jimin98@intizen.com (myasthenia gravis), (polymyositis), (inflammatory demyelinating polyneuropathy) 1-9). (cerebral demyelinating disease), 1. - 433 -
Korean Journal of Medicine : Vol. 59, No. 4, 2000 : 24 16, 1,. 13 cyclosporin prednisolone,.. :, Figure 1. Initial brain MRI. T he T2WI shows multiple small to moderate sized hyperintense lesions with mild surrounding edema in the subcortical white matter of both frontoparietal lobes., Babinski, Hoffman,.. :,,, 45 mmh2o, WBC 0/L, RBC 0/L, ph 7.4, 33 /dl, 69/dL.,,,. T 2 (Figure 1, 2),. (stereotactic biopsy), axon (macrophage) (demyelination), (Figure 4, 5). : dexamethasone 4 mg12,. 20,, dexamethasoneprednisolone 50 mg/d, 3. 1 (Figure 3), 12. Figure 2. Initial MRI. Following contrast administration, peripheral rim enhancements are demonstrated. Figure 3. Follow- up MRI. On T 2WI, there has been interval decrease in the extent of previous noted multiple hyperintense lesions. - 434 -
Ji- Min Lee, et al : A case of cerebral demyelinating disease after allogeneic bone marrow transplantation Figure 4. Bodian silver stain shows preserved axon in lesion.,,,,., (myasthenia gravis), (polymyositis), (inflammatory demyelinating polyneuropathy),. (MHC) class, (allogeneic stimulation) 10), (global neurologic signs) 11, 12). (cerebral demyelinating disease) (myelin),,,,, (multiple sclerosis),, (acute disseminated encephalomyelitis), (acute hemorrhagic leukoencephalitis).,,,,,,,,,. (pseudobulbar palsy),. Figure 5. Luxol- fast blue stain shows numerous macrophages containing granules of blue myelin debris and extensive demyelination in lesion.,,., (interferon 1b, interferon 1a, cyclophosphamide, cyclosporin, azathioprine, methotrexate). MRI methylprednisoloneprednisolone, INF, copolymer 1(copaxone), methotrexate, azathioprine, cyclophosphamide. methylprednisolone, prednisolone, INF, copolymer 1. baclofen, diazepam, cyclobenzaprine hydrochloride, clonazepam, tizanidine, clonidine hydrochloride, dantrolene, glucocorticoid, carbamazepine, phenytoin, amitriptyline. carbamazepine, acetazolamide, clonazepam, primidone, ondansetron, isoniazid stereotatic thalamotomy. bladder hyperreflexiaanticholinergics, bladder hyporeflexiacholinergics, laxatives, enemas, low fiber diet. cerebral demyelinating disease, 5 (acute Parkinsonian syndrome) MRI demyelinating leukoencepha- - 435 -
: 59 4 482 2000 lopathy 4 13), 20 multifocal remitting- relapsing cerebral demyelinating disease 14). 50 leukoencephalopathy 15). cerebral demyelinating disease,, w hite matter long tract, MRI 2, 24,, (multiple sclerosis),. progressive multifocal leukoencephalopathy(pml), PML,,,, (JC virus), MRI,. PML,,,,,,,,,.,.,,. 16,, cerebral demyelinating disease. RE F E R E N C E S 1) Bolger GB, Sullivan KM, Spence AM, Appelbaum FR, Johnston R. M yasthenia gravis after allogeneic bone marrow transplantation: relationship to chronic graft- versus- host disease. N eurology 36:1087-1091, 1986 2) Grav JM, Casademont J, Monforte R, Marin P, Granena A. M ysthenia gravis after allogeneic bone marrow transplantation: report of a new case and pathogenetic considerations. Bone Marrow Transplant 5:435-437, 1990 3) Seely E, Drachman D, Smith BR, Antin JH, Ginsburg D, Rappeport JM. Post bone marrow transplantation (BM T ) myasthenia gravis: evidence for acetylcholine receptor (A ChR) abnormality. Blood 64(Suppl):211a, 1984 4) Smith CI, Aarli JA, Biberfeld P, Bolme P. M yasthenia gravis after bone marrow transplantation: evidence for a donor origin. N Engl J M ed 309:1565-1568, 1983 5) Anderson BA, Young PV, Kean WF, Ludwin SK. Polymyositis in chronic graft- versus- host disease. Arch N eurol 39:188-190, 1982 6) Pier N, Dubowitz V. Chronic graft versus host disease presenting with polymyositis. Br M ed J 286: 2024, 1983 7) Maguire H, August C, Sladky J. Chronic inflammatory demyelinating polyneuropathy: a previous unreported complication of bone marrow transplantation. N eurology 39(Suppl):410, 1989 8) Eliashiv S, Brenner T, Abramsky O, Shahin R, Agai E, Naparstek E. Acute inflammatory polyneuropathy following bone marrow transplantation. Bone M arrow T ransplant 8:315-317, 1991 9) Adams C, August CS, Maguire H. N euromuscular complications of bone marrow transplantation. Pediatr N eurol 12:58-61, 1995 10) Hickey WF, Kimura H. Graft- vs.- host disease elicits expression of class and class histocompatibility antigens and the presence of scattered T lymphocytes in rat central nervous system. Proc N atl Acad Sci USA 84:2082-2086, 1987 11) Rouah E, Gruber R, Shearer W, Armstrong D, Hawkins EP. Graft- versus- host disease in the central nervous system: a real entity? Am J Clin Pathol 89:543-546, 1988 12) Marosi C, Budka H, Grimm G, Zeitlhofer J, Sluga E, Brunner C. Fatal encephalitis in a patient with chronic graft- versus- host disease. Bone M arrow T ransplant 6:53-57, 1990-436 -
5: (Cerebral demyelinating disease) 1 13) Lockman LA, Sung JH, William K. Acute Parkinsonian syndrome with demyelinating leukoencephalopathy in bone marrow transplant recipients. Pediatr N eurol 7:457-463, 1991 14) Kelly P, MRCPI, Staunton H, Lawler M, Brennan P, Jennings S, Unger ER, Sung JH, Farrell MA. M ultifocal remitting- relapsing cerebral demyelination twenty years following allogeneic bone marrow transplantation. J N europathol Exp N eurol 55:992-998, 1996 15) Li CK, Kwan WH, Matthew MK, Patrick MP. Unusual case of leukoencephalopathy after bone marrow transplantation. Pediatr Hematol Oncol 11:651-656, 1994-437 -