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유지숙(Jee Suk Yu),황세희(Se Hee Hwang),이백희(Beack Hee Lee),황용승(Yong Seung Hwang),안효섭(Hyo Seop Ahn) 대한소아신경학회 1993 대한소아신경학회지 Vol.1 No.1
Antineoplastic treatment-related leukoencephalopathy is defined as an entity in which acute or delayed changes in with matter, secondary to chemotherapy and/or radiation, produce cerebral, cerebellar, and/or brainstem dysfunction in the absence of demonstrable, causally microorganism. We experienced a case of antineoplastic treatment-related leukoencepholopathy in an 11 year-old girl who was diagnosed as having acute lymphoblastic leukemia when she was 8 years old. She had 3 episodes of isolated CNS relapse during maintenance chemotherapy. One month prior to admission, she had difficulties. in adaptation of new class and refused to go to school Six days prior to admission, she began to stammer over a few words and at that night, dysarthria became overt. Next morning, locomotor activities were slow and she was not able to salk without assistance and had right foot dragging. Brain MRI revealed diffuse demyelination in deep white matter with mild degree of diffuse ventricular dilatation and prominent subarachnoid space and sulcus. She had received craniospinal irradiation(whole brain 5,040 cGy, spine 1,350 cGy), intrathecal MTX 468mg, and intrathecal Ara-C 1,890mg. Supportive managements including L-tube feeding, steroid, and mannitolization were done without chemotherapy under the impression of antineoplastic treatment-related leukoencephalopathy. Bone marrow relapse was detected during admission. Her general conditions deteriorated and decorticate posture changed to decerebarte posture. She died at 54th hospital day but autopsy was not done.
부신백질이영양증의 임상적, 생화학적 소견 및 뇌자기공명영상 소견
유지숙(Jee Suk Yu),김기중(Ki Joong Kim),고태성(Tae Seong Ko),이백희(Baeck Hee Lee),황세희(Se Hee Hwang),황용승(Yong Seung Hwang),김인원(Kim In One),지제근(Je Geun Chi) 대한소아신경학회 1993 대한소아신경학회지 Vol.1 No.1
Seven cases of adrenoleukodystrophy who were diagnosed in the Department of Pediatrics, Seoul National University Hospital from January 1985 to April 1993 were reviewed. The results were as follows: 1) All of the patients were male and the onset of symptoms was between 5 and 13 years of age. They were all classified as X-liked childhood adrenoleukodystrophy. 2) The initial symptoms were behavior change, visual disturbance, skin pigmentation poor school perfomance and gait disturbance. 3) Skin pigmentation was noticed in 3 cases which showed elevated plasma ACTH level on ACTH stimulation test, the cortisol levels were not stimulated. One case out of 4 cases having no hypoadrenal symptoms showed no response to ACTH stimulation test. 4) Elevated CSF IgG and protein levels were noticed in 4 cases of 7(57%) and 4 cases out of 6(67%), respectively. 5) There is no correlation between the evoked potentials and the subjective visual and auditory disturbance. 6) There is correlation between the involved sites on brain MRI and the clinical manifestations. 7) Brain MRI showed typically bilateral, symmetrical hyperintense areas in the periventricular white matter of parieto-occipital areas in 5 cases out of 7 cases, One case showed frontal lobe involvement only without occipital lobe and another case showed multiple patch involvement in the internal capsules and thalamus. 8) Management was including restriction of saturated VLCFA in diet, steroid therapy, physiotherapy, IV globulin, and Lorenzo oil. Clinical courses were not reversed. In conclusion, we could confirm adrenoleukodystrophy using plasma VLCFA assay without brain biopsy, and earlier diagnostic approach was possible owing to brain MIR.