급성 출혈성 백질 뇌염에서 확산텐서영상 추적검사의 유용성 증례
고려대학교 의과대학 소아과학교실
최원지・변정혜・은소희・은백린・김건하
Case report
This report was presented at the annual meeting of Korea Child Neurology Society in 2016.
Submitted: 10 March, 2017 Revised: 28 March, 2017 Accepted: 29 March, 2017 Correspondence to Gun-Ha Kim
Department of Pediatrics, Korea University Guro Hospital, 148, Gurodong-ro, Guro-gu, Seoul, 08308, Korea
Tel: + 82-2-2626-1222, Fax: +82-2-2626-1249 E-mail: [email protected]
J Korean Child Neurol Soc 2017;25(1):54-57 pISSN 1226-6884•eISSN 2383-8973
Utility of Follow-up Diffusion Tensor Imaging in Acute Hemorrhagic Leukoencephalitis: a Case Report
Acute hemorrhagic leukoencephalitis (AHLE) is an acute, rapidly progressing, fulminant demyelinating disease. It is a rare disease of the central nervous system with high mortality; survivors commonly present with significant neurological deficit. We report the case of a 16-month-old girl who survived AHLE and presented with the associated neurologic deficit. The patient came into the emergency department with febrile seizure. She showed bilateral pinpoint-sized pupils and hyperactive deep tendon reflexes. Her mental status was initially drowsy and rapidly progressed to stupor. Extensive demyelination and microbleeds were found in the cerebral white matter, thalamus and left cerebellum on Magnetic resonance imaging (MRI) scans. Her mental status was improved by intravenous administration of immunoglobulin and methylprednisolone. Five months after being discharged, increased white matter connectivity was found on color-coded follow-up MR diffusion tensor imaging (DTI) as compared to previous MRI. We therefore suggest adding the DTI technique when a follow-up MRI is performed in patients with AHLE.
It could be useful to visualize the status of axonal injury and to encourage patients and their parents to continue the rehabilitation program.
Key Words: Acute hemorrhagic leukoencephalitis, Diffusion tensor imaging, Seizure, Child
Won Jee Choi, MD, Jung Hye Byeon, MD, PhD, So-Hee Eun, MD, PhD, Baik-Lin Eun, MD, PhD, Gun-Ha Kim, MD, PhD
Department of Pediatrics, College of Medicine, Korea University, Seoul, Korea
Copyright © 2017 by The Korean Child Neurology Society
http://www.cns.or.kr
Introduction
Acute hemorrhagic leukoencephalitis (AHLE) is a rare, fulminant demyelinating disease1-3). AHLE was first described by Weston Hurst in 19414). Since Hurst's description, less than 100 adult and 10 pediatric cases of AHLE have been reported3,5) and there is limited evidence regarding the long-term prognosis of AHLE. We report the case of a patient diagnosed with AHLE who had undertaken follow-up Magnetic resonance imaging (MRI) with diffusion tensor imaging (DTI) to visualize neuronal plasticity.
J Korean Child Neurol Soc 2017;25(1):54-57 55 http://www.cns.or.kr
Case Report
A 16-month-old girl came into our emergency department with febrile seizure. Five days prior to the onset of febrile convulsion, an upper airway infection occurred. Her tempera- ture was 39.2℃, pulse 122 beats/min, blood pressure 108/52 mmHg, and respiratory rate 28 breaths/min. She had another generalized tonic-clonic seizure at the emergency department.
The seizure stopped 35 min after an intravenous injection of phenobarbital. Her mental status rapidly deteriorated to coma and the deep tendon reflex was increased. On the 1st day of admission to ICU, GCS was 3, body temperature, 37.8℃, blood pressure, 122/43 mmHg and respiratory rate, 28/min. The results from cerebrospinal fluid (CSF) analysis were normal. The patient was treated with vancomycin, ceftriaxone, and acyclovir until the results of CSF analysis and serologic tests were negative. CSF multiplex polymerase chain reaction (PCR) test for bacteria (e.g.
Streptococcus pneumoniae, Neisseria meningitidis, Haemo- philus influenzae, Listeria monocytogenes, Group B Strepto- coccus), and viruses (e.g. herpes simplex virus (HSV) type 1,
HSV type 2, enterovirus, Epstein-Barr virus (EBV), and varicella zoster virus) showed negative result. An electroencephalogram showed diffuse delta waves. Initial MRI revealed bright T2 signal intensity from the cerebral white matter and the thalamus, while Gradient echo (GRE) sequences showed patches of low signal intensity denoting parenchymal hemorrhages in both cerebellar hemispheres. (Fig.1).
The MRI images suggested the diagnosis of AHLE. The patient was treated with methylprednisolone, 30 mg/kg/d for 5 d, and intravenous immunoglobulin (IVIG), 2 g/kg for 3 d. After the patient was admitted to the intensive care unit, she was progressively deteriorating and she showed sunset eye sign. To control the intracranial pressure, she was treated with intubation, hyperventilation, and administration of hypertonic saline, mannitol. On the 3rd day of admission, a cranial compu- ted tomography (CT) scan with contrast showed hypodensity of the white matter, but there were no signs of sulcal effacement and cerebral edema. On the 5th day of admission, her mentality improved slowly and she started to open her eyes. On the 8th day of admission, she was transferred from the intensive care
Fig. 2. Follow-up MRI after 5 months. (A) T2 weighted image (T2WI) shows diffuse brain atrophy. Compared to the diffusion tensor image (DTI) obtained 5 months earlier (B), the follow-up DTI (C) shows increased white matter connectivity.
Fig. 1. MRI on 1st hospital day. (A) T2 weighted image (T2WI) shows high signal intensity relative to the white matter and thalamus in both hemispheres, and in the left cerebellar hemisphere. (B) Apparent diffusion coefficient (ADC) sequence shows diffuse diffusion restriction. (C) Gradient echo (GRE) sequence shows multiple hemorrhagic lesions in both hemispheres.
56 Cho Y, et al. • Cerebral Palsy from Consumptive Coagulopathy Caused by Protein C Deficiency http://www.cns.or.kr
unit (ICU) unit to a general ward and started physiotherapy.
After 1 month, she could respond by smiling and move her head from side to side, but she could not control her neck; in addition, she showed increased tonicity in both upper and lower extre- mities. She was transferred to another hospital for rehabilitation.
Six months later, increased white matter connectivity was found on color-coded MRI with DTI as compared to the previous scan collected 5 months earlier (Fig. 2B and 2C).
Discussion
AHLE is characterized by hyperacute inflammatory demyelia- tion of the white matter with hemorrhagic necrosis and auto- immune encephalopathy1-3). This disease is generally recognized as the most severe form of acute disseminated encephalo- myelitis (ADEM)3,6-9). Both are usually preceded by either infec- tion of the upper respiratory tract or viral infection such as HSV, EBV, rubella, measles, mumps or influenza virus or vaccination.
The illness may begin with fever, headache, seizures, finally leading to altered consciousness and death, which usually occurs within hours to days6). Both diseases could be caused by an autoimmune process directed against myelin as shown in experimental autoimmune encephalomyelitis (EAE), an animal model of CNS inflammation and demyelination10,11). There are distinctions between AHLE and ADEM. AHLE is usually diag- nosed in young adults, whereas ADEM affects children. AHLE is usually fatal, while ADEM generally has a favorable outcome3,6). MRI is important to differentiate AHLE from ADEM and other demyelinating diseases1). AHLE shows multiple white matter lesions observed as hyperintensity with hypointense hemorrhages on T2-weited image; ADEM has no hemorrhagic areas1,3,12,15,16).
Diffusion tensor imaging has been established as a useful diagnostic tool in CNS disorder19). DTI has been used to under- stand injury of axonal tracts and myelin. DTI parameters can measure changes in the microstructure and offer the potential to evaluate pathologic changes in acute demyelinating diseases13). Fractional Anisotropy (FA) or Apparent Diffusion Coefficient (ADC), parameters acquired from DTI, reflects the local anisotropy or diffusivity of the structure17,18). Balasubramanya mentioned that DTI findings represent restricted diffusion in the acute stage of ADEM and free diffusion in the subacute stage 20). Werring reported that follow up DTI studies may provide a further understanding of the disease process after stroke11). In our AHLE case, DTI was also useful to visualize the status of axonal injury, although we could not quantify it; six months
since the beginning of rehabilitation, increased white matter connectivity was found on color-coded MRI with DTI.
We suggest adding the DTI technique when a follow-up MRI is performed in patients with AHLE. It could be useful to visualize the status of the axonal injury and encourage patients and their parents to continue the rehabilitation program.
요약
급성출혈성백질뇌염 급격히 진행하는 염증성 탈수초 질환으로 진 단과 추적관찰에 있어서 뇌자기공명영상이 중요한 역할을 한다. 16개 월 여아가 열성경련을 주소로 내원하였다. 내원 후 의식은 혼미상태로 악화되어 뇌자기공명영상을 시행하였으며, T2강조영상에서 고음영 병변과 경사에코에서 미세출혈이 확인되었다. 임상증상과 뇌자기공명 영상소견으로 급성출혈성백질뇌염으로 진단하였으며 면역글로블린 과 메틸프레드니솔론을 투여하고 재활치료를 진행하였다. 발병 한달 후 이따금씩 웃는 모습 관찰되고 고개 돌리기 가능하였으나 고개 가 누지 못하고 심부건 반사 증가 지속되었다. 재활치료 유지하였으며 발 병 5개월 후 확산텐서영상을 포함하여 뇌자기공명영상을 추적관찰 하였으며, 확산텐서영상에서 발병 당시 시행한 영상보다 뇌백질 연속 성이 증가한 것을 확인하였다. 저자들은 확산텐서영상을 포함한 뇌자 기공명영상을 추적관찰하여 신경손상 호전상태를 시각화하는데 유 용하다는 것을 확인하였기에 보고하는 바이다.
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