Hiroyuki Yamamoto1, Jun Natsume2, Hiroyuki Kidokoro2, Naoko Ishihara2, Motomasa Suzuki3, Takeshi Tsuji4, Tetsuo Kubota5, Akio Yamada6, Michio Ozeki7, Zenichiro Kato7, Yoshiki Kawamura8, Tetsushi Yoshikawa8, Akihisa Okumura9, Naoki Ando10, Shinji Saitoh10, Yoshiyuki Takahashi2, Kazuyoshi Watanabe2, Seiji Kojima2. 1. Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan. Electronic address: h-yamamoto@med.nagoya-u.ac.jp. 2. Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan. 3. Department of Neurology, Aichi Children's Health and Medical Center, Obu, Japan. 4. Department of Pediatrics, Okazaki City Hospital, Okazaki, Japan. 5. Department of Pediatrics, Anjo Kosei Hospital, Anjo, Japan. 6. Department of Pediatrics, Social Insurance Chukyo Hospital, Nagoya, Japan. 7. Department of Pediatrics, Graduate School of Medicine, Gifu University, Gifu, Japan. 8. Department of Pediatrics, Fujita Health University School of Medicine, Toyoake, Japan. 9. Department of Pediatrics, Aichi Medical University, Nagakute, Japan; Department of Pediatrics, Juntendo University School of Medicine, Tokyo, Japan. 10. Department of Pediatrics and Neonatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.
Abstract
OBJECTIVE: To clarify the clinical and radiological spectrum of posterior reversible encephalopathy syndrome (PRES) in children, and to identify the prognostic factors. METHODS: The records of 40 children with PRES were reviewed. Acute clinical symptoms, MRI including apparent diffusion coefficient (ADC) maps in the acute and follow-up periods and neurological sequelae, including epilepsy, were noted. RESULTS: Age at onset ranged from 2 to 16 years. Underlying disorders were hematological or neoplastic disorders (n = 20), renal diseases (n = 14) and others (n = 6). In the acute period, 31 patients had seizures, 25 had altered consciousness, 11 had visual disturbances and 10 had headache. Of 29 patients who had ADC maps in the acute period, 13 had reduced diffusivity as shown by ADC within PRES lesions. Of 26 patients with follow-up MRI, 13 had focal gliosis or cortical atrophy. No patients had motor impairment, and four patients had focal epilepsy. No clinical variables were associated with focal gliosis or cortical atrophy on follow-up MRI, but lesional ADC reduction in the acute period was prognostic for focal gliosis or cortical atrophy on follow-up MRI (p = 0.005). CONCLUSIONS: To the best of our knowledge, this is the largest cohort study to date involving PRES in children. Acute symptoms in pediatric patients are similar to those reported in adults, but altered consciousness was more frequent in children. Lesional ADC reduction in the acute period was common and was a good predictor of later, irreversible MRI lesions.
OBJECTIVE: To clarify the clinical and radiological spectrum of posterior reversible encephalopathy syndrome (PRES) in children, and to identify the prognostic factors. METHODS: The records of 40 children with PRES were reviewed. Acute clinical symptoms, MRI including apparent diffusion coefficient (ADC) maps in the acute and follow-up periods and neurological sequelae, including epilepsy, were noted. RESULTS: Age at onset ranged from 2 to 16 years. Underlying disorders were hematological or neoplastic disorders (n = 20), renal diseases (n = 14) and others (n = 6). In the acute period, 31 patients had seizures, 25 had altered consciousness, 11 had visual disturbances and 10 had headache. Of 29 patients who had ADC maps in the acute period, 13 had reduced diffusivity as shown by ADC within PRES lesions. Of 26 patients with follow-up MRI, 13 had focal gliosis or cortical atrophy. No patients had motor impairment, and four patients had focal epilepsy. No clinical variables were associated with focal gliosis or cortical atrophy on follow-up MRI, but lesional ADC reduction in the acute period was prognostic for focal gliosis or cortical atrophy on follow-up MRI (p = 0.005). CONCLUSIONS: To the best of our knowledge, this is the largest cohort study to date involving PRES in children. Acute symptoms in pediatric patients are similar to those reported in adults, but altered consciousness was more frequent in children. Lesional ADC reduction in the acute period was common and was a good predictor of later, irreversible MRI lesions.