Clinical spectrum of reversible posterior leukoencephalopathy syndrome

• Published in: Pediatric neurology 2001-05, Vol.24 (5), p.361-364
• Main Authors: Kwon, Soonhak, Koo, Jahoon, Lee, Sangkwon
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Biological and medical sciences; Blood-Brain Barrier - physiology; Brain Edema - diagnosis; Brain Edema - etiology; Child; Child, Preschool; Cyclosporine - adverse effects; Diagnosis, Differential; Disorders of higher nervous function. Focal brain diseases. Central vestibular syndrome and deafness. Brain stem syndromes; Female; Follow-Up Studies; Hemosiderin - metabolism; Humans; Lactic Acid - metabolism; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Male; Medical sciences; Nervous system (semeiology, syndromes); Neurologic Examination; Neurology; Occipital Lobe - pathology; Parietal Lobe - pathology; Remission, Spontaneous; Risk Factors; Syndrome; Tropical medicine
• ISSN: 0887-8994; 1873-5150
• DOI: 10.1016/S0887-8994(01)00265-X

Reversible posterior leukoencephalopathy syndrome is a recently recognized disorder with characteristic radiologic findings that mainly involve the white/gray matter of the parieto-occipital lobes. This complex syndrome is associated with cyclosporine A therapy or a variety of other conditions in which blood pressure rises acutely. Twelve patients from a variety of conditions who met the diagnostic criteria for this syndrome were studied. Interestingly, three of these patients had intra-abdominal neurogenic tumors, which have rarely been reported. Initial cranial magnetic resonance imaging scans revealed fairly symmetric areas of increased T 2 signal involving both white and gray matter of parieto-occipital lobes in the majority of the patients. However, the lesions were often located outside the parieto-occipital regions. Four patients had occipital region magnetic resonance spectroscopy during the acute phase, which revealed high lactate peak and normal N-acetyl aspartate/creatine and choline peaks. With appropriate treatment, most patients recovered from this syndrome and experienced almost complete resolution of brain lesions on follow-up magnetic resonance imaging. One patient, however, continued to have small residual hemosiderin deposits on a follow-up magnetic resonance imaging with neurologic sequellae. In conclusion, a better understanding of this complex syndrome may obviate unnecessary investigations and allow management of the associated problems in prompt and appropriate ways.