A brainstem variant of reversible posterior leukoencephalopathy syndrome.
Reversible posterior leukoencephalopathy syndrome (RPLS) is caused by various heterogeneous factors, the commonest being hypertension, followed by nonhypertensive causes such as eclampsia, renal diseases and immunosuppressive therapy. Patients with RPLS exhibit bilateral white and gray matter abnormalities in the posterior aspects of the cerebral hemispheres. However, this syndrome may affect the brainstem predominantly, and these cases are designated as hypertensive brainstem encephalopathy. We present here two patients with reversible brainstem encephalopathy: one with hypertension and the other without hypertension. These patients presented with swelling and diffuse hyperintensities of the brainstem in fluid-attenuated inversion-recovery (FLAIR) and T2-weighted MRI, but with relatively mild clinical symptoms. They recovered without major neurological deficits, but had residual lacunar lesions in the pons. Reversible brainstem encephalopathy with characteristic MRI features was found in both hypertensive and nonhypertensive patients. These patients were diagnosed with a brainstem variant of RPLS, which is potentially fully reversible after an adequate treatment, and therefore should be carefully differentiated from other brainstem disease conditions.
Kitaguchi
H
H
Department of Neurology, Graduate School of Medicine, Kyoto University, Sakyo-ku, Kyoto 606-8507, Japan.
Tomimoto
H
H
Miki
Y
Y
Yamamoto
A
A
Terada
K
K
Satoi
H
H
Kanda
M
M
Fukuyama
H
H
D000368:Aged; D001933:Brain Stem / Q000473:pathology*; D006801:Humans; D020343:Hypertensive Encephalopathy / Q000145:classification / Q000473:pathology*; D007022:Hypotension / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D013577:Syndrome