Background and Purpose: We have encountered a peculiar atrophic change in the midbrain in some patients with parkinsonian syndromes. We discovered these patients had vertical supranuclear gaze-palsy, an eye movement disorder. The purpose of this study was to elucidate whether this atrophic pattern of the midbrain (which we have termed morning glory sign) is related to the vertical eye movement disorder, in particular to progressive supranuclear palsy (PSP).Methods: We reviewed T 2 -weighted axial images obtained from 42 patients with parkinsonian syndromes, includingˆve patients with PSP, 23 patients with Parkinson's disease, and 14 patients with multiple system atrophy (MSA). We focused on a speciˆc atrophy of the midbrain, the morning glory sign, which is a concavity of the lateral margin of the tegmentum of the midbrain.Results: The morning glory sign was detected in four of theˆve patients with PSP and in one (striatonigral degeneration; SND) of the14 patients with MSA. All morning glory sign patients had vertical supranuclear gaze-palsy, as did the one PSP patient without the morning glory sign. Vertical supranuclear gaze-palsy was seen in no other patients (23 patients with Parkinson's disease and 13 patients with MSA) who lacked the morning glory sign.Conclusions: Morphologically, the morning glory sign is believed to be related to vertical supranuclear gaze-palsy. This sign should be considered a useful clue when diagnosing PSP.

In a review of magnetic resonance (MR) images from patients with spinocerebellar degeneration (SCD), we found 2 rare patients considered to be in late-stage cerebellitis who showed isolated cerebellar atrophy. The patients were negative for the spinocerebellar ataxia (SCA) genes and had no symptoms of hypothyroidism, history of malignant tumors, or history of alcohol and drug (phenytoin) abuse, which may cause cerebellar atrophy. MR images demonstrated generalized atrophy of the cerebellum, excluding the brainstem or cerebrum. In these cases, moreover, slightly high intensities were noted in the affected cerebellar cortices on fluid-attenuated inversion recovery (FLAIR) images. The distribution of widening of the folia and cortical high intensities on FLAIR images might be important clues with which to diagnose late-stage cerebellitis.

The aim of the present study was to demonstrate characteristic signal changes of the pontine base on T2-weighted images of patients with SCA 1, and to elucidate the relationship between abnormal high-intensities of the pontine base on T2-weighted images and the findings on multishot diffusion-weighted images. We assessed abnormal signals of the pontine base on T2-weighted images from 50 controls and six patients with SCA 1 diagnosed by genetic analysis. At the same time, we evaluated the degeneration of the transverse pontine fibers in the pontine base by multishot diffusion-weighted imaging. A midline high-intensity was seen in the pontine base on T2-weighted images in two of the 50 controls and five of the six patients with SCA 1. The midline high-intensity had a sensitivity of 83.3% for patients and a specificity of 96.0% for controls. Multishot diffusion-weighted imaging demonstrated the degeneration--the amorphous-pattern signal--of the transverse pontine fibers in four (66.7%) of the six patients. In the other two patients, the zebra-pattern signal was seen in the pontine base. The midline high-intensity on T2-weighted images appears to be one of characteristic MRI findings of SCA 1. Multishot diffusion-weighted imaging suggested that the midline high-intensity should reflect the degeneration of the transverse pontine fibers.