Our new finding on magnetic resonance imaging (MRI) of Machado-Joseph disease (MJD) patients indicates degeneration of the lenticular fasciculus (LF), a major outflow of the internal segment of the globus pallidus (GPi). We examined the clinical, radiological, and autopsy findings of one MJD patient and then retrospectively reviewed the MRI images of another 15 patients looking for a similar abnormal signal intensity. The significance of the clinicoradiological correlation of the MRI finding was confirmed by examining the MRI images of 130 control subjects. In the autopsy case, abnormal linear high intensity areas were observed along the bilateral medial margins of the internal segments of the GPi on T2 weighted, FLAIR, and proton density images, but not on T1 weighted images. Pathologically, this abnormal signal intensity was consistent with degeneration of the LF. The same finding was also observed in the other 15 patients. In two patients the finding was only unilaterally observed. No control subject showed this MRI finding. In MJD patients, abnormal linear high intensity areas indicating LF degeneration are usually observed along the medial margin of the GPi on T2 weighted, FLAIR, and proton density sequences. To our knowledge, this MRI finding has not previously been described.T he lenticular fasciculus (LF), which is a major outflow of the internal segment of the globus pallidus (GPi), is severely degenerated [1][2][3] in Machado-Joseph disease (MJD), which is one of the most prevalent familial cerebellar degenerative diseases in Japan. 4 We found in MJD patients that abnormal linear high intensity is observed on T2 weighted magnetic resonance imaging (MRI) along the medial margin of the internal segment of the GPi. This study was carried out to confirm that this MRI finding indicates LF degeneration. An autopsied case of MJD was clinicopathologically examined and then MRI images of other MJD cases were reviewed. The clinicoradiological relationship was examined and the significance of the MRI finding confirmed by examining MRI images of control subjects. METHODSPatients and control subjects There were 16 MJD patients (table 1). The diagnosis of MJD was made based on clinical presentation, positive family history for autosomal dominant cerebellar degeneration, and DNA analysis. The control subjects consisted of 130 consecutive patients (aged between 12 and 77 years old; mean age 51.7 years), who presented at our hospital during 2003 because of headache or dizziness, but showed no neurological abnormality other than exaggerated deep tendon reflexes. Molecular analysis of the MJD1 geneMolecular analysis of the MJD1 gene was performed as described by Kawaguchi and colleagues. 5MRI examination MRI examinations were performed using two different systems. A 1.0 T system (SMT-100X, Shimadzu, Kyoto, Japan) was used until April 2000, and a 1.5 T system (Magnetom Symphony, Siemens, Munich, Germany) thereafter. Standard brain examinations were carried out using T1 and T2 sequences with contiguous 10 mm thick slices. ...

CaseThoracic endovascular aortic repair (TEVAR) is becoming the standard therapy for blunt thoracic aortic injury (BTAI). However, the long‐term outcomes of TEVAR for BTAI remain unclear. A 36‐year‐old man was admitted to our emergency department with dyspnea. He had been involved in a serious traffic accident 6 years earlier, requiring TEVAR for BTAI.OutcomeAcute heart failure and pneumonia were diagnosed on this admission. His respiratory condition improved, but paraplegia developed 10 h after hospitalization. Magnetic resonance imaging showed an intraspinal longitudinal area of signal hyperintensity, and spinal cord infarction was diagnosed.ConclusionAlthough the causal relationship between the TEVAR and spinal cord infarction remains unclear, paraplegia as a long‐term complication after TEVAR does not appear to have been reported previously, and so represents a potentially important complication.