2020
DOI: 10.1007/s10072-020-04326-4
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Type B diencephalic–mesencephalic junction dysplasia, a congenital brainstem malformation that may be silent until adulthood: a case report

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Cited by 5 publications
(9 citation statements)
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“…Axial MRI of the midbrain shows a midline cleft with the classic appearance of the “butterfly” sign caused by extension of the third ventricle and other diencephalic structures into the midbrain 3 . To the best of our knowledge, only 2 cases of type‐B DMJD in adulthood have been reported in the literature 1,5 . These patients presented with slowly progressive symptoms including: cognitive dysfunction, movement disorders, hemiparesis, hearing loss, speech dysfunction, and gait dysfunction.…”
Section: Figmentioning
confidence: 99%
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“…Axial MRI of the midbrain shows a midline cleft with the classic appearance of the “butterfly” sign caused by extension of the third ventricle and other diencephalic structures into the midbrain 3 . To the best of our knowledge, only 2 cases of type‐B DMJD in adulthood have been reported in the literature 1,5 . These patients presented with slowly progressive symptoms including: cognitive dysfunction, movement disorders, hemiparesis, hearing loss, speech dysfunction, and gait dysfunction.…”
Section: Figmentioning
confidence: 99%
“…The patient’s obstructive hydrocephalus was promptly relieved following a third ventriculostomy as evidenced by his MRI changes resulting in significant reduction of headaches suggesting that this was a primary factor in their development. In the other two type‐B patients, their symptoms progressed slowly over time and were not diagnosed until past the age of 40 years 1,5 . It is possible that if this had not developed headaches that this malformation would have gone unnoticed.…”
Section: Figmentioning
confidence: 99%
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