We describe a patient with frontotemporal dementia (FTD), a tauopathy, who also showed clinical and polysomnographic features of REM sleep behavior disorder (RBD). The patient is a 78-year-old male with a 1 year history of behavioral dysfunction involving emotion, character and social functioning. Brain imaging and the results of neuropsychological testing were consistent with a diagnosis of FTD. Sleep symptom onset occurred some years before the behavioral changes, and consisted of unpleasant dreams, vocalization, and prominent motor behaviors. A polysomnography confirmed the diagnosis of RBD. Our findings support the hypothesis that RBD, although more frequent in synucleinopathies, might be a pathological stage in the development of almost every neurodegenerative disorder in which the pathological process involves the cerebral structures that regulate muscle tone during REM sleep.
Dysembryoplastic neuroepithelial tumor (DNT) is a clinically benign stable lesion, most frequently located in the temporal and frontal lobes, often responsible for epilepsy in young adults. We describe an unusual case of DNT in the brainstem of a 45-year-old woman. Brain MRI showed a multicystic-like lesion localized in the left inferior pons, involving the ipsilateral cerebellar peduncle and partially dislocating the fourth ventricle. The specific pattern of MRI and CT appearance of DNT and its benign course (our patient is clinically stable with unchanged MRI images at two year follow-up) may help differentiate this tumor from other lesions, i.e. gangliogliomas and glioneural malformations.
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