Four patients with a cerebrovascular disease characterized by palato-ocular myoclonus (POM) were compared with two patients with dentatorubropalli-doluysian atrophy (DRPLA) exhibiting dysfunction of the dentate nucleus of the cerebellum and the cerebellofugal pathway, a part of Guillain-Mollaret's Triangle. A clinical comparison revealed significant imformation.
1) PMO was noted in patients with lesions probably involving the tractus tegmentaris centralis and nucleus olivaris inferior.
2) In these four patients, the frequency of POM was 2.0 to 4.0Hz.
3) Intention or action myoclonus was pronounced in patients with lesions of the cerebellofugal pathway (tractus dentatorubralis), but typical POM was not noted.
4) In lateralized disease, palatal myoclonus (PM) was directed to the most affected hemisphere. In patients with a symmetric disturbance, a vertically directed upward traction was noted.
5) In those patients with a right-left difference in PM, horizontal nystagmus mixed with ROM was directed to the side ipsilateral to the pronounced traction of the palate.
6) In one patient with vertical pendular oscillation due to pontine disturbance (accompanied by a bilateral PPRF disturbance), PM was noted only if there was concomitant presence of disease of the tractus tegmentaris centralis.
7) When other myoclonus was also noted with pendular oscillation, the term ROM is recommended. When myoclonus dose not accompany this sign, the term of acquired pendular nystagmus (APN) or acquired pendular oscillation (APO) is recommended.
8) A vague statement has been made regarding the appearance of POM in the presence of a disturbance of Guillain-Mollaret's Triangle. However, although rhythmic myoclonus limited to the palate may appear with some lesions, myoclonus may be limited to the eyeballs (APN) in association with other lesions. In addition, both types of myoclonus may coexist.
9) The oral administration of Clonazepam was effective in the treatment of APN.
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