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New form of familial Parkinson‐dementia syndrome and progressive supranuclear palsy

To the Editor: In reporting the association of blepharospasm with brainstem lesions, Jankovic and Patel’ suggested several possible explanations including “denervation supersensitivity” of the facial nuclear complex similar to the presumed disorder of inferior olive in palatal myoclonus.’ Further evidence for a comparable mechanism in the two syndromes was provided by a patient who had both syndromes. This 60-year-old man noted sudden onset of nausea, vomiting, vertigo, and right-sided and midline ataxia in 1970. He recovered, aside from mild dysarthria and right limb incoordination. In 1976, he developed a left-sided resting and action tremor, with bilateral rigidity and bradykinesia worse on the left. He improved on Sinemet therapy, but within a few months, he had severe blepharospasm that persisted after Sinemet was discontinued. Drug-free, he was functionally blind most of the time, and the blepharospasm increased every time levodopa was reintroduced. In 1978, he had a 2-day episode of dysphasia. In 1979, a right thalamotomy improved the tremor, which returned 2 years later. Between 1979 and 1982, several medical and surgical attempts to improve the blepharospasm were unsuccessful. In 1982, palatal myoclonus was first documented. In 1983, he had frequent episodes of blepharospasm, palatal myoclonus, and a side-to-side tremor of the head, as well as resting and postural tremors of the hands, legs, and trunk (more on the left), moderate bilateral bradykinesia and rigidity, mild ataxia of the right arm, nasal dysarthric speech, and occasional inappropriate laughing. Palatal myoclonus was asynchronous with the blepharospasm as well as the head and limb tremors. CT (figure, A and B) showed severe focal atrophy involvingthe superior aspect of the right and, to a lesser extent, the left cerebellar hemisphere, with slight prominence of the lateral and third ventricles (figure, B) as well as cortical sulci. This patient was similar to patient three of Jankovic and Patel, with complicated parkinsonism after posterior circulation infarcts. The complex course, with disease in more than one vascular territory, made it impossible to define the localization of the lesion, but accounted for our patient’s blepharospasm. However, the lesions demonstrated by CT were similar to those documented by Jankovic and Patel. The argument that denervation supersensitivity underlies palatal myoclonus is based primarily on the delay between the acute brain insult and appearance of the movement disorder.2 However, the other mechanisms (disinhibition and sprouting of surviving neurons) that Jankovic and Patel propose for blepharospasm in brainstem lesions are also possible explanations for palatal myoclonus. Association of both disorders in our patient furthers the argument that a similar mechanism, probably in different brainstem regions, may cause these two conditions.