Brain, Vol. 117, No. 4, 775-788, 1994
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research-article |
Symptomatic and essential palatal tremor
1. Clinical, physiological and MRI analysis
1Human Motor Control Section, Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health Bethesda 2Department of Neurology, Johns Hopkins Hospital Baltimore, Maryland, USA
Correspondence to:
0Correspondence to: Mark Hallett, Building 10, Room 5N266, NINDS, NIH, Bethesda, MD 20892, USA
Palatal tremor (brief, rhythmic involuntary movements of the soft palate) apparently comprises two different nosological entities: essential palatal tremor (EPT) and symptomatic palatal tremor (SPT). The site of the abnormality in EPT is unknown, whereas SPT is believed to arise from a lesion of the brainstem or cerebellum (within the Guillain-Mollaret triangle). The clinical and physiological properties of these conditions were studied in four patients with EPT and six patients with SPT. Patients with EPT had normal cerebellar function, but those with SPT had clinical signs of cerebellar dysfunction. The palatal movements were consistent with activation of the tensor veli palatini muscle in EPT and of the levator veli palatini muscle in SPT. During sleep, EPT stopped, whereas SPT continued with only slight variations in the tremor rate. The cycle of palatal tremor could not be reset by stimulation of trigeminal afferents in either EPT or SPT patients, and Valsalva's manoeuvre did not consistently affect the rhythm of the tremor in either group. The palatal tremor cycle exerted remote effects on the tonic electro-myographic activity of the upper and lower extremities only in patients with SPT. These effects were present only on the side of the cerebellar signs (opposite the side with the enlarged inferior olive) in patients with a unilateral syndrome. Essential palatal tremor patients had only polysynaptic brain-stem reflex abnormalities, whereas SPT patients had abnormalities of monosynaptic, oligosynaptic and polysynaptic brainstem reflexes. Magnetic resonance imaging showed no evidence of structural abnormalities in EPT patients, but SPT patients had a hyperdense signal of the ventral upper medulla (the region of the inferior olive) on T2-weighted images. These observations support the hypothesis that EPT and SPT are two different diseases. In SPT, cerebellar dysfunction ipsilateral to the palatal tremor may be due, in part, to abnormal function of the contralateral hypertrophic inferior olive. The proposed basis of SPT is a disturbance of electrotonic coupling between the cells of the inferior olive induced by a lesion of the dentato-olivary pathway. Similar mechanisms could be responsible for postural tremors in general. The pathophysiological basis of EPT remains unknown.
palatal tremor; palatal myoclonus; tremor; MRI; inferior olive
Received November 11, 1993. Revised March 9, 1994. Accepted March 11, 1994.
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