L. J. Findley

Assessing tremor severity.

A clinical rating scale which measured the severity of tremor in 20 patients (12 with essential tremor and 8 with dystonic tremor) was assessed at specific anatomical sites for both inter and intra-rater reliability using four raters. The scores obtained with the scale were compared with the results of upper limb accelerometry, an activity of daily living self-questionnaire and estimates of the tremor induced impairment in writing and drawing specimens. The results show that, for the purposes of routine assessment and therapeutic trials, a clinical rating scale can produce reliable results which are a more valid index of tremor induced disability than standard postural accelerometry.

Primary orthostatic tremor: further observations in six cases

SummaryThe clinical and physiological features of six new patients with primary orthostatic tremor are described. We suggest that use of the term primary orthostatic tremor be confined to the clinical syndrome in which unsteadiness when standing is the predominant complaint and accompanied by characteristic electrophysiological findings of a rapid (frequency around 16 Hz), regular leg tremor which is not influenced by peripheral feedback, is synchronous between homologous leg muscles, and in certain postures of the upper limbs, between muscles of the arm and leg. The fast frequency of muscle activity in primary orthostatic tremor of the legs causes unsteadiness when standing (presumably due to partially fused muscle contraction) but only a fine ripple of muscle activity is visible. In contrast, the slower frequency of other leg tremors, for example essential tremor, results in obvious leg movement which is evident in many leg postures, is variable over time and can be reset by a peripheral nerve stimulus. Essential tremor and orthostatic tremor do not respond to the same therapies, suggesting differences in the pharmacological profiles of the two conditions. Accordingly, there are clinical, physiological and pharmacological differences between primary orthostatic and essential tremor. Whether these factors are sufficient to regard these tremors as separate conditions is discussed.

Frequency/amplitude characteristics of postural tremor of the hands in a population of patients with bilateral essential tremor: implications for the classification and mechanism of essential tremor.

Amplitude/frequency characteristics of postural hand tremor in 59 patients with bilateral essential tremor of various degrees of severity were assessed using accelerometric recordings and spectral analysis. Intra-subject comparisons of tremor characteristics between the more and less affected hands were used to control for variability of tremor due to age factors and intersubject differences in amplitude and frequency. Statistical analysis distinguished three different patient groups. Some patients had low amplitude (less than 0.1-0.015 cm) tremor in the less affected limb (which tended to be 7 Hz or more in frequency in the young) and a larger amplitude tremor in the more affected hand which was 1 Hz or more lower in frequency. Other subjects had either bilaterally small or bilaterally large amplitude tremors of similar frequencies. These findings imply that there is a downwards step in frequency between symptomatic tremors of small and large amplitude. The amplitude and frequency of the small amplitude tremors were unrelated but frequency declined with age. The frequency of the large amplitude tremor was generally determined by amplitude but a wide range of amplitudes were compatible with similar frequencies. The frequency of large amplitude tremor also declined with age. It was concluded that there are two types of essential tremor, the smaller amplitude tremor probably derives from an exaggeration of some or all of the mechanisms of normal physiological tremor whereas the larger amplitude tremor probably arises from a separate pathological central nervous mechanism. It is not known if or how one may transform into, or be replaced by, the other during progression of the disease.

Metoprolol and propranolol in essential tremor: a double-blind, controlled study.

Single oral doses of propranolol (120 mg), metoprolol (150 mg) and placebo were given in a randomised, double-blind fashion to 23 patients with essential tremor. Both beta blockers were significantly more effective than placebo in reducing the magnitude of tremor. The decrease in tremor produced by metoprolol (47, sem 9%, n = 23) was not significantly different from that observed propranolol (55, sem 5%, n = 23). Tachycardia on standing was antagonised by both drugs to a similar extent. These findings suggest that metoprolol may represent a valuable alternative to propranolol in the treatment of essential tremor. The data is consistent with the hypothesis that the tremorolytic effect of beta blockers in these patients may be unrelated to peripheral beta-2 adreno-receptor blockade, being possibly mediated by other central or peripheral modes of action of these drugs. However, it cannot be excluded that at the dose used, metoprolol had lost its relative cardio-selectivity and that the reduction in tremor was mediated by competitive antagonism at beta-2 receptor sites in skeletal muscle.

Modulation of postural tremors at the wrist by supramaximal electrical median nerve shocks in essential tremor, Parkinson's disease and normal subjects mimicking tremor.

The response of postural wrist tremors to supramaximal median nerve stimulation was examined in patients with hereditary essential tremor (n = 10) and Parkinsons disease (n = 9), and in normal subjects mimicking wrist tremor (n = 8). The average frequency of on-going tremor was the same in all three groups. Supramaximal peripheral nerve shocks inhibited and then synchronised the rhythmic electromyographic (EMG) activity of all types of tremor. The duration of inhibition ranged from 90 to 210ms, varying inversely with the frequency of on-going tremor. There was no significant difference in mean duration of inhibition or in the timing of the first peak after stimulation on the average rectified EMG records between the three groups. The degree to which supramaximal peripheral nerve shocks could modulate the timing of rhythmic EMG bursts in the forearm flexor muscles was also quantified by deriving a resetting index. No significant difference in mean resetting index of the three groups was found. These results suggest that such studies cannot be used to differentiate between the common causes of postural wrist tremors.

Electrophysiological aids in distinguishing organic from psychogenic tremor

The clinical differentiation of tremors of organic and psychogenic origin can be difricult. We describe a patient with unilateral upper limb tremor that was initially considered to have a psychogenic cause, but subsequent frequency analysis of EMG signals and accelerometer recordings indicated that the tremor was organic in nature. An ischemic lesion in the contralateral lentiform nucleus found on MRI supported this conclusion. Quantitative electrophysiologic studies may thus be useful in distinguish organic from psychogenic tremor.

Phenylethylmalonamide in essential tremor. A double-blind controlled study.

A randomised double-blind placebo-controlled trial of phenylethylmalonamide, the major metabolite of primidone was performed in eight patients with essential tremor. Phenylethylmalonamide was given in a daily dose of 400 mg for one week and 800 mg for a second week. The compound had no statistically significant effect on the amplitude of tremor assessed by an accelerometric method, tests of performance, clinical evaluation and patient self assessment. No side effects occurred. Serum levels of phenylethylmalonamide on a daily dose of 400 mg were 11-27 micrograms/ml and on 800 mg daily were 16-48.5 micrograms/ml.

Electrophysiological observations on an unusual, task specific jaw tremor

A patient with no other neurological signs or symptoms presented with a prominent tremor restricted to the mandible. This 5-6 Hz tremor was interesting in that it was normally confined to the digastric muscles and was highly task specific. In the course of her normal daily activities, it began only when the patient drank from a cup or glass. The localisation of this tremor to a muscle that has no muscle spindles and no reciprocal inhibitory reflexes suggests that such tremors must be capable of being generated centrally.