Abstract
To study prevalence, specific patterns and response to treatment of tremor in dementia with Lewy bodies (DLB), in comparison with other tremulous disorders prevalence, qualitative and quantitative features of tremor were studied in an incident cohort of 67 dopaminergic treatment naive DLB, 111 Parkinson’s Disease (PD) and 34 Essential Tremor (ET) patients. Tremulous DLB patients (tDLB) were compared with tremulous PD (tPD) and ET patients and followed for 2 years. Double blind placebo-controlled acute drug challenge with l-Dopa and alcohol was performed in all ET, 24 tDLB and 27 tPD. Effects of dopaminergic chronic treatment in all tDLB and tPD patients and primidone in 8 tDLB were also assessed. Tremor occurred in 44.76 % of DLB patients. The tDLB patients presented a complex pattern of mixed tremors, characterized by rest and postural/action tremor, including walking tremor and standing overflow in 50 % tDLB. Standing tremor with overflow was characteristic of tDLB (p < 0.001). Head tremor was more frequent in tDLB than tPD and ET (p = 0.001). The tDLB tremors were reduced by acute and chronic dopaminergic treatments (p < 0.01) but not by alcohol or primidone. Tremor occurs commonly in DLB patients with a complex mixed tremor pattern which shows a significant response to acute and chronic dopaminergic treatments. Recognizing that there is a clinical category of tremulous DLB may help the differential diagnosis of tremors.Electronic supplementary materialThe online version of this article (doi:10.1007/s00415-013-6853-y) contains supplementary material, which is available to authorized users.
Highlights
The last consensus on diagnostic criteria for dementia with Lewy Bodies (DLB), states that ‘‘tremor is less frequent than in Parkinson’s disease (PD)’’ [1] but does not detail the types and relative prevalence of tremor, despite earlier studies reporting a prevalence of 40 % for rest and 30 % for action tremor [2, 3].Generally rest tremor is considered specific for Parkinson’s Disease (PD), while postural and action tremor are attributed to essential tremor (ET), exceptions to this rule are clearly reported [4,5,6]
Within two years from the Essential Tremor (ET) diagnosis, six patients were re-classified as dementia with Lewy bodies (DLB) due to the appearance of cognitive decline with all core and supportive consensus elements [1], including REM sleep behaviour disorder (RBD), hallucinations, EEG abnormalities, and cognitive fluctuations
In these patients DLB categorization, instead of PD with Dementia (PDD), was considered acceptable despite the fact that tremor had appeared before or was concomitant with cognitive decline, as the tremor observed at first referral was purely intentional-kinetic and all six had been addressed to referral as ET patients and two of them were temporarily treated with primidone until our re-evaluation
Summary
The last consensus on diagnostic criteria for dementia with Lewy Bodies (DLB), states that ‘‘tremor is less frequent than in Parkinson’s disease (PD)’’ [1] but does not detail the types and relative prevalence of tremor, despite earlier studies reporting a prevalence of 40 % for rest and 30 % for action (kinetic-postural) tremor [2, 3].Generally rest tremor is considered specific for PD, while postural and action (or intentional) tremor are attributed to essential tremor (ET), exceptions to this rule are clearly reported [4,5,6]. Several reports showed that some ET patients, during follow-up may present with additional PD features, dopamine transporter capitation abnormalities, or cognitive abnormalities, and may present with Lewy bodies at the autopsy [16,17,18,19,20,21]. These observations were, more or less dismissively, challenged in three different editorials [22,23,24]. These discussions did not consider at all that the action (kinetic, postural) tremors, of DLB may be erroneously attributed to ET
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