Gerschlager, W. et al. Natural history and syndromic associations of orthostatic tremor: a review of 41 patients. Mov. Disord. 19, 788-795

Department of Neurology, Krankenhaus der Barmherzigen Brüder, Vienna, Austria
Movement Disorders (Impact Factor: 5.68). 07/2004; 19(7):788 - 795. DOI: 10.1002/mds.20132


Orthostatic tremor (OT) is a rare condition characterized by unsteadiness when standing still that is relieved when sitting or walking and is thought to arise from a central generator in the cerebellum or brainstem. OT is considered to be a distinct, discrete condition, and little is known about its demographic characteristics, natural history, associated features, and treatment response. We have reviewed these aspects in 41 OT patients fulfilling current diagnostic criteria, seen at our institution between 1986 and 2001. We classified 31 (75%) as having idiopathic “primary OT” either with (n = 24) or without an associated postural arm tremor. We found that 10 of 41 (25%) cases had additional neurological features, and we defined this group as having “OT plus” syndrome. Of these 10, 6 had parkinsonism; 4 of these had typical Parkinson's disease (PD), 1 had vascular and 1 had drug-induced parkinsonism. Among the remaining 4 patients, 2 had restless legs syndrome (RLS), 1 had tardive dyskinesia, and 1 orofacial dyskinesias of uncertain etiology. One patient with PD and the patient with vascular parkinsonism also had RLS. Age at onset was significantly earlier in the “primary OT” (mean ± SD, 50.4 ± 15.1) than in the “OT plus” (61.8 ± 6.4; z = 2.7; P = .006) group. In 7 of the 10 “OT plus” patients, OT leg symptoms preceded the onset of additional neurological features. OT appeared to be underdiagnosed, and on average, it took 5.7 years from the initial complaints until a diagnosis was made. In general, treatment response to a variety of drugs such as clonazepam, primidone, and levodopa was poor. In most cases, OT symptoms remain relatively unchanged over the years, but in 6 of 41 cases (15%), the condition gradually worsened over the years, and in some of these cases, symptoms spread proximally to involve the trunk and arms. OT may not be a discrete disorder as commonly believed and associated features like parkinsonism present in nearly 25% of cases. Dopaminergic dysfunction may have a role in the pathophysiology of this disorder. © 2004 Movement Disorder Society

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    • "It has been described in 3 brothers without a family history of ET [29] and one pair of monozygotic twins [30]. We found a higher proportion of patients had a family history of hand tremor (23/45) compared with previous studies [6] [7]. This may be because we assessed hand tremor while standing as well as sitting. "
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    ABSTRACT: Objective To evaluate the clinical characteristics, associated features, and treatment response of a large orthostatic tremor series seen over a 26-year period. Methods We reviewed the medical records of 45 patients seen between 1987 and 2013 who fulfilled the diagnostic criteria for orthostatic tremor. Results The mean age at onset was 59.5 years and 23/45 (51%) were men. A family history of any tremor was noted in 23/45 (51%) patients. A family history of orthostatic tremor was reported in 3/45 (7%) patients. 40/45 (89%) had primary orthostatic tremor with (n=30) or without (n=10) an associated postural arm tremor. We found that 5/45 (11%) had orthostatic tremor plus additional neurological features. One patient was diagnosed with dementia with Lewy bodies preceded by orthostatic tremor for 20 years. Prospective follow-up data was available for 30/45 patients and averaged 54.4 months. Treatment response to medications was modest and inconsistent. In 11/30 cases, orthostatic tremor worsened over the follow-up period. One patient with primary orthostatic tremor underwent thalamic deep brain stimulation surgery. Conclusions In our population of orthostatic tremor patients, mild postural hand tremor was a frequent finding. Over half of our patients had a family history of tremor, but a family history of orthostatic tremor was uncommon. Additional neurological features were seen in the minority of patients and we report possibly the first case of dementia with Lewy bodies associated with orthostatic tremor. Our series is the largest series of orthostatic tremor reported in the literature and contributes to understanding the clinical characteristics of this rare disease.
    Full-text · Article · Jul 2014 · Parkinsonism & Related Disorders
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    • "Większość dowodów wskazuje na umiejscowienie generatora w pniu mózgu lub móżdżku [14– 16]. Niektóre badania dowodzą, że w patogenezie OT rolę odgrywa również układ dopaminergiczny (stwierdzono zmniejszony wychwyt dopaminy w prążkowiu w badaniu SPECT), ale odpowiedź na leczenie L-dopą i agonistami dopaminy jest różna [17] [18] [19] [20] [21]. Elektromiografia jest pomocna w diagnostyce różnicowej drżenia. "
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    ABSTRACT: Objectives Orthostatic tremor is a specific form of tremor which is characterized by a subjective feeling of unsteadiness during stance, not present in other positions. The diagnosis can be confirmed in the electromyographic or accelerometric study by recording the tremor (e.g. in the quadriceps muscle) of frequency 13–18 Hz occurring exclusively in the upright position. Case report In the present case, despite the presence of the classic features of orthostatic tremor the proper diagnosis has been much delayed. Conclusions The paper presents the characteristic features of this rare movement disorder, indicates some diagnostic possibilities, and emphasizes the need for disseminating the knowledge about the disease which can be treated successfully.
    Full-text · Article · Mar 2014 · Postepy Psychiatrii i Neurologii
    • "Though OT is mostly an idiopathic condition and is generally non-familial, Wee et al.,[4] however, hinted at the familial nature of the disease and Gerschlager et al.,[8] touched upon the same in their work. The latter group observed that none in the pure OT group reported any movement disorder in the relatives. "
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    ABSTRACT: Orthostatic tremor (OT), is usually a disease of old age and is characterized by quivering movements of the legs during quiet standing or in the state of isometric contraction in the lower limbs. This is relieved on walking or on lying down. It is diagnosed by surface electromyography, particularly over the quadriceps femoris muscles which shows a distinctive frequency of 13 to 18 Hz on standing. Some investigators consider it as a variant of essential tremor (ET) and the two conditions often co-exist. The disease is usually non-familial. Two brothers presented with tremor in the lower limbs on standing and on the outstretched hands without any family history. Subsequently, they were proved to be suffering from OT and ET by clinical examination and surface EMG. Simultaneous occurrence of OT and ET in two young brothers without any family history in the previous generation has not been described before and they also appeared at a much earlier age than what is described in the literature.
    No preview · Article · Apr 2013 · Annals of Indian Academy of Neurology
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