Orthostatic tremor (OT) is a rare, disabling movement disorder characterized by the development of a high-frequency tremor of the lower limbs and feelings of unsteadiness upon standing, which compel the patient to sit down or walk. Medical therapy is often unsatisfactory. Previous reports suggest that deep brain stimulation of the ventral intermediate nucleus of the thalamus may improve clinical outcomes. The authors report 3 patients who had intractable orthostatic tremor treated with bilateral deep brain stimulation of the ventral intermediate nucleus of the thalamus-caudal zona incerta, resulting in improved and sustained clinical improvements in symptoms, although there were no apparent changes in the underlying tremor frequency or onset.First reported in 1970 by Pazzaglia and colleagues, the term orthostatic tremor (OT) was coined by Heilman in 1984 to describe a tremor of the legs or trunk induced by standing, causing feelings of unsteadiness or shakiness, compelling patients to sit or walk. 1,2 Although falls are uncommon, symptoms invariably progress and can cause considerable distress to quality of life. Development of a concurrent upper-limb tremor is not uncommon, and 11% to 15% of patients have additional neurological features, such as parkinsonism or ataxia, termed "OTplus." 3,4 Electromyography (EMG) reveals a characteristic, high-frequency tremor of 13 to 18 Hz in the lower limbs that demonstrates high intermuscular coherence, which can be used to differentiate it from other types of tremor. Clinical examination may reveal a barely detectable ripple of leg muscles, although auscultation with a stethoscope over the thigh, quadriceps, and hamstrings can reveal a repetitive thumping soundthe so-called helicopter sign. 5 Although anecdotal evidence suggests that treatment with clonazepam, gabapentin, and other antitremor drugs may offer some benefit to patients, the results are often unsatisfactory. 3,6 The pathophysiology of OT is unclear; OT is generally thought to originate from a central oscillator, but involvement of the spinal cord has also been postulated, and surgical treatment for refractory OT has been reported in a small number of patients. Deep brain stimulation (DBS) of the ventral intermediate (ViM) nucleus of the thalamus has been reported in 8 patients, and spinal cord dorsal column stimulation also has been reported in 3 patients (Table 1).In this report, we describe the clinical and electrophysiological effects of high-frequency DBS of the ViM nucleus of the thalamus-caudal zona incerta in 3 patients suffering from refractory OT.
CasesPatient 1 (a 46-year-old woman) complained of a 20-year history of unsteadiness during standing that was relieved on sitting, leaning, or walking. The latency between standing and the onset of unsteadiness remained at 30 seconds since onset, although, in the year preceding assessment, she also developed an alcohol-responsive kinetic tremor of the right hand and sub-1