Essential tremor is characterized by its low frequency, mainly affecting upper extremities and less frequently lower limbs and head. The origin of this disease is not well known, but it has been described in familiar forms as an autosomic dominant hereditary transmission pattern. We report three patients with essential tremor refractory to pharmacotherapy who underwent stereotactic thalamotomy of the ventrolateral nucleus. In all cases the procedure was unilateral. In two patients, the tremor completely remitted and in one patient the tremor decreased more than 50%. Tremor was defined by the Unified Parkinson’s scale. There were no complications related to surgery. We conclude that stereotactic ventrolateral nucleus thalamotomy can be of benefit in patients with essential tremor due to its low morbidity in selected patients.
Essential tremor
Caracterización Clínico-Epidemiológica del Temblor Esencial en Familias de Holguín y Matanzas, Cuba.
Essential tremor (ET) is a bilateral mostly simmetric disorder, usually postural but kinetic and rest tremor can be present, it involves the arms and hands. A tranversal study was made to get an epidemiological, functional and fenomenological characterization of ET. Patients with clinical criteria of ET were selected from movement disorders clinic from two cuban provinces; the Fahn, Tolosa and Marín functional disability scale was applied to the sample. A total of 105 patients were evaluated, 64.7% female, 40.0% over 60 years, 80.9% were white, in 36.1% tremor began between 21 and 40 years, superior limbs were affected in 90.4%, 85.9% showed a beginning age anticipation, 87.7% were from Holguín, just 8 sporadic cases; in 53.8% associated diseases were found, an autosomal dominant inheritance was evident. Functions included in the scale’s part B showed a moderate disability (25-49%) and those which belong to part C had low disability (1-24%).
Conclusions. Most of the sample was from Holguín. Female, whites and older than 60 years were predominant, tremor mostly began between 21 and 40 years, superior limbs were the most affected body part, dominant autosomal inheritance was predominant, with anticipation in begining age. Association with some other diseases was frequent, many patients showed functional disability.
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Características Clínicas del Temblor Esencial en México
We studied the clinical and genetic characteristics of 100 patients with definite diagnosis of essential tremor (ET), evaluated at the clinic of movement disorders of the National Institute of Neurology of Mexico. All patients hab laboratory (metabolic and endocrine tests), neurophysiologic studies, and neuroimaging studies to evaluate the presence of other potential causes of movement disorders. The study included 100 patients with definitive diagnosis of ET, 33 men and 67 women. The mean age at the time of diagnosis was 45,92 years (40.92 in men and 48.4 in women). Mean time from the onset of tremor to diagnosis was 10.60 years (13,71 in males and 9.07 in females). Family history of tremor was found in 56% of the patients. Patients with maternal inheritance had lower age of onset (35.61 years vs. 46.11 years, p<0.05). Five percent of the patients exhibited almost a total lack of capacity in writing and in fine and precise activities. This study showed that patients with ET inherited frim the mother exhibit a lower age of onset. A narrow relationship between the sex of the progenitor and ET probably exists, The torpid evolution of the tremor was noted in 5% of the cases, emphasizing the need for early therapy before deterioration becomes incapacitating.
