The ‘essentials’ of essential palatal tremor: a reappraisal of the nosology

Abstract

Essential palatal tremor (EPT) is an uncommon disorder, distinct from symptomatic palatal tremor (SPT), but characterized by superficially similar rhythmic movements of the soft palate. While the pathophysiology of SPT has been relatively well defined, this is not the case in EPT. Based on an analysis of 103 published cases, we reviewed EPT in the context of other movement disorders with similar features and outline possible pathophysiological mechanisms. Phenomenologically it remains best classified as a tremor. Four major causes, including a central generator, peripheral/mechanical, voluntary/special skill and psychogenic, appear to account for the majority of cases of EPT, although there is considerable overlap in the pathogenic mechanisms underlying these categories. Among the cases reviewed, a large proportion fit into the latter two categories, although there are others where multiple mechanisms are likely at play. Based on our reappraisal, we suggest a change in designation to 'isolated palatal tremor', with primary and secondary subtypes. This retains the distinction from SPT and emphasizes the non-uniform, heterogeneous nature of the disorder.

Palatal myoclonus

“…Beynin MRG incelemesi normaldir ancak fonksiyonel MRG çalışmaları alt oliva ve komşu beyin sapı yapılarında artmış aktiviteye işaret edebilir. [7] Esansiyel palatal miyoklonus, tensor veli palatiniye lokalize olmakla beraber EPM olgularının %30'unda yumuşak damak dışı kasların da tutulduğu bildirilmiştir. [8] Semptomatik palatal miyoklonus, farengeal pleksusun sinirlendirdiği levator veli palatini kaslarının yumuşak damağı kaldırıp geri çekmesi ile oluşur; komşu kasların da aynı anda kasılmaları tabloya eşlik edebilir hatta uzuv kaslarına yayı-labilen uzak etki oluşabilir.…”

Essential palatal tremor with hemifacial and vocal cord tremor

“…SPT is usually associated with olivary pseudohypertrophy or focal lesions involving dentato-rubro-olivary pathway ‘Guillain-Mollaret triangle’ 1 2. The common aetiologies of SPT are stroke, tumour, trauma, abscess, arteriovenous malformations, ectatic arteries, and some metabolic and demyelinating illness 1 2. In SPT, tremor may involve facial, ocular and extremity muscles with absence of ear clicks.…”

“…EPT does not have definable aetiology with younger age of presentation. It is associated with ear-clicking 2. Absence of extra palatal movement disorders as well as normal neuroimaging favours the diagnosis 2.…”

Tremor Tedavisinde Botulinum Toksini

“…Esansiyel PT'de objektif tinnitus en belirgin özelliktir ve sosyal s›k›nt› yarat›r. Antikonvülsan ve sedatifler baflta olmak üzere çeflitli ilaçlar denenmifl, ancak semptomlarda kayda de¤er iyileflme sa¤lanamam›flt›r (18,19,20).…”