Palatal tremor, progressive multiple cranial nerve palsies, and cerebellarataxia: A case report and review of literature of palatal tremors in neurodegenerative disease

We describe a patient with an unusual clinical presentation of progressive multiple cranial nerve palsies, cerebellar ataxia, and palatal tremor (PT) resulting from an unknown etiology. Magnetic resonance imaging showed evide nce of hypertrophy of the inferior olivary nuclei, brain stem atrophy, and marked cerebellar atrophy. This combination of progressive multiple cranial nerve palsies, cerebellar ataxia, and PT has never been reported in the li terature. We have also reviewed the literature of PT secondary to neurodege nerative causes. In a total of 23 patients, the common causes are sporadic olivopontocerebellar atrophy (OPCA; 22%), Alexander's disease (22%), unknow n etiology (43.4%), and occasionally progressive supranuclear palsy (4.3%) and spinocerebellar degeneration (4.3%). Most patients present with progres sive cerebellar ataxia and approximately two thirds of them have rhythmic t remors elsewhere. Ear clicks are observed in 13% and evidence of hypertroph y of the inferior olivary nucleus in 25% of the patients. The common neurod egenerative causes of PT are OPCA/multiple system atrophy, Alexander's dise ase, and, in most of them, the result of an unknown cause.