Progressive ataxia and palatal tremor associated with dense pontine calcification: A unique case.

Intracranial calcifications are associated with a variety of disorders, but marked pontine calcification without an underlying structural lesion has rarely been reported. Here we present a unique case with progressive ataxia palatal tremor (PAPT) associated with marked pontine calcification. A previously healthy 53-year old woman first noticed balance problems, slurred speech, and a constant oculopalatal tremor at age 48. Within 4 years of symptom onset, she progressed to requiring support when walking, developed swallowing difficulties, and intermittently had double incontinence. Cognition was intact. Medical history included type 2 diabetes (dietary management only), hypothyroidism (euthyroid for more than a decade), hypercholesterolemia, and arterial hypertension. She was taking lisinopril, simvastatin, levothyroxine, and fluoxetine. There was no family history of any neurological disorder. On examination at age 52, she had cerebellar dysarthria and a synchronous ocular-facial-palatal myoclonus. She had titubatory head tremor and torticollis to the left. Examination of extraocular movements showed an elliptical ocular tremor synchronous with the palatal tremor, left lateral rectus palsy, and slow horizontal saccades but normal conjugate vertical eye movements and convergence. She had bilateral gazeevoked nystagmus and finger–nose ataxia. Her gait was ataxic, requiring the support of 2 people in order to walk (Video 1). Sensation, power, and reflexes were normal. Follow-up at age 53 showed further deterioration of her dysarthria and gait. She is currently wheelchair bound (Video 2). Routine blood tests including thyroid function, parathyroid hormone, lupus anticoagulant, white cell enzymes, -----------------------------------------------------------Additional Supporting Information may be found in the online version of this article.