Benign tremulous
parkinsonism (BTP) is characterized by prominent
resting tremor combined with action and postural components, and with only subtle rigidity and
bradykinesia. This
tremor is frequently disabling and poorly responsive to
therapy with
levodopa. Thus, BTP could be considered either as a distinct clinical disorder or a variant of PD. We present a case of a 57-year-old man who had a 3-year history of severe and functionally disabling
resting tremor with action and postural features bilaterally but with left dominant hand predominance. There was only very mild rigidity and
bradykinesia and no postural instability. His
tremor was refractory to dopaminergic
therapy, including
carbidopa/levodopa. The
dopamine transporter (DAT) imaging showed reduced tracer uptake in the putamen bilaterally, more so on the right side. He was treated with
deep brain stimulation (DBS) targeting the right ventral intermediate nucleus of the thalamus. His
tremor resolved immediately after procedure. The DAT imaging abnormalities indicate the presynaptic
dopamine deficiency. In some autopsied BTP cases classic
alpha-synuclein pathology of PD was observed. Thus, despite the lack of
levodopa responsiveness BTP likely represents a variant of PD and not a distinct
neurodegenerative disorder. DBS should be considered for patients with BTP PD variant despite their poor responsiveness to
levodopa treatment.