title: Deep brain stimulation for levodopa-refractory benign tremulous parkinsonism.
abstract: 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.
authors: Department of Neurology, Mayo Clinic, Jacksonville, United States.
authors: Takuya
authors: T
authors: Konno
authors: Department of Neuroscience, Mayo Clinic, Jacksonville, United States.
authors: Owen A
authors: OA
authors: Ross
authors: Department of Neurosurgery, Mayo Clinic, Jacksonville, United States.
authors: Robert E
authors: RE
authors: Wharen
authors: Department of Neurology, Mayo Clinic, Jacksonville, United States.
authors: Ryan J
authors: RJ
authors: Uitti
authors: Department of Neurology, Mayo Clinic, Jacksonville, United States. Electronic address: Wszolek.zbigniew@mayo.edu.
authors: Zbigniew K
authors: ZK
authors: Wszolek
mesh_terms: D000978:Antiparkinson Agents; D046690:Deep Brain Stimulation / Q000379:methods*; D050483:Dopamine Plasma Membrane Transport Proteins / Q000378:metabolism; D004351:Drug Resistance; D006801:Humans; D007980:Levodopa; D008297:Male; D008875:Middle Aged; D020734:Parkinsonian Disorders / Q000000981:diagnostic imaging / Q000235:genetics / Q000628:therapy*; D010375:Pedigree; D049268:Positron-Emission Tomography; D013788:Thalamus; D016896:Treatment Outcome; D014202:Tremor / Q000000981:diagnostic imaging / Q000628:therapy
chemical_list: D000978:Antiparkinson Agents; D050483:Dopamine Plasma Membrane Transport Proteins; D007980:Levodopa
keywords: Benign tremulous parkinsonism; Deep brain stimulation; Dopamine transporter imaging; Parkinson's disease; Tremor