None of them were examined by us for the independent verification of their diagnosis. Additionally, his mother, maternal grandmother, and sister had ET. His father had akinetic-rigid type of PD without any tremor. However, his father, two paternal aunts and his maternal grandfather had PD. His past medical history was unremarkable in particular he denied a history of head trauma, encephalitis, and exposure to industrial toxins or psychotropic medications. He also had mild, bilateral upper extremity rigidity and reduced arm swing, with left side predominance. His tremor forced him to proceed with earlier retirement from his work as travel agent. He was able to cope with basic daily living activities but with significant compromise. He had only mild tremor in his right hand when operating a computer mouse. He had severe and persistent resting tremor with postural and kinetic components that affected mostly his left hand (video 1). Case report A 57-year-old, left-handed American man presented with a 3-year history of hand tremor. Here we present a case of BTP with levodopa-refractory resting tremor in which the tremor was completely resolved after deep brain stimulation (DBS).Ģ. Others suggest that BTP is a variant of classic PD. argued that BTP may be a distinct disorder similar to but distinguishable from PD. In some cases BTP can resemble essential tremor (ET) due to significant action and postural features. It is common for patients with BTP to have a positive family history of Parkinson’s disease (PD) or tremors. These symptoms either do not progress or progress very slowly over the time. Other cardinal parkinsonian signs such as rigidity, bradykinesia, and postural instability are usually mild. The tremor is often problematic because it is refractory to dopaminergic therapy. Action tremor with postural and kinetic components is usually present as well. Introduction Benign tremulous parkinsonism (BTP) is characterized by severe resting tremor that is usually asymmetric and predominantly affects the upper extremities. Wszolek, MD, Department of Neurology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL, 32224, United States, Tel.: +1 9, Fax: +1 9, Conflict of interest None declared.ġ. DBS should be considered for patients with BTP PD variant despite their poor responsiveness to levodopa treatment.īenign tremulous parkinsonism Parkinson’s disease deep brain stimulation dopamine transporter imaging tremor Thus, despite the lack of levodopa responsiveness BTP likely represents a variant of PD and not a distinct neurodegenerative disorder. In some autopsied BTP cases classic alpha-synuclein pathology of PD was observed. The DAT imaging abnormalities indicate the presynaptic dopamine deficiency. His tremor resolved immediately after procedure. He was treated with deep brain stimulation (DBS) targeting the right ventral intermediate nucleus of the thalamus.
The dopamine transporter (DAT) imaging showed reduced tracer uptake in the putamen bilaterally, more so on the right side. His tremor was refractory to dopaminergic therapy, including carbidopa/levodopa. There was only very mild rigidity and bradykinesia and no postural instability. We present a case of a 57-yearold 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. Thus, BTP could be considered either as a distinct clinical disorder or a variant of PD. This tremor is frequently disabling and poorly responsive to therapy with levodopa. Of Neurosurgery, Mayo Clinic, 4500 San Pablo Road South, Jacksonville, Floridaīenign tremulous parkinsonism (BTP) is characterized by prominent resting tremor combined with action and postural components, and with only subtle rigidity and bradykinesia. Of Neuroscience, Mayo Clinic, 4500 San Pablo Road South, Jacksonville, Florida
Of Neurology, Mayo Clinic, 4500 San Pablo Road South, Jacksonville, Florida 32224 doi:10.1016/j.pjnns.2016.05.008.ĭeep brain stimulation for levodopa-refractory benign tremulous parkinsonism Takuya Konno, MD, PhD1, Owen A. Published in final edited form as: Neurol Neurochir Pol. Author manuscript available in PMC 2017 September 01. HHS Public Access Author manuscript Author Manuscript
0 kommentar(er)
