Benign tremulous parkinsonism (BTP) is characterized by a prominent tremor that occurs both at rest and with action in conjunction with other mild features of parkinsonism. The progression of symptoms is typically slow and there is often a positive family history. Although BTP is included within the phenotypic spectrum of Parkinsonism its exact relationship with idiopathic Parkinson's disease remains unclear. Treatment of BTP is challenging especially considering the poor response to levodopa, therefore surgical therapies such as deep brain stimulation surgery are sought for treatment of these tremors. In this review, we will summarize the clinical features, diagnosis, neuropathology and treatment for BTP.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4874026 | PMC |
http://dx.doi.org/10.1186/s40035-016-0057-1 | DOI Listing |
Cureus
November 2024
Norton Neuroscience Institute, Norton Healthcare, Louisville, USA.
Hirayama disease (HD) is a rare disorder characterized by insidious asymmetric neurogenic atrophy primarily involving the upper extremities. HD most commonly affects adolescent males and has a favorable prognosis for arrest of progression. Electrodiagnostic (EDX) studies show chronic denervation changes in the distal upper extremity muscles.
View Article and Find Full Text PDFCureus
March 2024
Department of Clinical Neuroscience and Therapeutics, Hiroshima University, Hiroshima, JPN.
We report a case involving a 31-year-old male without any known precipitating injuries presenting with involuntary finger movements and rare seizures. There was a noted family history of tremulous movements. Yet the characteristics of his finger movements were irregular and not typical of essential tremor (ET).
View Article and Find Full Text PDFClin Park Relat Disord
June 2023
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Neurology Unit, Milan, Italy.
We describe a case of severe adult-onset progressive tremulous cerebellar ataxia with pyramidal signs associated with a rare homozygous truncating pathogenic variant in the gene (p.Arg5371*). This contrasts the initial views on SYNE1-related ataxia as a relatively benign, slowly progressive condition, with important implications for clinic-genetic counselling.
View Article and Find Full Text PDFJ Mov Disord
September 2023
Chulalongkorn Centre of Excellence for Parkinson's Disease and Related Disorders, Department of Medicine, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hospital, Thai Red Cross Society, Bangkok, Thailand.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!