Since only some of the patients initially diagnosed as BTP eventually displayed typical pathological features of PD, it was felt that BTP was a misnomer (Table 1). In an editorial on study by Selikhova et al., it was recommended that patients with rest and action tremors and minimal Parkinsonism features should be categorized as “monosymptomatic tremor at rest”. This subtype typically progresses the slowest amongst the various PD subtypes and the presence of significant motor findings in the beginning may be minimal. Some argue BTP represents one end of the clinical spectrum of PD which is tremor-predominant PD. Postmortem findings indicate fewer pathological changes in the substantia nigra further consolidating the concept that BTP is a distinct entity. The prognosis is much better compared to idiopathic PD. Most of them present at a relatively younger age of onset in the fifth decade and have a family history which suggests a strong underlying genetic etiology. These patients display a poor and unpredictable response to levodopa therapy. The gait disorder essentially comprises of mild stooping or a reduced arm swing instead of classic shuffling gait with progressive deterioration of balance.
Weak and tremulous series#
Īccording to the Mayo Clinic series which was also later reinforced by other groups, BTP is characterized by rest and action tremor at onset that remains persistent as the dominant feature for at least 8 years with only a minimal progression of other parkinsonism signs. Since then a debate has surfaced whether BTP represents a unique disorder or the clinicians are facing a variant within the spectrum of tremor-predominant idiopathic PD. The authors referred to this condition as benign tremulous Parkinsonism (BTP). Prior to this report, there were only few cases briefly mentioned in the literature. The progression of symptoms was notably slow, there was a significant family history and unlike typical PD, there were fewer nonmotor features. Although this constellation of features was tempting to be categorized as PD, the clinicians at Mayo Clinic observed unique differences. These patients had a pronounced asymmetric resting tremor in conjunction with mild to moderate postural and action tremor and mild parkinsonian signs. Benign tremulous parkinsonism was first characterized extensively in cohort of 16 patients followed at Mayo Clinic who shared similar phenotype and exhibited a unique clinical course. The basic difference between the arm tremors seen in these two conditions is that essential tremor is characterized by postural and action tremor whereas PD tremor is mostly a resting tremor. The most common cause of tremors is essential tremor and the other important differential is idiopathic Parkinson’s disease (PD). Tremor is a common symptom seen in movement disorders clinical practice.
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