Search

Patients with orthostatic tremor (OT) can be classified as having "primary OT," with or without postural arm tremor but no other abnormal neurological features, or "OT plus." We describe a patient with OT, with postural tremor of the arms and restless legs syndrome (RLS), who developed features typical of progressive supranuclear palsy (PSP). PSP can be accompanied by OT.

Background: Manganese intoxication may lead to a levodopa-resistant, akinetic-rigid syndrome. A new form of presumed manganese poisoning has been reported in drug-addicted persons from Russia, Ukraine, and Estonia who have intravenously injected self-prepared methcathinone hydrochloride (Ephedrone)., Patient: A 36-year-old man from Azerbaijan with hepatitis C and only modest hepatic synthetic dysfunction developed rapid-onset, levodopa-resistant parkinsonism with profound hypophonia., Conclusion: Ephedronic encephalopathy outside the region of the former Soviet Union may become a more widespread public health problem as a result of global travel and the easy availability of the recipe for synthesis of methcathinone on the Internet.

The Unified Parkinson's Disease Rating Scale (UPDRS) is widely used for the clinical evaluation of Parkinson's disease (PD). We assessed the rater variability of the UPDRS Motor examination (UPDRS-ME) of nurse practitioners, residents in neurology, and a movement disorders specialist (MDS) compared to a senior MDS. We assessed the videotaped UPDRS-ME of 50 PD patients. Inter-rater and intra-rater variability were estimated using weighted kappa (kappa(w)) and intraclass correlation coefficients (ICC). Additionally, inter-rater agreement was quantified by calculation of the mean difference between 2 raters and its 95% limits of agreement. Intra-rater agreement was also estimated by calculation of a 95% repeatability limits. The kappa(w) and ICC statistics indicated good to very good inter-rater and intra-rater reliability for the majority of individual UPDRS items and the sum score of the UPDRS-ME in all raters. However, for inter-rater agreement, it appeared that both nurses, residents, and the MDS consistently assigned higher scores than the senior MDS. Mean differences ranged between 1.7 and 5.4 (all differences P < 0.05), with rather wide 95% limits of agreement. The intra-rater 95% repeatability limits were rather wide. We found considerable rater difference for the whole range of UPDRS-ME scores between a senior MDS and nurse practitioners, residents in neurology, and the MDS. This finding suggests that the amount by which raters may disagree should be quantified before starting longitudinal studies of disease progression or clinical trials. Finally, evaluation of rater agreement should always include the assessment of the extent of bias between different raters.

We evaluated the effects of bilateral pallidotomy in patients with advanced Parkinson's disease. Thirteen patients with Parkinson's disease had a staged bilateral pallidotomy if they had severe response fluctuations, dyskinesias, painful dystonia, or bradykinesia despite optimum pharmacological treatment. Assessment scales were the Unified Parkinson's Disease Rating scale (UPDRS), the Schwab and England scale, and a questionnaire on the effects of disability in activities of daily living and adverse effects. Postoperative magnetic resonance imaging was evaluated for lesion location and extension. The median off-phase UPDRS motor score was reduced from 43.5 to 29 after the first pallidotomy, and it was further reduced to 23.5 after the second pallidotomy (n = 8). The UPDRS activities of daily living off-phase score improved from 28.5 to 20.5 after the first pallidotomy and to 19 after the second pallidotomy (n = 6). The Schwab and England scale off-phase score showed an improvement after both procedures, first from 40 to 60, and thereafter to 90 (n = 8). On-phase dyskinesias were reduced substantially. Eight patients had adverse effects, of whom five had problems with speech. One patient became hemiplegic due to a delayed infarction. Ten patients experienced further benefit from the second procedure. Bilateral pallidotomy reduces dyskinesias. A second contralateral pallidotomy may reduce parkinsonism, although to a lesser degree compared with the first pallidotomy and with an increased risk for adverse effects., (Copyright 2002 Movement Disorder Society)