Progression of parkinsonism in multiple system atrophy

BACKGROUND: Progression of parkinsonian motor impairment is usually rapid and relentless in multiple system atrophy (MSA). However, it may also be subject to considerable variation. Prospective natural history studies using validated rating scales are required to accurately determine the progression of parkinsonism in MSA. OBJECTIVE: To assess the progression of parkinsonism in patients with the Parkinson variant of MSA. METHODS: Parkinsonian motor impairment was assessed on regular therapy at two time points (mean follow-up 11.8 months, SD 1.4) using the Hoehn and Yahr scale (H&Y), the Schwab and England ADL scale (SES) and the motor examination section of the UPDRS (UPDRS-III) in 38 patients with clinically probable MSA-P. RESULTS : We examined 38 patients with probable MSA-P (mean age 63.2 years, SD 7.4; mean disease duration 4.1 years, SD 3.0). The mean difference of UPDRS-III between baseline and follow-up was 10.8 (95% CI 8.6-12.9), consistent with an average annual 28.3 % increase of UPDRS-III baseline scores. Several variables were associated with faster progression of parkinsonism including low baseline global motor disability as assessed by H&Y and SES, low baseline UPDRS-III score, and short disease duration. UPDRS-III progression was unrelated to gender, age at symptom onset, and age at baseline visit. CONCLUSION: This is the first observational study on UPDRS rates of decline in MSA. The observed 28.6% annual increase of UPDRS-III scores illustrates the rapid progression of motor impairment in MSA. Furthermore, motor progression appeared to be accelerated during the early disease stages.Our data allow sample size calculations that may be helpful for the planning of future therapeutic trials.     

Application of the International Cooperative Ataxia Scale rating in multiple system atrophy.

We assessed the International Cooperative Ataxia Scale (ICARS) as a means of extracting and rating cerebellar signs in multiple system atrophy (MSA). Cross-sectional analysis of internal consistency, factor structure, and correlation with parkinsonism severity (Unified Parkinson's Disease Rating Scale [UPDRS] III) of the ICARS, in 50 unselected MSA patients (mean age, 67.6 years; mean disease duration, 5.5 years), 50 age-matched and disease duration-matched Parkinson' disease (PD) patients, and 50 control subjects. Fifteen patients (30%) had MSA-C (cerebellar subtype) and 35 (70%) MSA-P (parkinsonism subtype), and 66% had at least one cerebellar sign. The total ICARS score was much higher (fivefold) in MSA compared to PD patients. The ICARS score was twofold higher in MSA-C than in MSA-P patients. MSA-C patients had a higher score than MSA-P mainly on posture and gait disturbances and kinetic functions subscores. All the ICARS items were significantly more severe in MSA than in PD patients, who in turn scored higher than the controls. In MSA, internal consistency was excellent (Cronbach = 0.93). Factor structure analysis revealed four clinically distinct subscores, in accordance with the scale structure, which accounted for 70% of the variance. The ICARS showed less consistency and accuracy in PD patients; however, the ICARS scores significantly correlated with the UPDRS-III scores in both MSA and PD patients. The ICARS appears a useful tool to extract and rate the severity of cerebellar signs in MSA; however, it is clearly contaminated by parkinsonian features.     

Placebo-controlled trial of amantadine in multiple-system atrophy

OBJECTIVE: Multiple-system atrophy (MSA) often presents as atypical parkinsonian syndrome with rapid progression and poor response to levodopa. Reports on the open-label use of amantadine in MSA suggest variable antiparkinsonian efficacy. The authors therefore conducted a double-blind, placebo-controlled crossover trial of amantadine in MSA patients. METHODS: Eight patients were enrolled in this study. They received either amantadine 200 mg twice daily or placebo for 3 weeks, followed by a 1-week washout and the alternate treatment of 3 weeks. Antiparkinsonian effects were evaluated using the Unified Parkinson's Disease Rating Scale part II (UPDRS-II, activities of daily living) and UPDRS-III (motor examination) before and at the end of each treatment phase. Timed tests were also performed according to the CAPIT protocol. RESULTS: There was a trend toward reduction of UPDRS-III scores during amantadine treatment (P = 0.058). Delta values of the treatment arm were higher than those of the placebo arm. However, this difference (-2.25 pt; 95% CI, -6.7-2.2) failed to reach significance. Reduction of UPDRS-III subscores for akinesia, rigidity, tremor, postural instability, and gait disorder failed to reach significance too. The hand-arm movement test revealed nonsignificant improvement in the amantadine arm. CONCLUSION: This study suggests that amantadine fails to provide clinically significant antiparkinsonian benefit to patients with MSA. The authors cannot exclude mild effects due to the limited sample size.     

Unilateral thalamotomy for the treatment of tremor dominant Parkinson's disease

BACKGROUND AND PURPOSE: To assess the effectiveness of unilateral thalamotomy for the treatment of parkinsonian tremor and other motor signs of Parkinson's disease (PD). MATERIAL AND METHODS: Between 1999 and 2004, 41 patients with idiopathic tremor dominant PD were treated surgically in the Neurosurgical Department of Postgraduate Medical Center in Warsaw. Stereotactic thalamotomy was performed with Leksell stereotactic frame (model G) using intraoperative macrostimulation. The patients were assessed according to the Unified Parkinson's Disease Rating Scale version 3. (UPDRS) before and after thalamotomy in the off state. The progression of PD was also evaluated according to the Hoehn and Yahr scale in the off state and also Schwab and England was used to assess the disability of the patients. The patients were evaluated before thalamotomy in the off state, and 3, 12, 24 and 36 months after surgery, according to the above mentioned clinical rating scales. RESULTS: The authors report their results among 41 patients who underwent stereotactic thalamotomy 3 years postoperatively. At 3 years follow-up (in the group of 19 patients) the contralateral tremor from the presurgical value of 11.2 (items 20 - 21 UPDRS) decreased to 2.6. The rigidity in contralateral limbs at 3 years follow-up was 1.7 (item 22 UPDRS) when compared to 2.8 (item 22 UPDRS) preoperative value. Thalamotomy had no effect on bradykinesia or other manifestations of PD such as balance or gait disturbance. There were 13 transient and 6 permanent complications. CONCLUSIONS: Thalamotomy using intraoperative macrostimulation in carefully selected patients is a beneficial operation for the control of medically refractory parkinsonian resting and postural tremor. The effect of unilateral thalamotomy on tremor is long lasting.     

Anomalies of asymmetry of clinical signs in parkinsonism.

Parkinson's disease (PD) is characterized by a minimum of two of three features: tremor, rigidity, and bradykinesia. Asymmetry of these features is often considered to support a diagnosis of PD in contrast to other parkinsonian syndromes. All major manifestations of PD are often more pronounced on the side first manifesting features of PD. Significant dissociation of features on the contralateral side, along with other variants of presentation involving the contralateral side, are rarely observed. To determine the frequency and significance of unusual asymmetry in parkinsonism, we retrospectively examined 613 patients clinically diagnosed as idiopathic PD for presence of unusual asymmetries of clinical features. Three groups of patients with unusual asymmetrical clinical findings were identified. Group 1 comprised 10 patients followed for an average of 6 years presenting with rest tremor most prominent in one lower limb and contralateral upper limb. Group 2 comprised 24 patients followed for an average of 5.5 years with action tremor most prominent on the side contralateral to the side of most prominent rest tremor. Group 3 comprised 33 patients followed for an average of 10 years who had parkinsonian signs of greatest severity on one side but subsequently, over an average of 5.4 years, became gradually more prominent on the opposite side. In Group 3, 15 of 33 patients (45%) demonstrated evolution to a rigid form of parkinsonism with disappearance of rest tremor over an average of 7.1 years after presentation. A small percentage (11%) of Parkinson's patients in our clinic demonstrated anomalous asymmetrical clinical findings, which indicates that (1) the disease process may begin in different topographic sites on each side; (2) rest tremor and action tremor may have different anatomical bases; (3) the disease process may progress at different rates on different sides; and (4) tremor becomes less pronounced with progression of disease in some patients with Parkinsonism.     

Function of the cerebellum in Parkinsonian rest tremor and Holmes' tremor.

We describe a patient who developed Parkinson's disease (PD) 17 years after resection of his right cerebellum because of a Lindau tumor. He showed a classic 4.3-Hz resting tremor on the left side but a 3.1-Hz resting, postural, and intention tremor on the right side compatible with midbrain tremor (Holmes' tremor). We conclude that the generator of the tremor in PD cannot be located within the olivocerebellar loop. The cerebellum, however, seems to modulate the tremor frequency of parkinsonian rest tremor and may prevent the rest tremor from transforming into a postural and goal-directed tremor.     

Myocardial sympathetic degeneration correlates with clinical phenotype of Parkinson's disease.

In idiopathic Parkinson's disease (PD), different clinical subtypes are distinguished due to predominant motor symptoms: a tremor-dominant type (TDT), an akinetic rigid type (ART), and a mixed type (MT). We compared myocardial sympathetic innervation, measured by MIBG scintigraphy, in different subtypes of PD at early and advanced stages of PD. We applied MIBG scintigraphy in 102 patients with PD. About 57 patients were at Hoehn and Yahr (H&Y) stage 1, 22 at H&Y stage 2, and 23 at H&Y stages 3 and 4. For quantification of myocardial MIBG uptake, the heart-to-mediastinum (H/M) count-ratio was calculated. At all H&Y stages, myocardial MIBG uptake was significantly higher in TDT patients than in ART or MT patients (P < 0.05; ANOVA). Furthermore, at each H&Y stage, myocardial MIBG uptake correlated significantly with severity of hypokinesia (P < 0.05; Spearman's correlation) and rigidity (P < 0.05), but not with severity of resting or postural tremor. The significant correlation between myocardial sympathetic degeneration and severity of hypokinesia and rigidity suggests that myocardial sympathetic degeneration and hypokinetic-rigid symptoms develop in a closely coupled manner in early as well as advanced PD. No such correlation can be found between myocardial sympathetic degeneration and parkinsonian tremor.     

123I-IPT brain SPECT study in essential tremor and Parkinson's disease

OBJECTIVE: To investigate nigral neuronal damage in patients with isolated postural tremor and those with postural and rest tremor without parkinsonism. METHODS: Using [123I]-N-(3-iodopropen-2-yl)-2 -carbomethoxy-3beta-(4-chlorophenyl) tropane SPECT, we measured the basal ganglia-occipital cortex/occipital cortex ([BG-OCC]/OCC) uptake ratios in 21 control subjects and patients with isolated postural tremor (n = 9), postural and rest tremor (n = 6), and PD (n = 11). RESULTS: In the patients with PD, the means (+/-SD) of the (BG-OCC)/OCC ratios of the ipsilateral (2.35+/-0.37) and the contralateral (1.97+/-0.33) sides to the more severely affected limbs were significantly lower than the mean of the bilateral (BG-OCC)/OCC ratios of the age-matched control subjects (3.83+/-0.66). The mean (+/-SD) of the bilateral (BG-OCC)/OCC ratios of the patients with isolated postural tremor (3.60+/-0.83) was comparable with that of the age-matched control subjects. However, the mean (+/-SD) of the bilateral (BG-OCC)/OCC ratios of the patients with postural and rest tremor (2.61+/-0.18) was lower than that of the control subjects (p < 0.05). The mean of the bilateral (BG-OCC)/OCC ratios of the patients with postural and rest tremor was comparable with that of the side ipsilateral to the severely affected limbs of the patients with PD. However, it was higher than that of the side contralateral to the limbs more severely affected by PD. Four of the six patients with postural and rest tremor had (BG-OCC)/OCC ratios lower than 2 standard deviations from the mean of the age-matched control subjects. CONCLUSIONS: Later in their clinical courses, some patients with postural tremor may acquire rest tremor in association with mild substantia nigra neuronal loss.     

Benign tremulous Parkinson's disease

OBJECTIVE: To analyze the long-term outcomes of patients presenting with pure parkinsonian tremor and to determine whether or not such patients develop the other features of Parkinson's disease (PD) eventually. METHODS AND MATERIALS: Two hundred fifty-one patients with PD followed at our referral center were examined regularly. In this study, we evaluated the long-term follow up of the the patients with parkinsonian tremor without bradykinesia or rigidity. RESULTS: The mean disease duration was 5 years (range: 2-10 yrs.) at the last follow-up visit. This final group included 7 female and 16 male patients with a mean age of 66.6 +/- 10.8 years. Four groups of patients were identified. First group consisted of 15 patients presenting with rest tremor; most prominently in one upper limb and/or contra-lateral upper limb. In the second group, there were 3 patients who had parkinsonian tremor with greatest severity in one lower limb and ipsilateral upper limb. Group 3 comprised 2 patients who had parkinsonian tremor in only one lower limb. The fourth group comprised 3 patients with isolated jaw tremor. CONCLUSIONS: Some patients with pure parkinsonian tremor may remain without bradykinesia or rigidity for a long time, which may be considered a benign form of Parkinson's disease.     

An imaging study of parkinsonism among African-Caribbean and Indian London communities.

We previously reported on 131 parkinsonian patients of African-Caribbean and Indian origin attending movement disorders clinics in six London Hospitals, of whom approximately 20% manifested atypical parkinsonism with a late-onset, akinetic-rigid predominant syndrome, postural instability and minimal resting tremor refractory to levodopa therapy and dopamine agonists (see Hu et al., Neurology 2000;54[Suppl.3]: A188 and Hu et al., Mov Disord 2000;15[Suppl.3]:S212). To better elucidate the phenotype of these atypical patients (18)FDG/(18)F-dopa positron emission tomography (PET) were performed in a subgroup to look for cortical and striatal metabolic changes suggestive of multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), or dementia with Lewy bodies. Magnetic resonance imaging (MRI) rating of cerebral vascular lesion load, putaminal atrophy, and neuropsychological testing were also performed. Discriminant function analysis of (18)F-dopa/(18)FDG striatal metabolism in 43 patients failed to separate atypical ethnic minority from typical Caucasian Parkinson's disease (PD) patients. Additionally, atypical Indian and African-Caribbean patients did not show cortical reductions in glucose metabolism suggestive of PSP, CBD, or DLB. Cerebral vascular lesion load rated in these patients did not differ between atypical and typical PD groups, and none of the atypical patients had MRI changes suggestive of MSA or PSP. Our results suggest the atypical parkinsonian phenotype seen in African-Caribbean and Indian patients represents a levodopa-refractory form of PD separate from MSA or PSP in most patients.     

The Bradykinesia Akinesia Incoordination Test (BRAIN TEST), an objective and user-friendly means to evaluate patients with parkinsonism.

The BRAIN TEST, a computerized alternating finger tapping test, was performed on 154 patients with parkinsonism to assess whether the test could be used as an objective tool to evaluate reliably the severity of Parkinson's disease (PD). Patients were instructed to tap two marked computer keyboard keys as fast and as accurately as possible for 60 seconds. The test generates the following variables: (1) kinesia score (KS)--number of keystrokes/min, (2) akinesia time (AT)--cumulative time that keys are depressed, (3) dysmetria score (DS)--a weighted score generated from incorrectly hit keys and corrected for speed, and (4) arrhythmia score (AS)--variance of the time interval between individual keystrokes. Among parkinsonian patients, we found a significant correlation between the four test parameters and PD rating scores of the Hoehn & Yahr, Schwab & England, and Unified PD Rating Scales (KS, AS, and AT p <0.001 and DS p <0.05). When compared with 73 parkinsonian patients 73 age- and sex-matched control subjects showed significantly higher KS and lower AT (p <0.001) as well as lower DS and AS (p = 0.05). The BRAIN TEST is a reliable and practical tool for evaluating the severity of parkinsonism and for distinguishing subjects with parkinsonism from normal control subjects. A version of the BRAIN TEST is available by FTP on the worldwide web (http://www.anaesthetist.com/software/brain.htm).     

Asymmetric and Upper Body Parkinsonism in Patients with Idiopathic Normal-Pressure Hydrocephalus

MethodsINPH subjects were selected in consecutive order from a prospectively enrolled INPH registry. Fifty-five INPH patients (37 males) having a positive response to the CSFTT constituted the final sample for analysis. The mean age was 73.7±4.7 years. The pre-tap mean Unified Parkinson''s Disease Rating Scale motor (UPDRS-III) score was 24.5±10.2.ResultsThere was no significant difference between the upper and lower body UPDRS-III scores (p=0.174). The parkinsonian signs were asymmetrical in 32 of 55 patients (58.2%). At baseline, the Timed Up and Go Test and 10-meter walking test scores were positively correlated with the total motor score, global bradykinesia score, global rigidity score, upper body score, lower body score, and postural instability/gait difficulties score of UPDRS-III. After the CSFTT, the total motor score, global bradykinesia score, upper body score, and lower body score of UPDRS-III significantly improved (p<0.01). There was a significant decrease in the number of patients with asymmetric parkinsonism (p<0.05).ConclusionsIn the differential diagnosis of elderly patients presenting with asymmetric and upper body parkinsonism, we need to consider a diagnosis of INPH. The association between gait function and parkinsonism severity suggests the involvement of similar circuits producing gait and parkinsonian symptoms in INPH.     

Exacerbation of postural tremor with emergence of parkinsonism after treatment with neuroleptic drugs.

Neuroleptic medication in three patients with prior isolated postural arm tremor led to a conspicuous deterioration; the postural tremor increased in amplitude, tremor appeared at rest, and other signs of mild parkinsonism developed. Withdrawal of neuroleptic drugs led to improvement in tremor and disappearance of parkinsonism. Positron emission tomography showed no reduction in uptake of [18F]dopa into nigrostriatal terminals suggesting that these patients did not have Parkinson's disease. Neuroleptic drugs can convert postural essential arm tremor into that characteristic of Parkinson's disease in patients with no evident nigrostriatal lesion.     

Does deep brain stimulation of the nucleus ventralis intermedius affect postural control and locomotion in Parkinson's disease?

The purpose of this study was to evaluate the effect of unilateral stimulation of the nucleus ventralis intermedius (VIM) on parkinsonian signs like postural stability and locomotion with respect to the severity of Parkinson's disease (PD). Seven patients with idiopathic PD were included in the study. Changes in visual cues on postural stability and step initiation were assessed on a fixed platform system with VIM stimulation switched either on (VIM ON) or off (VIM OFF), and compared with a control group of seven age-matched normal individuals. Sway scores (area and path) were significantly (p <0.05) higher in the parkinsonian patients with VIM OFF than with VIM ON as well as compared with the control subjects. No correlation was obtained between extent of sway scores and severity of contralateral tremor after cessation of VIM stimulation. Locomotion parameters, by contrast, were not influenced by VIM stimulation: latency until step initiation and walking-cycle time were the same among parkinsonian patients as among normal individuals, both in the presence and in the absence of VIM stimulation. In conclusion, our results indicate that tremor suppression by VIM stimulation improves postural stability.     

Diagnosing multiple system atrophy with greater accuracy: combined analysis of the clonidine-growth hormone test and external anal sphincter electromyography.

The clonidine-growth hormone test (CGHT) has been proposed as a means of differentiating multiple system atrophy (MSA) from idiopathic Parkinson's disease (PD). However, it is controversial whether the CGHT is valid. We sought to confirm the validity of the CGHT and to compare the diagnostic accuracy of the CGHT with that of external anal sphincter electromyelography (Sph-EMG) for MSA. We performed the CGHT and the Sph-EMG on 21 PD patients, 23 patients with probable MSA of parkinsonian type (MSA-p), and 22 patients with probable MSA of cerebellar type (MSA-c). We compared the sensitivity, specificity, and positive and negative predictive values (PPV and NPV) of CGHT, Sph-EMG, and a combination of the two tests. We also evaluated the correlations of Unified Parkinson's Disease Rating Scale (UPDRS) scores with the results of the two tests. There was no significant difference between the UPDRS scores for the PD and MSA-p groups. Serum growth hormone concentrations after clonidine significantly increased in PD (mean increase +/- SEM, 4.19 +/- 0.92 ng/ml; P < 0.0001), but remained unchanged in both MSA-p (0.83 +/- 0.61 ng/ml) and MSA-c (1.45 +/- 0.58 ng/ml). The growth hormone responses to clonidine in MSA-p were significantly different from those in PD (P < 0.05). Abnormal, denervated Sph-EMG was observed in 95.7% of MSA-p, 86.4% of MSA-c, and 33.3% of PD patients. Compared to Sph-EMG, the CGHT was less sensitive but more specific in both MSA-p and MSA-c. The result of neither test correlated with the severity of parkinsonism. Interestingly, combining the results of the CGHT and Sph-EMG markedly increased the specificity (85.7% in the CGHT and 66.7% in Sph-EMG vs. 95.2% in the combination study) and the PPV in both MSA-p (85.7% and 75.9% vs. 94.4%) and MSA-c (82.4% and 73.1% vs. 91.7%). We confirm that the CGHT can distinguish MSA-p from PD. Its sensitivity is lower and its specificity higher than Sph-EMG. Compared to either test alone, combined testing with the CGHT and Sph-EMG increased specificity and PPV, thereby enhancing accuracy in the diagnosis of MSA.     

Parkinsonism in fragile X-associated tremor/ataxia syndrome (FXTAS): Revisited

BackgroundParkinsonian features have been used as a minor diagnostic criterion for fragile X-associated tremor/ataxia syndrome (FXTAS). However, prior studies have examined parkinsonism (defined as having bradykinesia with at least rest tremor or postural instability) mostly in premutation carriers without a diagnosis of FXTAS. The current study was intended to elaborate this important aspect of the FXTAS spectrum, and to quantify the relationships between parkinsonism, FXTAS clinical staging and genetic/molecular measures.MethodsThirty eight (38) FXTAS patients and 10 age-matched normal controls underwent a detailed neurological examination that included all but one item (i.e. rigidity) of the motor section of the Unified Parkinson's Disease Rating Scale (UPDRS).ResultsThe FXTAS patient group displayed substantially higher prevalence of parkinsonian features including body bradykinesia (57%) and rest tremor (26%), compared to the control group. Furthermore, parkinsonism was identified in 29% of FXTAS patients. Across all patients, body bradykinesia scores significantly correlated with FXTAS clinical stage, FMR1 mRNA level, and ataxic gait of cerebellar origin, while postural instability was associated with intention tremor.InterpretationParkinsonian features in FXTAS appear to be characterized as bradykinesia concurrent with cerebellar gait ataxia, postural instability accompanied by intention tremor, and frequent rest tremor, representing distinctive patterns that highlight the need for further clinical studies including genetic testing for the FMR1 premutation. The association between FMR1 mRNA level and bradykinesia implicates pathophysiological mechanisms which may link FMR1 mRNA toxicity, dopamine deficiency and parkinsonism in FXTAS.     

Olfaction in essential tremor patients with and without isolated rest tremor.

An olfactory deficit is present in patients with essential tremor (ET), but it is often milder than that in patients with Parkinson's disease (PD). In both, the deficit occurs early in the disease. Isolated rest tremor without other signs of parkinsonism can occur in patients with ET. If the rest tremor in these patients represents a manifestation of ET rather than early PD, we hypothesized that their University of Pennsylvania Smell Identification Test (UPSIT) scores would be similar to those of ET patients without rest tremor. The mean UPSIT score in 13 ET patients with isolated rest tremor did not differ from that of 58 ET patients without rest tremor (29.3 +/- 4.3 vs. 29.4 +/- 6.4; P = 0.69). Several ET patients with rest tremor had UPSIT scores that fell outside of the range that is seen in 95% of patients with PD. These data raise the possibility that some ET patients with isolated rest tremor may not have early PD and that the pathological process that is responsible for their ET is also involving the basal ganglia.     

Coherence analysis of local field potentials in the subthalamic nucleus: differences in parkinsonian rest and postural tremor

Implantation of electrodes in the subthalamic nucleus (STN) for deep brain stimulation is a well-established method to ameliorate motor symptoms in patients suffering from Parkinson's disease (PD). This study investigated the pathophysiology of rest and postural tremor in PD. In 14 patients with PD, we recorded intraoperatively local field potentials (LFPs) in the STN (at different recording depths) and electromyographic signals (EMGs) of the contralateral forearm. Using coherence analysis we analysed tremor epochs both at rest and hold conditions in patients of the akinetic-rigid or of the tremor-dominant PD subtype. Data analysis revealed significant LFP-EMG coherence during periods of rest and postural tremor. However, strong differences between both tremor types were observed: local maxima (cluster) of rest and postural tremor did not match. Additionally, during rest tremor coherence occurred significantly more frequently at single tremor frequency than at double tremor frequency in tremor-dominant as well as in akinetic-rigid patients. In contrast, during postural tremor in patients with akinetic-rigid PD coherence was predominantly at double tremor frequency. The data suggest a specific topography of 'tremor clusters' for rest and postural tremor. Furthermore, we presume that the same tremor mechanisms exist in patients with tremor-dominant and akinetic-rigid PD, but to different degrees.     

Diagnostic utility of positron emission tomography for parkinsonism after chronic manganese exposure]

Positron emission tomography (PET) with [18F] 6-fluoro-L-dopa (18F-FDOPA) was performed in three South Korean patients with parkinsonism who developed after chronic manganese exposure. A 51-year-old man (patient 1) suffered from masked face, marked postural tremor of hands, dystonia in the neck and the upper extremities, severe retropulsion and lateropulsion which were typical for chronic manganese intoxication. 18F-FDOPA scan was normal. Other two patients, a 46-year-old man (patient 2) and a 47-year-old man (patient 3), showed tremor at rest and rigidity predominantly on the right side, bradykinesia, stooped posture and postural instability; all of these were typical for Parkinson's disease (PD). There was reduced uptake of 18F-FDOPA in the striatum, particularly in the posterior putamen predominant on the left side, in both patient 2 and 3. From these results, patient 1 was diagnosed as pure manganism, while patient 2 and 3 were primarily as PD, because loss of nigrostriatal fibers was obvious with asymmetry of affection in the putamen. PET with 18F-FDOPA provides valuable information for differentiation between PD and manganism, although it is not clear whether development of parkinsonian symptoms in patient 2 and 3 was modified by excessive manganese exposure.     

重复经颅磁刺激治疗帕金森病

目的探讨重复经颅磁刺激对帕金森病患者的治疗作用。方法采用重复经颅磁刺激方法对8例帕金森病患者进行治疗,另选择7例帕金森病患者作为对照。采用改进的H&Y(HoehnandYahr)评分标准、SchwabandEngland日常生活能力评分量表以及帕金森病症状评分量表(UPDRS)进行疗效评估。结果8例接受重复经颅磁刺激治疗的患者,于治疗第3、6和9个月时进行H&Y评分和UPDRS评分,与治疗前相比,这两种评分均明显下降(P<0.05),而SchwabandEngland日常生活能力评分则明显提高,治疗前后相比差异具有显著性意义(P<0.05);对照组治疗前后相比差异无显著性意义(P>0.05)。结论重复经颅磁刺激有助于缓解帕金森病患者的症状。