[Effect of safinamide on morning-off phenomenon in elderly patients with Parkinson's disease].

AIM: Morning-off is a symptom experienced by patients with Parkinson's disease (PD), which markedly reduces patients' quality of life. The present study evaluated the effect of safinamide on morning-off in elderly PD patients. METHODS: This observational study included 30 PD patients treated with 50 or 100 mg/day of safinamide in the evening. Using patient-reported outcomes, we evaluated the effect of safinamide on daily/morning ON-time, daily/morning OFF-time, Unified Parkinson's Disease Rating Scale (UPDRS) Part III score, and non-motor symptoms. Data at baseline (treatment start) and at 4, 8, 12, and 16 weeks after baseline were recorded. RESULTS: The PD patients (75.8±7.5 years old) in this study, who tended to be older than in previous phase 2/3 or 3 studies, may represent real-world Japanese PD patients. Compared with baseline, safinamide significantly increased the daily ON-time at eight weeks and morning ON-time at four weeks. Safinamide significantly reduced the daily OFF-time and morning OFF-time at four weeks. The UPDRS Part III score was significantly reduced by 1 point at 12 weeks. Safinamide showed a tendency to reduce non-motor symptoms, such as anxiety, pain, and depressive feelings. There was no marked difference in these parameters between patients treated with 50 and 100 mg of safinamide. CONCLUSIONS: Our results suggest that safinamide administered in the evening can benefit elderly patients who experience wearing off, especially morning off, and non-motor symptoms.

Early detection of cognitive impairment in Parkinson's disease with the use of the Wisconsin Card Sorting Test: correlations with Montreal Cognitive Assessment and smell identification test.

Cognitive function is often impaired in early Parkinson's disease (PD). The Wisconsin Card Sorting Test (WCST) is a neuropsychological test of "set-shifting" ability. To see whether WCST is useful for detecting early changes of cognitive function in PD, we examined the correlations of WCST with the Montreal Cognitive Assessment (MoCA) and the Odor Stick Identification Test (OSIT). Subjects were 48 PD patients (age 66 ± 10 years; Hoehn & Yahr stage 2.3 ± 0.8; mean duration 3.1 ± 2.5 years). WCST sub-scores for categories achieved (CA), perseverative errors of Nelson type (PEN), and difficulties of maintaining set (DMS) were evaluated. MoCA-J (Japanese version) and OSIT-J (Japanese version) were done in that order, followed by the WCST. In PD patients, CA was 2.2 ± 2.0, PEN was 7.0 ± 6.4, and DMS was 2.3 ± 2.0, and all were worse than those of age-matched normal subjects. MoCA-J scores significantly correlated with PEN. OSIT-J scores were also significantly correlated with CA and DMS. As MoCA-J and OSIT-J show high sensitivity and specificity for detecting mild cognitive impairment in PD, WCST may also be a useful supplementary diagnostic tool for early and mild cognitive impairment in PD patients.

Association of Smell Identification Deficit with Alzheimer's Disease Assessment Scale-Cognitive Subscale, Japanese Version Scores and Brain Atrophy in Patients with Dementia.

INTRODUCTION: Olfactory dysfunction is commonly associated with Alzheimer's disease (AD) and may be related to disorder of the central olfactory processing system. In this work, therefore, we examined the relationships between olfactory changes and the most affected cognitive domain or degree of brain atrophy in patients with AD and mild cognitive impairment (MCI). METHODS: The subjects were 55 AD patients and 27 MCI patients. Smell identification tests were performed using Odor Stick Identification Test for Japanese -(OSIT-J). The severity and nature of cognitive dysfunctions were evaluated using the AD Assessment Scale-cognitive subscale, Japanese version (ADAS-Jcog). MRI with voxel-based specific regional analysis system for AD software was used for evaluation of brain atrophy. RESULTS: -OSIT-J scores were significantly correlated with total -ADAS-Jcog scores, as well as with ADAS-Jcog subscale items of word recall task, orientation (memory domain) and ideational praxis. Smell identification deficit was proportional to the degree of atrophy of the medial temporal lobe. CONCLUSION: Smell identification deficit in AD/MCI is strongly associated with the memory domain of cognitive function and with atrophy of the medial temporal lobe.

Characteristic deterioration of ADAS-Jcog subscale scores and correlations with regional cerebral blood flow reductions in Alzheimer's disease.

The Alzheimer Disease Assessment Scale (Japanese version) cognitive subscale (ADAS-Jcog) is composed of a number of subscale tasks. However, it is not clear which subscale tasks are most susceptible to impairment in Alzheimer's disease (AD) or what is the relationship between reduction in regional cerebral blood flow (rCBF) and decreased ADAS-Jcog scores. Subjects were 32 AD patients, aged 52-86 years. We examined the relationship between subscale tasks that showed marked score changes and brain regions that showed reduced rCBF over a 2-year period. rCBF was measured by single-photon emission computed tomography (SPECT) with technetium-99m ethyl cysteinate dimer (99mTc-ECD), and the SPECT imaging data were analyzed with the easy Z-score imaging system (eZIS) and voxel-based stereotactic extraction estimation (vbSEE) methods. Total score of ADAS-Jcog deteriorated from 19.5 ± 7.0 to 35.7 ± 15.2 after 2 years. Subscale scores were significantly worse in all fields, particularly in orientation, word recall, remembering test instructions, commands, constructional praxis, and ideational praxis, in that order. Significant correlations were found between (1) word recall and commands and rCBF in the left middle temporal lobe, (2) naming objects/fingers and rCBF in the left temporal (middle, inferior) lobe, and (3) constructional and ideational praxis and rCBF in the right parietal (superior, inferior) lobe, temporal (superior, middle) lobe, angular gyrus, and cingulate gyrus. We identified the brain regions associated with specifically impaired subscales of ADAS-Jcog during progressive deterioration of AD over 2 years.

Microbleeds, Cerebral Hemorrhage, and Functional Outcome After Stroke Thrombolysis.

Background and Purpose- We assessed whether the presence, number, and distribution of cerebral microbleeds (CMBs) on pre-intravenous thrombolysis MRI scans of acute ischemic stroke patients are associated with an increased risk of intracerebral hemorrhage (ICH) or poor functional outcome. Methods- We performed an individual patient data meta-analysis, including prospective and retrospective studies of acute ischemic stroke treated with intravenous tissue-type plasminogen activator. Using multilevel mixed-effects logistic regression, we investigated associations of pre-treatment CMB presence, burden (1, 2-4, ≥5, and >10), and presumed pathogenesis (cerebral amyloid angiopathy defined as strictly lobar CMBs and noncerebral amyloid angiopathy) with symptomatic ICH, parenchymal hematoma (within [parenchymal hemorrhage, PH] and remote from the ischemic area [remote parenchymal hemorrhage, PHr]), and poor 3- to 6-month functional outcome (modified Rankin score >2). Results- In 1973 patients from 8 centers, the crude prevalence of CMBs was 526 of 1973 (26.7%). A total of 77 of 1973 (3.9%) patients experienced symptomatic ICH, 210 of 1806 (11.6%) experienced PH, and 56 of 1720 (3.3%) experienced PHr. In adjusted analyses, patients with CMBs (compared with those without CMBs) had increased risk of PH (odds ratio: 1.50; 95% confidence interval: 1.09-2.07; P=0.013) and PHr (odds ratio: 3.04; 95% confidence interval: 1.73-5.35; P<0.001) but not symptomatic ICH. Both cerebral amyloid angiopathy and noncerebral amyloid angiopathy patterns of CMBs were associated with PH and PHr. Increasing CMB burden category was associated with the risk of symptomatic ICH ( P=0.014), PH ( P=0.013), and PHr ( P<0.00001). Five or more and >10 CMBs independently predicted poor 3- to 6-month outcome (odds ratio: 1.85; 95% confidence interval: 1.10-3.12; P=0.020; and odds ratio: 3.99; 95% confidence interval: 1.55-10.22; P=0.004, respectively). Conclusions- Increasing CMB burden is associated with increased risk of ICH (including PHr) and poor 3- to 6-month functional outcome after intravenous thrombolysis for acute ischemic stroke.

A Recurrent Case of Ischemic Stroke Caused by Vasospasm due to Giant Cell Arteritis.

A 73-year-old man was admitted with sudden right upper-limb weakness. He had a temporal headache on the left side and had a 4-month history of fever. Meandering of the left temporal artery (TA) with induration and high inflammatory responses (white blood cell count 22,500 per microliter, C-reactive protein 35.0 mg/dL, and elevated sedimentation rate [ESR] 80 mm/h) were observed. Glycometabolism and lipid metabolism were normal, and autoimmune antibodies were negative. Cultivation tests revealed no bacteria in either blood culture or cerebrospinal fluid. Brain magnetic resonance imaging (MRI) showed ischemic lesion in the left frontal lobe, while magnetic resonance angiography (MRA) and carotid ultrasonography showed unstable plaque lesions in the left extracranial internal carotid artery (ICA). According to reported criteria (age > 50 years, new onset of headache, abnormality of the TA, and raised ESR), we diagnosed giant cell arteritis (GCA) with acute ischemic stroke (IS) and gave the patient antithrombotic therapy (aspirin 100 mg, cilostazol 200 mg). After admission, hemiparesis progressed but fluctuated. Subsequent MRI showed new lesions in the left watershed area. MRA also showed vasospasm in the middle cerebral artery and C5 portion of the ICA. Considering the correlation with GCA pathophysiology, oral prednisolone therapy was administered. Steroid therapy has prevented stroke recurrence and improved the symptoms and vasospasm. We wish to emphasize that GCA can induce IS via vasospasm, and steroid therapy is recommended.

Cerebral microbleeds on T2*-weighted images and hemorrhagic transformation after antithrombotic therapies for ischemic stroke.

To assess the predictive value of cerebral microbleeds (CMBs) on gradient-echo T2*-weighted magnetic resonance imaging for hemorrhagic transformation (HT) after antithrombotic therapy for an acute ischemic stroke, we prospectively examined the relationship between CMBs on T2*-weighted images before the start of therapy and the appearance of HT in a series of patients treated with antithrombotic therapies. The subjects were consecutive acute ischemic stroke patients admitted to Tokai University Hospital (187 subjects, mean age±SD: 74±11 years). The prevalence of CMBs was not significantly different between the subjects with and without HT on computed tomography (CT) (19% versus 36%, P=.081). In both the subgroup of patients treated with anticoagulants and the subgroup treated with antiplatelets, the prevalence of HT was not significantly different between the subjects with and without CMBs (anticoagulants, 9% versus 21%, P=.161; antiplatelets, 0% versus 9%, P=.542). The odds ratios (ORs) of increasing the National Institutes of Health Stroke Scale score (1.14, 95% confidence interval [CI]: 1.04-1.26, P=.005) and decreasing the Alberta Stroke Program Early CT Score on diffusion-weighted images (ASPECTS-DWI) (1.32, 95% CI: 1.10-1.59, P=.003) were significantly increased for the appearance of HT, but the OR of CMBs (.35, 95% CI: .09-1.41, P=.140) was not significantly increased for the appearance of HT. In conclusion, the severity of neurological deficits and the ASPECTS-DWI are closely correlated to the development of HT related to anticoagulants/antiplatelets but not to CMBs on T2*-weighted images.

Phase I study of intravenous low-dose granulocyte colony-stimulating factor in acute and subacute ischemic stroke.

BACKGROUND: Granulocyte colony-stimulating factor (G-CSF; filgrastim) may be useful for the treatment of acute ischemic stroke because of its neuroprotective and neurogenesis-promoting properties, but an excessive increase of neutrophils may lead to brain injury. We examined the safety and tolerability of low-dose G-CSF and investigated the effectiveness of G-CSF given intravenously in the acute phase (at 24 hours) or subacute phase (at 7 days) of ischemic stroke. METHODS: Three intravenous dose regimens (150, 300, or 450 µg/body/day, divided into 2 doses for 5 days) of G-CSF were examined in 18 patients with magnetic resonance imaging (MRI)-confirmed infarct in the territory of the middle cerebral artery. Nine patients received the first dose at 24 hours poststroke (acute group) and 9 patients received the first dose on day 7 poststroke (subacute group; n = 3 at each dose in each group). A scheduled administration of G-CSF was skipped if the patient's leukocyte count exceeded 40,000/µL. Patients received neurologic and MRI examinations. RESULTS: We found neither serious adverse event, drug-related platelet reduction nor splenomegaly. Leukocyte levels remained below 40,000/µL at 150 and 300 µg G-CSF/body/day, but rose above 40,000/µL at 450 µg G-CSF/body/day. Neurologic function improvement between baseline and day 90 was more marked after treatment in the acute phase versus the subacute phase (Barthel index 49.4 ± 28.1 v 15.0 ± 22.0; P < .01). CONCLUSIONS: Low-dose G-CSF (150 and 300 µg/body/day) was safe and well tolerated in ischemic stroke patients, and leukocyte levels remained below 40,000/µL.

Relationship between chronic kidney disease and white matter hyperintensities on magnetic resonance imaging.

Renal dysfunction may be related to cerebral small-vessel disease. This study aimed to assess the relationship between mild renal dysfunction and various white matter hyperintensities on magnetic resonance imaging (MRI). A total of 2106 subjects (1368 men and 738 women; mean age, 56 ± 10 years) without a history of stroke were enrolled in the study. Kidney function was evaluated in terms of estimated glomerular filtration rate (eGFR), calculated using the relationship 194Cr(-1.094) × age(-0.287) × 0.739 (if female), where Cr is serum creatinine concentration. White matter hyperintensity on T2-weighted MRI was classified as deep and/or subcortical white matter hyperintensity (DSWMH), periventricular hyperintensity (PVH), or asymptomatic cerebral infarction (ACI). The prevalence of ACI, DSWMH, and PVH was significantly correlated with degree of eGFR reduction; in the subgroups with eGFR ≥ 90, 60∼89, and <60 mL/min/1.73 m(2), the following prevalences were found: ACI, 7%, 6%, and 16%; DSWMH, 18%, 21%, and 37%; PVH: 7%, 10%, and 21%. The odds ratios for ACI, DSWMH, and PVH of eGFR <60 mL/min/1.73 m(2) were significantly increased, to 2.11 (95% confidence interval [CI], 1.23-3.61; P = .006), 2.26 (1.53-3.34; P < .001), and 2.81 (1.67-4.72; P < .001), respectively. Our data indicate that mild renal dysfunction may be associated with an increase in cerebral small-vessel disease independent of hypertension.

Chronic kidney disease in patients with ischemic stroke.

To examine the significance of renal dysfunction in patients who have sustained ischemic stroke, we examined the relationship between the renal function evaluated in terms of estimated glomerular filtration rate (eGFR) and the subtype of brain infarction (BI) in patients with ischemic stroke. A total of 639 patients with BI were enrolled in this study, with 314 subjects without stroke or transient ischemic attack registered as age-matched controls. eGFR was calculated according to the equation 194 × Cr(-1.094) × Age(-0.287) (-0.739 if female), where Cr is serum creatinine concentration, and was classified into four stages: stage I, eGFR ≥ 90 mL/min/1.73 m(2); stage II, eGFR 60 ~ 89 mL/min/1.73 m(2); stage III, eGFR 30 ~ 59 mL/min/1.73 m(2); and stage IV, eGFR <29 mL/min/1.73 m(2). Stage III-IV was significantly more prevalent in the BI group (38%) than in the control group (22%; P < .001). The odds ratio for stage III-IV was significantly higher in the BI group (1.93; 95% confidence interval [CI], 1.35-2.76). Among the BI subgroups, the odds ratios of stage III-IV for the atherothrombotic type (1.81; 95% CI, 1.23-2.68) and the cardiogenic type (2.25; 95% CI, 1.32-3.83) were significantly higher than that of the control group, but that of stage III-IV for lacunar type was not (1.67; 95% CI, 0.98-2.84). Our results indicate that ischemic stroke is frequently associated with renal dysfunction. Chronic kidney disease might be independent risk factor for infarction, especially for cardiogenic and atherosclerotic types.

Case report: Corneal endothelial degeneration and optic atrophy in dentatorubral-pallidoluysian atrophy quantified by specular micrography and optical coherence tomography.

Introduction: Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disease with various neurological manifestations. Corneal endothelial degeneration and optic atrophy have been reported separately; however, there are no reports of corneal endothelial degeneration with optic atrophy. Cases: Herein, we present four related patients with DRPLA: two patients (69-year-old woman and 80-year-old man) who exhibited both corneal endothelial degeneration and optic atrophy and another two (49- and 51-year-old women, respectively) who exhibited only corneal endothelial degeneration. We quantified the reduction in corneal endothelial cell density (ECD) and hexagonality using specular microscopy and thinning of the circumpapillary retinal nerve fiber layer (RNFL) using optical coherence tomography (OCT). Conclusion: This is the first report of DRPLA accompanied by corneal endothelial degeneration and/or optic atrophy, which were both quantified based on the corneal ECD and the circumpapillary RNFL thickness using specular micrography and OCT, respectively. The pathophysiological mechanism is unclear; however, the involvement of the nuclear receptor TLX interacting with atrophin-1 may be implicated in ophthalmic manifestations of DRPLA. Therefore, we recommend performing specular micrography and/or OCT when patients with DRPLA experience visual disturbances.

Effect of edaravone on the estimated glomerular filtration rate in patients with acute ischemic stroke and chronic kidney disease.

The oxygen free radical scavenger edaravone is used in patients with acute ischemic stroke in Japan, but adverse reactions, especially decreased renal function, have raised concerns. To examine whether a patient's estimated glomerular filtration rate (eGFR) at admission can predict renal function deterioration after edaravone treatment, we retrospectively evaluated the effect of edaravone on eGFR in Japanese patients with acute ischemic stroke and chronic kidney disease (CKD). The baseline eGFR in the edaravone-treated group (73.5±20.3 mL/min/1.73 m(2); n=408) at admission was significantly (P < .05) higher than that in the non-edaravone-treated group (51.9±25.2 mL/min/1.73 m(2); n=41). The change in eGFR after treatment was categorized into 3 grades: nonexacerbation (≤10%), 10%-30% exacerbation, and >30% exacerbation. There was no significant difference in exacerbation grade between the edaravone-treated and non-edaravone-treated groups (χ(2) =3.134; P=.21). We next subdivided the edaravone-treated group according to eGFR at admission as either CKD (eGFR <60 mL/min/1.73 m(2); n=111) and non-CKD (n=297). No significant decrease in eGFR was seen even in the edaravone-treated CKD group (most of whom were in stage 3 CKD). Decreased eGFR in stroke patients was found to be associated with stroke subtype (cardiogenic stroke), but not with infection. The present study demonstrates that eGFR at admission is not a good predictor of renal deterioration in edavarone-treated acute ischemic stroke patients, including those with stage 3 CKD.

PARK2 Patient Presenting with Dopa-Responsive Dystonia.

We report a 34-year-old female PARK2 patient presenting with dopa-responsive dystonia (DRD). She noticed difficulty in raising her foot while walking at the age of 24. Her lower limb symptoms were identified as dystonia later, and she was started on Menesit, which resulted in improvement of her symptoms. She was diagnosed as DRD and has been on continuous treatment since then. The specific binding ratio (SBR) of 123I FP-CIT SPECT was significantly lower than those of controls of the same age, but 123I-meta-iodobenzylguanidine myocardial scintigraphy showed a normal heart to mediastinum ratio. The Montreal Cognitive Assessment, Japanese version, was normal for her age. DRD is an inherited dystonia that typically begins during childhood and may be caused by mutations of the GCH1 (GTP cyclohydrolase), SPR (sepiapterin reductase), or TH (tyrosine hydroxylase) genes. Our patient was diagnosed as PARK2, known as autosomal-recessive juvenile Parkinson's disease, based on genetic analysis. Although there was no family history of the disease, the decrease in SBR of 123I FP-CIT SPECT enabled us to diagnose PARK2 and to differentiate this from DRD due to other genetic disorders.

Unilateral Dorsolateral Medullary Hemorrhage Presenting with Severe Dysphagia.

We report an 87-year-old woman with right dorsolateral medullary hemorrhage. She did not show all of the usual symptoms of Wallenberg syndrome and her main symptom was severe dysphagia. Dorsolateral medullary hemorrhage may be overlooked, because it is rare and does not exhibit the typical Wallenberg syndrome presentation usually seen in patients with infarction at the dorsolateral medulla.

Levodopa-Responsive Primary Slow Orthostatic Tremor: A Premotor Sign of Parkinson's Disease?

We present a case of primary orthostatic tremor (OT) responsive to dopaminergic medication. The patient was a 62-year-old woman, who had leg tremor on standing for 2 years. No parkinsonian or other neurological signs were observed. Surface electromyography of the quadriceps muscles showed regular 5-6 Hz muscle discharges. [123I]-FP-CIT DAT-SPECT imaging revealed decreased specific binding ratio values in the striatum compared with age-matched controls. Her leg tremor almost completely disappeared following administration of levodopa 200 mg and pramipexole 0.75 mg. Since her OT with low-frequency discharge was responsive to dopaminergic medication, we speculate that it may be a premotor sign of Parkinson's disease.

The role of high-sensitivity C-reactive protein in asymptomatic intra- and extracranial large artery diseases.

BACKGROUND: It remains unclear whether high-sensitivity CRP (hs-CRP) is predictive of atherosclerosis in the intracranial artery. The aim of this study is to assess the role of hs-CRP in asymptomatic intracranial artery occlusive diseases. METHODS: Of the 3,366 apparently healthy subjects who received a brain checkup, 138 with > or =25% intracranial artery stenosis on magnetic resonance angiography, 267 with > or =25% extracranial carotid artery stenosis on B-mode ultrasonography and 435 without intracranial artery or extracranial carotid artery stenosis (age-matched controls) were selected for this study. RESULTS: The mean CRP concentration in the subjects with intracranial artery stenosis was not significantly different from that in the control subjects, and the differences of mean CRP concentrations among the subgroups with 25-49, 50-74 and 75% or greater stenosis in the intracranial artery were not significant. The odds ratios of hs-CRP for extracranial carotid artery stenosis tended to increase with increasing CRP concentrations, but those of hs-CRP for intracranial artery stenosis showed no significant difference. CONCLUSION: The degree of atherogenic inflammation in asymptomatic intracranial artery stenosis may be less than that in extracranial carotid artery stenosis.

[Patient with innominate artery steal accompanied with cerebral infarction].

A 49-year-old man suddenly suffered left hemiplegia, and was brought to our hospital by ambulance at the beginning of August, 2006. He had a history of hypertension, and had received replacement of a synthetic graft in the ascending aorta and aortic arch with innominate artery for dissecting aneurysm in the aorta 2 years before. On diffusion-weighted magnetic resonance images obtained after admission, cerebral infarction was detected at the right corona radiata, and MR angiography (MRA) showed obstruction of the right middle cerebral artery. He was given intravenous tissue-plasminogen activator (t-PA) a few hours after arrival, and his hemiplegia was improved on the following day. At 11 days after onset, recanalization of the right middle cerebral artery was seen by MRA. On Doppler ultrasonographic examination, obstruction and thrombus in the innominate artery were observed. Retrograde flow of the right vertebral artery was demonstrated by both pulse-Doppler ultrasonography and velocity-coded color MRA. This patient is a rare example of innominate artery steal and ischemic cerebrovascular disease with obstruction of the innominate artery. Cerebral infarction in this patient might have developed via artery-to-artery embolism, with the thrombus in the innominate artery, rather than through a hemodynamic mechanism with innominate artery steal.

Neurological safety of fingolimod: An updated review.

Fingolimod (FTY) is the first oral medication approved for treatment of relapsing-remitting multiple sclerosis (RRMS). Its effectiveness and safety were confirmed in several phase III clinical trials, but proper evaluation of safety in the real patient population requires long-term post-marketing monitoring. Since the approval of FTY for RRMS in Japan in 2011, it has been administered to approximately 5000 MS patients, and there have been side-effect reports from 1750 patients. Major events included infectious diseases, hepatobiliary disorders, nervous system disorders and cardiac disorders. In the present review, we focus especially on central nervous system adverse events. The topics covered are: (i) clinical utility of FTY; (ii) safety profile; (iii) post-marketing adverse events in Japan; (iv) white matter (tumefactive) lesions; (v) rebound after FTY withdrawal; (vi) relationship between FTY and progressive multifocal leukoencephalopathy; (vii) FTY and progressive multifocal leukoencephalopathy-related immune reconstitution inflammatory syndrome; and (viii) neuromyelitis optica and leukoencephalopathy.

123I-Meta-iodobenzylguanidine (MIBG) myocardial scintigraphy in patients showing scans without evidence of dopaminergic deficits (SWEDDs).

OBJECTIVE: Scans without evidence of dopaminergic deficits (SWEDDs) in dopamine transporter single-photon emission computed tomography (DAT-SPECT) are found in 3.6-19.6% of patients with clinically suspected Parkinson's disease (PD). We investigated whether combined use of 123I-meta-iodobenzylguanidine (MIBG) myocardial scintigraphy would be helpful to differentiate PD among SWEDDs patients. PATIENTS AND METHODS: 145 patients with clinically suspected PD underwent both DAT-SPECT and MIBG myocardial scintigraphy. Striatal binding ratio (SBR) of DAT-SPECT and heart-to-mediastinal (H/M) ratio and washout rate (WR) of MIBG myocardial scintigraphy were calculated. RESULTS: Among 18 SWEDDs patients (12.4%), 11 were finally diagnosed with PD based on follow-up for at least two years after the DAT-SPECT and MIGB myocardial scintigraphy scans. Among the latter group, 8 patients showed an H/M ratio of less than 2.2, and 9 showed WR above 30%. CONCLUSION: Our results indicate that the combination of low H/M ratio and high WR of MIBG myocardial scintigraphy of SWEDDs patients may be helpful for detection of PD patients.

Early Motor Fluctuations in a Patient with Striatonigral Degeneration.

We report a 44-year-old female with striatonigral degeneration (SND) who showed wearing-off oscillations after 4 months of levodopa treatment. The patient presented with asymmetric left-side dominant rigidity, and levodopa was effective at first. However, she began to show wearing-off oscillations of motor symptoms, which gradually worsened thereafter. Fluid-attenuated inversion recovery sequence magnetic resonance imaging (MRI) showed linear lateral putamen hyperintensities, and positron emission tomography (PET) studies using 18F-fluorodopa (FD) and 11C-N-methylspiperon (NMSP) showed a marked decrease of radioactivity in the right putamen, especially in the posterior putamen. The results of MRI and 2 PET studies with FD and NMSP were well consistent with the diagnosis of SND.