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Objective : Auditory P300 has been reported to be abnormal in demented patients with Parkinson's disease. However, it is still controversial which factors in Parkinson's disease influence P300 parameters. Methods : Forty patients with Parkinson's disease were included. Patients were divided into two groups: patients with dementia (PDD) and without dementia (PDND). An ‘odd-ball’ paradigm was used for auditory event-related potentials. Results : P300 latency was markedly delayed in PDD patients. Age and DRS1 (attention) were the most important factors influencing P300 latency. Conclusions : Although there have been reports of P300 in the past, its abnormalities reflect the deficit of attention in Parkinson's disease.

Objective We compared brain perfusion image using 3D-SSP analysis of 123I-IMP SPECT between Parkinson's disease patients with auditory verbal hallucination and those without auditory hallucination. Methods Eighty-three cases with Parkinson's disease were studied. In 6 of these patients, auditory hallucination was noted. Among them, four cases had verbal hallucination and two other cases had elementary hallucination. Auditory hallucination was not found in the other 77 cases. Results Right thalamic perfusion was significantly increased in the verbal hallucination group compared to the group that lacked auditory hallucination. Conclusion In Parkinson's disease, the right thalamic hyperactive state may be related to verbal hallucination.

The pathophysiology of depression and anxiety in Parkinson’s disease remains obscure. We aimed to compare the fractional anisotropy (FA) values of Parkinson’s disease (PD) patients with and without depression to investigate the nature of depression in PD. Twenty-eight patients were divided into two groups: those with depression and those without. Diagnosis of depression was made using the DSM-IV criteria. Patients in the two groups were matched for Hoehn Yahr stage. There were significant reductions in FA values in the bilateral frontal ROIs possibly representing anterior cingulate bundles. The anterior cingulate bundles play an important role in depression in PD, and some aspects of depression in PD have pathological processes in common with de novo depression.

Background Few imaging studies investigated frontal dysfunctions in Parkinson's disease. Objectives We investigated relationships between Wisconsin Card Sorting Test (WCST) and brain perfusion in patients with non-demented Parkinson's disease. Methods Patients were divided into two groups according to WCST score: (1) CA (number of category achieved)≤2 or (2) CA≥3. We performed three-dimensional stereotactic surface projection and volume of interest analysis using 123I-IMP scintigraphy. Results Hypoperfusions of the bilateral posterior cingulate, rostrodorsal prefrontal, and left frontopolar cortices were shown in CA≤2 group, with the left cingulate and right rostrodorsal prefrontal cortices showing prominent hypoperfusion. CA and PE (perseverative errors) scores significantly correlated with perfusion of the left posterior cingulate cortex. PE score also correlated with perfusion of the right rostrodorsal prefrontal cortex. Conclusion Data indicated participations of various regions in task achievement. Careful interpretation of WCST scores is required.

Background Although excessive daytime sleepiness (EDS) in Parkinson's disease (PD) has received a great deal of attention, the underlying pathological mechanism of its development and the relative contributions of brain function to this process are poorly understood. Objective To compare the fractional anisotropy (FA) values of PD patients with EDS and those without EDS, and to investigate the mechanism of EDS in this disease. Methods Eleven patients with PD with EDS, 26 patients with PD without EDS and 10 controls participated in this study. Patients with an ESS (Epworth Sleepiness Scale) score ≥10 were defined as having EDS. The FA values of 5 regions of interest (ROI) including the fornix were compared between the three groups. Results There were significant reductions in FA values of the fornix fiber in patients with EDS. ESS scores had significant correlation with FA values of the fornix. Conclusions Our study is the first to find the fornix fiber degeneration in PD patients with EDS. These results indicate that fornix dysfunction may have some correlations with EDS in PD.

Pathophysiology of hallucinations in Parkinson’s disease is poorly understood. This study investigated relationships between visual hallucinations and visual acuity. Twenty-six consecutive patients with Parkinson’s disease participated in this study. Patients were divided into two groups: patients with visual hallucinations (VH group) and those without visual hallucinations (no-VH group). Unaided and corrected eyesight was evaluated in all patients, and if frequent use of prescription glasses or contact lenses was involved, eyesight using these lenses was also measured as the patient’s own best eyesight. If a patient did not use prescription glasses or contact lenses, the patient’s own best eyesight was defined as the unaided eyesight. Multivariate regression analysis demonstrated that agonist use and best eyesight were different after the backward elimination method. Visual hallucinations were closely related not to uncorrected eyesight or unaided eyesight but to the patient’s best eyesight. It is suggested that impaired visual acuity is a risk factor for visual hallucinations.

Objective – To compare brain perfusion image using three-dimensional stereotactic surface projection (3D-SSP) analysis of N-isopropyl-p-123I iodoamphetamine (123I-IMP) single photon emission computed tomography (SPECT) between patients with Parkinson's disease with orthostatic hypotension and those without orthostatic hypotension. Materials and methods – Fifteen patients with Parkinson's disease and orthostatic hypotension and 13 patients with Parkinson's disease without orthostatic hypotension were studied. We compared brain perfusion image between the two groups by 3D-SSP. Results – Bilateral anterior cingulate gyrus perfusion of the patients with orthostatic hypotension was significantly decreased compared to that of the patients without orthostatic hypotension. Conclusions – The disorder of anterior cingulate gyrus may participate in the autonomic failure in Parkinson's disease.

Background: Decreased cardiac metaiodobenzylguanidine (MIBG) uptake was reported in Parkinson’s disease and this contributes to the differential diagnosis between Parkinson’s disease and other forms of parkinsonism such as multiple system atrophy. However, decreased MIBG uptake of the thyroid has not been demonstrated. Objective: To compare MIBG uptake of the thyroid among Parkinson’s disease, multiple system atrophy and controls. Methods: Twenty-six patients with Parkinson’s disease, 11 patients with multiple system atrophy and 14 controls were examined in this study. Planar images were taken 15 minutes (early images) and 3 hours (late images) after intravenous injection of 111 MBq 123 I-MIBG. Results: MIBG uptake of the thyroid on early images decreased significantly in Parkinson’s disease compared to controls (p < 0.0001) and multiple system atrophy (p = 0.018). MIBG uptake of the thyroid on early images decreased significantly also in multiple system atrophy compared to controls (p = 0.027). On late images, thyroid uptake differed significantly only between Parkinson’s disease and controls (p = 0.010). Conclusions: Our study is the first to demonstrate decreased MIBG uptake of the thyroid in Parkinson’s disease. Sympathetic nervous denervation of Parkinson’s disease occurred not only in the heart but also in the thyroid.

Objective: The pathophysiology of hallucinations in Parkinson disease is poorly understood. This study investigated the relation between visual hallucination and visual evoked potentials (VEPs) in Parkinson disease. Methods: Nineteen patients with Parkinson disease were studied. The authors divided patients into 2 groups: patients with visual hallucinations (VH group) and those without visual hallucinations (no-VH group). VEPs using a checkerboard stimulus were recorded under a drug-free state. Results: On multivariate regression analysis, only the average P100 latency was selected and remained significant after the backward elimination method. Conclusion: The authors demonstrated a close association between visual hallucinations and elongated VEP latency in Parkinson disease. VEPs may become one of the predictors for visual hallucination.

Background Intellectual deterioration occurs in 10-40% of patients with Parkinson’s disease. However, there are many conflicting studies on its relation with brain perfusion and the nature of this dementing process remains controversial.Objective To compare cortical perfusion by SPECT using 123I-IMP between Parkinson’s disease patients with dementia and those without dementia and to investigate the correlation between dementia in Parkinson’s disease and brain perfusion in various areas.Methods Fifty-two cases of Parkinson’s disease and 10 control cases were studied. The Parkinson’s disease with dementia group included 30 cases and the Parkinson’s disease without dementia group included 22 cases.,Results By multiple logistic regression method, we demonstrated significant hypoperfusion in the occipital cortex in Parkinson’s disease with dementia.Conclusions The cause of dementia in Parkinson’s disease may vary. We demonstrated that occipital hypoperfusion was closely correlated to dementia in Parkinson’s disease compared to frontal, parietal and temporal perfusion.

We analyzed the CD16+CD57– lymphocyte subset, which is considered to have strong natural killer (NK) cell activity, in peripheral blood from patients with chronic immune-mediated neuropathies and patients with other neurological diseases. We found that the ratio of CD16+CD57– NK cells to total lymphocytes was increased in 4 of 6 patients with multifocal motor neuropathy (MMN) with persistent conduction block. Since the CD16 molecule is an Fc receptor for immunoglobulin G (IgG), high-dose intravenous immunoglobulin (IVIg) may interfere with CD16+CD57– NK cells via Fc receptor blockade. In addition, cyclophosphamide (Cy) is often used to suppress NK cells. Therefore, our findings may partly account for the effectiveness of IVIg or Cy, which is the current treatment of choice for MMN.

Objective : To investigate the physiological abnormality in writer's cramp, a focal dystonia which specifically affects writing. Methods : We recorded brain potentials that precede hand and neck movements (contingent negative variation or CNV) in 11 patients and 11 age-matched normal subjects. A 1000 Hz tone burst (S1) was delivered to the right or left ear in random sequence, and 2 s after, a 2000 Hz tone burst (S2) was delivered to both ears simultaneously. For the response task to S2, the subjects were instructed to extend their fingers ipsilateral to the ear to which S1 was given in one experiment or to rotate the head to the side of the S1 presentation in another. All the patients had symptoms in the right hand only, and performed both tasks normally. CNV amplitudes were compared between normals and patients using unpaired t test. Results : They showed normal CNV for neck movement but significantly decreased CNV amplitudes for movements both in the affected and unaffected hands. Conclusions : Our findings suggest that motor programming is specifically abnormal for the affected body part, including the asymptomatic contralateral limb, and that the clinical symptom may result from a deficient compensatory mechanism for abnormal motor programs or subroutines.

慢性炎症性脱髄性ポリニューロパチーは慢性の経過をとる運動知覚性ニューロパチーの一群で,近位部優位の末梢神経系有髄線維の障害による.診断は,臨床的・病理学的所見や脳脊髄液所見より行われるが,特に電気生理学的所見が重要である.治療は,副腎皮質ステロイドホルモン療法, azathioprine・cyclophosphamideなどの免疫抑制療法,血漿分離療法が一般的であるが,最近免疫グロブリン療法が注目されている.

A 27-year-old woman was admitted to our hospital with tetraparesis, dysesthesia and hypoesthesia of all regions below the breasts, urinary disturbance, and difficulty in breathing. Since age 21 idiopathic thrombocytopenic purpura (ITP) was diagnosed and steroid therapy was continued. At age 26, she had splenectomy for her ITP. On admission, steroid pulse therapy was administered with a tentative diagnosis of transverse myelitis. Symptoms gradually ameliorated. At age 29, she gradually lost her left vision, and multiple sclerosis was diagnosed and steroid therapy was administered, and her left vision gradually ameliorated. There are several reports describing other autoimmune disorders that arise after splenectomy. Since the spleen acts as a major pool of type 2 helper T cells, it is plausible that peripheral type 1 helper T cell activity may increase after splenectomy, promoting the development of autoimmune disorders. We considered there would be a close relation between splenectomy for ITP and multiple sclerosis in this case.

Patients with focal dystonia take advantage of certain cutaneous or proprioceptive sensory inputs to alleviate their symptoms ("sensory trick"). We examined the effects of increasing muscle spindle activity by the tonic vibration reflex maneuver and decreasing it by intramuscular injection of lidocaine. The vibration was applied to the palm or the tendon of forearm muscles in 15 patients with writer's cramp and 15 age-matched normal subjects. In 11 patients, the vibration induced dystonic postures or movements typical of those seen during writing. Normal subjects showed either no response to the vibration or a gradually developing tonic vibration reflex only in the wrist and finger flexors, which produced visible movements with a significantly longer latency (12.5 +/- 6.7 seconds [mean +/- standard deviation]) than what was observed in the patients (2.7 +/- 2.5 seconds, p < 0.0001). Local injection of lidocaine (0.5%, 5-40 ml/muscle) attenuated the tendon reflex with relatively little effect on the M response. Injection into muscles with increased activity produced marked reduction of dystonic movements and significant clinical improvement in 13 patients, whereas injection into the other muscles had no effect. The clinical benefit lasted for 1 to 24 hours after injection. In 13 patients who had additional injections of 10% ethanol, which blocks sodium channels for a longer period than does lidocaine, the duration of action was prolonged to 5 to 21 days. These findings suggest that muscles causing dystonic movements have abnormal sensitivities to vibration at rest and that muscle afferents may play a pivotal role in producing dystonic movements.(ABSTRACT TRUNCATED AT 250 WORDS)

To investigate the pathophysiology of dystonia, we recorded contingent negative variation (CNV) in 12 patients with cervical dystonia and in 12 age-matched normal subjects. In a simple reaction time paradigm, the subjects were given a pair of a warning stimulus and a subsequent stimulus that triggered head rotation to either side or extension of the fingers. In normal subjects, CNVs for head rotation were not affected by neck muscle pre-activation simulating torticollis, and were always symmetrical with equally high amplitudes over the frontal and central leads. By contrast, CNVs for finger movement had the maximum over the central lead and showed a characteristic distribution; those for the right finger movement had the left hemisphere dominance, whereas those for the left finger movement had similar amplitudes over both hemispheres. In patients with rotatory torticollis (rotatocollis), the components of CNVs for head rotation were markedly attenuated in all the leads, except for the initial negative deflection (orienting response). As a whole, cervical dystonia patients had significantly lower amplitudes of late CNVs for head rotation than normal subjects (P < 0.001), whereas late CNV amplitudes in finger extension did not differ in the two groups. Their reaction times for head rotation were similar, but durations of EMG activities were prolonged in the patients because of co-contractions of the antagonists. The task-specific CNV amplitude loss is therefore not explained by reaction times or by the abnormal neck muscle activities prior to the movement, but it reflects a failure of neural activities preparing for a phasic neck movement, resulting in co-contraction of the agonists and the antagonist. Dystonia may be associated with defective retrieval or retaining of specific motor programmes or subroutines in response to sensory stimuli.

Lymphocytic hypophysitis (LYH) is a rare neuroendocrine disorder characterized by autoimmune inflammation of the pituitary gland. Visual disturbance is one of the most common and serious symptoms of LYH. Most of the visual symptoms in LYH are secondary to compression of the optic chiasm and some reports have described direct inflammatory involvement of the optic pathways. We describe a 30-year-old man with a 9-day history of bilateral blurred vision. Ophthalmic examination demonstrated severely impaired vision without temporal hemianopsia. Hypothyroidism, hypocortisolism, and hypogonadism were detected in laboratory tests. Central diabetes insipidus was diagnosed by a hypertonic saline infusion test. MRI revealed thickening of the pituitary stalk and enlargement of the hypophysis, which was enhanced with gadolinium. High intensity of the posterior lobe was not recognized on T1-weighted images. These findings established a clinical diagnosis of lymphocytic panhypophysitis. Methylprednisolone pulse therapy was introduced and his visual acuity gradually recovered. The anterior pituitary function improved, but desmopressin was still required. Pattern-reversal visual evoked potentials (VEP) have been widely used to detect optic nerve lesions caused by multiple sclerosis and brain tumors. However, there have been no previous reports of their usefulness for LYH. The P100 latency in our case was slightly prolonged and the amplitude was markedly reduced. These findings are similar to ischemic optic neuropathy and other conditions in which axonal damage is prominent. The prolonged latency and low amplitude on VEP examination in this case showed rapid improvement in parallel with the recovery of visual acuity. Taken together, our case implies the usefulness of pattern-reversal VEP for the diagnosis of optic neuritis in LYH, especially for the evaluation of its pathogenic mechanisms.

Objective – Dementia occurs frequently in patients with Parkinson’s disease (PD). However, the nature of the dementing process remains controversial. We evaluated various cognitive functions in patients with PD, compared fractional anisotropy (FA) values between PD patients with and without dementia. Methods – Thirty-seven consecutive patients with Hoehn-Yahr stage III or IV PD participated in this study. Patients were divided into two groups: (i) PD with dementia group (PDD) and (ii) PD without dementia group (PDND). There were 11 PDD and 26 PDND cases. Ten controls were also studied. Results – The PDD group showed significant FA reduction in the bilateral posterior cingulate bundles compared with PDND. FA values in the left posterior cingulate bundle showed significant correlations with many cognitive parameters. Interpretation – Our results showed that the posterior cingulate areas play some important roles in the dementing process in PDD. However, as the pathological processes responsible for dementia in PD patients may be multifaceted, further studies are necessary.

Introduction – It is generally assumed that executive dysfunctions in Parkinson's disease (PD) are caused by degeneration of the basal ganglia or frontal cortex or both. However, there have been few studies investigating the relationship between executive dysfunctions and cerebral pathological change. The objective of this study was to evaluate various cognitive functions in non-demented patients with PD, and to compare the fractional anisotropy (FA) values of PD patients with and without executive dysfunction. Materials and Methods – Twenty-one consecutive non-demented patients with PD were enrolled in this study. Patients were divided into two groups on the basis of their Wisconsin Card Sorting Test score. Results – There was significant FA reduction in the left parietal white matter in the group in which the number of categories achieved was ≤2 relative to the group that achieved >2. Conclusion – Accumulating evidence suggests that conventional ‘frontal’ tasks correlate with both frontal lobe and parietal lobe function, and we suggest that pathological changes in the left parietal lobe may cause, in part, disturbances in executive tasks in PD.

Thrombosis involving a brain infarction frequently occurs in patients with a malignant tumor. Although nearly all types of tumor have been reported in association with a hypercoagulable state, pleural mesothelioma-associated Trousseau syndrome is extremely rare.A 69-year-old female was admitted to our hospital with cough, sputum, and breathing difficulties. She was diagnosed as having a mesothelioma from a percutaneous pleural biopsy. Although there were no risk factors for atherosclerosis, brain infarctions showed frequent relapses, even under anticoagulant therapy, and there was a marked hypercoagulable state.Attention should be paid to this syndrome when unexplained brain infarctions occur in patients with pleural mesothelioma.

We examined patients with and without auditory hallucinations, using n-isopropyl-p-[123I]iodoamphetamine single photon emission computed tomographic imaging. We assessed verbal hallucinations in the present study: patients with nonverbal auditory hallucinations were excluded. A total of 11 patients with verbal and visual hallucinations and 17 patients with visual hallucinations only were enrolled. Patients with both verbal and visual hallucinations revealed significant hypoperfusion in the bilateral prefrontal cortex and right superior temporal gyrus compared to patients with visual hallucinations only. There were no significant hyperperfusion in patients with verbal plus visual hallucinations. These results may support the release hallucination theory in verbal hallucinations of Parkinson's disease, although another explanations may be more appropriate and further studies are required.

STUDY OBJECTIVES The underlying pathologic mechanism of excessive daytime sleepiness (EDS) in Parkinson disease and the relative contributions of brain function to this process are poorly understood. We compared brain perfusion images between patients with Parkinson disease and EDS and those without EDS using n-isopropyl-p-1231 iodoamphetamine single photon emission computed tomography. DESIGN Clinical study. SETTING Sumitomo Hospital. PATIENTS Thirteen patients with Parkinson disease with EDS (EDS group) and 27 patients with Parkinson disease without EDS (no-EDS group) were studied. Whether or not each case had EDS was determined according to the response to the Epworth Sleepiness Scale: patients with an Epworth Sleepiness Scale score > or = 10 were included in the EDS group, and patients with an Epworth Sleepiness Scale score < or = 9 were included in the no-EDS group. MEASUREMENTS AND RESULTS There were significant hypoperfusions in the left parietal and temporal association cortex in the EDS group. In the multivariable logistic regression model, attention and decreased regional cerebral blood flow of the left parietal association cortex and right caudate and increased regional cerebral blood flow of the right thalamus were the independent and significant factors. CONCLUSIONS The cortical hypofunction relative to hyperfunction of the brain stem may relate to EDS in Parkinson disease. This is the first imaging study about EDS in Parkinson disease, and further studies are required.

Background Nature of the dementing process in Parkinson's disease, and particularly its relationship with Alzheimer's disease, diffuse Lewy body disease or frontal dementia remains controversial. Objective We hypothesize that origins of dementia in Parkinson's disease are heterogeneous, so we compared cortical regional cerebral blood flow (rCBF) between Parkinson's disease patients with and without dementia. Patients Forty consecutive patients with Hoehn-Yahr stage III or IV Parkinson's disease were used (13 patients had dementia (PDD group), and 27 patients had no dementia (PDND group)). Results There were significant rCBF reductions in the left parietal association cortex and left frontal association cortex in PDD. Multiple logistic regression analysis demonstrated that only rCBF of the left frontal association cortex was significant. PDD patients were divided into three groups according to rCBF patterns: frontal hypoperfusion group, Alzheimer's disease-like group, and diffuse Lewy body disease-like group. Conclusions Controversial study results involving PDD patients may be mainly due to heterogeneity in dementing processes in Parkinson's disease.

Depression is common in individuals with Parkinson's disease. However, the pathophysiology of depression in Parkinson's disease remains obscure. Here we compared brain perfusion images of Parkinson's disease patients with and without depression to investigate correlations between depression and brain perfusion images in Parkinson's disease. We divided 40 consecutive patients with Parkinson's disease into two groups: patients with minor depression (n = 22) and patients without depression (n = 18). We then compared brain perfusion images between the two groups. As a result, hypoperfusion of the left superior and inferior frontal gyrus was demonstrated in depressed patients. These results were partially in agreement with previous studies on de novo and parkinsonian major depression. We could not conclude on whether pathophysiological mechanisms differed between de novo depression and depression with Parkinson's disease, and between major and minor depressions.

We described a 69-year-old woman with neurological manifestations due to mixed connective tissue disease (MCTD). The patient demonstrated subacute cognitive decline, seizure and gait disturbance with no connective tissue manifestation. She had been diagnosed with dementia at another hospital, later in our hospital, serological examinations disclosed high titers of anti-RNP antibody. Cognitive dysfunction in this patient was dramatically ameliorated by steroid therapy. Three months later, she developed edema of the hands, synovitis and acrosclerosis. The patient was finally diagnosed as having MCTD. We emphasized MCTD as a rare cause of "treatable dementia".

Background Peripheral neuropathy with adult T-cell leukemia/lymphoma (ATLL) has seldom been reported. Review summary A 69-year-old woman with smoldering-type ATLL presented with pain and muscle weakness of the bilateral upper and lower limbs and gait disturbance. Anti-human T lymphotropic virus type I antibody was positive in the serum but negative in the cerebrospinal fluid. Magnetic resonance imaging (MRI) showed no abnormal signals in the spinal cord or brain. Nerve conduction studies disclosed severe peripheral neuropathy. Sural nerve biopsy disclosed both axonal degeneration and demyelinated fibers, and marked perivascular inflammatory infiltrates mainly consisting of T lymphocytes without malignant changes were seen. Steroid therapy was markedly effective, and the patient became able to walk without assistance. Conclusions This is the first reported case of ATLL and peripheral neuropathy in which a nerve biopsy was performed. In our patient, the marked perivascular inflammatory infiltrates mainly consisting of T lymphocytes suggested either immune-mediated vasculitis or tumor invasion. Further studies are needed.

The objective was to compare brain perfusion image using 3-dimensional stereotactic surface projection analysis of N-isopropyl-p-123I iodoamphetamine single photon emission computed tomography between Parkinson’s disease patients with a high frontal assessment battery score and those with a low frontal assessment battery score. Thirty nondemented patients with Parkinson’s disease were studied. Patients were divided into 2 groups: a high-scoring group whose frontal assessment battery score was 12 or more and a low-scoring group whose frontal assessment battery score was 11 or less. The high-scoring group included 21 patients, and the low-scoring group included 9 patients. They underwent N-isopropyl-p-123I iodoamphetamine single photon emission computed tomography, and we analyzed the data by the 3-dimensional stereotactic surface projection method. Results showed that left inferior parietal lobule and left supramarginal gyrus perfusion of the low-scoring group were significantly decreased compared with the high-scoring group. It is concluded that_patients with Parkinson’s disease may have frontal lobe dysfunction, but the decreased frontal assessment battery score may be caused not by progressed frontal lobe dysfunction but by parietal lobe dysfunction added to their preexisting frontal lobe impairment.

Objectives To compare MIBG (metaiodobenzylguanidine) uptake between Parkinson's disease patients with and without autonomic symptoms to investigate meanings of MIBG scintigraphy and correlations between autonomic symptoms and MIBG uptake. Methods Forty consecutive patients with Hoehn–Yahr III or IV Parkinson's disease and 12 controls were enrolled. We compared cardiac MIBG uptake between patients with and without orthostatic hypotension. Similar comparisons were performed for constipation and bladder dysfunction. Results MIBG uptake did not significantly correlate with age, disease duration, Hoehn–Yahr stage, Unified Parkinson's Disease Rating Scale (UPDRS) motor score or levodopa equivalent daily doses. There were no significant correlations between orthostatic hypotension/constipation and cardiac MIBG uptake. Only bladder symptoms significantly correlated with MIBG uptake. Conclusion Meanings of MIBG uptake abnormalities in Parkinson's disease and relationship between MIBG uptake and clinical parameters remain to be resolved in future studies.

Gait disturbance is a cardinal symptom in patients with Parkinson's disease. Among the gait disturbances, freezing of gait is a unique and troublesome symptom, but its mechanism is unclear. We compared brain perfusion images using three-dimensional stereotactic surface projection analysis of N-isopropyl-p-123I iodoamphetamine single photon emission computed tomography between Parkinson's disease patients with freezing of gait and those without. Twenty-four cases (freezing of gait group) with Parkinson's disease with freezing of gait, and 31 Hoehn and Yahr stage-matched cases (no freezing of gait group) with Parkinson's disease without freezing of gait were studied. Bilateral Brodmann area 11 perfusion of the freezing of gait group decreased significantly compared to that of the no freezing of gait group. The Brodmann area 11 may play important roles in gait, and impairment in this region may have a close relationship with freezing of gait in Parkinson's disease. © 2005 Movement Disorder Society

We investigated the clinical efficacy and tolerability of 45 mg/day mosapride, a selective 5-hydroxytryptamine type 4 (5-HT4) agonist, in an open-label study involving five patients with Parkinson's disease (PD) who had response fluctuations (RFs). 'On' time and motor function scores were determined, and gastric motility was measured by a radionuclide gastric emptying (GE) test, the most reliable quantitative method available. We found that mosapride therapy significantly shortened GE half-time, reduced RFs, and improved motor functions in all patients. There were no adverse reactions. We conclude that selective 5-HT4 agonist therapy is beneficial for patients with PD who have RFs.

We compared MIBG uptake at various parts of the body in controls and patients with Parkinson's disease and multiple system atrophy. In the heart, MIBG uptake in Parkinson's disease (early H/M: 1.668+/-0.325, late H/M: 1.500+/-0.402) was less than that in multiple system atrophy (early H/M: 2.395+/-0.186, late H/M: 2.530+/-0.391) and controls (early H/M: 2.635+/-0.508, late H/M: 2.575+/-0.635) (early: P0.0001, late: P0.0001). There were no significant differences in uptake by the lung, thyroid, or liver in the three groups. Only on early images, uptake in the shoulder in multiple system atrophy (early S/M: 0.473+/-0.78) and Parkinson's disease (early S/M: 0.470+/-0.710) was decreased compared to that in controls (early S/M: 0.560+/-0.118) (P=0.0252). MIBG is reported to be taken up in the terminal part of sympathetic nerves and demonstrates sympathetic nerve activity, especially on late images. The cause of differences between the heart and other parts of the body remains unknown. We consider the following possibilities: (a) differences in the sympathetic nervous system between Parkinson's disease and multiple system atrophy are more subtle in organs other than the heart; (b) the cause of MIBG uptake reduction by the heart in Parkinson's disease involves factors in addition to sympathetic nervous system damage; and (c) MIBG uptake by organs other than the heart involves not only the sympathetic nervous system but also non-neuronal components. In conclusion, MIBG uptake by organs other than the heart cannot differentiate Parkinson's disease from multiple system atrophy at present.

Background The patterns of regional cerebral blood flow in Parkinson's disease and multiple system atrophy remain inconsistent. Objectives To compare brain perfusion images of 123 I-IMP SPECT between Parkinson's disease, multiple system atrophy with predominant parkinsonian features (MSA-P) and controls. Methods Eighty-two patients with Parkinson's disease, 10 patients with MSA-P and 14 controls were studied. We performed 3D-SSP and volume of interest analysis using 123 I-IMP scintigraphy. Results Occipital perfusion of MSA-P increased compared to that of Parkinson's disease and perfusion in the cerebellum and primary sensorimotor cortex of Parkinson's disease increased compared to that of MSA-P. Perfusion in the putamen of MSA-P decreased compared to that of Parkinson's disease. Conclusion Our study demonstrated perfusion differences in 123 I-IMP SPECT between the two diseases.

A 50-year-old man who first noticed shaking of the hands at age of 18 and had previous history of three convulsive episodes was admitted to our department because of the generalized tonic seizure with loss of consciousness. Family history showed that 7 members had similar shaking and one had generalized seizure. On neurological examination, rhythmic myoclonic jerks were noted in the bilateral upper limbs, and was remarkably more severe on the left. Other neurological findings were normal. Based on the benign clinical course, familial history, and the normal range of pyruvate and lactate levels in serum and cerebrospinal fluid, we diagnosed familial essential myoclonus and epilepsy (FEME)/benign adult familial myoclonic and epilepsy (BAFME). After admission, we performed electrophysiological test. EEG showed spike discharge mainly in the left parietooccipital lobe. Jerk-locked back averaging (JLA) showed spikes 30 msec precedings the trigger in the left parietooccipital lobe. Because JLA spikes are commonly found 20 msec precedings the trigger, we consider the subtraction time of 10 msec was due to the conduction through the corpus callosum. Some reports showed that in progressive myoclonus epilepsy patients, transcallosal excitation of one hemisphere by the other was critical for generalized seizure. In cases of FEME/BAFME, epileptic focus in the hemisphere ipsilateral to the myoclonus limbs has not previously reported. Our case suggested that generalization of the seizure was caused by the interhemispheric excitation through the corpus callosum.

We report two cases of Parkinson's disease in which visual hallucinations disappeared after cataract surgery. Patient 1 was a 72-year-old woman with Parkinson's disease, visual hallucinations and musical hallucinations. Patient 2 was a 77-year-old woman with Parkinson's disease and visual hallucinations. Both patients had severe bilateral cataracts. Though it was difficult to control their visual hallucinations with medication only, cataract surgery made them disappeared quickly. The visual hallucinations of Parkinson's disease are similar to those of Charles Bonnet syndrome. For example, both hallucinations often happen in dim light, at night and when patients are awake with eyes open. Though there have been many reports describing visual hallucinations in Parkinson's disease, there have been few reports discussing the relation between these hallucinations and impaired visual acuity. Similar to the hallucinations of Charles Bonnet syndrome, impaired visual acuity should be related to the visual hallucinations of Parkinson's disease. When Parkinson's disease, visual hallucinations and severe cataract coexist, visual hallucinations may disappear after cataract surgery.

We report a 65-year-old woman with sudden onset of paresthesia on the left side of the lip and left thumb. Neurological examinations did not demonstrate any disturbance of higher brain function or motor function except for subjective thermohyperesthesia of the left thumb. Brain MRI demonstrated a small high intensity lesion on T2-weighted images (T2 WI) and diffusion-weighted images (DWI) in the right anterior precentral gyrus. She was diagnosed with cerebral infarction presenting with pure cheiro-oral syndrome (COS). Her neurological symptoms were completely abolished 4 or 5 days later. The thalamus is the region responsible for COS in the majority of cases while reports that a cortical infarction causes COS are rare, because it is difficult to show a small cortical lesion on CT or conventional T2WI. DWI is superior to T2 WI in discriminating between acute and chronic ischemic lesions as well as in detecting small cortical lesions adjacent to the cerebrospinal fluid. In this patient, the lesion was vaguely hyperintense on T2 WI and difficult to differentiate from an artifact caused by cerebrospinal fluid. The precentral gyrus infarction detected on DWI was thought to cause COS in this patient. Not only lesions of the postcentral gyrus as a primary sensory cortex but also those of the precentral gyrus or opercula causing COS were described in previous reports, indicating that the hand and mouth sensory areas may be widely distributed. Cortical mapping studied by electrical stimulation through subdural grid electrodes also supports this finding. Further examination of the relation between neurological symptoms and localization on MR images such as DWI is needed to clarify the distribution of the sensory cortex.

Type A botulinum toxin has widened its clinical range of applications, but the risk of developing antibodies limits the repeated use of high-dose injection. To minimize the risk, mixing different types of toxin might reduce the antigenic presentation of a specific toxin and associated proteins. At the same time, inhibition of the neuromuscular release process at the multiple sites might potentiate the clinical response or the duration of action. We compared the effectiveness of a mixture of type A and type F botulinum toxins with that of type A or type F toxin alone for treating patients with blepharospasm in a double-blind study. Fifty-four patients had 10 units of toxin injection, a mixture of type A and F toxins (including 5 units of each) on one side and either type A or F toxin on the other side of the orbicularis oculi muscle. Clinical evaluation at 4 and 10 weeks after the injection revealed that the peak clinical effect at 4 weeks was similar among the three preparations. The duration of action of the mixture was intermediate between type A and type F alone, as assessed at 10 weeks, when there was a tendency of conserving the beneficial effect on one eye at the expense of that on the other. Although there was no apparent potentiation of the clinical efficacy, the combination of these different types of toxin might be used for decreasing the risk of antibody development.

In order to clarify the abnormality in cortical motor preparation for voluntary muscle relaxation of the hand in patients with focal hand dystonia, Bereitschaftspotentials (BPs) preceding voluntary muscle contraction and relaxation were recorded in eight patients (three with simple writer's cramp and five with dystonic writer's cramp), and were compared with those from 10 normal subjects. Voluntary muscle relaxation: after keeping the right wrist in an extended position for > 5 s, the subject let the hand drop by voluntarily terminating muscle contraction of the wrist extensor without any associated muscle contraction. Voluntary muscle contraction: the right wrist was flexed by voluntarily contracting the wrist flexor muscle. Scalp EEGs were recorded from 11 electrodes placed over the frontal, central and parietal areas. In the control group, the BP measured at the movement onset was maximal at the left central area (C1), and distributed predominantly over the left hemisphere equally in both the contraction and relaxation tasks. In the focal hand dystonia group, BP was maximal at C1 in the contraction task, whereas, in the relaxation task, it was maximal at the midline central area (Cz) and symmetrically distributed. At the left central area, the BP amplitude in the focal hand dystonia group was diminished significantly in the relaxation task compared with the contraction task (P < 0.05). The present results demonstrate for the first time that the cortical preparatory process for voluntary muscle relaxation, or motor inhibition, is abnormal in focal hand dystonia.

POEMS syndrome is a syndrome that presents with polyneuropathy, organomegaly, endocrinopathy, M-proteins and skin changes. Elevated vascular endothelial growth factor (VEGF) has recently been reported in POEMS syndrome. We report a case of POEMS syndrome with high VEGF titers. Steroid, plasmapheresis and intravenous gamma-globulin had little effect. Various immunosuppressive agents were discontinued due to side effects. Although administration of aspirin did not decrease VEGF, ticlopidine decreased VEGF significantly. This case suggests that ticlopidine is a candidate for supportive therapy in POEMS syndrome and we should measure VEGF before and after the administration of ticlopidine in other cases.

Catalepsy is a generalized condition of diminished responsiveness shown by a trance-like state, posturing, or maintenance of physical positions for a prolonged period of time. It is an internal reaction to escape from stress and one of the characteristic symptoms of catatonia. Interactions among various neurotransmitters such as dopamine, serotonin, and γ-aminobutyric acid (GABA) are involved in the onset of catalepsy, but the mechanism has not yet been completely elucidated. An 87-year-old man with Parkinson's disease on l- dopa therapy showed catalepsy. Eight years after the onset, he developed episodes of akinetic mutism with catalepsy, which gradually became prolonged. He became almost unable to take oral medication, so was admitted to our department, where intravenous l -dopa injection therapy was started. At 20 min after l -dopa injection, catalepsy was evoked. Suspecting that l -dopa was involved in catalepsy, we reduced the dose. With a reduction in l -dopa, the frequency of akinetic mutism with catalepsy decreased. In addition, single-photon emission computed tomography (SPECT) with three-dimensional stereotactic surface projection (3D-SSP) demonstrated decreased cerebral blood perfusion in the prefrontal cortex and increased perfusion in the motor cortex. Some reports have shown that stress can increase dopamine release in the medial prefrontal cortex, and excessive dopamine causes dopamine receptor down-regulation. In this case, besides chronic stress, l -dopa was a factor in causing excessive dopamine in the prefrontal cortex. Moreover, increased perfusion in the motor cortex is suspected of being associated with continuous movement. Some reports have shown that various modulations originate in the prefrontal cortex and affect the motor area, and that disturbance of such modulation is involved in the onset of catalepsy. In conclusion, we speculate that stress and l -dopa caused the down-regulation of dopamine receptors in the prefrontal cortex and subsequent dysfunction of this area. As a result, modulation originating in the prefrontal cortex and affecting the motor area did not function appropriately.