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PubMed1995

[Autonomic failure as the cause of vertigo and syncope].

BACKGROUND: Vertigo and syncope often occur during orthostatic conditions. A possible cause is asympathicotonic dysregulation with a fall of blood pressure and no increase in heart rate. After the exclusion of secondary disturbances of autonomic innervation. g. in diabetes mellitus, and pharmacological side effects, e.g. tricyclic antidepressants, primary reasons must be considered. We present 2 cases of primary autonomic failure. PATIENTS: Patient 1 had Parkinson-syndrome, impaired bladder function and orthostatic dysregulation with a maximal drop of blood pressure to 60/40 mm Hg. Patient 2 showed orthostatic dysregulation with a minimal blood pressure of 70/30 mm Hg without further symptoms, in a tilt table examination catecholamines were measured during orthostatic conditions. Patient 1 had normal, and patient 2 very low resting catecholamine plasma levels. Neither patient reacted with a rise of plasma catecholamines when tilted to an upright position. These findings are diagnostic of multiple system atrophy (MSA) in patient 1 and pure autonomic failure (PAF) in patient 2. DISCUSSION: In PAP only dysautonomic symptoms are found. It has a favourable prognosis. MSA consists of an autonomic dysregulation in combination with parkinsonism, cerebellary and bulbar symptoms, prognosis quo ad vitam is poor. Diagnosis is often established by a tilt table examination and radiology findings, and is often late. Treatment of both diseases consists of pharmacological, e.g. fludrocortisone, vasopressin analogues and ergotamine tartrate as well as non-pharmacological blood pressure stabilizing measures.

Adult↗

PubMed1995

Immunocytochemical characterization of glial fibrillary tangles in Alzheimer's disease brain.

Neurofibrillary tangle is a major cytoskeletal pathology in Alzheimer's disease brains, and has been considered to develop exclusively in neuronal cells. We examined brains with Alzheimer's disease and observed argyrophilic fibrillary tangles not only in cortical neurons but also in subcortical glial cells in the frontal and temporal white matter. The tangles in glial cells were immunolabeled by antibodies against tau and ubiquitin, and double immunocytochemistry analyzed by confocal laser scanning microscopy demonstrated that the cytoplasms of tangle-bearing glia were labeled by antibodies against transferrin and 2'3'-cyclic nucleotide 3'-phosphohydrolase. Ultrastructurally, they were made up of bundles of straight filaments 16 nm in diameter and constricted filaments. These results indicate that fibrillary tangles resembling neurofibrillary tangles may develop in oligodendrocytes in brains with Alzheimer's disease and are distinguishable from glial cytoplasmic inclusions observed in multiple system atrophy brains. We referred to them as glial fibrillary tangles. Glial fibrillary tangles commonly occurred in this disease condition, and glial cells might be involved under the pathological processes similar to neuronal cells.

Aged↗

PubMed1994

[Ambulatory measurement of blood pressure and plasma catecholamines in the study of orthostatic hypotension].

The purpose of the study was to investigate the interest of ambulatory blood pressure (BP) monitoring (ABPM) and plasma catecholamines in the management of orthostatic hypotension (OH). Fifteen patients, 4 men, 11 females, 53.3 +/- 21.1 years old, with OH were included in the study: 7 with dysautonomia (G1) = multiple systemic atrophy, pure autonomic failure, OH in elderly people; 8 with OH from other origin (G2) = hypovolemia, neurodystonia, vaso-vagal syncope. ABPM and plasma catecholamines assays (HPLC) in lying then in standing position were carried out in all patients. BP was 131.2 +/- 31.9/78.1 +/- 12.0 mmHg in lying and 112.1 +/- 25.3/75.4 +/- 15.8 in standing position (n = 15). The systolic (S) standing-induced (delta) decrease in BP after 1 min and 10 min (delta SBP) correlated with standard-deviation and variation coefficient of mean SBP (r = 0.78, p < 0.01; r = 0.82, p < 0.01 for delta sBP 1 min and r = 0.80, p < 0.01; r = 0.81, p < 0.01 for delta sBP 10 min), but not with norepinephrine (NorE) or epinephrine levels. There was a significant correlation between diastolic nycthemeral BP variability expressed by mean night-time DBP/mean day-time DBP ratio/diastolic N/D) and standing-induced decrease in DBP after 1 min (r = 0.59, p < 0.05). delta SBP 1 min and 10 min, delta DBP 1 min (p = 0.02, p = 0.05, p = 0.01) and systolic and diastolic N/D (p = 0.02; p < 0.01) were significantly different in G1 and G2.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1995

Iodine-123-IBF SPECT evaluation of extrapyramidal diseases.

UNLABELLED: Iodine-123-IBF is a dopaminergic antagonist suitable for SPECT imaging of D2 receptors. Initial animal studies demonstrated that its affinity for D2 receptors is approximately four times that of the commonly used SPECT D2 ligand [123I]IBZM. In this study we investigated whether this higher affinity would lead to an improved accuracy in differentiating between various extrapyramidal diseases. METHODS: SPECT imaging was performed in 17 patients with idiopathic Parkinson's syndrome (IPS); 4 patients with progressive supranuclear palsy (PSP), 2 patients with multiple system atrophy (MSA) and 7 age-matched control subjects. SPECT imaging was performed 5, 60, 120 and 180 min following intravenous bolus injection of 150-250 MBq of [123I]IBF. The ratio of ligand uptake in the basal ganglia and frontal cortex was determined as a measure of receptor status. RESULTS: In PSP and MSA patients, the basal ganglia-to-frontal cortex ratio reached a plateau at 2 hr; in the control subjects and the IPS patients the ratio was steadily increasing. At 3 hr the basal ganglia-to-frontal cortex ratio was 2.66 +/- 0.29 (control subjects), 3.01 +/- 0.41 (IPS), 2.09 +/- 0.22 (PSP) and 2.10 (MSA). In the IPS patients with predominantly one-sided symptoms, the striatum contralateral to symptoms showed a tendency towards relatively increased ligand uptake. Despite the higher affinity of IBF for the D2 receptor compared to IBZM, the separation of individual PSP and MSA patients from the control subjects was not as clear cut as reported for IBZM due to a relatively high variation in the control subjects. We hypothesize that the latter is due to imaging in nonequilibrium conditions. CONCLUSION: The data suggest that IBF-SPECT can help in discriminating extrapyramidal disease. The accuracy might be improved by an administration protocol that allows imaging in "true equilibrium" conditions, such as a bolus injection followed by a constant infusion.

Aged↗

PubMed1995

Postural hypotension enhanced by exercise in patients with chronic autonomic failure.

The effect of supine exercise on blood pressure (BP), measured while recumbent and after head-up postural change, was investigated in three groups with marked postural hypotension due to chronic autonomic failure: 15 with associated neurological impairment (Shy-Drager syndrome, SDS, multiple system atrophy); 15 with pure autonomic failure (PAF) and two with a deficiency of the enzyme dopamine beta hydroxylase (DBH deficiency). Fifteen normal subjects were controls. In controls, exercise increased supine BP, and there was no postural fall before or after exercise. In SDS and PAF, however, exercise produced a substantial fall in BP, which was greater in PAF. In both groups, BP fell to a lower level on standing after than before exercise. In DBH deficiency, there was little change in BP with exercise, but BP fell to a lower level on standing after exercise. In all three groups with autonomic failure, there were more symptoms of postural hypotension on standing after exercise. The influence of exercise on both supine and postural BP, therefore, should be considered in the clinical and laboratory assessment of autonomic dysfunction.

Aged↗

PubMed1994

[Significance of serum myosin light chain-1 level in neuroleptic malignant syndrome].

In Parkinson disease, the interruption of medication often results in neuroleptic malignant syndrome, which is caused by acute inhibition of the dopaminergic system. It is a serious question in the management of Parkinson disease, whether episode of pyrexia is derived from neuroleptic malignant syndrome or other origins. In this syndrome, muscle damage enhances serum titers of myogenic enzymes and proteins. Myosin light chain-1 (MLC-1), which is a small fragment of myosin, has been reported to show long lasting elevation compared with CK in neuromuscular diseases and cardiac infarction. Then, we evaluated the clinical significance of serum MLC-1 in six patients with neuroleptic malignant syndrome, i.e., four cases of Parkinson disease, one multiple system atrophy and one schizophrenia with acute administration of haloperidol. Muscle breakdown was observed by the elevation of serum MLC-1 titer, which sustained several days after normalization of serum CK titer. In two cases of Parkinson disease, high level of serum MLC-1 was observed in spite of normal body temperature, which suggested pre-clinical stage of neuroleptic malignant syndrome. Thus, we concluded that the estimation of serum MLC-1 is useful in those patients with high risk of neuroleptic malignant syndrome.

Adult↗

PubMed1993

[A case of rabbit syndrome--its unique pharmacological feature].

A 61-year-old woman developed cerebellar ataxia as an initial symptom when she was 52 years old. Her neurological symptom was gradually followed by autonomic nervous system disturbance, pyramidal sign, and rigidity. She was diagnosed as suffering from multiple system atrophy (MSA), when she was 53 years old. Magnetic resonance imaging revealed atrophy of the cerebellum and brainstem. She was accompanied by rabbit syndrome (RS), when she was 61 years old. She had not been given any neuroleptics, which might produce RS, before she developed RS. Her regular involuntary movement was localized in lips, and its frequency was about 3 Hz. While sleeping, she did not have the involuntary movement. We had a chance to conduct pharmacological examination on RS. The administration of atropine or trihexyphenidyl did not change her symptom of RS, and the intravenous injection of levodopa deteriorated the movement. Haloperidol, sulpiride, or chlorpromazine was significantly effective on her involuntary movement of RS. These results indicated that our patient had the supersensitivity in her dopamine receptor. Such supersensitivity might result from the denervation in MSA, because she had not been administered any neuroleptics. RS is generally considered to be a kind of extrapyramidal sign, and we have not been aware of any report about RS, which levodopa deteriorated and neuroleptics improved. The mechanism of her symptom is discussed.

Antipsychotic Agents↗

PubMed1993

Renin responses in patients with orthostatic hypotension: case reports.

In orthostatic hypotensive patients renin is reported to increase in response to head-up tilt unless the lesion involves postganglionic sympathetic efferent nerves. The authors examined responses of plasma renin activity (PRA) to acute blood pressure reduction in 4 patients with orthostatic hypotension. When the coexistence of normal plasma norepinephrine (PNE) and normal or high-normal pressor response to alpha stimulant is considered to be a sign of preserved function of postganglionic fibers, 2 of the patients had sympathetic lesions mainly confined to central and/or preganglionic fibers. They were diagnosed as having multiple system atrophy (MSA). The other 2 were diagnosed as having idiopathic orthostatic hypotension with possible postganglionic lesions. Although 1 MSA patient showed an increase in PRA from 2.0 to 4.7 ng/mL/hr in response to the tilt, the other MSA patient showed no PRA response to the tilt. The head-up tilt did not provoke an apparent PRA response in patients with idiopathic orthostatic hypotension. The authors infused sodium nitroprusside (SNP) in the 3 patients whose PRA did not respond to the head-up tilt. The infusion decreased blood pressure by 40 mmHg or more, but PRA responses were inappropriately small or suppressed. In all cases, PNE did not respond at all to the head-up tilt and to the infusion of SNP. These findings suggest that possible preservation of postganglionic fibers does not necessarily indicate an intact responsiveness of PRA. The PRA profiling may not be an accurate predictor for the site of the fiber degeneration in orthostatic hypotension.

Aged↗

PubMed1993

Classification of autonomic disorders.

Recent advances in our understanding of the pathophysiology of cardiovascular regulation and the metabolism of catecholamines have enabled us to develop an improved system of classification of autonomic disorders. Patients with autonomic impairment, clinically unassociated with other neurological abnormalities, are considered to have the Bradbury-Eggleston syndrome (idiopathic orthostatic hypotension, pure autonomic failure). Individuals whose autonomic failure is accompanied by degeneration in other neurological systems are classified as having the Shy-Drager syndrome (multiple system atrophy with autonomic failure). Patients in whom a deficiency of the enzyme dopamine-beta-hydroxylase is present from birth have many features suggestive of the Bradbury-Eggleston syndrome but manifest normal sweating and biochemically have an elevated plasma and urinary dopamine level. Recognition of these individuals is of particular importance because they are uniquely responsive to treatment with oral dihydroxyphenylserine (L-DOPS). A fourth disorder is baroreflex failure; this disorder is usually due to surgery, trauma, radiation or other injury to the ninth or tenth cranial nerves or the medullary nuclei which their fibers innervate. Patients with baroreflex failure have oscillations between hypertension and hypotension, but these alterations are poorly correlated with posture. Very high levels of plasma norepinephrine are found during the hypertensive phase of baroreflex failure. Baroreflex failure is generally responsive to treatment with clonidine. In conclusion, the diagnosis and therapy of autonomic disorders has improved due to the more precise taxonomy now current.

Autonomic Nervous System Diseases↗

PubMed1993

Dopamine D2 receptor imaging and measurement with SPECT.

Only recently it has become possible to label and visualize dopamine D2 receptors in the living human brain using an [123I]labeled ligand and the SPECT technique. Iodobenzamide (S(-)IBZM) is a substance with high affinity and high specificity for D2 receptors and was used in controls and in patients with different extrapyramidal disorders. After the i.v. administration of 5mCi (185 MBq) of [123I] labeled S(-)IBZM data collection was performed with a rotating double head scintillation camera between 60 and 110 minutes. In a semiquantitative approach a ratio was calculated between mean counts/pixel in the striatum and a region in the lateral frontal cortex, which gives an index of receptor density. In a group of 21 controls this ratio is 1.73 +/- 0.09. A highly significant age-related decline is found in controls and in 57 patients with PD. PD patients without L-DOPA or dopamine agonist treatment do not differ from controls (1.72 +/- 0.09; n = 18), whereas patients under dopaminergic therapy show a significantly lower binding ratio (1.65 +/- 0.13; n = 39; p = 0.017) suggesting receptor downregulation. Comparing the striatum ipsi- and contralateral to clinical symptoms in 18 hemiparkinsonian patients shows slightly but significantly higher values on the contralateral side, indicating receptor supersensitivity (1.71 +/- 0.11 vs. 1.66 +/- 0.09; p = 0.0014). No correlation between D2 receptor binding and clinical stage, duration of disease or duration of dopaminergic therapy exists. Markedly reduced ratios are measured in 7 patients with progressive supranuclear palsy and multiple system atrophies and in 18 patients with Huntington's disease (1.40 +/- 0.09 and 1.37 +/- 0.12 respectively; p = 0.0001).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1993

Choreas, hereditary and other ataxias, tics, myoclonus, and other movement disorders.

Developments in the field of Huntington's disease have focused on the potential benefits of predictive testing. Markers have been described for autosomal dominant cerebellar ataxia and for certain subtypes of Friedrich's ataxia. Argentophilic neuronal and glial inclusions appear to be the first specific pathologic hallmark of multiple system atrophy. "Pure" hereditary spastic paraplegia is not a multisystem disorder of the central nervous system, but a monomorphic and stereotyped disease. Advances in Tourette's syndrome are limited because the presumed gene eludes identification. A new type of myoclonus, propiospinal myoclonus, has been described. Clinical and electrophysiologic criteria for defining primary orthostatic tremor have been proposed. Understanding of the neurophysiologic substrate of essential tremor and myoclonus is improving. New neurologic disorders presenting clinically with prominent movement disorder continue to be described.

Ataxia↗

PubMed1996

Oxidative stress in Parkinson's disease and other neurodegenerative disorders.

The cause of cell death in neurodegenerative diseases remains unknown but the formation of free radicals and the occurrence of oxidative stress may be a common component of many, if not all, such disorders. For example, in substantia nigra in Parkinson's diseases key alterations occur, in iron handling, mitochondrial function and antioxidant defences, particularly reduced glutathione. These indices of oxidative stress are accompanied by evidence of free radical mediated damage in the form of increased lipid peroxidation and oxidation of DNA bases. The alterations in oxidative stress occurring in Parkinson's disease appear not be related to the administration of L-DOPA. Some alterations of oxidative stress are found in other basal ganglia in degenerative disorders (multiple system atrophy, progressive supranuclear palsy, Huntington's disease) but these have not been investigated to the same extent. Similarly, examination of biochemical changes occurring in Alzheimer's disease, motor neurone disease and diabetic neuropathy also suggest the involvement of free radical mediated mechanisms as a component of neurodegeneration. It is probable that irrespective of the primary cause of individual neurodegenerative disorder, the onset of oxidative stress is a common mechanism by which neuronal death occurs and which contributes to disease progression. Clearly, therapeutic strategies aimed at limiting free radical production and oxidative stress and/or damage may slow the advance of neurodegenerative disease.

Alzheimer Disease↗

PubMed1995

Autonomic disorders in Parkinson's disease.

Patients with idiopathic Parkinson's disease (IPD) often show signs and symptoms of autonomic involvement, related to the disease itself or to its progression. The more frequently disturbances reported are connected with loss of extrapyramidal motor control, i.e. dysphagia, gastric emptying and the most common constipation. They concern about 73% of the patients. A high frequency of urinary symptoms, ranging from 37% to 71%, is also reported in IPD, in particular detrusor hyperreflexia causing urgency, frequency of micturing or urgency incontinence. Another autonomic groups of symptoms are related to the failure of cardiopressor adaptability which involve 15% of the subjects and are more typical of late onset cases or forms bordering with the Multiple System Atrophy, finally resulting in orthostatic hypotension (OH).

Autonomic Nervous System Diseases↗

PubMed1995

Epidemiological, genetic, pharmacological, kinesiological, nuclear medical (IBZM-SPECT), standard and functional MRI studies on Parkinson's disease and related disorders and economic evaluation of Parkinson's disease therapy--clinical projects in the BMFT-research program Munich: "Parkinson's disease and other basal ganglia disorders".

The results of selected clinical research projects related to epidemiological, genetic, pharmacological, kinesiological, and neuroimaging aspects (SPECT, PET, MRI, functional MRI) of basal ganglia disorders such as Parkinson's disease, Progressive Supranuclear Palsy, Multiple System Atrophy and Wilson's disease are summarized. A retrospective pharmacoeconomic analysis of Parkinson's disease is presented. These studies are part of a nationwide research program of the German ministry of research and technology (BMFT) entitled "Parkinson's disease and other basal ganglia disorders" and were carried out at the Department of Neurology, LMU München.

Basal Ganglia Diseases↗

PubMed1995

[Cardiovascular alpha-, beta 1- and beta 2-adrenoceptor functions in neurogenic orthostatic hypotension].

It has been believed that patients with neurogenic orthostatic hypotension (NOH) usually develop denervation supersensitivity of cardiovascular alpha-, beta 1- and beta 2-adrenoceptors, because the majority studies have shown augmented cardiovascular responses to intravenously given noradrenaline (NA) or isoprenaline (IP) in these patients. This view, however, leaves room for discussion. First, drugs were administered by means of drip infusion method in most of the previous studies. Since this method necessarily provokes baroreflex, which is buffering fluctuations in blood pressure and heart rate, the results may reflect diminished baroreflex rather than denervation supersensitivity. Second, adrenoceptor functions are modulated by multiple factors, such as ageing, thyroid hormone and chronic inflammation. It is, thus, inappropriate to explain the whole picture of functional change in adrenoceptors by the denervation mechanism only. In order to look over the cardiovascular adrenoceptor functions in NOH, we performed bolus infusion tests of NA and IP on the patients. The subjects comprised 7 cases of NOH with pre-ganglionic sympathetic deficit (NOH-I), 6 cases with post-ganglionic deficit (NOH-II) and 15 healthy controls. NOH-I group included 6 patients with multiple system atrophy and one with Parkinson disease, while NOH-II group consisted of 3 patients with idiopathic orthostatic hypotension (pure autonomic failure) and 3 with diabetic autonomic neuropathy. Both NA and IP infusion tests were carried out under the continuous measurement of blood pressure and heart rate. In NA test, different bolus doses (0.01, 0.02, 0.05 and 0.1 microgram/kg) of NA were intravenously administered, and a degree of subsequent rise in mean blood pressure was used as an index for alpha-adrenoceptor function. IP test was performed in the same manner, and an increase in heart rate and a fall in mean blood pressure in response to the drug (0.001, 0.002 and 0.005 microgram/kg) were measured as indices for beta 1- and beta 2-functions, respectively. A rise in blood pressure following the administration of any dose of NA did not statistically differ among three groups. An increase in heart rate in IP test was generally lower in both NOH groups, and a significant difference was obtained between NOH-I and control when a given dose was 0.005 microgram/kg) (p < 0.02). A fall in blood pressure in IP test was significantly greater in NOH-I compared to control when doses were 0.002 and 0.005 microgram/kg (p < 0.02 and 0.01, respectively). It was also greater in NOH-II than in control when a dose was 0.002 microgram/kg (p < 0.01). In disagreement with most of the previous studies, the present results suggest that alpha-adrenoceptor function is hardly altered, beta 1-function is suppressed, and beta 2-function is augmented in NOH.

Adult↗

PubMed1996

Gallyas-positive, tau-negative glial inclusions in Parkinson's disease midbrain.

Gallyas-positive, tau-negative oligodendroglial cytoplasmic inclusions are specific for multiple system atrophy, while both Gallyas- and tau-positive glial fibrillary tangles are a common feature of tau-related neurological disorders. We examined the midbrain of Parkinson's disease (PD) patients and found significant numbers of Gallyas-positive glial inclusions in 17 of the 20 cases. They were most frequent in the dorsomedial part of the substantia nigra. Immunocytochemically, the glial inclusions were negative for tau and tubulin. Glial fibrillary acidic protein immunoreactivity was present in inclusion-containing cells. No such inclusions were observed in Alzheimer's disease (AD) or aged controls. These findings indicate that the glial inclusions present in the PD midbrain are different from those reported previously and unrelated to brain aging or the concurrence of AD lesions.

Aged↗

PubMed1996

[Effects of octreotide on experimental orthostatic neurogenic hypotension].

The synthetic somatostatin analogue, octreotide, has recently been proposed for the treatment of both postprandial and orthostatic hypotension (OH) in humans with autonomic failure related to multiple system atrophy (MSA) or diabetes mellitus. However, pharmacodynamic data are not still available in experimental models of orthostatic hypotension. We investigated in a model of neurogenic orthostatic hypotension, obtained by chronic sinoaortic denervation (SAD) in chloralose-anaesthetized dogs, the effects of octreotide (0.1 mg/kg, subcutaneous route) during a double-blind cross-over study vs placebo. Blood pressure (BP) and heart rate (HR) average values, SBP and HR short-term variabilities (using fast Fourier transformation) in both low (LF: 50-150 mHz) and high frequency range (respiratory rate +/- 50 mHz) and plasma noradrenaline (NA) levels (HPLC) were measured in supine position and during head-up tilt test (HUT: 80 degrees, 10 min) before and 45 min after drug administration. In controls, as expected, head-up tilt test induced a significant increase in DBP (+14 +/- 8 mmHg), HR (+36 +/- 21 beat/min), NA (296 +/- 118 vs 141 +/- 63 pg/ml), SBP-LF (25 +/- 5 vs 14 +/- 3%) whereas HR-HF significantly decreased. The changes during head-up tilt test were not modified after placebo or octreotide administration. In SAD dogs, head-up tilt test elicited a dramatic fall in SBP (-74 +/- 39 mmHg), DBP (-20 +/- 15 mmHg) without any significant change in HR (-5 +/- 12 beat/min), NA (708 +/- 213 vs 606 +/- 331 pg/ml), SBP-LF (16 +/- 3 vs 16 +/- 3%), HR-HF (8 +/- 2 vs 7 +/- 1%). Octreotide or placebo failed to significantly modify any of the measured parameters during head-up tilt test performed 45 min after drug administration. At the dose used, octreotide elicited a 80% decrease in insulin plasma levels after 45 min in both normal and SAD dogs. These results suggest that 1) this experimental model of orthostatic hypotension in SAD dogs is reproductible and can be used to investigate the pharmacological effects of antihypotensive drugs, 2) cardiovascular and biochemical characteristics of the SAD model are similar to those observed in MSA and 3) octreotide, in these experimental conditions, is not able to correct the BP fall during head-up tilt test.

Animals↗

PubMed1997

[PET study of dopamine metabolism and dopamine D2 receptor in juvenile parkinsonism].

Positron emission tomographic(PET) study using 18F-6-fluoro-L-dopa (18FDOPA) can provide efficient information on the pre-synaptic function of nigrostriatal dopaminergic neurons. In juvenile parkinsonism(JP), the accumulation of 18FDOPA is markedly decreased in the caudate nucleus and putamen on both hemispheres. This finding is different from those in dystonia syndromes such as dopa-responsive dystonia (DRD) and hereditary progressive dystonia with marked diurnal fluctuation(HPD), and it is rather similar to late onset of Parkinson's disease. Furthermore, we studied dopamine D2 receptor binding activity on the post-synaptic sites of the striatum using 11C-YM-09151-2(11C-YM), a highly selective dopamine D2 receptor antagonist. In JP, 11C-YM was highly accumulated in the striatum, and D2 receptor binding activity is not significantly different from that of age-matched young normal subjects, but much higher than that of aged subjects. This finding suggests that post-synaptic dopamine receptor function keeps still normal or hypersensitive in JP, and may be different from other degenerative disorders such as multiple system atrophy. Glucose metabolism using 18F-fluoro-2-deoxy-D-glucose(18FDG) was also within normal range in the cerebral cortex in JP, but was more increased in the striatum than in the cerebral cortex in some patients. These PET studies can provide efficient informations about the pathologic condition of JP.

Age of Onset↗