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PubMed1992

[Shy-Drager syndrome].

Shy-Drager syndrome is characterized by severe orthostatic hypotension and other autonomic dysfunctions, cerebellar ataxia, parkinsonism, and upper and lower motor neuron symptoms. The disease starts in the 4th to 6th decades with severe dizziness, blackout or syncopi on standing up, and the duration of the illness is 6-7 years in the majority of the cases. The pathological lesions responsible for the autonomic dysfunctions are in the hypothalamus, locus saeruleus, Edinger-Westphal and other nuclei in the brain stem, intermediolateral column and Onuf's nuclei in the spinal cord. Oppenheimer has postulated a concept of multiple system atrophy to such widely distributed degenerative neurological disorders. However, olivopontocerebellar atrophy of Dejerine and Thomas, striatonigral degeneration and this syndrome can be distinguished clinically as well as pathologically.

Adult↗

PubMed1992

[Idiopathic orthostatic hypotension (IOH) and sympathotonic orthostatic hypotension (SOH)].

IOH occurs as progressive autonomic failure (PAF) without any neurologic symptoms indicating multiple system atrophy or Parkinson's disease. The responsible lesion for IOH is yet obscure but has been suggested to be in the peripheral sympathetic nerves, since postganglionic sympathetic neurons in IOH fail to release norepinephrine and there present extensive supersensitivities to exogenous pressors. SOH is characterized as marked tachycardia induced by hypotensive stress like standing, and is less sensitive to the administered catecholamines. Careful examinations by some pharmacological studies are essential to diagnose IOH and SOH in patients with orthostatic hypotension.

Autonomic Nervous System Diseases↗

PubMed1992

[Progressive autonomic failure with Parkinson's disease].

Progressive or primary autonomic failure (AF) is a disease of unknown-etiology, and presents generalized and extensive autonomic disturbances because of selective neuronal degeneration in the whole of autonomic nervous system. AF is classified into three categories; (1) pure autonomic failure, without associated neurological disorders, (2) AF with Parkinson's disease (PD), and (3) AF with multiple system atrophy (Shy-Drager syndrome) (Bannister, 1988). AF with PD is pathologically characterized by neuronal cell degeneration in the intermediolateral column and the substantia nigra, together with Lewy bodies mainly in the pigmented nuclei in the brain stem. Patients with PD occasionally develop syncope or dizziness due to orthostatic hypotension and/or postprandial hypotension as well as urorectal disturbances as the initial symptoms, and are followed by parkinsonism. Levodopa is usually effective for parkinsonism but the prognosis is rather poor. AF with PD could be regarded as a form of 'the Lewy body disease' according to Kosaka's clinicopathological entity (1984).

Aged↗

PubMed2006

Dual-tracer dopamine transporter and perfusion SPECT in differential diagnosis of parkinsonism using template-based discriminant analysis.

UNLABELLED: Clinical differential diagnosis in parkinsonism can be difficult especially at early stages. We investigated whether combined perfusion and dopamine transporter (DAT) imaging can aid in the differential diagnosis of parkinsonian disorders: idiopathic Parkinson's disease (IPD), progressive supranuclear palsy (PSP), multiple system atrophy (MSA), dementia with Lewy bodies (LBD), and essential tremor (ET). METHODS: One hundred twenty-nine patients were studied, retrospectively (69 males; 24 MSA, 12 PSP, 8 LBD, 27 ET, and 58 IPD; mean disease duration, 3.5 +/- 3.7 y). Diagnosis was based on established clinical criteria after follow-up of 5.5 +/- 3.8 y in a university specialist movement disorders clinic. Group characterization was done using a categoric voxel-based design and, second, a predefined volume-of-interest approach along Brodmann areas (BA) and subcortical structures, including striatal asymmetry and anteroposterior indices. Stepwise forward discriminant analysis was performed with cross-validation (CV) using the leave-one-out technique. RESULTS: Characteristic patterns for perfusion and DAT were found for all pathologies. In the parkinson-plus group, MSA, PSP, and LBD could be discriminated in 100% (+CV) of the cases. When including IPD, discrimination accuracy was 82.4% (99% without CV). 2beta-Carbomethoxy-3beta-(4-iodophenyl)nortropane imaging as a single technique was able to discriminate between ET and neurodegenerative forms with an accuracy of 93.0% (+CV); inclusion of perfusion information augmented this slightly to 97.4% (+CV). CONCLUSION: Dual-tracer DAT and perfusion SPECT in combination with discrimination analysis allows an automated, accurate differentiation between the most common forms of parkinsonism in a clinically relevant setting.

Algorithms↗

PubMed2006

[Abeta, tau and alpha-synuclein and glial cells].

Many neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease are now characterized by abnormal accumulation of certain proteins in the brain. The key molecules include amyloid beta-protein (Abeta), tau and a-synuclein, all of which are involved in the pathogenesis and provide histopathological hallmarks of the diseases. Abeta is continuously produced in and removed from the brain. Microglia and astrocytes take up and degrade soluble Abeta. In the Alzheimer brain, once-deposited, insoluble Abeta is also removed by phagocytosis by activated microglia. The success of these removal processes, however, is at best partial. The phagocytic removal of insoluble Abeta is associated with neuroinflammation, a potentially neurotoxic reaction. Tau is accumulated in astrocytes under a diversity of pathological conditions in several forms: thorn-shaped astrocytes; tuft-shaped astrocytes; astrocytic plaques. Thorn-shaped astrocytes are associated with gliosis and are not disease-specific. Tuft-shaped astrocytes are characteristic of progressive supranuclear palsy (PSP) and astrocytic plaques of cortico-basal degeneration (CBD). Tau accumulation in oligodendrocytes is referred to as coiled bodies and occurs in PSP, CBD, Pick's disease and some other so-called taupathies. a-Synuclein is accumulated in oligodendrocytes, which is referred to as glial cytoplasmic inclusions (GCI). Occurrence of GCI is diagnostic to multiple system atrophy. Transgenic mouse models in which tau or alpha-synuclein is overexpressed in glial cells indicate that neuronal degeneration occurs following tau/alpha-synuclein accumulation in glial cells, supporting a notion that these abnormal glial cells play pathogenic roles.

Alzheimer Disease↗

PubMed2006

Reduced cardiac uptake and enhanced washout of 123I-MIBG in pure autonomic failure occurs conjointly with Parkinson's disease and dementia with Lewy bodies.

UNLABELLED: The purpose of this study was to investigate myocardial uptake of 123I-metaiodobenzylguanidine (MIBG) in patients with pure autonomic failure (PAF), which has pathologic features in common with idiopathic Parkinson's disease (IPD) and dementia with Lewy bodies (DLB). METHODS: Six patients with PAF, 130 with IPD, 21 with DLB, 9 with corticobasal degeneration (CBD), 11 with progressive supranuclear palsy (PSP), and 11 with multiple-system atrophy (MSA) underwent myocardial 123I-MIBG scintigraphy, as did 16 control patients. RESULTS: Resulting heart-to-mediastinum (H/M) ratios were significantly lower in patients with PAF, IPD, or DLB than in patients with CBD, PSP, or MSA and in the controls. H/M ratios were lower for delayed images than for early ones in patients with PAF, IPD, or DLB, whereas the ratios were higher for delayed images in patients with CBD and in the controls. CONCLUSION: Cardiac sympathetic denervation and enhanced washout of 123I-MIBG from sympathetic nerve terminals may develop in parallel in patients with PAF, IPD, or DLB.

3-Iodobenzylguanidine↗

PubMed2006

Botulinum toxin type A treatment for Parkinsonian patients with moderate to severe sialorrhea.

PURPOSE: To investigate the effect of botulinum toxin type A (BTX-A; Botox) in reducing saliva in patients with Parkinsonism. METHODS: Fifteen patients with clinical diagnosis of idiopathic Parkinson's disease, dementia with Lewy bodies, or multiple system atrophy were enrolled in this open clinical trial. A total of 40-unit dose of Botox was injected into the bilateral parotid and submandibular glands. Objective measuring of saliva production with dental rods, subjective Drooling Score, personal impression of clinical improvement, and the duration of response were used for the global assessment of sialorrhea after BTX-A treatment. RESULTS: All patients showed objective reduction in saliva production following BTX-A treatment and the mean production was reduced at a significant level. The severity of sialorrhea assessed by Drooling Score was 5.87 +/- 0.92 (range: 5-8) and 3.60 +/- 1.18 (range: 2-6) respectively (p<0.001) before and after BTX-A injection. The mean duration of BTX-A response extended for 16.3 +/- 5.7 weeks (range: 5-24). No severe adverse effect nor worsening of existing dysphagia was observed in all Parkinsonian patients. CONCLUSIONS: Parkinsonian drooling may undermine patient's health and daily activity. BTX-A local injection is a safe and effective measure in counteracting sialorrhea, even in patients associated with moderate dysphagia.

Aged↗

PubMed2006

Neuroimaging in Parkinson's disease.

Structural imaging studies often reveal relatively limited findings in Parkinsonian disorders, as the most profound changes are neurochemical and hence better revealed by functional studies such as PET or SPECT. However, newer magnetic resonance techniques such as spectroscopy, diffusion weighted imaging, diffusion tensor imaging and magnetization transfer have shown promise in differentiating between idiopathic Parkinson's and the atypical parkinsonian disorders such as multiple system atrophy and progressive supranuclear palsy. We review here recent advances in functional imaging as well as in structural studies of basal ganglia disorders. Functional studies may give insights into mechanisms underlying disease pathogenesis, as well as neurochemical alterations.

Diffusion Magnetic Resonance Imaging↗

PubMed2006

PET measurement of cardiac and nigrostriatal denervation in Parkinsonian syndromes.

UNLABELLED: Scintigraphic imaging with (123)I-metaiodobenzylguanidine ((123)I-MIBG) has demonstrated extensive losses of cardiac sympathetic neurons in idiopathic Parkinson's disease (IPD). In contrast, normal cardiac innervation has been observed in (123)I-MIBG studies of multiple-system atrophy (MSA) and progressive supranuclear palsy (PSP). Consequently, it has been hypothesized that cardiac denervation can be used to differentiate IPD from MSA and PSP. We sought to test this hypothesis by mapping the distribution of cardiac sympathetic neurons in patients with IPD, MSA, and PSP by using PET and (11)C-meta-hydroxyephedrine ((11)C-HED). Also, the relationship between cardiac denervation and nigrostriatal denervation was investigated by measuring striatal presynaptic monoaminergic nerve density with PET and (11)C-dihydrotetrabenazine ((11)C-DTBZ). METHODS: (11)C-HED and (11)C-DTBZ scans were obtained for patients with IPD (n = 9), MSA (n = 10), and PSP (n = 8) and for age-matched control subjects (n = 10). Global and regional measurements of (11)C-HED retention were obtained to assess the extent of cardiac sympathetic denervation. (11)C-DTBZ binding was measured in the caudate nucleus, anterior putamen, and posterior putamen. RESULTS: As expected, extensive cardiac denervation was observed in several of the patients with IPD. However, substantial cardiac denervation was also seen in some patients with MSA and PSP. (11)C-DTBZ studies demonstrated striatal denervation in all patients with IPD and in most patients with MSA and PSP. No correlation was found between cardiac (11)C-HED retention and striatal (11)C-DTBZ binding. CONCLUSION: Cardiac sympathetic denervation was found to occur not only in IPD but also in other movement disorders, such as MSA and PSP. This finding implies that scintigraphic detection of cardiac sympathetic denervation cannot be used independently to discriminate IPD from other movement disorders, such as MSA and PSP. Cardiac sympathetic denervation was not correlated with striatal denervation, suggesting that the pathophysiologic processes underlying cardiac denervation and striatal denervation occur independently in patients with parkinsonian syndromes. These findings provide novel information about central and peripheral denervation in patients with neurodegenerative disorders.

Adult↗

PubMed1990

[Vasopressin effect and pathophysiological analysis on postprandial hypotension].

In order to clarify the effect of arginine vasopressin (AVP) on postprandial hypotension (PPH) in patients with multiple system atrophy, a loading test of 75 g glucose in 225 ml water was performed during infusion of AVP (0.3 U/min). AVP (0.3 U/min) infusion prior to the loading was significantly effective for PPH in all patients: PPH following to oral intake of glucose was completely prevented. We conclude that infusion of AVP maintained circulating systemic blood volumes by reduced portal venous flow (after a reduction of portal venous inflow) as a result of vasoconstrictive effect of AVP on the splanchnic vessels in patients with PPH.

Adult↗

PubMedDate not supplied

A case of late onset striatonigral degeneration.

We report the case of a 78-year-old patient who had a parkinsonian syndrome which did not respond to levodopa treatment. The duration of the illness was four years. The main clinical features were bradykinesia and rigidity. He also showed pseudobulbar and pyramidal syndromes. The prominent pathological findings were a severe neuronal loss in the depigmented substantia nigra and in the putamen, which showed a brownish discoloration. Although the late age of onset is unusual, the findings suggest striatonigral degeneration. The link of this disease to multiple system atrophy is discussed.

Aged↗

PubMed1989

Characterization of glutamate dehydrogenase isoproteins purified from the cerebellum of normal subjects and patients with degenerative neurological disorders, and from human neoplastic cell lines.

Glutamate dehydrogenase (GDH) was purified to homogeneity from cerebellar tissue of three normal subjects and seven patients with four distinct types of degenerative neurological disorders. Nonequilibrium pH gradient gel electrophoresis showed that the purified enzyme consists of four major isoproteins designated GDH 1, 2, 3, and 4. With one exception, the relative abundance and isoelectric points of the GDH isoproteins decrease and the molecular weights increase progressively going from isoprotein 1 to isoprotein 4. The enzyme isolated from the brain of one patient with a variant form of multiple system atrophy displayed marked reduction of GDH isoprotein 1. The Km values of the patients' GDH for alpha-ketoglutarate, glutamate, NADH, and NADPH were significantly increased as compared to GDH obtained from normal and neurologic control subjects. In addition, glutamate levels were reduced markedly in the patient's cerebellum. Pulse-chase studies have shown that both the human hepatoma HepG2 and the human glioma U373 cell lines synthesize exclusively GDH isoprotein 2. The different GDH isoproteins do not have a precursor-product relationship and may represent products of different GDH mRNA species.

Adolescent↗

PubMed1988

[The neuropathology of Parkinson syndrome].

Parkinsonism is due to an alteration of the dopaminergic nigro-striatal pathway the main morphological basis of which is a lesion of the substantia nigra (SN). Parkinson's disease, the etiology of which is unknown, is responsible for 90 p. cent of the cases of parkinsonism. It is characterized by a neuronal loss involving mainly the pigmented brainstem nuclei (substantia nigra, locus coeruleus and dorsal vagal nuclei) and a specific neuronal inclusion: the Lewy inclusion body. Involvement of other structures such as the nucleus basalis of Meynert, hypothalamus, reticular formations of the brainstem, spinal lateral horns, sympathetic ganglia, may be responsible for dementia and/or autonomic failure which are often associated with the extrapyramidal signs. Various other pathological processes, infectious, toxic, vascular, tumoral or traumatic, involving usually the SN, are occasionally responsible for parkinsonism. A number of degenerative diseases involving the SN may develop parkinsonism as major presenting sign. Most of them are diseases of the basal ganglia such as multiple system atrophy, primitive pallidal atrophies, Steele-Richardson-Olszewski disease, Hallervorden-Spatz disease. However, changes in the SN at the origin of parkinsonism are occasionally found in cortical degenerative diseases such as senile dementia of Alzheimer's type, Pick disease or the complex of Guam, in cerebellar atrophies or in motor neuron disease.

Aged↗

PubMed1988

Studies of the neurogenic bladder.

A series of 36 patients with specific neurological lesions affecting those parts of the nervous system thought to be involved in the control of bladder function have been studied by urodynamic, electromyographic and neurohistochemical means and the results compared with those obtained in 20 control subjects. In patients with pelvic nerve injury urethral sphincter electromyography (EMG) revealed abnormal motor units and the density of bladder innervation was significantly reduced (P less than 0.01). By contrast, in patients with distal autonomic neuropathy the innervation of the striated muscle of the urethra was unaffected and although there was an almost total loss of nerves from the bladder muscularis, the subepithelial plexus of nerves was preserved. In patients with progressive autonomic failure and multiple system atrophy, the bladder neck was incompetent in every case and the striated muscle of the urethra was affected by a process of denervation and re-innervation. These findings serve to distinguish patients with autonomic failure from those with idiopathic Parkinson's disease and influence the selection of patients for transurethral surgery.

Autonomic Nervous System Diseases↗

PubMedDate not supplied

[Cerebellar ataxia and hypogonadism. Description of 4 cases].

We describe the association of cerebellar ataxia and hypogonadism in four female patients. Clinical, neurophysiological, neuroradiological and endocrinological features are reported. Post-mortem findings in one case are also described. We show in our patients that, beside the cerebellum, both the supratentorial white matter and the peripheral nerve are involved. We suggest that this association can be considered as a "multiple system atrophy". Furthermore, since hypogonadism is hypergonadotropic in three patients and hypogonadotropic in one, the endocrine pattern appears to be heterogeneous.

Adolescent↗

PubMed1987

Genetics of Parkinson's disease.

The hypothesis that heredity plays an important role in the etiology of PD has long seemed attractive. It was initially suggested by positive family histories and later by reports of kindreds of "parkinsonism." It was reinforced by the first systematic family study of PD conducted a half-century ago by Mjones. However, closer study of the clinical data indicates that familial aggregations of parkinsonism have reflected various other disorders, notably OPCA and other forms of multiple system atrophy. Case control studies have failed to document a prevalence of PD among first-degree relatives of index cases significantly exceeding that found in control groups. More recently, the concordance rate for PD among monozygotic twins has been found to be unexpectedly low and not significantly different from that among dizygotic twins or the prevalence among first-degree relatives. Thus, the best available data do not support a role of heredity in the etiology of PD. A nonhereditary, possibly congenital genetic defect is, however, not excluded.

Chromosome Aberrations↗

PubMed1995

[Clozapine in the treatment of mental manifestations of Parkinson disease].

BACKGROUND: Medical treatment of Parkinsonian syndromes is often complicated by psychiatric side effects such as confusional states, hallucinations and psychosis. Recent pilot studies report good clinical results with the atypical neuroleptic clozapine. PATIENTS AND METHODS: We report on 15 patients with Parkinsonian syndromes: 11 with idiopathic Parkinson's disease (IPD), 3 with multiple system atrophy (MSA) and 1 with postencephalitic Parkinsonism (SPP). The mean age was 68.8 +/- 10 years; the mean duration of Parkinsonian symptoms was 6.8 +/- 5.7 years. The Hoehn & Yahr grade was: 3.5 +/- 0.8. Eleven patients were suffering from psychotic episodes, 10 from hallucinations, 8 from confusional states. Clozpine was introduced at nighttime and dosage was modified until the appearance of clinical effect or intolerable side effects. RESULTS: We report on an observed cumulative duration of clozapine treatment of 13 patient-years. The average treatment duration was 10.5 +/- 10.4 months. The mean daily dose was 33.3 +/- 30 mg (range: 6.2-100). There was at least transitory improvement of psychiatric symptoms in all patients. There was constant and complete improvement in 7 patients (46%) and satisfactory improvement in 5 patients (33.3%). The levodopa dosage was unchanged (mean dosage 563 +/- 232 mg), and the dosage of dopamine agonists was significantly increased. None of our patients experienced motor deterioration. Side effects comprised sialorrhoea, sedation, orthostatic hypotension, and delirium tremens and an epileptic seizure in one patient each. Two patients died suddenly at the 63rd and at the 86th day of treatment respectively, outside the hospital. These deaths seemed to be unrelated to the treatment. There was no agranulocytosis. CONCLUSION: Clozapine is an efficient antipsychotic drug in Parkinsonian patients with no motor side effects in the dosages used. The effective dosage is very low in comparison to psychiatric patients. However various side effects may occur and close monitoring is required.

Adult↗

PubMed1995

[Multivariate analysis of spinal automatism in neurological diseases].

We investigated the relationship between spinal automatism (SA) and other neurological signs and symptoms (pyramidal, extrapyramidal, cerebellar, autonomic) by multivariate analysis in cervical spondylotic myelopathy (CSM) 112 cases, amyotrophic lateral sclerosis (ALS) 121 cases, and multiple system atrophy (MSA) 115 cases (olivo-ponto-cerebellar atrophy (OPCA) 55 cases, Shy-Drager syndrome (SDS) 42 cases, striato-nigral degeneration (SND) 18 cases). SA elicitation maneuver we used was pinprick stimulation at the dorsal pedal skin, and all cases showed twitched or phasic triple flexion pattern. As SA was elicited in 19 cases in CSM (17.0%), 23 cases (19.0%) in ALS, 36 cases (31.3%) in MSA, SA was more elicited in MSA than in ALS and CSM (p < 0.05, p < 0.01 by chi-squared test). In MSA, SA tended to be elicited more likely in SDS (21 cases, 50.0%) and in SND (8 cases, 44.4%) than in OPCA (7 cases, 12.7%) (p < 0.01 by chi-squared test). In MSA, the longer the duration of the disease became, the more SA was elicited, but not in ALS and CSM. Multivariate analysis (quantification method type II) showed that clinical signs as a statistical contribution factor for SA elicitation ranked Babinski's sign, micturition disturbance in ALS and CSM and Babinski's sign, orthostatic hypotension in MSA in order: In all 3 diseases, Babinski's sign and autonomic disturbance ranked higher. This conclusion suggested that SA was not always related to the pyramidal tract damage and was associated with the damage of small myelinated fibers in and/or around the pyramidal tract.

Adult↗