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PubMed1997

[Striatonigral degeneration].

Striatonigral degeneration (SND) is sporadic, middle-aged on set degenerative disease of the nervous system which etiology is unknown. SND is considered one of multiple system atrophy (MSA). Clinically parkinsonian symptom is dominant and then it is difficult to distinguish from idiopathic Parkinson's disease (PD). Pathologically neuron cell loss and gliosis are recognized principally striatum (mainly putamen) and substantia nigra. Putaminal hypointensity and slit-hyper intensity in the outer margin of putamen are often seen on T2-weighted 1.5 Tesla MRI. PET with [18 F] fluorodeoxyglucose indicates a considerably decreased glucose utilisation in the striatum of SND, whereas glucose utilisation are normal in PD. Striatal dopamine D1, D2 receptors are reduced. Response to Levodopa is poor or absent.

Age of Onset↗
PubMed1997

[Striatal dopamine D2 receptor in Parkinson disease and its related disorders assessed by C-11 NMSP and PET].

Dopamine D2 receptor binding potential (BP) was measured by C-11 N-methylspiperone (NMSP) and PET in normal healthy volunteers and patients with various neurodegenerative diseases including Parkinson disease (PD), multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), Huntington disease (HD) and so on. In normal aging process, striatal BP was decreased according to the age. In never-medicated Parkinson disease, BP was slightly elevated, which may reflect denervation hypersensitivity in postsynaptic site. In contrast, BP was reduced in MSA (SND type) and HD. C-11 NMSP and PET is useful in better understanding of pathophysiology and differential diagnosis of Parkinson disease and its related disorders.

Aging↗
PubMed1997

[Analysis of Parkinson's disease and related syndromes using 123I-IMP-SPECT with the ARG method].

We studied regional cerebral blood flow (rCBF) in 8 patients with non-demented Parkinson's disease (PD). 1 patient with progressive supranuclear palsy (PSP), 1 patient with multiple system atrophy (MSA), and 7 normal control subjects using single photon emission computed tomography (SPECT) with the IMP-ARG method. Regions of interest were studied in the cerebral cortex (upper frontal, lower frontal, temporal, occipital, parietal), thalamus, basal ganglia, and cerebellum. In patients with PD, rCBF was normal in 4/8, and decreased in occipital lobe in 4/8. In patient with PSP, rCBF was decreased in the upper and lower frontal lobes, and in the cerebellum. In patient with MSA, rCBF was diminished in the cerebellum. The results of our study were almost compatible with the conventional rCBF study by positron emission tomography (PET), however, the decrease of rCBF in occipital lobe had rarely been reported, suggesting that might be related to visuospatial dysfunction in Parkinson's disease.

Aged↗
PubMed1996

[Orthostatic hypotension and postprandial hypotension].

Both orthostatic hypotension (OH) and postprandial hypotension (PPH) may result from lesions in any part of the baroreflex arc, which comprises central autonomic network, afferent pathways, efferent pathways, and neuro-vascular junction. Nonetheless, most attention has been centered to the efferent pathways to date. In the present report, I discussed on the contribution of neuro-vascular junction and central autonomic network to the development of OH and PPH. I also referred to the essential difference in pathophysiology between OH and PPH. (1) Difference between OH and PPH: Essential difference is in triggers inducing the initial blood pressure fall. The trigger in OH is gravity, while that in PPH is abnormal release of vasodilative gastroenteric peptides; the former is equally delivered to all persons living on earth, but the latter varies from person to person. (2) Neuro-vascular junction: Previous studies, in which catecholamine drip infusion tests were carried out on patients with OH, suggested that all of cardiovascular alpha-, beta 1, and beta 2 adrenoceptors gain denervation supersensitivity in OH. This view does, however, underestimate the blood pressure buffering effect of baroreflex. Because long-standing alteration in blood pressure by drip infusion of catecholamine necessarily provokes baroreflex, it is reasonable that augmented cardiovascular responses in OH are largely due to baroreflex failure. We performed bolus infusion tests of noradrenaline and isoprenaline on patients with OH, and found that alpha-adrenoceptor-mediated rise in blood pressure was comparable to control, beta 1-mediated increase in heart rate was blunted, and beta 2-mediated fall in blood pressure was enhanced in OH. It is, therefore, likely that beta 2-mediated vasodilation exceeds alpha-mediated vasoconstriction in OH. In such condition, noradrenaline may produce a paradoxical hypotensive effect, which contributes to the development of OH or PPH. (3) Central autonomic network (CAN): Clinical symptoms due to lesions within CAN are usually not manifested when the efferent sympathetic pathways are highly impaired, as in multiple system atrophy. Some variants of OH and PPH may result from lesions within CAN, however. For example, we have experienced a case of sympathotonic OH associated with herpes simplex encephalitis, in which the efferent pathways do not seem to be involved.

Eating↗
PubMed1997

[PET and SPECT in Parkinson's disease].

Positron emission tomography (PET) with [18F] fluorodopa (FD) assesses nigrostriatal function in vivo. Measurements deriving from FD PET have direct lineal relationship to the nigral cell count in normal ageing and in Parkinson's disease (PD), although in PD the progression of the decline in nigrostriatal dopaminergic function is faster than the controls. The functional anatomy of the movements has been studied using PET and SPECT. The supplementary motor area (SMA) dysfunction is responsible for bradykinesia in Parkinson. The SMA is undereactive in PD during self-generated movements, and this underactivation is reversed by the administration of apomorphine and modulate by the stereotaxic ventral pallidotomy. PET has been used, also, to attempt the differentiation of PD, multiple system atrophy (MSA) and PSP, in vivo, using a number of different radioligands (opiod, dopamine receptor binding). [11C] raclopride PET and [123I] IBF SPECT seemed a promising agents to assessing D2 receptor status in humans. Studies of D2 status have demonstrated normal or increased D2 receptor density in PD and decreased receptor density in PSP and MSA. Using SPECT has been demonstrated marked differences of the DA transporters located on dopaminergic terminals in the striatum in healthy controls and PD patients. Also the correlation of SPECT measures of transporters of DA and motor severity suggests that this may be a useful marker of disease severity in PD.

Brain↗
PubMed1997

Parkinson's disease: clinical features.

Idiopathic Parkinson's disease (IPD) is a common and universal condition. Although its cause is still unknown, we now have some insights into pathogenetic mechanisms and genetic factors that may be important in causing the selective neuronal loss and presence of Lewy bodies that characterize its pathology. Clinically, as well as the classic features of akinesia, rigidity and often rest tremor, patients may present a wide range of other symptoms including pain, other sensory symptoms, impaired olfaction, personality change, mild executive cognitive deficits, dementia and depression, an extraordinary richness of symptoms and signs rendered even more extraordinary by the long-term effects of drug treatment. While there may be little difficulty recognizing typical cases of IPD, there has been, at least until recently, a considerable misdiagnosis rate in both atremulous (confusion with ageing, vascular disease, multiple system atrophy (MSA) or progressive supranuclear palsy (PSP)) and tremulous (confusion with essential tremor (ET), dystonic tremor, and MSA) forms. However, increasing awareness of the clinical features of all these conditions, together with adherence to exacting diagnostic criteria, is leading to improved diagnosis, which is crucial for patients (who want to know what the future holds for them), for their treatment (giving them the right drug and not the wrong one) and for research (since all the different diseases above have different aetiologies and pathology).

Aging↗
PubMed1997

Other causes of parkinsonism.

This chapter deals with the 'other causes of parkinsonism with the exclusion of idiopathic Parkinson's disease, multiple system atrophy, progressive supranuclear palsy and corticobasal degeneration. It is a cursory glance over the endless list of 'secondary' causes of parkinsonism or parkinsonism due to acquired and inherited metabolic and neurodegenerative diseases. With the exception of drug-induced parkinsonism and 'vascular' parkinsonism, most such cases are rare. However, single rare causes must not be overlooked as there can be specific therapeutic consequences. Parkinsonism is often present amid a constellation of other symptoms. Careful evaluation of the personal, familial and environmental context, mode of onset and progression, clinical features, and drug response, together with imaging, biochemical and molecular tests, help in determining the underlying disease.

Antipsychotic Agents↗
PubMed1998

Neuropathology of movement disorders.

This article reviews the cytoskeletal abnormalities, morphologic lesion patterns, and resulting pathophysiology of the most frequent neurodegenerative movement disorders caused by dysfunction of the basal ganglia and related neuronal loops. The following topics are discussed: Among the akinetic-rigid Lewy body disorders is idiopathic Parkinson's disease, which reveals specific lesion patterns of pathophysiologic and therapeutic relevance. Dementia with Lewy bodies characterized by cortical Lewy bodies appears intermediate between Parkinson's and Alzheimer's diseases. Tau pathologic disorders may show some clinical and morphologic overlap. Multiple system atrophy has ubiquitous oligodendroglial inclusions as a cytopathologic hallmark. Secondary parkinsonism includes drug-related, toxic, and other symptomatic disorders. Hyperkinetic disorders include CAG-related inherited diseases, showing specific genetic defects and morphologic lesions. Dystonia syndromes show inconsistent pathologic findings, and myoclonus may be related to a variety of disorders. Consensus data on clinical and neuropathologic criteria already existing for some disorders, together with molecular genetic and biochemical data will provide further insight into the complex pathophysiology and pathogenesis of movement disorders.

Humans↗
PubMed1998

P53- and CD95-associated apoptosis in neurodegenerative diseases.

Apoptosis is likely to be an important mechanism of cell loss in neurodegenerative diseases, but the signaling cascades activated before DNA fragmentation have not yet been determined. p53 or CD95 gene up-regulation precedes apoptosis in many cell types, and a potential role for these molecules in apoptosis of neurons and glial cells has already been demonstrated in Alzheimer's disease (AD). To determine whether apoptosis in other neurodegenerative diseases is mediated by similar mechanisms, p53 and CD95 expression were examined in postmortem central nervous system tissues from patients with diffuse Lewy body disease (DLBD), Pick's disease (PkD), progressive supranuclear palsy (PSP), multiple system atrophy (MSA), Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS), and Down's syndrome plus Alzheimer's disease (DN+AD). Quantitative immunoblot analysis demonstrated higher temporal lobe levels of p53 and CD95 proteins in DLBD, PkD, and DN+AD, and higher temporal lobe levels of CD95 only in MSA and PSP relative to PD and aged controls (for all, p < 0.01). In histologic sections, increased p53 immunoreactivity was localized in neuronal and glial cell nuclei, neuronal perikarya, and dystrophic neuritic and glial cell processes in the frontal (Area 1 1) and temporal (Area 21) lobes in DLBD, PkD, and DN+AD, the motor cortex and spinal ventral horns in ALS, and the striatum and midbrain in DLBD, MSA, PD, and PSP. Increased CD95 expression and nuclear DNA fragmentation were present in the same cell types and structures that manifested increased nuclear p53 immunoreactivity. The results suggest that p53- or CD95-associated apoptosis may be a common mechanism of cell loss in several important neurodegenerative diseases. In addition, the presence of abundant p53-immunoreactive neurites and glial cell processes appears to be a novel feature of neurodegeneration shared by these distinct diseases.

Adult↗
PubMedDate not supplied

[Pharmacologic approach to autonomic failure].

Four different forms of primary autonomic failure (multiple system atrophy, pure autonomic failure, Parkinson's disease and dopamine beta-hydroxylase deficiency) have been described. The first part of the article will focus on the interest to pharmacology of elucidating pathophysiological mechanisms underlying autonomic involvement at the central level (growth hormone response to clonidine acute challenge), presynaptic level (plasma catecholamine levels after yohimbine administration) and on post-synaptic receptors (binding studies, pressor responses to noradrenaline). The second part will discuss efficacy and side-effects of some of the many drugs which are currently proposed for the treatment of one of the most disabling symptoms related to autonomic failure, orthostatic hypotension. Special attention will be paid to drugs acting on blood composition (fludrocortisone, erythropoietin), on post-synaptic alpha-adrenoceptors (midodrine and clonidine) and on noradrenaline spill-over (yohimbine and L-Threo-DOPS).

Adrenergic Agonists↗
PubMed1998

[Clinical impact of cerebral dopamine-D2 receptor scintigraphy].

The present review describes findings and clinical indications for the dopamine D2 receptor scintigraphy. Methods for the examination of D2 receptors are positron emission tomography (PET) using 11C- or 18F-labelled butyrophenones or benzamides or single photon emission tomography (SPECT) using 123l-iodobenzamide (IBZM) respectively. The most important indication in neurology is the differential diagnosis of Parkinsonism: patients with early Parkinson's disease show an increased D2 receptor binding (D2-RB) compared to control subjects. However, patients suffering from Steele-Richardson-Olszewski-Syndrome or Multiple System Atrophy show a decreased D2-RB and are generally non-responsive to treatment. Postsynaptic blockade of D2 receptors results in a drug induced Parkinsonian syndrome, which can be diagnosed by D2 scintigraphy. Further possible indications occur in psychiatry: the assessment of receptor occupancy is useful in schizophrenic patients treated with neuroleptics. Additionally, D2 receptor scintigraphy might help to clarify the differential diagnosis between neuroleptic malignant syndrome and lethal catatonia. The method might be useful for supervising neurobiochemical changes in drug dependency and during withdrawal. Assessment of dopamine D2 receptor binding can simplify the choice of therapy in depressive disorder: patients showing a low D2 binding are likely to improve following an antidepressive drug treatment whereas sleep deprivation is promising in patients with high D2 binding.

Benzamides↗
PubMed1998

[Myopathology of the intrinsic laryngeal muscles in neurodegenerative diseases, with reference to the mechanism of vocal cord paralysis].

To investigate the mechanism of the vocal cord abductor paralysis (VCAP) in the neurodegenerative diseases, the intrinsic laryngeal muscles (the crycothyroid, the interarytenoid, and the posterior crycoarytenoid muscles) from 41 autopsied cases were histologically examined: 10 cases of amyotrophic lateral sclerosis (ALS), 10 of Parkinson's disease (PD), 9 of multiple system atrophy (MSA), 4 of Machado-Joseph disease (MJD), 4 of progressive supranuclear palsy (PSP), 1 of familial amyloidotic polyneuropathy (FAP), and 3 of cerebrovascular diseases as a control. According to the distribution of the neurogenic changes among above-described three intrinsic laryngeal muscles, three forms were raised: 1. The totally paralytic form showing that all the three muscles developed neurogenic atrophy. This form includes ALS, MJD, and FAP. 2. The posterior muscle-paralytic form showing that only the posterior crycoarytenoid muscle was selectively involved. This form includes MSA. 3. The nonparalytic form showing no morphological abnormalities in any of the intrinsic laryngeal muscles. This type includes PD and PSP. In this nonparalytic form, supranuclear mechanism such as pyramidal or extrapyramidal tract involvement may cause VCAP through the increased laryngeal muscles tone. Considering that VCAP can be seen in any of the above-described forms, our results indicate that the mechanism of VCAP is different among the neurological disorders.

Atrophy↗
PubMed2004

Central nervous system atrophy and clinical status in multiple sclerosis.

In this review, the authors focus on clinical aspects of central nervous system (CNS) atrophy in multiple sclerosis (MS), including the relationship between atrophy and disability, disease course, disease duration, quality of life, and fatigue. Cross-sectional studies have demonstrated a moderate but significant correlation between brain or spinal cord atrophy and physical disability in patients with MS. Longitudinal studies (>/= 5 years) have shown that CNS atrophy is a significant predictor of subsequent long-term neurologic deterioration. The clinical relevance of CNS atrophy is reinforced by studies showing that atrophy accounts for more variance than conventional lesion measures in predicting disability. Impaired quality of life and both urodynamic and sexual dysfunction, but not fatigue, are associated with brain atrophy. It is likely that once the level of CNS atrophy reaches a critical threshold, patients begin to suffer clinical impairment and disease progression. Longitudinal studies suggest that CNS atrophy may occur in patients with clinically isolated demyelinating syndromes who are at high risk for developing clinically definite MS. Longitudinal natural history studies in relapsing-remitting, secondary progressive, and primary progressive MS have suggested that patients develop CNS atrophy at a faster rate in the first few years of disease than later in the disease course. Similarly, long-term follow-up studies have shown a poor relationship between disease duration and the rate of brain atrophy. The authors conclude that measurement of atrophy of the CNS is emerging as a clinically relevant biomarker of the MS disease process.

Atrophy↗
PubMedDate not supplied

Multimodal evoked potentials in multiple system and late onset cerebellar atrophies.

Forty subjects were clinically examined using scales for cerebellar, pyramidal, parkinsonian, and mental status and by quantitative evaluation of neuroimages. The patients were classified into two groups: cerebellar-plus and "pure" cerebellar syndromes. Patients with "pure" cerebellar syndrome were diagnosed as autosomal dominant cerebellar ataxia III (ADCA III) or "pure idiopathic" late-onset cerebellar ataxia (ILOCA) in this series. Patients with cerebellar-plus syndrome were diagnosed as multiple system atrophy (MSA), subclassified as either ILOCA-plus, ADCA I, ADCA II or autosomal recessive LOCA. We have used visual (VEP), brainstem auditory (BAEP) and somatosensory (SEP) evoked potentials in order to establish their diagnostic validity. Cerebellar-plus syndrome and "pure" cerebellar syndrome showed overlapping VEP, BAEP and SEP abnormalities. VEP P100 latency, however, shows a certain ability to differentiate between the two groups (p = 0.08) and appears useful in distinguishing between sporadic cerebellar-plus syndromes (MSA or ILOCA-plus) and "pure" cerebellar syndromes (p < 0.02). The incidence of prolonged N9-N13 latency was significantly higher in the latter subgroup (p < 0.04) as well. Within cerebellar-plus syndromes, VEP, BAEP and SEP abnormalities were more frequent in inherited cases (ADCA I and II, along with autosomal recessive LOCA) than in sporadic ones. The most apparent differences were a higher incidence of abnormal BAEPs at brainstem level (p < 0.002), and of both peripheral and possible central SEP impairment in hereditary cerebellar-plus syndrome than in sporadic cerebellar-plus syndrome (p < 0.03). EP investigation is useful to a certain extent in differentiating between some variants of LOCA.

Adult↗
PubMed1995

Transcranial magnetic stimulation in multiple system and late onset cerebellar atrophies.

Central motor conduction time (CMCT) after transcranial magnetic stimulation (TMS) of the cortex, electromyography and nerve conduction velocity were performed in 24 patients with multiple system (MSA) and late onset cerebellar atrophy (LOCA) (often olivopontocerebellar atrophy--OPCA -). CMCT was abnormal in 7 patients with OPCA and one with LOCA. CMCT abnormalities (43% of cases) and increased threshold (68%) were more often found within OPCA group than in another multisystem atrophy and LOCA. Reduction in amplitude of the response after TMS was significantly correlated with cerebral hemispheres's atrophy. Increased threshold was correlated with upper vermal hemisphere atrophy and enlargement of the fourth ventricle. Electrophysiologic signs of mixed peripheral neuropathy were found in 8 patients. TMS abnormalities were not related to peripheral nerve involvement. Marked variation in CMCT suggests heterogeneity in these diseases. However, the percentage of CMCT abnormalities in OPCA group suggests that TMS seems to play a role in the neurophysiological diagnosis of these heterogeneous disorders.

Adult↗
PubMed2004

Magnetic resonance imaging surrogates of multiple sclerosis pathology and their relationship to central nervous system atrophy.

This article focuses on the various magnetic resonance imaging metrics currently used in multiple sclerosis and discusses how they relate to central nervous system atrophy. The authors discuss the significance of T2 lesion burden, gray matter damage, T1 hypointense lesions (black holes), contrast-enhanced lesions, magnetization transfer imaging, diffusion imaging, and magnetic resonance spectroscopy. These magnetic resonance imaging surrogates exhibit different sensitivities for each of the underlying pathogenic processes of multiple sclerosis. By exploiting the complementary nature and varying sensitivities of these magnetic resonance imaging surrogates, it is possible to create a more comprehensive picture of the degenerative process of multiple sclerosis.

Atrophy↗